'J

ANTENATAL PATHOLOGY AND HYGIENE

PRINTED TOR

WILLIAM GREEN & SONS

1SV .MORRISON AND GIISB LIMITED

October 1904.

*p

MANUAL

OF

ANTENATAL PATHOLOGY

AND HYGIENE

THE EMBRYO

BY

J. W. BALLANTYNE, M.D.: F.R.C.P.E., F.R.S.Edin.

LECTURER ON .MIDWIFERY AND GYNECOLOGY, MEDICAL COLLEGE FOR WOMEN, AND SURGEONS' HALL,

EDINBURGH ; EXAMINER IN MIDWIFERY IN THE UNIVERSITY OF EDINBURGH ; PHYSICIAN,

ROYAL MATERNITY AND SIMPSON MEMORIAL HOSPITAL, EDINBURGH;

HONORARY FELLOW OF THE GLASGOW OBSTETRICAL AND GYNECOLOGICAL SOCIETY,
AND OF THE AMERICAN ASSOCIATION OF OBSTETRICIANS AND GYNECOLOGISTS.

EDINBURGH

WILLIAM GREEN &

PUBLISHERS
1904

SONS

PREFACE

The companion volume to this, which appeared in 1902, dealt
specially with the diseases of the fcetus ; this is concerned with
the pathology of the embryo, and, to a small extent, with the
morbid tendencies of the germ. The two volumes are intended to
give the reader an idea of the whole extent and variety of the
pathological processes of antenatal life.

The pathology of the embryo is practically synonymous with the
subject of Teratology, or at least with that part of it which deals
with single as distinguished from double monsters. Now, Teratology
may be studied in three different ways : a simple description may be
given of the various monstrosities, with regard to their anatomy,
physiology, social and economic results, and the like ; or teratological
phenomena may be used for the purpose of explaining embryological
problems or as a means of clearing up difficulties in the domain of
foetal physiology ; or, finally, the whole subject may be approached
from the side of causation, and attention be focused upon the genetic
aspects of abnormal formations. While not neglecting the first and
second methods of study, I have endeavoured to specialise the third
plan ; for, after all, the most important matter is to reach a solution
of the problem of causation, because, if that be reached, there begins
to be hope that preventive treatment may be discovered. In my
opiniou, therefore, Chapters VII. to XII., which discuss teratogenesis
on general principles, are the most important of all.

The greater part of this volume, then, is given to the considera-
tion of the pathology of the embryo, and only three chapters at the
end are set aside for the pathology of the germinal period of life.
This fact requires a word or two of explanation. I had originally
intended to treat the pathology of the germ, which may, roughly
speaking, be regarded as synonymous with double monsters and
morbid heredity, on the same scale of completeness as that of the
foetus and embryo ; but this, I soon found, would have entailed the
writing of a third volume not much smaller in size than the present.

vi PREFACE

For such a task I have not the time, even if I have the ability, which
I doubt. I determined, therefore, simply to present in Chapters
XXXI., XXXII., and XXXIII. a very brief sketch of the plan I had
drawn out for the treatment of this division of the whole subject.
If it do no more, it will serve to give an idea of the morbid processes
which may be believed to be initiated or determined in germinal life.
The importance of these may be estimated when it is said that I
believe that they include the double monsters (both symmetrical and
asymmetrical), most neoplasms, plural births, and the mysterious
phenomena of morbid heredity. I wish, however, to emphasise the
fact that I am giving only a sketch and not a fully tilled-in picture
of this important section of antenatal pathology.

I have paid special attention to the bibliographical notices and

references, and, as in the first volume, I have tried as far as possible

to give the reader references which themselves contain literature

li>is. I have endeavoured to verify all these, and to give for each

the volume, the page, and the year of publication ; this has been a

task of great magnitude, and unaided I should have been forced to

abandon it, but, with the help of the Index Catalogue of Washington,

the Index Medicos, and Tarufti's fine work (Storia delta teratologic) in

eight volumes, it has been accomplished. The reader will never

know how many inaccurate references have been corrected, and how

many weary hours have been spent in putting right the errors which

greater care on the part of earlier writers would have prevented.

Here is a single example and a glaring one. In Tarufti's Storia (vol.

vi. p. 149) I found a reference to the case of a woman who had given

birth to six anencephalic foetuses; it was Adam Mencer, A. Medical

Times <<,,</ Gazette, Dicem. 1861. I looked up this reference and

could find no trace of it ; I looked up the indexes of adjacent volumes

ami fared no better. I then thought that perhaps Mercer Adam was

meant, and I referred to his articles on Teratology in the Edinburgh

and London Monthly Journal of Medical Science, and there, in his

second article, I found it, not the case indeed, but a reference back

to one reported by J. Martin in the New York Journal of Medicine

for March 184!). Then, as it happened, the only set of this Journal

to which I could gain access lacked the very volume I wanted.

Then I went to the Index Catalogue and found that J. Martin's paper

had also appeared in the Medical Examiner of Philadelphia for 1849 ;

I looked up this and got the original reference at last. So Tarufn's

Adam Mencer, A. Medical Times and Gazette, Dicem. 1861, had become

Martin, J., Med. Exam. Phila., 1849, n.s., v. 23 !

I may take this opportunity of thanking the Committee and

PREFACE vii

Council of the Koyal College of Physicians, Edinburgh, who granted
me, in 1902, one half of the Cullen Prize, in recognition of my work
on Antenatal Pathology.

I have again to thank my friend Dr. John Thomson for the care
and trouble which he has taken with the revision of the proof sheets,
as well as for the suggestions which he has from time to time made,
and always for the benefit of the work. It is also a pleasure to
me to express my gratitude to the many medical men who have
placed specimens at my disposal which have made my study of
the subject a possibility. A list of these will be found in the
Appendix.

J. W. BALLANTYNE.

24 Melville Street, Edinburgh,
September 16, 1904.

CONTENTS

LOOK III

THE PATHOLOGY OF THE EMBRYO

CHAPTER I

PAGE

Divisions of Antenatal Life ; Embryonic Period; Contrast between Fu'tal
and Embryonic Physiology ; Functions of Organs and Formation of
Organs ; Contrast between F( etal and Embryonic Pathology ; Noso-
graphy and Teratology ; Necessity for the Preliminary Study of
Embryology ........ 1

CHAPTER II

Embryology, the Anatomy and Physiology of the Embryo ; Definition ;
General Principles of the Subject : Progress towards the Complex and
Special ; Varieties of Heterogeneity ; Mechanism of Organogenetic
Alterations and Evolutions ; the Specificness of Ontogenesis ; the
Increase in Embryological Complexity in the higher Animal Forms ;
the Principle of " Recapitulation," Definition, Description, and
possible Exceptions ; Anisotropy and Isotropy . ... 8

CHAPTER III

Chronology of Embryonic Life : Preliminary Considerations : Conditions
found (1) at the Beginning of the Second "Week after Impregnation ;
(2) at the End of the Second Week ; (3) at the Beginning of the Third
Week after Impregnation ; and (4) at the End of the Third Week :
Recapitulation . . . . . . . .16

CHAPTER IV

Chronology of Embryonic Life (rant.) : Conditions found in the Fourth

Week after Impregnation . . . . . .38

CHAPTER V

Chronology of Embryonic Life (cord.) : Conditions found in the Fifth

Week after Impregnation . . . • • .52

61

CONTENTS

CHAPTER VI

Chronologj of Embryonic Life (eont.): Conditions found in the Sixth
W'l-fk after Impregnation ....••

CHAPTER VII

General Principles of the Pathology of the Embryo, or Teratology: (1)
j Physiological Factor— Nature and Illustrations ; (2) Amniotic Factor-

Nature, .Mode of Action, and Illustrations; (3) Germinal Factor—
Nature, Mode of Action, and Illustrations . . • .72

CHAPTER VIII

Teratogenesis or the Theories of the Causation of Monstrosities : Theories
of the Past ; General Survey ; Theory of the Supernatural, with the
Gods, or the Evil Spirits, or the Stars as Teratogenic Factors ; Theory
of the Natural, with Seminal and Menstrual Conditions and Hybridity
as Teratogenic Factors . . . . . . .81

CHAPTER IX

v* Teratogenesis (cont.) : Theories of the Past (cont.) : Mental Impressions as a

( 'ause of Monstrosities : History of the Theory among the Hebrews,
the Ancient Greeks, the Romans, Jews, and Christian Fathers; in
the Middle Ages ; in the Sixteenth Century ; in the Seventeenth,
Eighteenth, and Nineteenth Centuries; at the Present Time:
Summary ......... 105

CHAPTER X

Teratogenesis (cont.) : Theories of the Present; Traumatism and Mechanical
Pressure as a Cause of Monstrosities ; Extra-genital Maternal Trauma-
tism ; Injuries affecting the Mother's Abdomen : Intra-abdominal
Pressure ; Intra-uterine Pressure ; Funic Pressure, with Literature . 129

CHAPTER XI

The Nosological Theory in Teratogenesis : Foetal Diseases as Causes of
Monstrosities, the Theory and its Critics; Disease of the Fcetal
Annexa as a Cause of Monstrosities ; Amniotic Disease in Terato-
genesis ; the Amniotic Theory and its Developments ; the Facts and
the " Fancies," the Adherents and the Critics of the Theory ; Con-
genital Amputations and Foetal Skin Diseases ; Regeneration of lost
Parts; Conclusions; Literature .....; 1">8

CHAPTER XII

The Embryological Theory in Teratogenesis: Historical Sketch of the
Theory ; Embryology an Explanation of Teratology ; Arrested Develop-
ment; the Amniotic Factor in Teratogenesis; Experimental Terato-
geny in the hands of Geoffroy Saint-Hilaire, Allen Thomson, Panum,
Schrohe, Lombard ini, Dareste, F6re, and others ; the Germinal Factor :
Summary ......... 203

CONTENTS xi

CHAPTER XIII

ClassiH cation of Teratology : Historical Sketch of the Systems of Empe-
docles, Obsequens, Lycosthenes, St. Isidore, Weinrichius, Schenkius,
Aldrovaudus, Licetus, Huber, Haller, Buffon, Malacarne, Penada,
Meckel, Breschet, Charvet, Saint-Hilaire, Gurlt, Otto, Bischoff,
Sangalli, Forster, Davaine, Billard, Ahlfeld, Cleland, Hirst and
Piersol, and Thompson Lowne ; Difficulties of Teratological Taxonomy ;
"Connecting Links" ; Taruffi's System of Classification ; Teratological
Nomenclature ; the Author's Classification based upon Taruffi's ;
Special Teratology of Embryonic Life, and of Germinal . . 225

CHAPTER XIV

Teratological Types : The Types and their Bibliographies : Microsomia or
Dwarfism ; Growth Processes ; Embryonic, Foetal, and Postnatal Micro-
somia ; Generalisations ; Literature : Macrosomia or Giantism ; Em-
bryonic, Fcetal, and Postnatal ; Literature : Unilateral Macrosomia or
Hemihypertrophy : Unilateral Microsomia or Hemi-atrophy . . 240

CHAPTER XV

Teratological Types : Merosomatous Terata : Partial Macrosomia affecting
(1) the head and face, (2) the extremities, and (3) one extremity
or part of an extremity ; Pathogenesis and Treatment : Partial
Microsomia ........ 263

CHAPTER XVI

Teratological Types : Merosomatous Terata (cunt.) : Monstrosities affecting
the Spine and Head : Iniencephaly ; Definition ; Illustrative Instances ;
Morbid Anatomy and Clinical History ; Associated Malformations ;
Teratogenesis ; Literature ...... 272

CHAPTER XVII

Merosomatous Terata (cont.) : Malformations affecting the Spine ; Spina
Bifida or Rhachischisis ; Synonyms ; Varieties ; Holo-Rhachischisis ;
Mero-Rhachischisis ; Spina Bifida Occulta ; Localised Rhachischisis
without a Sac ; Spina Bifida with Rachicele ; Morbid Anatomy ;
Teratogenesis ; Treatment ; Bibliography .... 285

CHAPTER XVIII

Merosomatous Terata (cont.) : Malformations of the Spine (cont.) : Minor
Types of Spinal Deformity; Spinal Curvature ; Scoliosis; Cyphosis ;
Lordosis ; Spinal Retroflexion ; Spondylolisthesis ; Numerical Anomalies
in Vertebrae ; Fusion of Neighbouring Vertebrae ; Assimilation ; Fcetus
Anurus ; " Tails " ; Tailed Races ; Sporadic Cases of Homo Caudatus ;
Spurious Tails ; True Tails, Varieties, Complications, Teratogenesis ;
Sacral Dimples and Infundibula ; Miscellaneous Malformations of the
Spine ; Malformations of the Spinal Cord(Double Central Canal; Hetero-
topia of White and Grey Matter ; Hydromyelia and Syringomyelia) . 310

xii CONTENTS

CHAPTEB XIX

PAGE

Merosomatous Terata {cont.) : Malformations of the Cranium ; Anencephaly
or Acrania; Definition; Nomenclature; Historical Notes; Clinical
Details; Morbid Anatomy, Cranial Vault, Varieties of Defect, Face,
Basis Cranii, Cerebral Structures, Eyes, Spinal Cord, Suprarenal
Capsules, Sectional Appearances, Musculus Sternalis; Associated Mal-
formations ; Teratogenesis ; Literature ..... 332

CHAPTER .XX

Merosomatous Terata (cont.) : Malformations of the Cranium. Cephalocele :
Definition, Synonyms, Historical Note, Clinical History, Morbid
Anatomy, Position, Sac, Foramen, Contents (Hydro - meningocele,
Encephalocele, Hydro-eneephalocele, Hydro-meningo-encephalocele),
Basis Cranii, Associated Malformations, Comparative Teratology,
Teratogenesis. Hydrocephalus : Varieties, Definition, Clinical History,
Morbid Anatomy, Associated Malformations, Surgical Treatment.
Microcephalia : Definition, Historical Note, Clinical History, Morbid
Anatomy (Cranium, Brain, Varieties, Other Parts, Associated Mal-
formations), Teratogenesis, Surgical Treatment. Miscellaneous Mal-
formations (Cranial Perforations, Porencephaly, etc.). Omphalocephaly
(Dareste) ......... 352

CHAPTER XXI

Merosomatous Terata (cont.) : Malformations of the Face and Mouth ;
Genal Fissures ; Definition, Synonyms, Clinical Details, Morbid
Anatomy, Varieties (Fronto-maxillary, Intermaxillary, Anomalous),
Teratogenesis, I 'omparative Teratology : Median Fissure of the Face,
of Upper Lip, of Nose, of Lower Lip : Hare-lip ; Definition, Synonyms,
Clinical History, Morbid Anatomy, Varieties (Unilateral, Bilateral,
Simple, Complicated, Fissure of Jaw, Cleft Palate), other Associated
Malformations, Comparative Teratology, Teratogenesis, Treatment:
Fistula of Lower Lip : Fissure of the Tongue : Miscellaneous Mal-
formations of the Face (Asymmetry, Brachyrhynchus, Cranium
IVogenium, Prognathism) and Mouth (Synchelia, Synechia', Tongue-
Tie, Microstomia, Microglossia, Distomus, Macrostomia, Achelia,
Brachychelia, Microchelia, Macrochilia, Dicheilus, Macroglossia, Con-
genital Teeth) . . . . . . . .373

CHAPTER XXII

Merosomatous Terata (out.): Malformations of the Eyes and Nose ; Cyclopia:
Definition, Historical Note, Clinical History, Morbid Anatomy (Eye,
Nasal Apparatus, Facial Rones, Cranium and Brain, Associated
Malformations), Comparative Teratology, Teratogenesis, Literature ;
Cebocephalus ; Trigonocepbalus ; Cryptophthalmus ; Anophthalmias ;
Microphthalmia ; Hydropnthalmus ; Coloboma of Eyelid ; Miscellane-
ous Malformations of tin- Eyelids (MicroUepharon, Blepharophimosis,
Polyblepharon, Ectropion, Entropion, Distichiasis, Epicanthus, Ptosis
Congenita) ; .Malformations of the Lachrymal Apparatus, Cornea, Iris,
Choroid, Retina, Lens, and Vitreous : Miscellaneous Malformations of
tbe Nose ......... 393

CONTENTS xiii

CHAPTER XXIII

Merosomatous Terata (eont.) : Malformations of the Lower Jaw and Ear ;
Nomenclature and Classification ; Micrognathus ; Agnathus ; Cyclotia ;
Aprosopus ; Absence of External Ear ; Preauricular Appendages ;
Fistula Auris Congenita ; Minor Malformations of the Pinna and of
its various Parts (Helix, Anti-helix, Tragus, etc.) ; Distomus and
Accessory Maxilla; ....... 423

CHAPTER XXIV

Merosomatous Terata (cont.) : Malformations of the Neck : Fistula Colli ;
Definition ; History ; Varieties (Lateral, Median, Supra-hyoidean,
Infra-hyoidean, Complete, Incomplete) ; Morbid Anatomy ; Associ-
ated Malformations ; Teratogenesis ; Literature : Cervical Auricles :
Cervical Cysts : Cystic Hygroma of the Neck : Cysts of the Thyroid
and of Aberrant Thyroids : Median Fissure of the Neck : Cervical
Patagium : Cervical Ribs : Malformations of the (Esophagus : Mal-
formations of the Larynx and Epiglottis : Muscular Anomalies of the
Neck ......... 450

CHAPTER XXV

Merosomatous Terata (cont.) : Malformations of the Thorax : Anomalies
of the Clavicle (Absence, Incomplete Development, Bifurcation) ;
Anomalies of the Scapula (Foramina, Projections, Elevation) ; Anom-
alies of the Ribs (Bifurcation, Incomplete Formation) ; Anomalies
of the Mammary Glands (Absence, Hypoplasia, Athelia, Polymastia) ;
Anomalies in the Muscles (Pectoralis, Sternalis, Trapezius, etc.) ; Con-
genital Diaphragmatic Hernia, Illustrative Cases, Varieties, Clinical
Characters, Morbid Anatomy, Teratogenesis, Literature ; Malformations
of the Sternum ; Fissures of the Sternum ; Ectopia Cordis ; Pleurosomus 407

CHAPTER XXVI

Merosomatous Terata (cont.) : Malformations of the Thoracic Organs : Mal-
formations of the Heart : Individual Anomalies of Interauricular
and Interventricular Septa, Pulmonary Artery, Aorta, and Valves ;
Associations of Cardiac Anomalies ; Associated Malformations ; Tera-
togenesis ; Symptomatology ; Malformations of the Lungs, Trachea,
Bronchi, and Thymus Gland ...... 41)5

CHAPTER XXVII

Merosomatous Terata (cunt.) ; Malformations of the Abdomen : Gastroschisis :
History, Nomenclature, Classification, Clinical Characters, Morbid
Anatomy, Teratogenesis, Literature ; Hernia of the Umbilical Cord ;
Intestinal Diverticula ; Intestinal Stenosis and Atresia ; Hour-glass
Stomach ; Malposition of Intestines ; Anomalies of Appendix Vermi-
formis ; Congenital Hernia and Hydrocele ; Anomalies of Liver and
Gall Bladder ; Anomalies of Spleen, Suprarenal Capsules, Pancreas,
and Blood Vessels ; Congenital Umbilical Neoplasms . . .513

xiv CONTENTS

CHAPTER XXVIII

PAGE

Merosomatous Teratu (cont.) : Malformations of the Pelvic Organs : Extro-
version of the Bladder ; Definition, Synonyms, Historical Notes,
Clinical Features, Morbid Anatomy (in the Male and in the Female),
Cloacal Variety, Teratogenesis, Literature; Urinary Umbilical Fistuhe ;
Miscellaneous Malformations of the Bladder (Double Bladder, Diver-
ticula); Malformations of the Kidneys (Absence, Single Kidney,
Displacement, Lobulation, Accessory Kidney, Cystic Kidney) ; Mal-
formations of the Ureters ; Malformations of the Penis (Absence,
Duplication or Di phallus, Double Urethra, Penile Canals, Atresia of
Urethra, Epispadias, Hypospadias); Malformations of the Testicles ;
Malformations of the Female Genitals ; Malformations of the Anus
and Rectum ; Hermaphroditism ...... 539

CHAPTER XXIX

Merosomatous Terata (rout.) : Malformations of the Limbs : Sympodia
(Definition, Frequency, Synonyms, History, Clinical Characters, Morbid
Anatomy, Teratogenesis) ; Monopodia ; Ectromelus or Amelus ; Hemi-
melus ; Phocomelus; Absence of Individual Bones; Miscellaneous
Defects ; Double Hand and Double Foot ; Polydactyly and Hyper-
phalangy : Malformations of the Foetal Annexa ; Anomalies of the
Placenta, Umbilical Cord, and Vessels ..... 568

CHAPTER XXX

Heterotaxy or Transposition of the Viscera : Definition, Synonyms, Historical
References, Literature, Illustrative Cases, Morbid Anatomy, Varieties
(Total, Partial, Irregular), Associated Malformations, Transposition in
Animals and in Double Terata, Clinical Aspects, Diagnosis, Prognosis,
Teratogenesis : Antenatal Diagnosis of Monstrosities : Retrospect and
Conclusions . . . . . . . .594

BOOK IV
THE PATHOLOGY OF THE GERM

CHAPTER XXXI

Pathology of Germinal Life: Physiology of Germinal Life; Ovum and
Sperm Production, Maturation, Fertilisation, Segmentation, Blastocyst
Formation : Pathology of the Blastocyst ; Hydatid Moles, Abortion Sacs
without Embryos, Amniotic Defects, Heterotaxy : Pathology of the
Morula Mass; Fractional Individuals, Neoplasms: Pathology of
Fertilisation; Polyspermy, Double Terata, Hybridity : Pathology
of Maturation ; Parthenogenesis, Dermoids, Teratomata or Em-
bryoraata ........ c>Q7

CONTENTS xv

CHAPTER XXXII

PAGE

Polysomatous Terata : Uniovular or Monochorionic Twins : Allantoido-
angiopagous Twins ; Definition, Synonyms, Varieties (Paracephalus,
Acephalus, Amorphus), Teratogenesis : United Twins or Double Terata ;
Symmetrically United (Syncephalus, Dicephalus, Thoracopagus), Asym-
metrically United, Teratogenesis : Triplets, Normal, Malformed,
United : Quadruplets : Quintuplets : Sextuplets : Septuplets . . 623

CHAPTER XXXIII

Pathology of Germinal Life (cont.) : Physiology of the Egg- and Sperm-
Cell ; Normal or Ancestral Heredity ; Pathology of the Egg- and
Sperm-Cell ; Popular and Scientific Ideas regarding Morbid Heredity
and its Various Phenomena ; Degeneration ; Effect of Impregnation
upon Heredity and Degeneration ; Arrest of Growth, Development,
and the Performance of Functions ; Etiological Uniformity in the
Various Periods of Life ; Treatment of Antenatal Morbid States, and
especially of those arising in the Germinal Epoch . . . 647

Appendix ......... 661

Index of Subjects ........ 669

LIST OF ILLUSTRATIONS

COLOURED PLATES

TAGE

XV. Vertical Mesial section of Inieneephalic Foetus. (Specimen

No. 44) . . . . . . .274

XVI. Vertical Mesial section of Inieneephalic Foetus. (Specimen

No. 251) .278

XVII. Vertical Mesial section of Inieneephalic F<etus. (Specimen

No. 225) ....... 280

Will. Vertical Mesial section of Infant with Spina Bifida and Hydro-
cephalus. (Specimen No. 233) ..... 300

XIX. Vertical Mesial section of Foetus with Commencing Hydro-
cephalus. (Specimen No. 245) ..... 360

XX. Vertical Mesial section of Foetus with Hydrocephalus and Spina

Bifida. (Specimen No. 246) ..... 362

XXI. Vertical Mesial section of Foetus with Anencephalus and Spina

Bifida. (Specimen No. 226) ..... 342

XXII. Vertical Mesial section of Fietus with Anencephalus. (Specimen

No. 47) . . . . . . . . 346

XXIII. Coronal section of Fietus with Hernia of the Diaphragm.

(Specimen No. 166) . . . . . .478

FIGURES IN THE TEXT

1. Divisions of Antenatal Life

2. Divisions of Early Antenatal Life

3. Section through Peters' Ovum .

4. Section through Embryonic Part of Peters' Ovum

5. Section through Leopold's Early Ovum

6. Section of Mall's Early Ovum .

7. 8. Sections of Graf Spee's Early Ova .

9. Section of Guicciardi's Early Gestation Sac

10. Embryo at beginning of Third Week (His)

11. Embryo at beginning of Third Week (His)

12. Embryo at end of Third Week (His) .

13. Embryo of Fourth Week (His)

b

3

18
20
21
22
26
27
29
30
31
34
40

XVI 11

LIST OF ILLUSTRATIONS

1 I. Mall's 26 Day Embryo .

15. Reconstruction of .Mall's 26 Day Embryo

16. The Same ; I )eeper View

17. Embryo of the Fifth Weefc (His)

18. Head of Cyclops Fo-tus .

19. Foetus with Cyclopia

20. Foetus with Sympodia .

21. Chaldean Tablet of Monstrous Births

22. Maternal Impression Story

23. Schaeffer's Case of Spontaneous Amputation

24. Watkinson's Case of Spontaneous Amputation

25. Foetus with Amniotic Adhesions to Face

26. Foetus with Amniotic Adhesions and Exomplialos

27. Foetus with Amniotic Adhesions and Anencephaly

28. Section of Foetus with Anencephaly and Exomplialos

29. Foetus with Exomphalos and Spina Bifida

30. Foetus with Exomphalos and Facial Fissures .

31. Foetus with Sacral Projection and Band

32. Foetus with Anencephaly and Adherent Membranes

33. Foetus with Spina Bifida and Amniotic Thread

34. Foetus with Exomplialos and Cutaneous Band

35. Twin Foetus, showing early stage of Sympodia

36. "Wolffs Case of Amniotic Adhesions to Fingers

37. Infant with Spontaneous Amputation of Hand

38. Abortion Sac with Dwarf Embryo

39. Case of Hemi-Hypertrophy

40. Siamese Woman with Bilateral Macrosomia .

41. Case of Macrodactyly ....

42. External Appearances of Iniencephaly

43. Sectional Appearances of Iniencephaly

44. External Appearances of Iniencephaly

45. Sectional Appearances of Iniencephaly

46. External Appearances of Iniencephaly

47. Small Diencephalic Foetus

18. External Appearances of Iniencephaly

49. External Appearances of Iniencephaly

50. Fcetus with Spina Bifida and Anencephaly

51. Foetus with Spina Bifida, Mero-acrania, etc. .

52. Spinal Column and Cord in Spina Bifida

53. Fcetus with Lumbar Spina Bifida

54. Foetus with Sacral Spina Bifida and Exomphalos

55. Infant with Soft Tail (Harrison)

56. Fcetus with Sympodia and "Tail"

57. Vertical Mesial section of Anencephalus

58. Vertical Mesial section of Head in Anencephaly

59. Occipital Encephalocele

60. Occipital Encephalocele (E. Charles) .

61. Occipital Encephalocele

02. Hydrocephalus and Absence of Thumb

PAGE

42
44
4G
54
85
86
87
89
120
151
152
168
170
172
173
174
176
177
178
179
181
182
184
187
244
258
266
267
273
274
276
277
278
279
280
281
287
291
294
295
296
324
325
342
343
353
353
354
360

LIST OF ILLUSTRATIONS

xix

63. Foetus with Sympodia (Uromelic) and Hare- Lip

64. Congenital Tooth in Extra- Alveolar Sac

65. Infant with Unilateral Anophthalmia

66. Hypoagnathus and Synotus (Eogers) .

67. Aprosopus, with Deformity of Hand

68. Section of Preauricular Appendage

69. Microscopic section of Preauricular Appendage

70. Infant with Congenital Tumour on Face

71. Infant with Congenital Tumour on Face (Side View)

72. Case of Cervical Fistula (J. Thomson) .

73. Cystic Hygroma of the Neck ....

74. Foetus with Diaphragmatic Hernia and Anencephaly

75. Sectional Appearances of Anencephaly and Gastroschisis

76. Foetus with Cyllosomus (Haultain)

77. Foetus with Hernia of Umbilical Cord

78. Infant with Ectopia Vesicae (Michin) .

79. Fcetus with Gastroschisis and Double Genital Tubercle

80. Infant with Diphallus (Double Penis) .

81. Case of Diphallus (Jenisch) ....

82. Penis-like Body with Double Urethra (?)

83. Fcetus with Sympodia .....

84. Case of Ectromelus or Amelus (G. Thomson) .

85. Fcetus with Phocomelus (Baxter Tyrie)

86. Case of Double Hand (Dichirus)

87. External Appearances of Foetus Anideus and Membranes

88. Sectional Appearances of Foetus Anideus

89. Dicephalic Twin Fcetus .....

90. Dicephalic Twin, Variety Tripus Tribrachius .

91. Thoracopagous Twins .....

92. Dermal Aspect of Fcetus in Fcetu

93. Section of Fcetus in Fcetu ....

94. The " Biddenden Maids "

95. " Broadside " of the " Biddenden Maids "

PAGE

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439
440
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459
480
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MANUAL OF
ANTENATAL PATHOLOGY AND

HYGIENE

BOOK III

THE PATHOLOGY OF THE EMBRYO
CHAPTER I

Divisions of Antenatal Life ; Embryonic Period ; Contrast between Foetal and
Embryonic Physiology ; Functions of Organs and Formation of Organs ;
Contrast between Foetal and Embryonic Pathology; Nosography and
Teratology ; Necessity for the Preliminary Study of Embryology.

Divisions of Antenatal Life.

As was pointed out in the first chapter of the first section of this
work (Manual of Antenatal Pathology and Hygiene — The Fcetus,
p. 7, 1902), antenatal life may be divided into three periods — the
foetal, the embryonic, and the germinal. These periods differ con-
siderably in length : the foetal occupies thirty-two weeks of intra-
uterine life, or thirty-four if the short neofcetal period be included ;
the embryonic, five weeks, or seven if the neofcetal period be reckoned
with it ; while the germinal period stretches back almost indefinitely
into the lives of parents and earlier ancestors, if the theory of the
continuity of the germ-plasm be accepted.

The greater part of intrauterine life is foetal, and the smaller part
is embryonic. It has sometimes been stated that the earliest part of
embryonic life is in progress while the blastodermic vesicle is still in
the Fallopian tube ; but there seems to be good evidence to show
that during the whole of these two periods (foetal and embryonic)
the new organism is inside the body of the uterus, save only in the
pathological condition of extrauterine pregnancy when development
takes place wholly or in part in the interior of the Fallopian tube.
It is doubtful whether any part of germinal life is carried on in

VOL. II. — I

2 ANTENATAL PATHOLOGY AND HYGIENE

utero. This period ends with the appearance of the first traces of
the embryo in the embryonic area of the blastodermic vesicle; and, if
it be believed that impregnation and the first stages of development
occur in the Fallopian tube, then it is quite likely that no part of
germinal life is enacted in the cavity of the uterus. Whether this be
so or not, there can be no doubt that by far the greatest part of the
germinal period of existence takes place in the sexual glands of the
parents, where the ova and spermatozoa lie quiescent but probably
capable of being impressed by chemical and other changes occurring
in their immediate neighbourhood. These matters will be more
clearly understood with the help of the scheme of the divisions of
antenatal life on page 3 (Fig. 1).

It was also pointed out in the first section of this work that each
period of antenatal life had to a large extent a pathology peculiar to
itself ; it was stated that morbid causes acting upon the organism in
the foetal period produced results different from those arising in the
embryonic period or in the germinal, although all the while the
morbid causes might be the same. There was, therefore, a pathology
of the foetus, a pathology of the embryo, and a pathology of the germ.
In the first section of this Manual the pathology of the foetus was
fully dealt with, while those of the embryo and germ were merely
referred to incidentally when either happened to throw light upon
or to be associated with truly foetal morbid processes. In this, the
second section of the work, it is the pathology of the embryo that
calls for special consideration, while merely passing references will be
made to that of the foetus. Further, an attempt will be made to
separate the pathology of the embryo from that of the germ, although
it must be frankly confessed that such a separation is at present
largely a matter of conjecture. This volume, then, is occupied with
the pathology of the embryo.

As with the foetus so with the embryo, it will be found most con-
venient to make the consideration and description of its anatomy
and physiology precede the study of its pathology. "When great
differences exist between the physiological conditions of two epochs
of life, great differences will also be noted in their pathological
manifestations. At any rate, the physiology of the foetus has been
shown to have a far-reaching effect upon its pathology — to penetrate
it through and through (durch unci durch durchdringen) to use
Graetzer's expressive phrase (Die Krankheiten des Fcetus, p. 3, 1837) ;
and it may be expected that the physiological characters of embryonic
life will leave their impress upon the morbid conditions then arising.
As a matter of fact, a more complete and striking difference could
scarcely be imagined than that which exists between the results of
pathological activity in the foetus and in the embryo : in the one case
diseases are met with, which, while they present certain features
which are peculiar, are yet evidently of the same nature as the
maladies of postnatal life ; in the other case, the morbid conditions
found are so extraordinarily dissimilar from anything else in the
whole field of pathology as to suggest that an altogether special
cause has been at work in their production. Yet, if our generalisa-

DIVISIONS OF ANTENATAL LIFE

FIG. 1.

25
34

19
18
17
10
IS
14
IS
13
11
10
9
8

THE DIVISIONS OF ANTENATAL LIFE.

POSTNATAL PERIOD..

NEONATAL PERIOD

F(ETAL PERIOD

NEOFCETAL PERIOD

EMBRYONIC PERIOD

Po«t conceptlonal

ceptlonal.

Otuminaturation.

(a) Ovular lilt.

•

■Spermatogenesis
lb) Sperminal Hie.

X

GrowVh.

Organogenesis

1 Mi[ rt-fc-,|3' i .,n

4 ANTENATAL PATHOLOGY AND HYGIENE

tion be correct, the explanation of this dissimilarity may be contained
in the existence of great differences between the physiological con-
ditions of these two periods of antenatal existence. Is then the
physiology of embryonic life strikingly unlike that of foetal life?
Let us see.

The Contrast between Foetal and Embryonic Physiology.

The foetus, in its physiology, shows resemblances of a real kind
to the child at birth and to the adult. It is true that these resem-
blances are in some measure obscured by the dissimilar surroundings
in which foetal, as contrasted with postnatal, life is carried on ;
nevertheless in the unborn infant as in the child or adult there is
the picture of a congeries of organs regularly performing their func-
tions of circulation, digestion, assimilation, excretion, and the like.
There are organs, and these organs are functionally active. In the
foetus some of the organs are less active and some doubtless are more
active than in the child or adult; some also are active in somewhat
different ways; yet the essential resemblance is maintained. The
presence of an additional organ, the placenta, in the case of the
foetus, and the peculiarities of an intrauterine life, give to foetal
physiology a character peculiarly its own, but do not destroy the
likeness which it bears to postnatal physiology. The only circum-
stance which tends seriously to mask the resemblance between the
functions of the unborn and those of the new-born infant is the
presence of developmental processes in the former. Some of the
organs of the foetus can hardly be described as performing functions
or at any rate as performing nothing but functions, for they are still
themselves in process of being formed. To express this fact in
perhaps a more accurate way, some of the foetal organs, such as the
brain, are still occupied with the great function of development and
are not sufficiently elaborated to have any other functions. In this
respect certainly the physiology of the foetus stands somewhat apart
from the physiology of postnatal existence; in this one character
there is a real difference between the functional activities of these
two periods of life. But the difference is not great enough to pre-
vent the observer from recognising in the foetus the same kind of
physiological activity as is seen in the infant, child, or adult. Let us
turn now to the physiology of the embryo.

The physiology of the embryo is embryology : what is embraced
by the name Embryology is really almost the only function, if it can
so be called, of embryonic life. To gain a clear idea of embryonic
physiology it is necessary to think not of organs performing functions
but of organs being formed ; embryonic physiology has not to do with
organs and their special activities, but with the special activity which
] 'induces, builds up, and perfects the organs themselves. The great,
almost the only, function of the embryo is to form tissues and organs,
or, in one word, organogenesis. We have to imagine an aggregate of
cells arranging themselves, apparently in a disorderly fashion or in a
fashion of which the order is but dimly discerned, first into the three

FCETAL AND EMBRYONIC LIFE 5

layers of the blastoderm, and then, after many intermediate stages
and phases, into the organs which take on the particular functions of
foetal and postnatal life. There is no anatomy of the embryo apart
from its physiology ; the two are united together in the subject of
embryology, which, as it were, contains them. It is only in foetal
life that the distinction between matters anatomical and matters
physiological begins to become apparent ; after birth it is, of course,
quite evident. For the sake of strict accuracy, however, it must be
added that the later part of embryonic life is not without indications
of functional activity (in the ordinary sense of the term), and some of
the organs, e.g. the heart, are performing other acts than those of
formation or construction merely. In a similar manner the early
part of fcetal life shows signs of constructive and formative activity
in certain organs which have hardly yet begun to be operative in a
functional way. There is, therefore, no sharply marked boundary
between fcetal and embryonic life in this respect. The experienced
biologist ought not, on reflection, to look for it. There is rather a
gradual passage from the typical embryonic life of organogenesis to
the typical fcetal life of growth and functional activity through a
series of intermediate phases in which one or the other of these forms
of vital action seems to preponderate. These intermediate phases
were to be expected, Nature in her operations making no sudden
transitions. At the same time, and after due allowance has been
made for these connecting links, it can be recognised that the
principal manifestation of embryonic life stands out prominently as
organogenesis, while the chief sign of fcetal life is the functional
activity of the various organs which go to constitute the foetal
economy.

Contrast between Foetal and Embryonic Pathology.

The statements which have been made in the preceding paragraph
abundantly prove that there is a fundamental dissimilarity between
the physiology of the foetus and that of the embryo. It is indeed
scarcely possible to imagine two . forms of vital activity less alike.
We can now return with less surprise to the statement that the
pathology of the embryo is startlingly unlike that of the foetus. With
a physiology so special, there is little room for wonder if the path-
ology also be of its own kind. In fact, one is led to look for morbid
processes of a peculiar kind under circumstances which are so
peculiar. There is here a conjunction of conditions which may be
reasonably expected to produce something sui generis, something
quite unlike what happens at any other period of life, postnatal or
antenatal.

To state the matter concisely, the pathology of the embryo is
Teratology. Since the chief result of the physiological activity of
the embryo is the formation of parts and organs, so the chief result
of pathological processes in the embryo is the malformation of its
parts and organs. The one leads to normal formation, the other to
abnormal formation. Monstrosities and structural anomalies are the

G ANTENATAL PATHOLOGY AND HYGIENE

results of morbid agents acting upon the organism in the embryonic
period of its existence, or, to write more exactly, upon such parts of
the organism as are in the embryonic or formative stage. It will be
afterwards shown that there is good reason to believe that the morbid
agents or causes are in no way peculiar in the embryonic epoch, and
proof will be led that they are in fact just the same causes which
produce diseases by their action upon the foetal or adult organism.
In the case of the embryo they are called teratogenic and in that of
the foetus or adult they are named pathogenic; but there is no
sufficient reason for supposing that in their essential nature they
differ one whit. It is the result which is dissimilar, being in the one
instance teratological and in the other pathological. The mode of
action of the cause is no doubt different, and the organism upon which
it acts and its surroundings are indeed widely different ; it is in this
manner that the vast difference between the results — a monstrosity
and a disease — is produced. The difference then between terato-
genesis and pathogenesis is one of results rather than of causes ; this
I hope to make clear in the following chapters.

The pathology of the embryo, then, has to do with monstrosities
and malformations, with, in other words, the teratological states. At
the same time all that is teratological does not of necessity belong to
the embryonic period, neither are all the manifestations of pathological
activity in the embryonic period of necessity teratological. It has,
for instance, been shown (in the first section of this work) that some
malformations may arise in the foetal period of antenatal life ; these
are the structural anomalies of such parts as the brain, genital organs,
and skeleton whose development has continued after the termination
of the embryonic period properly so called. Again, united twins are
instances of teratological states which in all probability are deter-
mined in the germinal period of antenatal life. Further, some of the
organs and parts of the embryo are so far advanced in formation
before the end of the embryonic period as to be capable of some
degree of functional activity, and they may therefore become the
possible seats of disease ; but this is a matter about which little is
known, and it is hardly wise to speculate concerning it. These ex-
ceptions, however, do not greatly weaken the generalisation which
has just been made, namely, that the pathology of the embryo is
Teratology. Without doubt, the most numerous and the most
characteristic of the morbid states which arise in embryonic life are
teratological in their nature. The description of the morbid states
of the foetus forms part of Nosography, for these states are mainly of
the nature of diseases ; the description of the morbid states of the
embryo, on the other hand, is Teratology, for they are chiefly of the
nature of malformations and monstrosities.

I have endeavoured to place clearly before the reader the contrast
between foetal pathology and embryonic pathology, between Noso-
graphy and Teratology. I regard it as a fundamental and essential
difference, as a distinction which must be rightly appreciated and
constantly borne in mind if the phenomena of pathological activity
in the embryo are to be properly understood.

EMBRYOLOGY PRELIMINARY TO TERATOLOGY 7

Necessity for the Preliminary Study of Embryology.

In order that the manifestations of disturbed developmental pro-
cesses may be recognised and their mode of production made intelligible,
it is obvious that there must be a preliminary acquaintance with the
phenomena and laws of normal development. Embryology has to do
with the study of the normal development of the embryo, just as
Teratology has to do with the study of its abnormal development.
A knowledge of Embryology must precede the investigation of
Teratology, if that investigation is to be fruitful in results of scientific
value. The following five chapters will, therefore, be occupied with a
short sketch of Human Embryology ; thereafter it will be at least a
little less difficult to unravel some of the many tangled problems of
Teratology. Unfortunately it is not yet possible to set forth with
accuracy of detail the various changes which occur in embryonic
organogenesis ; about many of them the information obtainable is
fragmentary and uncertain, and about some of them there is no
information at all. Human Embryology is an incomplete narrative ;
a great part of the first volume is wanting and has to be supplied by
conjecture, and there are pages amissing throughout the rest of the
work. We have to supplement our information about human Em-
bryology by facts borrowed from Comparative Embryology, and we
can hardly take it for granted that what happens in the chick will
happen also in the human embryo. Since, then, we do not possess
a complete knowledge of the anatomy and physiology of the embryo
— Embryology, we cannot hope to explain with certainty all the
processes concerned with pathology of the embryo — Teratology. At
the same time it is by no means necessary to suspend investigations
into the causation of monstrosities and malformations on account of
this lack of accurate information regarding many parts of Embry-
ology. Indeed the inquiry into several types of malformation and
structural anomaly has repeatedly thrown light not only upon the
malformation or anomaly itself but also upon the normal process of
development the disturbance of which it represents. No surprise
need be felt that this result has followed ; for has not the investiga-
tion of the action of various organs in disease cleared up matters
which were obscure regarding the functions of these organs in health ?
Physiology and pathology are mutually serviceable in explaining each
other.

CHAPTER II

Embryology, the Anatomy and Physiology of the Embryo ; Definition ; General
Principles of t lie Subject: Progress towards the Complex and Special;
Varieties of Heterogeneity ; Mechanism of Organogenetic Alterations and
Evolutions ; the Specificness of Ontogenesis ; the Increase in Embryological
( "complexity in the higher Animal Forms ; the Principle of " Recapitulation,"
Definition, Description, and possible Exceptions ; Anisotropy and Isotropy.

r

Embryology, if the word be used in a restricted sense, is the science
which treats of the origin and formation of the parts and organs of
the embryo. It is the study of the anatomy and physiology of
embryonic life ; for it has to do with the description of the parts
which make up the embryo or its anatomy, as well as with the way
in which these parts, often after passing through several intermediate
forms, become the permanent organs of the embryo, or its physiology.
It is not possible to separate the anatomical from the physiological
part of the subject. It is quite possible, and indeed it is easy, to
describe the organs and parts of the adult body, e.g. the individual
bones of the skeleton, without referring to their functions, for they
are fully formed and do uot differ save in size at the various periods
of life. To attempt, however, to describe the parts of the embryo
is really to picture these parts as they appear at the various stages
in the development of the embryo, in other words it is to represent
their progressive elaboration. When an embryologist speaks of, say,
the face of the human embryo, he is thinking of the five processes
springing from the base of the primitive cerebral capsule, and
gradually growing round the cavity of the stoinodceum, and ulti-
mately uniting to form the facial framework ; a living picture is as
it were passing before his mind's eye. Embryology is, or ought to
be, a kinematographic representation, a lifelike reproduction of the
original moving scene of the whole embryonic period of antenatal
existence. The embryo is not like a finished piece of machinery
which can be studied both in action and at rest; it is unfinished, it
is like a piece of machinery in process of construction, and its
activities consist in a ceaseless progress towards a termination which
shall be also a completion. In one sense (a limited one) the human
embryo attains this finished stage with the close of the embryonic period
of antenatal life ; but in another sense (a wider one) it has not reached
it even at the moment of birth, for some construction or organo-
genesis goes on postnatally. In another and yet wider sense it may

be said of the human embryo that evolution has not brought it even

s °

PRINCIPLES OF EMBRYOLOGY 9

now to full fruition, and that the world has not yet beheld the final
phase in the ascent of man in the production of the embryo perfect
in every part and without dangerous structural relics and morbid
tendencies. It may never be brought so far, but Hope whispers that
it may approach a little nearer than now.

" Tutto clie questa gente maledetta
In vera perfezion giammai non vada,
Di la, piii che di qua, essere aspetta." 1

In a more usual and less restricted sense Embryology is the
science which treats of all the vital phenomena which occur in the
uterus and ovary before birth ; in this sense, therefore, it includes
foetal physiology and germinal activity as well as ovulation and even
menstruation. Its employment, however, in this extended sense is
not to be commended. It is but one instance, although a very
prominent one, of the general looseness of definition which has pre-
vailed regarding the terminology of antenatal affairs ; for instance,
the name embryo has been applied to the new organism in the uterus
both in the foetal and in the embryonic period, while to the word
foetus the same wide significance has been given ; again, to cite a
still more evident example, the term ovum lias been used not only
for the female sexual cell both before and after impregnation, but
also for the product of conception at the various months of intra-
uterine life. Such a usage of terms is sadly lacking in scientific
accuracy. In this work the word Embryology is employed as mean-
ing the science which treats of the origin and formation of the parts
and organs of the embryo ; 2 it deals with a part of antenatal
existence which, although somewhat short (five to seven weeks), is
so crowded with events of such importance as to cause it to take
rank above all the other periods of life, antenatal and postnatal. It
includes the study of all the changes through which the embryo
passes, from the appearance of its first rudiments in the embryonic
area up to the transformation of the " transition organism " into the
recognisably-human embryo or neofoetus, as has been sketched in the
first section of this work (Manual of Antenatal Pathology and
Hygiene, p. 80, 1902).

General Principles of Embryology.

The general principles of Embryology lie buried somewhat
deeply ; they are far from the surface, and not easily to be reached
or recognised when reached. It is difficult for the mind to picture a
stage in the life of the organism when almost the only manifestation
of vital activity is of the purely constructive kind, when, in other

1 Dante, Divina Commedia, Inferno, vi. 109 : —

"Though ne'er to true perfection may arrive
This race accurs'd, yet nearer then than now
They shall approach it."

(Cary's Transl.)

2 The word Embryography might, perhaps, be used in this sense ; but the writer does
not desire to complicate matters by the introduction of many terms.

10 ANTENATAL PATHOLOGY AND HYGIENE

words, the framework of the body is being built up. So complex
also is the process that certain portions of it, notwithstanding the
laborious researches of keen and able embryologists, are still im-
perfectly understood or only guessed at. It is even more difficult
to discover the laws which govern embryological processes, and to
trace the general principles which underlie so complex a series of
manifestations. One is almost driven to speculate whether, after all,
there are laws regulating the mysterious metamorphoses of the
embryo, whether indeed the parts do not fall together in kaleido-
scopic fashion, and confusion happen somehow' to give place to order.
But reflection assures the investigator that there must be laws,
otherwise he is forced to state that Embryology differs from all the
other sciences in being uncontrolled by rule. Yet in no other part
of biological study is it so difficult to trace and analyse the laws and
their results. Events move so rapidly, form succeeds form and
phase follows phase with such swiftness and with so much partial
superposition, that nothing less than an arrestment of the succession
of phenomena at various stages would serve to enable the embry-
ologist to trace the procession of occurrences. Such an arrestment
sometimes occurs, and pathology conies to our aid to help us to
understand the intricacies of physiology ; but the arrestment is
usually of a local and incomplete kind, and its immediate effect is
too often simply to increase the complexity of the problem we are
attempting to solve. At the same time progress has been made,
and further progress will yet be made by means of the careful study
of these partial arrestments, which we may at once call teratological
phenomena, and so ultimately the abnormal and the normal will
prove mutually elucidative. In what remains of this chapter it is
my purpose to try to educe from the embryological and teratological
data at command such general principles and laws as may seem to
be educible. Let the reader meanwhile bear in mind that some of
these principles rest upon very insecure foundations, and that after
all there is but little advantage in formulating statements which are
apparently definite, concise, and clear-cut if the facts do not warrant
them or if the facts are not sufficiently known to warrant them.
Facts must not be twisted to suit general principles, but general
principles must be allowed slowly and surely to emerge from the
rigorous investigation and comparison of facts. The principles which
follow may be said to be emerging from the crucible of modern exact
research ; they are not all pure gold, but they have appreciably been
cleansed from some impurities.

First, it may be stated with a large degree of confidence that the
maiii progress of events and changes in Embryology is from the
simple towards the complex and from the general towards the
special; there is a trend towards elaboration, and from a homo-
geneity which is at any rate apparent even if it be not real there
arises a heterogeneity which is both evident and real. From the
comparatively simple-looking impregnated ovum conies the superlat-
ively complicated embryo. From the successive divisions of that
wonderful unicellular organism come all the tissues and organs of the

HETEROGENEITY IN ONTOGENESIS 11

still more wonderful multicellular organism. To the series of changes
which are thus induced and in which we see the constant trend
towards an increasing complexity the name ontogenesis has been
given. It has, it is true, been employed in a somewhat wider sense
(" the origin and development of the individual living being "), but it
will be convenient to use it here as indicating the changes in the
impregnated ovum by which it becomes a fully formed embryo. As
this progressive differentiation is going on, generation after generation
of cells comes forward and plays its part, being always represented by
a yet more numerous progeny of cells, and so ontogenesis may be
regarded as a great genealogical tree (Delage x). In this genealogical
tree, however, the successive generations do not coexist, but one gives
place to the next and is indeed represented by it, for its cells have
divided to form it.

Second, the heterogeneity which is produced in the process of
ontogenesis is of two kinds. In one of these the differences are seen
in the cells themselves ; they grow unlike one another ; there is a
histological differentiation. In the other the differences are visible
in the arrangement of the cells into organs ; they are built together
in different ways into organs ; there is an organogenetic differentia-
tion. There are different sorts of cells, and they are arranged in
different sorts of ways.

Third, the startlingly divergent results found in the organs of the
fully formed embryo may probably all be ascribed to differences in
the rate of growth of the tissues composing the various organs and
parts. In this way the manifold twistings and overlapping^ and
separations and approximations which are seen in all embryos before
their parts settle down into their permanent relationships are to be
accounted for. A greater rapidity of growth of one part will cause a
projection outwards or inwards at that part ; a slowly growing part
will find itself surrounded, built in, so to say, by the more active
neighbouring areas ; two organs at first far from each other may come
into contact if both grow at a quicker rate than the body as a whole,
they may even do so if one only grows faster, but then the meeting-
place will not be the same. We speak in a loose fashion of the
movement of various parts of the embryo in development, but the
idea conveyed is erroneous if we mean by it that one organ without
altering its rate of growth approaches another or passes away from it.

Fourth, there is a specificness in the ontogenesis of different types
of animals. The embryos of different animals do not travel by the
same route to reach their later stages ; there is a wide diversity
between their ontogenetic processes, how wide we do not probably
fully realise as yet. Even when the fully formed organ or part in
two species of embryo seems to be exactly similar, it does not
apparently follow that it has reached this state by the same series of
alterations. The embryo of the chick differs from that of the
mammal in the details of its development ; further, each has an
amnion, but there is good reason to believe that it is not formed in
the same manner in each. This principle cannot be too clearly kept

1 Delage, Y., La structure du ■protoplasme ct hs theories sur Vheredite, 1895.

12 ANTENATAL PATHOLOGY AND HYGIENE

in mind ; for in the past it has been neglected, and what was observed
in the chick embryo was regarded as proven also for the human
embryo, with results which were disastrous so far as accuracy was
concerned.

Fifth, as a general rule, the higher an animal is in the zoological
series the more complex is its ontogenesis. At the same time the
progress towards complexity need neither be uniform nor constant.
The highest of one group of animals may have an ontogenesis much
more complex than the lowest of the group next in order above it.
There is a specificness even in this matter, and there is no complete
chain of types, representing a regular sequence of ascents.

Sixth, there is a general principle which may be stated in several
different ways, and which has given rise to an immense amount of
discussion. It has also been called the " great law of biogenesis." It
may be enunciated in this way — that ontogenesis never follows a
simple and direct route, but reaches its end by detours (" ces detours
de 1't'volution," Delage). In the forming of the completed embryo
there are stages passed through which apparently serve no useful
purpose ; there are organs which appear and disappear again leaving
sometimes rudiments which confessedly are dangers to the organism ;
some of these transitory organs have a temporary use, but others
seem to have none at all. The higher vertebrate embryo, for instance,
has three sets of urinary organs — the pronephros and its duct, the
mesonephros and its duct, and the metanephros and its duct ; and
yet the necessity for some of them is not apparent. Further, it has
for long been noted that many of these " detours " or temporary
organs and parts distinctly recall the stages or appearances seen in
animals lower down in the zoological scale; and so it came to be
thought that in ontogenesis there is a sort of recapitulation of
Comparative Anatomy, or, to put it in more modern terms, that
ontogeny gives a condensed phylogeny, or that there is a reminiscent
projection of phylogeny into ontogeny. Before, however, we discuss
the correctness of this conclusion, let us see that we have a clear idea
of what is intended by this general principle, this " great biogenetic
law."

Henry Drummond l gave a picturesque and vivid description of
what he understood and read into it. He wrote: "The human
embryo is a subtle phantasmagoria, a living theatre in which a weird
transformation scene is being enacted, and in which countless strange
and uncouth characters take part. Some of these characters are well
known to Science, some are strangers. As the embryo unfolds, one
by one these animal actors come upon the stage, file past in phantom-
like procession, throw oh" their drapery, and dissolve away into
something else. Yet as they vanish, each leaves behind a vital
portion of itself, some original and characteristic memorial, some-
thing itself has made or won, that perhaps it alone could make or
win— a bone, a muscle, a ganglion, or a tooth — to be the, inheritance
of the race." So Drummond pictured the human embryo "wander-
ing among the ghosts of departed types " ; in making a man, " Nature

1 Druninioiid, H., The Ascent of Man, p. S7, 1894.

LAW OF RECAPITULATION 13

introduced the framework of these earlier types, displaying each
crude pattern by itself before incorporating it in the finished work."
" Every creature that lives climbs up its own genealogical tree before
it reaches its mature condition."

This law, which as we shall see is not so really a law as has been
thought, may be stated in another way. Apparently the framework
of the body is not laid down once for all, but is rather constructed in
portions and even in overlapping portions, so to speak. One part
of the framework may be replaced in whole or in part by a new
substructure often of a type quite different from that which has
preceded it. Readjustment, adaptation, alteration, and variation are
processes constantly at work in the life of the embryo ; it is by
reorganisation that order is brought out of the seeming chaos of the
results of blastodermic activity. If one were to imagine a builder
constructing a palace by first erecting the scaffolding for a cottage ;
by then, after building operations had only just been commenced,
replacing this by the scaffolding for a villa ; and that again by the
framework necessary for a mansion; and then, finally rearranging,
and as far as possible utilising, all the materials for the formation of
the palace ; one would have an idea, albeit an imperfect one, of the
character and complexity of ontogenesis. The comparison is, of
course, almost ludicrously inadequate, for it gives no indication of
the marvellous manner in which in organogenesis all the materials
of the temporary scaffoldings are made use of in the construction of
the permanent edifice, are worked into it so exactly as to be indis-
tinguishable from each other in it. Only here and there in the
body, in the appendix vermiformis, the fossa ovalis, and the ductus
arteriosus, are to be seen the traces of some parts of the earlier
formations which have either been incompletely utilised or have been
imperfectly replaced by the later productions.

This same general principle of ontogenesis may be set forth in yet
another way, as evolution by atrophy. In order that a new scaffold-
ing may be set up it is necessary that the former atrophy ; if a new
plan of construction is to be followed it is needful that the old plan
be abandoned. To quote from Ernest Mehnert * (as rendered by
J. Arthur Thomson2), involution and evolution go hand in hand.
The ovum develops, the polar bodies degenerate; the blastoderm
develops, the merocytes degenerate ; the head grows and the tail
dwindles ; the higher nerve centres of the mammalian brain increase
and the occipital region diminishes ; the metanephros comes and the
pronephros goes ; the backbone strengthens and the notochord dis-
appears ; and so on throughout a continual necrobiosis.

In whatever way it may be stated, there can be no doubt that in
ontogenesis there is very evidently this principle of reaching the
final result by ways which appear to be roundabout and by scaffold-
ings that are temporary. When, however, the attempt is made

1 Mehnert, E., Biomechanik erschlossen cms clem Principe dcr Organogenese,
Jena, 1898.

2 Thomson, J. A., " Mehnert' s Principles of Development," Natural Science, xiv.
385, 1899.

14 ANTENATAL PATHOLOGY AND HYGIENE

to find a constant and determining rule or law dominating the
ontogenetic detours and the developmental scaffoldings, difficulties
at once emerge ; the explanation that the embryo of the higher
vertebrates passes rapidly through a series of stages which represent
the permanent forms of embryos lower in the zoological scale breaks
down at once upon close examination, and as yet no other satis-
factory hypothesis has arisen to take its place. But this fact demands
a separate paragraph, and it will be best to embody it in a general
principle, the seventh.

Scvmth, then, it may be affirmed that ontogeny does not give a
short recapitulation of evolutionary progress; it is not an epitomised
phylogeny. This is seen in the fact that the time at which an
organ appears in individual development does not always indicate the
historical or phylogenetic age of that structure. If there is, then,
a recapitulation in ontogenesis it is one in which Nature makes
mistakes ; or, to express the matter in more scientific language, there
are " time-displacements " in development. For instance, the heart
is regarded as a secondary differentiation (due to functional adapta-
tion) of that part of the primary ventral blood vessel where the
greatest resistance has to be overcome ; but in the organogenesis of
the human subject the heart appears before the associated main
vessels. The mammary glands, also, make their appearance in the
individual before the sebaceous glands from which they are supposed
to have been differentiated ; and the paired eyes in vertebrates arise
long before the much older pineal eye. It would seem, to revert for
a moment to a non-scientific way of speaking, as if Nature in telling
in the individual the story of his descent (or ascent) made occasional
slips and put incidents in a wrong order of occurrence. Doubtless
the mistakes are only seeming mistakes, and an explanation is hidden
away somewhere if it could but be found. Possibly, as Mehnert
suggests, when an organ rises in physiological importance and
structural differentiation it acquires a proportionate increase in its
rate of development. Progressive organs are accelerated in their
rate of development, and retrogressive ones tend to be belated ; and
their state of acceleration or belatedness (or retardation) is due to
the intensity of developmental energy in them. It is not yet clear
what exactly it is that exalts or depresses developmental energy. It
must apparently be conceded, therefore, that ontogeny is not in
point of the sequence of events a recapitulation of phylogeny ; but
it does not necessarily follow that there is no tendency for it to be
so. It would seem that there is a constant inclination towards a
chronological parallelism between ontogeny and phylogeny, along
with frequently occurring interruptions and disturbances due to
circumstances possibly of an environmental or hereditary character.
It is not true then that " every creature climbs up its own genea-
logical tree," but it would seem to be true that it tries to do so ;
perhaps it sometimes swings from branch to branch ! At the same
time, even if the recapitulation principle be accepted in this partial
form, it must not be forgotten into how marvellously short a
story has been condensed so inconceivably long a history. In

ANISOTROPY AND ISOTROPY 15

the first six weeks of the antenatal life of the human embryo are
represented in epitome all the ages that have gone to the making
of man ! And each life adds on something to the ever-lengthening
tale !

Further than these seven principles it does not seem wise in the
present state of our knowledge to go. It would be easy, extremely
easy, to add to the number, but the additions would be hypothetical
generalisations, and they would also be hypotheses about which
embryologists are far from being in agreement. There is, for
instance, the question whether the various parts of the embryo
exist already in the ovum and need only to be rearranged during
organogenesis, whether each organ, each tissue, and each cell is
represented by a distinct rudiment (anisotropy of the ovum) ; or
whether the substance of the ovum is homogeneous and contains no
parts specially set aside to form definite structures in the embryo,
whether it is isotrojric. The weight of evidence is apparently in
favour of the isotropic character of the ovum ; but it would seem as if
in certain animals it were sometimes, and in others always, anisotropic.
The evidence is chiefly of an experimental kind, and is not free from
fallacy. At any rate, it is obvious that under the circumstances it
would be unwise to attempt to formulate a general principle on the
data at command. The same remark applies to the problem of the
causes and manner of the differentiation of parts in the embryo, to
that of morphogenesis, to that of regeneration, and to many other
biological questions.

Enough has been said to show that there are general principles in
Embryology, general laws, the appreciation of which will facilitate the
understanding of the architectonic phase of antenatal life. The out-
standing peculiarity of Embryology is still its complexity, for the
statement of general principles does not lessen that — it rather throws
it into bolder relief ; but surely behind this complexity there is a real
simplicity if we could find out the great determining factor in
embryo-formation. The results of the action of gravity are complex
enough, but behind their perplexing manifestations is the simple law
of gravitation. There may be some such simple and universally
applicable principle of ontogenesis, if it could only be discovered.
We need for Embryology an Isaac Newton " of an extraordinary
genius and proficiency in these things."

CHAPTER III

Chronology of Embryonic Life : Preliminary Considerations : Conditions found
(1) at the Beginning of the Second Week after Impregnation ; (2) at the
End of the Second Week ; (3) at the Beginning of the Third Week after Im-
pregnation ; and (4) at the End of the Third Week : Recapitulation.

In this chapter and in the three that follow it an attempt is made to
give a chronological sketch of the phenomena of embryonic life. The
period of antenatal existence from the appearance of the first rudi-
ments of the embryo in the embryonic area up to the transformation
of that embryo into the neofoetus is dealt with. The neofcetal period,
as readers of the first section of this Manual (p. 80) will remember,
is the transition time intervening between embryonic and foetal life.
Its leading character — gradual assumption by the embryo of a re-
cognisably human appearance — has been already described (pp.
80-84), and need not be referred to again. Let the reader, however,
try to carry in his mind the visual image of the chief changes of these
two eventful weeks (the seventh and eighth) of antenatal life, when
the facial region becomes clearly and unmistakably a face, when the
limbs become truly limb-like and no longer bud-like, when the caudal
projection ceases to project as a free appendage, and when a wonder-
ful series of internal alterations, including the commencement of the
ossification of many of the future 1 tones, accompanies the external
modifications. Let him also rememl >er that the neofoetus is, from the
point of view of its physiology, foreshadowing the foetus in its in-
creasing dependence upon its placental connections.

It is with the embryo, then, before the neofcetal epoch that
we have to do in these four chapters. The terminus of embryonic
life we fix at the end of the sixth week of intrauterine existence.
But at what time may it be said that the typically embryonic life of
the new organism begins ? We have announced the term inns ad quern,
but what declaration have we to make regarding the terminus a quo?

It may be admitted at once that it is not yet possible to fix
exactly the date when human embryonic life begins and germinal
existence ceases. If we regard the antenatal life of forty weeks as
beginning with the impregnation of the ovum by the spermatozoon,
then true embryonic life must manifestly begin at a date later than
that. How much later, however, we are not in a position to state.
If we allow a week, we are probably giving too much ; if we decide
upon less than a week, we may not be giving enough. In the absence
of exact information, shall we say a week ? Shall we let it be under-

16

CHRONOLOGY OF EMBRYONIC LIFE 17

stood that provisionally we regard the beginning of truly embryonic
life as coinciding with the beginning of the second week of antenatal
life, reckoning from the date of impregnation of the ovum ? I am re-
minded that we do not know at what time subsequent to coitus
impregnation occurs. That I know well, and I have, therefore, in
my scheme of the divisions of antenatal life (Fig. 1), set apart a
period of half a week during which the delegated spermatozoon is
presumably travelling towards the destined ovum. I make the
supposition that three and a half days after coitus impregnation
occurs and antenatal life in its unified form (i.e. within the walls of
one cell) begins ; a week later I presume that embryonic existence
commences with the appearance of the first rudiments of the embryo
in the embryonic area ; and in five weeks after that I assume that
typically embryonic life is over and neofcetal existence has begun.
This conception is shown in schematic form in Fig. 2.

These exact periods of time are not, of course, insisted upon.
They are advanced only as possibilities, which may turn out to
be probabilities, but which may also prove to be quite otherwise. I
do not indeed think it likely that it will be found that the whole
period of antenatal life in the ordinary sense of the term (say, forty
weeks or two hundred and eighty days) is an exact multiple of
embryonic existence. Our knowledge of Nature's operations and
mode of working out her results does not lead us to expect that she
deals with exact multiples, as we understand them. Nature, we can
well believe, in her calculations is not limited to the simpler exercises
of arithmetic ; she can deal with fractions !

Let us commence our study of the chronology of embryonic life
by realising how important it is that we should form in our mind a
visual image of the human embryo in the various weeks of its kaleido-
scopic existence. It is a most difficult task ; nevertheless, however
difficult it is, it must be attempted, else all efforts to form rational
ideas regarding the pathological phenomena of these embryonic times
must end resultless or in error. In the later weeks (e.g. the fifth and
sixth) of intrauterine life, the difficulties are not so overpowering, for
observations of human embryos of these ages are not wanting, and
conclusions may fairly be drawn from them. But in the early weeks !
We know almost nothing of human blastoderms of less than seven
days' age, we know but little of embryos of the second week, and even
up to the end of the first month embryological facts are scanty. The
qucesita, indeed, are many, but the data are few. So the embryologist
has been driven to study the early stages in the lower animals, and
has not always been careful to distinguish between the occurrences
which are met with in them, and those which there is good evidence
to show take place in the human embryo. In the text-books the
details of Comparative Embryology are often interpolated among those
of Human Embryology ; and writers, in transcribing from original
sources of information, are oftentimes careless about distinguishing
between what has been found, for instance, in the embryo chick,
rabbit, or pig, and what in the human subject. All this is very dis-
couraging, and, in order to correct many misapprehensions, it would

VOL. II. — 2

18

ANTENATAL PATHOLOGY AND HYGIENE

FIG. 2.

THE DIVISIONS OF EARLY ANTENATAL LIFE.

Days.

42

41

40

39

38

37

36

35

34

33

32

31

30

29

28

27

26

25

24

23

22

21

20

19

18

17

16

15

14

13

12

11

10
9
8
7
6
5
4
3
2
1

NEOFCETAL PERIOD

EMBRYONIC PERIOD

GERMINAL PERIOD

OVULAR LIFE

SPERMINAL LIFE

Events

Vide
CHAPTER VT

Vide

CHAPTER V.

Formation of
Embryo.

Vide
CHAPTER IV.

Vide
CHAPTER III.

Vide
CHAPTER III.

Formation of
Blastocyst.

Impregnation.
Insemination.

SECOND WEEK AFTER IMPREGNATION 19

be well worth some embryologist's time and trouble to edit a text-
book in which all the facts known certainly regarding the human
embryo were printed in red letters. I do not depreciate the value of
Comparative Embryology, but I deny that its data can be unquestion-
ingly transferred to till up gaps in our knowledge of Human Embryo-
logy. Human Embryology so constructed is simply a series of scanty
facts laced together by a series of assumptions.

The Blastocyst in the Second Week after Impregnation.

A. At the Beginning op the Week.

What mental picture can we form of the human blastoc}-st as it exists
during the second week after impregnation 1 Two things, at least, we may
rest well assured of: (1) the appearances of the blastocyst at the beginning
and end of this week will be very different, and (2) the embryo will, in size
and apparent importance, seem almost insignificant in comparison with its
annexa and the decidual membranes. Development is very rapid in these
early days, and a week is a long time in respect of the changes which are
effected in it. At a later date than the second week, the truly embryonic
part of the new organism is little more than an appendage of its own
vesicles and membranes, and it may be concluded that this character is still
more emphasised at the time with which we have here to do. Of these
two things we feel sure within measure, but of what else ?

Let us try to picture to ourselves what has taken place towards the close
of the first week after impregnation. The impregnated ovum, now doubt-
less a blastodermic vesicle or blastocyst, has found its way into the uterine
cavity. In so doing it has not dropped into an open space, as diagrams de-
ceitfully suggest to the mind's eye, but has insinuated itself between uterine
walls in complete contact, and possibly may have had some difficulty in thus
making its way. It passes some distance from the uterine orifice of the
Fallopian tube down which it has come, perhaps creeping on amcebadike by
means of the plasmodial syncytium with which it is covered, perhaps waved
onwards by ciliary action aided by a partial uterine contraction, perhaps
lifted forward by a sort of congestive elevation of the mucous membrane
upon which it is resting (who can say 1) ; at length it reaches its destined
anchorage. But what is it that constitutes a suitable mooring place, and by
what means is the anchorage rendered tenable 1 Perhaps there is found an
area of mucous membrane without its epithelial covering (or its cilia) where
the blastodermic vesicle can reach connective tissue ; perhaps there is a
little slit in the mucosa under the margin of which it can edge itself and so
get below the surface ; perhaps it simply rolls into the furrow between two
ridges and lies there till the swelling ridges meet over it and shut it in ;
perhaps it falls astride a ridge, and projecting downwards on each side of it
fills also the adjacent furrows, and so remains till neighbouring parts grow
up and around it ; perhaps it bores its way downward through the epithel-
ium by the destructive or phagocytic action of its covering cells, and the
little opening through which it passes is closed with a coagulum. It is easy
to multiply suppositions. Curiously enough one of the most obvious sug-
gestions— that the ovum passes or sends processes into the mouths of the
uterine glands — is that which nearly all embryologists nowadays agree in
rejecting, and that apparently on good grounds.

According to the view we take of the manner of mooring of the ovum

20

ANTENATAL PATHOLOGY AND HYGIENE

in utero will be the theory we hold of the mode of formation of the decidua
reflexa (s. capsularis). John Hunter's conception of its origin has heen
abandoned, but there seems no reason sufficient to warrant the abandonment
of the name which lie gave to it ; names in medicine do not always retain their

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original and etymological meaning. I shall, therefore, still use the term de-
cidua reflexa, without, of course, holding the Hunterian view of its formation.
II. Peters' specimen (Ueber die Eiribettung des nienschlichen Eies,
Leipzig u. Wien, 1*99) gives us the means of imagining how the reflexa is
formed, for its age was five or six days, and this decidual membrane was
still in process of differentiation. The specimen showed the ovum entirely

EMBEDMENT OF THE OVUM

21

sunk in the compact layer of the decidua vera about the middle of the
posterior wall of the uterus. Over each side of the ovum was an extension
of the vera forming the outermost portion of the reflexa, and the central
portion was made up of a mass of fibrin. It may be supposed, therefore,
that the ovum, after sinking down into the decidua vera, begins to excavate
laterally as well as deeply ; and so it comes about that the overhanging rim
of the mucosa (the vera) forms the reflexa, while the opening made by the
ovum is plugged with a coagulum of fibrin. In Leopold's earliest specimen
(Uterus und Kind, Leipzig, 1897), the age of which may be stated as the
end of the first week, the reflexa is seen surrounding the ovum entirely, but
it is thin and fibrinous in its polar portion, and the outer surface of the
ovum is intimately attached to it (Fig. 5). The differentiation of the

En*.

— %k. —

'

Fig. 4. — Section through embryonic part of Peters' early ovum.
A.H., amniotic sac; D.S. , umbilical vesicle; E.Sch., em-
bryo; Wet., ectoderm; Mies., mesoderm; Ent., entoderm;
Sp., fissure in exo-coelom (?). (After Peters.)

mucosa forming the decidua vera into a compact (surface) and a spongy
(deeper) layer is quite evident at this age ; in the reflexa near its base are
some glands lined with cubical epithelium, in the interglandular tissue are
some large deeply-stained cells (maternal or foetal ?), and there is no
epithelium on the inner aspect of this reflexa.

The mucous membrane to which the ovum is moored or anchored is the
serotina, now often called the decidua basalis or placental decidua. It con-
sists, even at the age of Peters' specimen, of a superficial compact layer and a
deeper spongy one. In the former part are signs of oedema with dilated
capillaries and blood spaces, some of which communicate with lacunae in
the tissue surrounding the ovum (the trophoblast). In the spongy layer are
small arteries. Large polymorphic cells are found in the compact portion,
possibly decidual and maternal in nature, possibly also trophoblastic and

22

ANTENATAL PATHOLOGY AND HYGIENE

foetal. There is a loosening of epithelium going on in the glands of
the serotina. In Leopold's specimen the serotina immediately below the
ovum has half the thickness of the mucous membrane in its near neighbour-

h 1 ; the surface capillaries open into the spaces (intervillous) between the

processes of the trophoblast, so that even at this early date maternal blood
bathes the outer aspect of the ovum.

In this manner, therefore, or in a manner somewhat like it (for it is
unwise to draw deductions hastily from the interpretation of so few speci-
mens), the fertilised human ovum becomes anchored to the uterine wall.
The term anchorage, however, hardly meets the descriptive requirements of
the case, when we think of the ovum surrounded as it is on all sides by

Fi<;. 5. — Section through Leopold's early ovum (end of first week of pregnancy). M.,
muscular wall with ends of glands (de) in it; D.v., decidua vera, with compact
(Comp. ) and spongy (Amp. ) layers ; I.e., leucocytes in the lymphatics ; D.c, decidua
capsularis or reflexa, in which up to dr. cp. gland spaces are seen, and above i i it
consists mainly of fibrin ; b h is the ovum or chorionic sac resting on a spur of the
decidua ; a, amnion ; d, decidua serotina ; e, mouth of maternal vessel ; h, attached
villi;/, imbedded villi. (Vide, Leopold's Atlas, Uterus and Kind.)

maternal decidual tissues; it will be more correct to speak of it as planted
in or imbedded in the uterine mucous membrane.

Let us now construct our mental picture of the ovum itself, as it
lies thus imbedded at- the end of the first week after impregnation.
At this date it consists evidently of two very unequal parts, one of
which may be called extra-embryonic and the other embryonic. Let us
look first at the former part, which is not only much the larger of
the two, but is also apparently of vastly greater importance. The ovular
mass (blastodermic vesicle, blastocyst, chorionic vesicle, or whatever other
name is given to it) has measurements varying from 1*6: 0*8: 0*9 mm.
(Peters' specimen) to 40: 37 mm. (Leopold's specimen); it is, therefore,

THE TROPHOBLAST 23

a lenticular mass which lies thus in the uterine mucosa. Its outer aspect
consists of a thick layer of what may he conveniently termed trophoblast,
which has heen probably developed from the epiblast of the ovum. In
this trophoblast layer are numerous lacunae and vacuoles, some of which,
according to Peters, contain maternal blood. The processes or strands
between the lacunae consist of nucleated cells with distinct outlines, and near
their outer ends some of them divide irregularly. In some parts the bl< >< 11 1
lacunae are lined by a nucleated protoplasm in which no cell-outlines are
visible, syncytial, therefore, in nature (Peters). Much difference of opinion
exists as to the mode of formation of the syncytium which plays so im-
portant a part in the union between maternal and ovular tissues. Perhaps
the most likely view is that it is formed by the action of the maternal
blood upon the trophoblast cells ; much of the protoplasm of the syncytium
may then be of maternal origin, while its nuclei are the changed nuclei of the
trophoblast (vide Handler's article, Amer. Journ. Obst., xlvi. 145, 1902). The
trophoblast, it is thought, invades the compact layer of the uterine mucosa,
while the maternal blood has a corrosive action upon the trophoblast cells, con-
verting them into syncytium. It is to be noted that as yet there are no villi,
but here and there processes of trophoblast make their way into blood sinuses
in the decidua. We may provisionally call the interspaces intervillous.
Beneath the trophoblast is a thin layer of mesoblast, but the latter scarcely
passes at all into the processes formed by the former. In Leopold's early
ovum there is a later stage of .the development of the union between ovum
and decidual membranes. In it the ovular mass is covered with villi (save
in one small part) ; some of these are branched, some simple ; some have a
mesoblastic tissue core, others are purely buds of syncytium ; some merely
reach the decidua (serotina and reflexa), others are slightly imbedded in
it ; some show capillary formation in progress in them, others do not.
Probably the villi in Leopold's ovum are the transformed strands of tropho-
blast seen in Peters' specimen. Between the villi are to be seen heaps of
decidual cells, the beginnings, no doubt, of the trabecular which are found
in later stages of development. In the intervillous spaces, also, maternal
blood circulates freely, for maternal capillaries open into them.

It is to be noted then, that even at so early a period as the end of the
first week after impregnation, the ovular mass is surrounded by a sort of
primary placenta. The epiblastic proliferation or trophoblast would seem
(as J. C. Webster states in his Human Placentation, p. 63, 1901) to
have the following functions : it acts, first, as a means of attaching the
ovum to the mucosa ; second, its strands probably serve as pathfinders for
the future permanent villi ; third, it probably absorbs nourishment for the
ovum from the decidual tissues and from the serum and blood with which
it comes in contact. It is possible also that fragments of its syncytial
covering may be broken off and travel into the maternal circulation
(Bandler, New York Med. Journ., lxxvii. 54, 1903), a possibility which
takes on great importance when considered in connection with the de-
velopment of deciduoma malignum and the pathology of pregnancy. The
trophoblast, it may be added, may be regarded as the same structure to
which the names of chorion, exochorion, primitive chorion + decidual
layer, epiblast of non-embryonic part of blastodermic vesicle, and ecto-
placental formation have been given by various writers. It is matter for
great regret that there is such a plurality of names ; it is probable that
the term trophoblast will gradually supersede the others, with benefit to
the reader who does not wish to solve troublesome terminological puzzles.

Let us now proceed with our inquiry regarding the constitution of the

24 ANTENATAL PATHOLOGY AND HYGIENE

ovular mass at the end of the first week after impregnation. What lies
inside the trophoblast? As has been said, there is inside it a thin layer
of mesoblast (two to four thicknesses of cells, Peters), a little thicker
opposite the pro-embryon. Its cells arc rounded, oval, and spindle shaped.
Inside the sac formed in this way by trophoblast lined with mesoblast is
situated at one point the pro-embryon (for we can scarcely yet call it
"embryo"). Here between the' mesoblast lining the trophoblast and the
epiblast of the pro-embryon is placed a small cavity, that of the amnion.
It is completely closed, and is lined with a layer of very flat cells opposite
the pro-embryon and with the cylindrical cells of the embryonic region
themselves. It is to be noted that in this earliest known specimen (Peters')
the amniotic sac is already closed, so that we can know nothing of its
mode of formation ; but the fact of its being closed suggests the question
whether it was ever open. Probably the amnion in the human subject is
not formed by the upheaval of folds of extra-embryonic somatopleure at all,
but by a breaking down of epiblast tissue to form a cavity (Berry Hart),
or by inversion of the blastoderm (Mall J). At any rate we have to put an
amniotic cavity into our mental picture of th'e ovular mass at the end of
the first week after impregnation. On the side of the amniotic sac next
to the centre of the ovular mass is the epiblast of the pro-embryon, and to
the inner side of it again is the umbilical vesicle lined with hypoblast cells
and showing also some mesoblast cells outside these. In the pro-embryon
or embryonic area, it is to be noted there is as yet no appearance of the
primitive streak, so that an amniotic sac and an umbilical vesicle are in
existence before any trace of the embryo itself appears. This, also, seems
td he the state of affairs in F. P. Mall's early ovum (Johns Hoplrins Hosp.
Hull., iv. 119, 1893). Neither is there any indication of a cord-like struc-
ture or Bauchstiel, for as yet the amniotic and umbilical sacs are, as it wTere,
buried in the mesoblast of the chorionic vesicle. Graf Spee, who carefully
examined Peters' specimen, could not be sure which was the cranial and
which the caudal end of the pro-embryon. The remaining space inside the
uvular mass is the extra-embryonic coelom or simply the exo-ccelom.

Such then is the conception we have to form in our mind of the new
organism in the uterus about the end of the first week after impregnation.
It is, so to say, only a peep that we thus obtain of antenatal affairs at this
early period. There is a great lacuna in our knowledge, a lacuna which
extends from the time when the human ovum leaves the ruptured Graafian
vesicle up to the time when it is found implanted in the uterine mucosa
with its amniotic sac and umbilical vesicle closed and the decidua reflexa
nearly completed. This gap in our knowledge we may attempt to fill up
by observations upon the lower animals, but it is not safe to conclude that
what occurs in them will occur in the human subject. We may also
attempt to supply the missing parts of the kinematographic representation
of the progress of formative processes from our imagination, but it is still
less safe to pursue this plan. So we must be content to wait till yet
earlier specimens of human ova are met with. It is the safest method,
but the waiting may be long. Better, however, is it to wait for facts
than to construct theories which have soon to be demolished.

B. At the End op the Week.

In order to picture to ourselves the state of affairs in utero towards the

end of the second week after impregnation, we have the specimens of Graf

1 .Mall, F. P., " Development of the Human Coelom," Journ. of Morphol. , xii. 395, 1897.

SECOND WEEK AFTER IMPREGNATION 25

Spee (12 days), Merttens (8 days (?) but probably more), F. Burgio (12 to 13
days), Reichert (12 to 13 days), Breus (12 to 14 days), F. P. Mall (13
days), Keibel (13 to 14 days?), Merttens (14 days), His (13 to 15 days),
Leopold (14 to 15 days), Eternod, and some otbers.1 Of course it must
be remembered that these ages are only approximate, for it is extremely
difficult from the history of the case to fix the probable age of the con-
ception. Further, the appearances and measurements of the embryos
cannot be used as definite guides, for there is some reason to believe that
all embryos do not progress towards a given stage in their development
with the same degree of rapidity. With these considerations in full view
we may now proceed to form an idea of the condition of the new organism
at the end of the second week.

At this date there exists, as all the specimens show, a distinct and
recognisable Embryo ; no longer have we to do with a pro-embryon or
embryonic indication. At the same time, the embryonic part of the
ovular mass is still insignificant in point of size when compared with the
embryonic vesicles and the decidual membranes. We shall, therefore,
first consider the decidual membranes, the chorion, the amnion, and the
umbilical vesicle.

The deeidua reflexa (capsularis) now forms a complete fold over the
ovular mass, but the outer polar portion is mainly, if not entirely, fibrinous
in nature, showing neither surface epithelium nor decidual cells, nor glands.
The deeidua serotina (basalis) exhibits clearly the division into a compact
and a spongy layer. The outer parts of some of the glands are obliterated.
Large decidual cells (many of them spindle-shaped) are seen in the compact
layer, as are also many sinuses containing blood. Nucleated plasmodial
masses are to be discovered lying among the decidual cells. In the spongy
layer the gland spaces are large, and are lined with columnar or cubical
epithelium. I )ecidual cells and plasmodium are also found in the spongy
part. On the surface of the compact layer of the serotina next to the
ovular mass is plasmodium or syncytium continuous with that covering the
villi (for there are now distinct villi). The general opinion held at the
present time is that this plasmodium is not formed from the original
surface epithelium of the deeidua or from that of the gland ducts, but in
the way which has been already indicated (vide p. 23).

Inside the decidual membranes (reflexa and serotina) lies the ovular
mass or chorionic vesicle (as we may now call it). It has a diameter
varying from 3 to 5 mms., and it is furnished at this date with recognisable
villi, no longer with simple strands of trophoblast. These villi are mostly
simple, but a few are branched, and they may cover the whole chorionic
vesicle or be defective at the poles ; they measure about '5 mm. in length ;
and they are attached to the deeidua reflexa and serotina. They are
covered with a coating of sjaicytium, and below that lies a layer of distinct
cubical cells, Langhans' layer. Nearly all the villi have now a mesoblastic
core of connective tissue. The syncytial covering of the villi is, as has
been already stated, continuous with that on the surface of the serotina
and reflexa. There are distinct intervillous spaces containing maternal
blood, and into these spaces masses of syncytium here and there project.
About this date (end of second week) capillaries are found in the mesoblastic
core of the villi. At the point of attachment of a villus to the deeidua

1 The references of the contributions in which these embryos are described will be
found in Hertwig's ffandbveh der vergleichenden und experiments? len Eniwickclungslehre
der Wirbelthiere, Jena, 1901-1903, and in other recent Text-books of Embryology, e.g.,
that of M'Murrich.

26

ANTENATAL PATHOLOGY AND HYGIENE

there is usually a great proliferation of the cells of Langhans' layer, forming
a distinct knob.

What now are the contents of the chorionic vesicle at this age 1 Attached
to the inner aspect of the chorionic vesicle by a stalk (Haftstiel, Bauchstiel,
anlage of the umbilical cord) is a mass which consists of the amniotic sac,
the umbilical vesicle, and the embryo (Figs. 6, 7, and 8). The amniotic sac
is closed, and it covers the dorsal surface of the embryo lying close to it ; on
the ventral surface is a wide opening leading from the hypoblasts or ento-
dermal interior into the umbilical vesicle. Both the amniotic sac and the
umbilical vesicle are small structures in comparison with the chorionic
vesicle. The embryo, measuring about 1*5 mm. in length, consists of a
mass of cells lying between the amniotic sac and the umbilical vesicle, and

continuous at its caudal end with
the Haftstiel or umbilical stalk.
In it can be recognised a primi-
tive streak (thickening of epi-
blast cells), and in front of it a
canal (the neurenteric) by which
the amniotic sac communicates
with the archenteron which is
now showing signs of division
into an intra-embryonic part (the
gut tract), and an extra-embry-
onic part (the umbilical vesicle).
In front of the opening of the
neurenteric canal is the medul-
lary plate with the two medullary
folds forming the medullary
groove which is not yet con-
verted into a canal. These parts
are formed by a thickening
and an infolding of the epi-
blast ; similarly on the ventral
surface there is an infolding of

Fig. 6. —Diagrammatic section of one-half of hypoblast which gives rise to

the notochord, which is at first
a hollow rod of cells communi-
cating at its posterior end with
the neurenteric canal. In some
of the specimens said to be of the
age we are now considering, namely, end of second week after impregnation,
there are indications of five or seven protovertebrae (the primitive segments
of Minot, Urwirbel of the Germans) in the mesoblast near the notochord.
There may also be a trace of the anlage of the heart near the cephalic end of
the embryo in the form of two tubes in the mesoblast. According to Graf
Spee there is, about this time, a small diverticulum from the single cavity
of the gut tract and umbilical vesicle into the Bauchstiel, which he regards
as the beginning of the allantoic duct (Fig. 8). Vessels also can be
recognised in the wall of the umbilical vesicle ; it is not clear that these
yet communicate in any way with the heart rudiment to constitute a
vitelline circulation. In Eternod's specimen (Anat. Anz., xv. 181, 1898)
there was a curious, hitherto undescribed vein in the caudal part of the
umbilical vesicle (" anse veineuse vitelline ") ; there was also a single
allantoic vein due to the fusion of the original two veins.

6. — Diagrammatic section of one-half of
Mall's early ovum. Enlarged 10 times.
The villi are only shown in part. Ec,
ectoderm ; en. , entoderm ; mes., mesoderm ;
u.v., umbilical vesicle ; coe., ccelom ; all.,
allantois ; a., amnion. (After Mall.)

SECOND WEEK AFTER IMPREGNATION

27

Such is, perhaps, as complete a picture as we can yet form of the
appearance of the chorionic vesicle and its contents at this period of
antenatal life. It leaves, of course, much to be desired. We know
nothing of the changes taking place in the human ovum as it comes down
the Fallopian tube. We know not how it develops its thick covering of
epiblast cells or trophoblast. We can only suppose that soon after its
arrival in the uterus it is furnished with a covering, apparently possessing
all the cellular activity of an epithelioma, by means of which it eats its way
into the mucosa of the uterus. While it has all the cellular activity of an
epithelioma (and more), it, however, has not the unrestrained or ill-
regulated mode of growth of such a tumour, for very soon an orderly
development of parts begins to be evident, and a primitive placenta is

Figs. 7, 8. — Diagrammatic section of two early human ova. Enlarged
10 times. About half of each is represented, and the villi are
outlined over only a part of the ovum. H., Rauber's layer; a.,
amniotic sac ; u.v., umbilical vesicle ; ec, ectoderm ; en., entoderm ;
mcs., mesoderm ; all., allantois ; c, notochord ; n.c, neurenteric
canal; H., position of heart. (After Graf Spee and Mall.)

evolved, while embryonic membranes, vesicles, and structures commence to
appear. From the point of view of the antenatal pathologist it is much to
be regretted that there is no specimen showing how, in the human subject,
the amniotic sac is formed and how the umbilical vesicle arises, for these
are structures which are supposed to have much to do with the origin of
teratological conditions. In the earliest known human ovum both these
structures are already present, and they give no indication as to their
ontogenesis.

The question of the age and size of human embryos is a very difficult
one. F. P. Mall's settlement of it is the best that we have at the present
time. Mall [Johns Hopkins Hosp. Bull., xiv. 29, 1903) has found that for
embryos of from one to one hundred millimetres in length their ages can be
fairly correctly estimated by multiplying the length in millimetres by one

28 ANTENATAL PATHOLOGY AND HYGIENE

hundred, and then extracting the square root, when the result will be the
age in days. The formula is J 100 x length in mm. = age in days. For
foetuses of from 100 to 220 mm. in length, the length in millimetres equals
the age in days. It is useful to remember the following leading facts,
which are fairly well established : embryos of 1 mm. are about 12 days old ;
of 2*5 mm., about 14 days ; of 4*5 mm., about 19 days ; of 7 mm., about 26
days ; of 11*5 mm., about 34 days; and of 17 mm., about 41 days. After
forty-one days the embryo becomes the neofoetus, and its later measure-
ments will he found in the first section of this Manual, which deals
specially with the foetal period of antenatal life.

The Embryo in the Third Week after Impregnation.

Several embryos of the third week are available to enable the embry-
ologist to construct his picture of developmental events at this period; but,
us it happens, there is a scarcity of material showing the state of the
decidual membranes at this time. It is truly an hebdomada mirabilis for
the embryo, this third week after impregnation, fur during it there is a
marvellous differentiation of organs and tissues taking place, and a trans-
formation scene going on, the details of which are so complex as to baffle
the keenest observer. We cannot trace out the processes in their intricacy,
but we can at least recognise the results.

Through the growth of the chorionic vesicle and its contents, the decidua
reflexa comes in contact with a larger surface of the decidua vera, and the
potential space between these two membranes is diminished ; for at the be-
ginning of this third week the chorionic vesicle measures 14 mm. by 12 mm.
by 5 mm. (Leopold's fifteen day specimen). Decidual cells are seen in the
ivllexa as well as in the serotina, and in the lower and middle part of the
former there are enormously distended capillaries. In the compact layer of
the serotina are likewise numerous dilated capillaries. The outer ends of
the glands are very narrow, appearing as slits. In the spongy layer the
gland spaces are irregular, and contain much desquamated epithelium.
Here and there are extravasations of blood. The relations of the ovum
to its membranes are well seen in Guicciardi's fifteen days' specimen
(Fig. 9).

The mode of attachment of the chorionic vesicle to the reflexa and
serotina is such as has been already indicated. Possibly the villi are even
now becoming more numerous in relation to the serotina than to the reflexa,
indicating the commencement of the differentiation of the chorion into a
villus-carrying part (chorion frondosum, placental part) and a portion which
is bare (chorion lseve, non-placental part). As yet, however, there is a
circulation going on all round the chorionic vesicle (in its reflexal as well as
in its serotinal part), and no indication of a limitation of it to any one area.
The villi are covered with syncytium with Langhans' layer underneath, and
in their mesoblastic cores capillaries can be clearly seen. In Guicciardi's
specimen (Ann. di ostet., xxiv. 176, 1902) only a few villi were vascu-
larised.

With regard to the structures inside the chorionic vesicle in the third
week, it has to be noted that the amnion is at first a thin membrane
springing from the body of the embryo round the origin of the umbilical
vesicle. It, therefore, surrounds the back, sides, and cephalic and caudal
ends of the embryo, but has no relation as yet to the umbilical vesicle (or
yolk-sac) and to the part of the embryo winch communicates with that

THIRD WEEK AFTER IMPREGNATION

29

vesicle. It is closely applied to the embryo, there being as yet little liquor
amnii. During the third week the amniotic fluid increases relatively in
amount, but even at the end of the week the membrane is not far removed
from the embryo, and does not surround the umbilical vesicle and scarcely

an

■m,,\

uv

c/t

?r-

4' ' ■ '"' '- '

/ch

■ ■ .-•■■ ■■■' .'.■:xy ■ ■', '■ *\

■■■' .- .- -- ■"*•'■ .VV ■■.';•"•'■••■•.' ■ > vr---Ok\ >>T

1.

** jPf *"

1

't

&'

Fig. 9. — Guicciardi's Early Gestation Sac. Section showing — cs., caduca or decidua
serotina, at one pole of ovum ; cp., decidua vera ; cr., caduca or decidua reflexa ;
v., chorionic villi; Ich., tract in which the chorion comes into relation with the
decidua, a zone of amorphous tissue alone intervening; ch.-ch., chorionic epi-
thelium and connective tissue ; am., amnios ; E., embryo ; UV. , umbilical vesicle.
(Vide Annali di ostctricia, xxiv. p. 176, 1902.)

covers the heart. In its microscopic characters the amnion about this period
shows a differentiation : there is an epiblastic layer of somewhat endothelial
cells as before, but the mesoblastic tissue now consists of an outer layer
similar in appearance to the epiblast, called the mesothelium, and of an
inner homogeneous matrix next to the epiblast layer.

30

ANTENATAL PATHOLOGY AND HYGIENE

Embryo at the Beginning of the Third Week.

For our knowledge of the appearances of the human embryo, about the
beginning of the third week after impregnation, we are indebted to the
study of the specimens of J. Kollmann, of C. S. Minot (Nos. 195 and 143),
of His (Lg, Sch. 1, and L), of A. Kondino, and of Coste. The embryo
now measures from 2 -2 mm. to 2 '5 mm., and is still almost insignificant in
size when compared with the chorionic vesicle and the decidual membranes ;
but it already shows a marvellous advance in the differentiation of its parts
(Fig. 10). The cephalic and caudal ends are quite recognisable, and both of

Fig. 10. — Embryo at the beginning of the third week.
Embryo Lg. (After His.)

these project a little beyond the umbilical vesicle, which is now no longer
attached to the whole length of the ventral aspect of the embryo. (In
Kallmann's 2*2 mm. embryo the umbilical vesicle was attached for a dis-
tance of 1*5 mm., leaving the head to project 0'58 mm. and the tail 0-3
mm.) The cephalic end is already showing signs of enlargement, a feature
which is so characteristic at later stages of embryogenesis, and is bending
slightly towards the ventral aspect. There may be no branchial arches
(Kollmann's embryo), or there may be two (Minot's Nos. 143, 195) or
three (Coste's). No indication of the eye or ear may be found (Koll-
mann's embryo); or the optic vesicles may have already grown out, and the
epiblastic invagination which is to form the otocyst or auditory vesicle may
have begun to form (His' Embryo Lg). The oral invagination can be seen,
but the oral plate still separates it from the large primitive pharynx ; below

THIRD WEEK AFTER IMPREGNATION

31

it is the first branchial arch, which by and by becomes the mandible, and
below that is the first branchial slit, or rather groove, with the second or
hyoidean branchial arch. The brain (vesicular in character) forms a large
part of the whole embryo : there is a fore-brain from which the optic
vesicles have grown out; at the junction between the fore- and mid-brain
there is a sharp bend forwards (almost at a right angle) of the former part;
the hind-brain is relatively very long, and near its centre lies the open in-
vagination of the otocyst (Fig. 11). The greater part of the medullary

Fig. 11. — Embryo at the beginning of the third week,
seen in section. Enlarged about 37 times. (After
His.)

groove, therefore, is now closed in to form the medullary canal ; the part
posterior to the hind-brain is not yet differentiated into the spinal cord.
Between the fore-brain and the attachment of the umbilical vesicle, and
lying in a large pericardial space, is the prominent heart, which consists of
an inner tube formed of endothelial elements, and an outer tube of con-
tractile elements, which ultimately form the heart muscle. This heart tube
is asymmetrically bent : from the point where the great veins enter it, it
runs towards the head and left side, making the auricular limbs ; then it

32 ANTENATAL PATHOLOGY AND HYGIENE

turns to the ventral side and obliquely to the right, forming the ventricular
limb ; and then takes a curving course to the median line, forming the
aortic limb, and ends close behind the mouth. Between the two heart
tubes (endocardial and myocardial) is a considerable space ; and the inner
or endothelial tube is continuous at one end with the walls of the veins
and at the other with those of the aorta. We note, therefore, that by this
date the two endothelial tubes, which represented the heart in the second
week, have fused into or become one. From the aortic limb (outside the
pericardial sac) arises the aorta, which divides into two branches on each
side which, as the aortic arches, pass round the pharynx. The front branch
on each side curves over and joins the second, to form one of the two dorsal
aorta?. The dorsal aortae pass towards the caudal end of the embryo ; about
midway they fuse into one dorsal aorta, which near the tail bifurcates into
two vessels (the allantoic or umbilical arteries), which pass through the
Bauchstiel (or allantoic stalk) to the chorion. In an earlier stage, doubt-
less, the dorsal aortse pass, without fusing, to the umbilical vesicle as vitelline
arteries, but of this we have insufficient evidence from human embryos.
The veins which proceed to the heart consist of the cardinal, the jugular,
the omphalo-mesenteric or vitelline, and the umbilical. On each side of
the body the jugular vein (from the head end of the embryo) joins the
cardinal vein (from the tail end) to form the ductus Cuvieri, which passes
into the sinus venosus and through it into the heart. The ductus Cuvieri
is joined by the umbilical or allantoic vein of the same side, which comes
from the chorion by way of the Bauchstiel, and then takes, as Minot says,
a short cut through the somatopleure along the base of the amnion. The
omphalo-mesenteric or vitelline vein opens into the sinus venosus. There is
as yet no trace of the inferior vena cava, and, since nothing more than a slight
evagination or outgrowth from the fore-gut represents the liver, the veins
have not the complicated hepatic relations which they afterwards develop.

With regard to the intestine or embryonic part of the archenteron at
this age, it is to be noted that it is divided into three parts. Of these the
fore-gut is expanded anteriorly into the wide primitive pharynx with two
gill-pouches. The middle portion communicates freely with the umbilical
vesicle, and shows the anlage of the liver just where it is continuous with
the fore-gut. The posterior portion is distinct from the umbilical vesicle,
and ends in a dilatation (His' bursa), and from the under surface of this
dilatation arises the allantoic diverticulum which passes into the Bauchstiel
{vide His' Embryo Lg, Figs. 10 and 11). As already stated, the fore-gut is
shut off from the oral invagination by the oral plate (Rachenhaut).

At this period (beginning of third week after impregnation) the noto-
chord can be seen distinctly, flanked with a varying number of proto-
vertebr?e, or, to use Minot's more correct term, primitive segments. These
primitive segments, which perhaps even at this age show a division into the
myotome next to the medullary canal and the nephrotome (or intermediate
cell mass) which lies distally, vary in number from fifteen (Kollmann's
specimen) to twenty-nine (His' Embryo Lg). One of the most remarkable
features presented by embryos of the beginning of the third week is the pre-
sence of what has been called the dorsal flexure (seen well in Minot's Em-
bryo, No. 195, and in His' Embryo Lg). By this term is meant the bending
of the column of primitive segments in such a way as to form a deep dorsal
concavity, possibly in order to accommodate the long back of the embryo.
Minot says about this (Hitman Embryology, p. 313, 1892): "the facts
indicate that the back is too long for the somatopleure at the side of the
body, and that it finds room ... by becoming concave ; later it springs

THIRD WEEK AFTER IMPREGNATION 33

into a new position of equilibrium by becoming convex ; it is possible that
the change from the concave to the convex position is very abrupt, and it
is probable that the time of its occurrence is very variable." Tins flexure
may sometimes owe its existence to accident, but I agree with Minot that
His' observations prove it to be normal in certain instances at least. From
the teratologist's point of view, its presence is particularly interesting, as it
is apparently maintained permanently in some monstrosities {e.g. in inience-
phaly and exomphalos, vide Plates 15, 16, 17, and Fig. 28).

In order to complete our survey of the state of the embryo at this epoch,
we must now glance at its caudal extremity. The prolongation of the body
of the embryo by which it is attached to the chorion has now become a
distinct stalk (lUiuchstiel, body-stalk, allantoic stalk, later the umbilical
cord). On account of the bending forwards of the caudal extremity of
the embryo, the origin of the liauchstiel likewise comes to be situated
more on the ventral aspect. The stalk contains at this date the allantoic
arteries and veins, and the allantoic diverticulum from the archenteron. It
is appreciably nearer to the wide stalk of the umbilical vesicle, but it has
not yet come into contact with it.

Embryo at the end of the Third Week.

"We form our visual image of the human embryo at the end of the
third week after impregnation from the study of the specimens of Chiarugi,
His (Embryos, Rf, M, BB, and Lr), Allen Thomson, Ecker, Hecker, von
Baer, J. Muller, and R. Wagner. Organogenesis is still proceeding at a
very rapid rate, for the embryo at the end of the third week differs in
many particulars from the embryo at the beginning thereof ; and we have
not nearly enough observations of human embryos of these ages to enable
us to fill up the gaps in our knowledge of the means by which one stage
passes on into the next. It is undoubtedly a wonderful week (liebdomada
mirabUis) in the history of the embryo; all the story of embryological
development is wonderful, but the third week is so essentially a week of
beginnings that it arrests our attention, fascinates us, and finally amazes us
by the variety and complexity and rapidity of the changes which supervene
upon these beginnings. When the first rudiment of an organ or part has
appeared, it is not so difficult or so surprising to follow it in its progress
into its mature form ; but to see it emerging in the first instance out of a
sort of pre-enibryonic chaos, that it is which startles, astounds, and almost
stupefies us. Had not one of the writers of old time a dim adumbration
of all this when he wrote these words ?

"Thine eyes did see mine unperfect substance,
And in thy book were all my members written,
Which day by day were fashioned,
When as yet there was none of them."

To return to the description of the embryo at the end of the third week,
it may be stated generally that all the systems and parts which were found
indicated at the beginning of the week are now much further advanced in
development, while some new parts have for the first time become evident.
The umbilical vesicle is now attached by a relatively much smaller base to
the ventral aspect of the body than formerly, so that we may reasonably
begin to speak of the pedicle of the umbilical vesicle or yolk-stalk ; the
vesicle itself has become pear-shaped. It is still growing, indeed it con-
vol. II. — 3

34

ANTENATAL PATHOLOGY AND HYGIENE

tinues to increase till the end of the fourth week, but its rate of growth
is slackening. The embryo measures at this age from 2-66 mm. (His'
Embryo M) to 3'2 mm. (His' Embryo BB), or even to 4-2 mm. (His'
Embryo Lr). The measurements of the whole chorionic vesicle may be
7"5 mm. x S"0 mm. (His' Embryo M), or 11 mm. x 14 mm. (His' Embryo

BB), or 12 mm. x 9 mm. (Ecker's
Specimen), or 12 mm. x 12 mm. x
9 mm. (Chiarugi's Specimen).

The head projects well beyond
the umbilical vesicle, and shows
a slight twist to left or to right ;
this twist is part of the spiral form
which the whole embryonic body
has assumed, the caudal end ex-
hibiting a twist to the opposite side
as compared with the head. There
are now three (or four) branchial
grooves. The mouth is relatively
large ; it is deeper than at the be-
ginning of the week on account of
the rupture of the oral plate ; it
communicates, therefore, with the
fore-gut (Fig. 12). On each side of
the mouth at its upper corner can
be seen the maxillary process from
the first branchial arch or mandi-
bular process, which is also very
evident. The pharynx is still
wide, and four pouches can be
easily recognised ; each pouch lies
between two branchial arches.
A new development is the pres-
ence of a small projection on the
ventral floor of the pharynx in
the middle line ; this is the tuber-
culum impar of His ; and from it
arises part of the tongue. With
regard to the nervous system, it
must be noted that the medullary
or neural canal is now closed in
its whole extent. The fore-brain,
which has become quite distinct
from the mid-brain, is divided into
two parts, the secondary fore-brain
(prosencephalon or telencephalon)
and the inter-brain (thalamen-
cephalon or diencephalon) ; or rather, as Minot believes, the anlages of the
cerebral hemispheres arise from the anterior end and dorsal side of the fore-
brain, which, on account of the head bend which is present, grows downward
and forward. The optic vesicles are by this time clearly stalked, and the
otocyst is a closed pear-shaped vesicle. With regard to the primary head
bend or flexure, to which reference has been made, it is to be noted that
now it is so marked that no longer is the fore-brain at right angles to the
hind-brain (by a flexure situated in the mid-brain), but it has become so

'** ■'- "• ~-"

x

Fia. 12.— Embryo at the end of the third
week. Embryo BB. Enlarged about 37
times. (After His.)

THIRD WEEK AFTER IMPREGNATION 35

sharply bent that its floor is almost parallel with the floor of the hind-
brain. It must be remembered that the fore-, mid-, and hind-brain are
not at this stage of development anything more than vesicles. At the
junction of the hind-brain with the spinal cord is the medulla oblongata,
and in it even at this date it can be seen that the central canal is wider
in the dorsal than in the ventral portion ; at this point, also, there is
an indication of the second cerebral flexure or neck-bend which appears
first as a projection (His' NackenhocJcer). In front of this region is
the anlage of the cerebellum, and behind it is the spinal cord.

The heart, as seen in His' Embryo BB, is now an S-shaped tube, " the
venous end is convex toward the head, the arterial end convex toward the
tail " (Minot). From the arterial end spring the aortas which give off five
branches (aortic arches) on each side of the neck ; these branches unite again
on the dorsal side, and passing backward the resulting trunk joins that of
the opposite side to form the single median dorsal aorta, which subdivides
into the two umbilical or allantoic arteries. Each of the aortic arches
helps with the surrounding tissue to make a visceral or branchial arch ;
and between the five arches are the four visceral, branchial, or gill clefts or
grooves (for they are never really clefts in the human embryo). The
aidages of the true auricular cavities of the heart have now appeared,
and the two limbs of the ventricle are nearer together and form a
distinct apex where they join. The heart area of the embryo is quite
covered by amnion at the stage of development at present under con-
sideration. The veins consist of the cardinals and jugulars, of the
ducti Cuvieri formed by their union, the omphalo-mesenterics and the
umbilicals ; the three last named come into close relation with the anlage
of the liver and also with the septum tvansversum, while the cardinals are
anatomically associated with the developing Wolffian bodies (to which I
shall immediately refer). The development of the septum transversum
and of the diaphragm is a peculiarly complex and obscure part of organo-
genesis ; but upon it the researches of Franklin P. Mall (Johns Hopkins
Hasp. Bull, xii. 158, 1901) and others have thrown much light. The
septum transversum is, to begin with, a V-shaped bridge of mesodermal
tissue (irregular round cells, numerous vessels communicating with the
veins of the heart) which connects the umbilical vesicle with the embryo
at the juncture of the head with the amnion. It supports the omphalo-
mesenteric and umbilical veins, and contains in it the anlage of the liver
as well as that of the diaphragm, and all these structures are at a high
level in the body of the embryo in the neighbourhood of the structures of
the head; indeed nearly the whole coelom at this stage of development
lies in the head and neck region. The septum transversum serves to divide
the coelum anterior to it or headward of it (the parietal cavity of His)
from that which lies posterior (tailward) to it (the trunk cavity of His) ;
but laterally the two cavities communicate by passages, the parietal recesses,
each of which divides later into a ventral and a dorsal part. Ultimately
the parietal cavity becomes the pericardial sac, while the trunk cavity with
the dorsal parietal recesses becomes the pleuro-peritoneal cavity. By the
end of the third week the liver diverticulum, now enlarged and branching,
is found embedded in the septum transversum. Through the descent of
the septum all these associated parts greatly change their relations to the
framework of the body.

At this date, also, traces of the Wolffian bodies can be recognised as little
canals in the Wolffian ridges ; the latter are longitudinal ridges, one on
each side of the dorsal surface of the coelom of the abdomen. The tubules

36 ANTENATAL PATHOLOGY AND HYGIENE

of the Wolffian body (mesonephros) are evidently developed from the
nephrotomes or intermediate cell masses of the primitive segments of the
neighbourhood. The blood from these bodies passes to the cardinal veins.
Thirty or more primitive segments or protovertebrae can be recognised (His'
Embryos BB and Lr), and the dorsal flexure has now disappeared, a con-
vexity taking the place of the concavity which was present at the beginning
of the third week. A tendency is present for the embryo to bend forwards,
the cephalic and caudal extremities approximating, and this tendency
becomes more evident in the fourth week. The curving forward of the
caudal end of the body brings the insertion of the Eauchstiel still more to
the front, and so it and the umbilical vesicle lie nearer to each other than
formerly.

Recapitulation.

Thus, in these two weeks, we see the embryo developing from
a mere disc of tissue resting upon a relatively large umbilical vesicle
into an organism with a circulatory apparatus, a series of vesicles
constituting a nervous system, an intestinal canal, a body cavity
with a commencing septum dividing it into a thoracic and an ab-
dominal portion, a mouth consisting of a five-sided fossa, a pharynx
with branchial arches and clefts, a series of mesodermic somites or
primitive segments, and rudiments of eyes, ears, and Wolffian bodies.
This organism no longer rests, insignificant in size, upon its umbilical
vesicle, but is of relatively considerable size, and has its umbilical
vesicle appended to it by a recognisable vitelline stalk. All these
structures have appeared during these eventful weeks ; and they have
come into being by a series of changes of which we know very little
from the direct examination of human embryos, but about which
embryologists have formed many theories from the observation of the
early stages of development in the lower animals. We cannot, in
consequence, be at all sure of the manner in which many of the
transformations and alterations have been produced. We know
nothing, for instance, of the way in which the neurenteric canal is
closed, and but little of the relation of the allantoic stalk to the
Bauchstiel. In whichever direction we attempt to follow out the
problems of the subject, we immediately feel the pressure of the
limitations of our knowledge. At the same time the feeling of
restriction thus produced must not be suffered to engender in us the
idea that we are disqualified from ultimately searching out the
secrets of organogenesis. When we think of the progress that has
been made in the last twenty years, when we reflect how much the
finding of such a specimen as Peters' early ovum may mean for the
elucidation of our problems, and when we remember the hundreds of
skilled workers engaged in this field of research, we take courage
again and press forward. Every advance, it is true, but adds to our
perception of the regions yet unknown ; but this was to be expected,
for the domain of Embryology is of vast extent. We are not, there-
fore, to be hindered but stimulated by the prospect ever opening up
before us ; the wider view is in itself a gain. Let us then go on
carefully preserving early embryos, cutting them in serial sections,

RECAPITULATION 37

and reconstructing them again, so as to obtain an intimate know-
ledge of their inmost constitution. Scarcely any two will be found
perfectly alike, and we may at any time make a discovery rivalling
that of the detection of the neurenteric canal in the human embryo.
Let us also examine all uteri and appendages removed by hyster-
ectomy, vaginal or abdominal ; such operations are common now-a-
days ; we may thus add to the number of the known specimens of
early ova in situ in the uterus, we may even find an impregnated
ovum in the tube on its way thither.

CHAPTEK IV

Chronology of Embryonic Life (continued) : Conditions found in the Fourth

Week after Impregnation.

In the previous chapter an attempt was made to place in graphic
fashion before the reader's mind a picture of the conditions found
inside the uterus during the second and third weeks after impregna-
tion. The picture was confessedly an imperfect one : many details
were dimly represented because imperfectly known ; others were
only, so to say, outlined in the rough ; while others were altogether
wanting. Yet, after all, some idea was gained of the nature of the
processes of organogenesis going on at this period of antenatal life.
Let us now, in this chapter, try to do at least as much for the fourth
week of embryonic existence. The attempt is likely to be more
successful, for the number of known and examined specimens of
embryos of this period is much larger than at the earlier dates. Let
us pursue the same plan and consider first the extra-embryonic parts
of the gestation sac and then the embryo itself.

Extra-embryonic Parts in the Fourth Week.

Regarding the decidual membranes at this period of intrauterine life
we gain some information from the description given by E. Fraenkel (Arch,
f. Gyncek., xlvii. 139, 1894) of an aborted ovum, three and a half weeks
old, and containing an embryo which measured 4-3 mm. in length and
possessed thirty pairs of primitive segments. The sac formed by the
decidua reflexa (cajjsidaris s. circumflexa) measured 16 mm. in width at
its base by 10 mm. in height. Two parts of the reflexa could be easily
distinguished: a thin polar portion with no regular structure, with scarcely
any traces of glands, and with signs of commencing degeneration; and a
thicker basal portion, the inner half of which was more compact (no blood
sinuses and very few glands), while the outer half, especially near its
junction with the decidua vera, showed several gland orifices and many
blood sinuses. The decidual cells were more compressed in the inner than
in the o\iter half. In the decidua serotina the large decidual cells were
practically limited to the compact layer. Blood sinuses could be recognised
opening into intervillous spaces both in connection with the serotina and
the reflexa. C. Kupffer's specimen, of about the same age, showed similar
conditions (Miinchen. med. Wchnschr., xxxv. 515, 1888); in this case
also villi were found over the whole surface of the chorion although they
were most strongly developed in an equatorial band. An epithelial double

38

FOURTH WEEK AFTER IMPREGNATION 39

layer covered the chorion and the chorionic villi (Fraenkel's specimen), the
outer layer being, as at an earlier date, plasmodial or syncytial, and the
inner cellular. In the neighbourhood of the polar portion of the decidua
reflexa the syncytium was very thick and Avas covered with a blood co-
agulum ; in this spot the cellular layer was very thin. The stroma of the
chorion reached its greatest thickness opposite to the decidua serotina. The
villi were most numerous where the decidua serotina passed into the
decidua reflexa. Kastschenko (Arch. f. Anat. u. Entwchlngsgesch., p. 451,
1885) has described a fine network of threads in the syncytial layer at this
age of pregnancy, and he found the syncytium thickest over the ends of
the villi, while buds and strands extended from their sides in the form of
processes. The amniotic membrane in the fourth week has very similar
appearances to those described already (in the preceding chapter) as existing
at the third week. The amniotic sac, on account of the rapid growth of
the embryo, is at this time almost entirely filled by it, so that the amnion
is closely applied to the embryo. This space between the amniotic sac and
the wall of the chorionic sac, the extra-embryonic coelom, is still large.

The umbilical vesicle or yolk-sac is now quite extra-embryonic in
position, and is attached to the body by a more constricted pedicle, at first
short and later of a considerable length. Towards the end of this week the
Bauchstiel or allantois-stalk has grown partly round the proximal part of
the yolk-stalk, so that we can now speak of the umbilical cord, although it
is not yet very cord-like. The umbilical vesicle, in this week, reaches its
greatest development; it measures from 2 "7 by 3*0 mm. (His' embryo a) to
5-0 by 7*0 mm. (Mall's embryo of 26 days). The umbilical vesicle can be
recognised as differentiated from the yolk-stalk, and the cavity in the latter
(vitelline duct) is soon obliterated.

The Embryo in the Fourth Week.

For the study of human embryos of the fourth week (22 to 28 days)
several specimens are available, among which may be named His' embryos a,
D2, W, R, B, A, Eck 1, and Stt, and those of C. Kabl, Coste, Allen Thom-
son (fourth embryo), Hensen, Ecker, Fol, B. H. Buxton, Mall, J. Midler,
V. Magnus, and Waldeyer.

The embryo of this age measures from 5 to 7'5 mm. in length ; but the
organism is now so coiled upon itself that it is difficult to ascertain its real
length. The dorsal flexure of the preceding week has entirely disappeared
and is replaced by a well-marked and rounded convexity posteriorly, which
is due to the approximation on their ventral aspect of the caudal and
cephalic ends. The embryo is shaped like a C, the upper end of the C
being the fore-brain and the lower end the embryonic tail ; or, rather, the
organism is so rolled up that its dorsal outline describes more than a com-
plete circle (Fig. 13). In addition to the head -bend (in the region of the
mid-brain), there is also a well-marked cervical flexure or neck-bend (at the
posterior limit of the hind-brain) ; and the well-developed tail is sharply
bent forwards at the sacral bend. So it comes about that the length of the
embryo from the head-end to the tail-end does not represent the total extent
of the organism, thus His' embryo a measured 4 mm. but when unrolled
13 "7 mm.

The body shows still the spiral twist which was noted at the end of the
third week : the head is bent to the right and the tail to the left in His'
embryo a. The Bauchstiel or allantoic stalk is now becoming more stalk-

40

ANTENATAL PATHOLOGY AND HYGIENE

like ; on account of the marked ventral concavity of the trunk and the
development of the tail, it lies near to the yolk-stalk and partly surrounds
it, as has been already pointed out.

A very noticeable change in the external appearance of the embryo is
now visible, namely, the indication of the limb-buds. Possibly they may
be recognised in some specimens of the end of the third week ; at any rate,
they are quite distinct at the beginning of the fourth. They are at present
no more than slight swellings or flat processes arising from the ridge
(Wolffian ridge) which runs along the dorsal border of the embryonic
somatopleure. In structure they consist of rounded mesoblastic cells with
an external layer of epiblast. Each limb-bud has an upper, dorsal, or

Fig. 13. — Embryo of the fourth week. Embryo a (after His). Enlarged
20 times. G.V., vagus ganglion ; G.GL, ganglion of glossopharyn-
geal ; Gh., otic vesicle; G.ac, auditory ganglion; G.G., Gasserian
ganglion; Vh., heart; VI. , liver; U.E., lower extremity; Oh.,
chorion.

extensor surface, and a lower, ventral, or flexor one ; and there are two
borders, an anterior or cephalic, and a posterior or caudal. Into these
limb-buds grow processes from the myotomes of a number (not less than
seven) of the primitive segments. The extremities are at this time truly
appendicular huch and not limbs, for they are quite unsegmented. The
anterior ones are larger than the posterior. As I have pointed out in the
first section of this Manual, the limbs belong to the outlying parts of the
developing organism and so are late in showing differentiation ; thus it
comes about that in the fourth week, when other systems are far advanced
in development, the extremities are only little mesoblastic outgrowths.

The embryo is still somewhat translucent, so that the internal parts can
be dimly discerned. Thus it can be seen that there are at least twenty-

FOURTH WEEK AFTER IMPREGNATION 41

seven primitive segments or somites (Mall's 26 day embryo, Figs. 14, 15,
16), and there may be thirty or more (cervical 8, dorsal 12, lumbar 5,
sacral 5). There were thirty-five in Magnus' specimen. In the region of
the head the outlines of the five chief divisions of the brain can be recog-
nised, and the margins of the fossa rhomboidalis (future fourth ventricle)
are distinct ; the ganglia of some of the cranial nerves are also visible, and
to them reference will be made below.

Three or four branchial grooves and arches can be made out during this
week; they have irregular outlines, and are beginning to be transformed
into the more permanent arrangement of parts. Towards the end of the
week the sinus praecervicalis is well marked, and the fourth branchial arch
lies deeply in it, and largely under cover of the third arch ; the third arch,
also, is within the sinus. This cervical sinus is formed by the growth of
the second arch over the third and fourth arches till its tissues come into
contact with the body wall beyond them in the region of the faintly marked
fifth arch ; thus the epiblast of these arches and clefts is buried. This
change, however, is only beginning in the fourth Aveek. From the dorsal
or attached end of the first branchial or mandibular arch a projection can be
seen arising ; this is the superior maxillary process, and it can be recognised
both in the beginning and towards the end of the fourth week (e.g. in His'
embryo a, and in Mall's 26 day embryo). The ventral or free end of the
first arch is knob-like, so also is that of the second or hyoid arch ; in fishes
it forms the operculum for the gills, while in the human embryo, as above
stated, it serves to close in the sinus praecervicalis. It is to be noted that
neither now nor at any other time do the branchial furrows or grooves in
the human subject become continuous with the pharyngeal pouches ; the
epiblast conies into touch with the hypoblast (no mesoblast intervening) to
form the "closing membrane," but there is no communication between the
external branchial clefts and the internal pharyngeal pouches.

During this week another change in the external appearance of the
facial region of the embryo occurs, the appearance of the first indications
of a nose. On each side of the naso-frontal process which grows down
between the two superior maxillary processes is an epiblastic thickening,
the olfactory plate (as in His' embryo a) ; these two olfactory plates, after
the twenty-fifth day, become hollowed out (or, rather, their margins grow
up) to form the nasal pits (as in His' embryo a or in Mall's 26 day embryo).
The pits, however, remain shallow during this week ; further, their margins
do not grow up on the side next the mouth, and so they open freely into
the oral cavity. From the nasal pits the nasal mucous membrane ultimately
develops. Just outside and anterior to the nasal pits are situated the optic
vesicles which are now stalked and converted into optic cups ; and each
shows (at the end of this fourth week) a lens surrounded by a groove
which also passes down between the superior maxillary process and the
nasal pit of the same side. The lens is formed by the thickening of the
epiblast at the point where the optic vesicle comes nearest to the surface.
The otocyst or otic vesicle (formed from the auditory pit in the third week)
has now receded from the surface of the epiblast or ectoderm ; it becomes
pear-shaped rather than spherical, and forms a labyrinth in which vestibular
and cochlear portions and a recessus vestibuli can be dimly recognised.

Such are the more striking and evident external features of the embryo
of the fourth week. We must now try to form a picture in our mind of
the internal arrangements found at this epoch, and it will be convenient to
study in order the nervous, alimentary, circulatory, urinary, and supporting-
systems of the embryonic body.

42

ANTENATAL PATHOLOGY AND HYGIENE

The spinal cord is the less differentiated part of the nervous system at
this age, so it will he convenient to study it first. It consists of a tuhe
with a central cavity which is still relatively large and has an elongated
oval form, the long diameter of the oval being antero-posterior. The lateral
Avails are thick ; the anterior and posterior are thin, and are called the roof-
and floor-plates or mid-dorsal and mid- ventral laminae. At the beginning of
the fourth week two kinds of tissue-elements can be made out in the

o

Fig. 14. —External appearances of Mall's 26 Jay embryo. Enlarged about 18 times.
(Viilc Mall's article in the Joitrnal of Morphology, vol. v., 1891.)

neural tube wall : spongioblasts or sustentacular elements and neuroblasts or
young nerve cells (the future neurons). To begin with, the wall consists of
a single layer of columnar cells or spongioblasts, but soon at the point where
these cells border the lumen of the canal other elements appear between
them called germinating or germinal cells, and from these arise the neuro-
blasts. The spongioblasts multiply and form the sustentative or sustenta-

DEVELOPMENT OF NERVOUS SYSTEM 43

cular part of the spinal cord, and more particularly what is called the
neuroglia. The inner ends of the spongioblasts unite to form a close net-
work next the canal cavity, the inner limiting membrane or ependymal
layer. There is an outer layer formed by the branched prolongations of
the ependyma cells, which forms the marginal velum or Randschleier of
His. Between these two layers is a very delicate network, which becomes
the. mantle layer, and into its meshes the neuroblasts wander. This
wandering would seem to take place by an accumulation of the protoplasm
on the distal side of the nucleus, and this indicates the commencement of
the formation of the axis-cylinder process or axon of the future neuron.
Later, other processes arise from the neuroblast, the dendrites. The axons
pass out through the marginal velum, and some form the ventral nerve roots.

While these changes have been going on inside the spinal cord the cells
of the neural ridge or crest (line of union of the neural tube) begin to form
the posterior or dorsal root ganglia ; these cells arrange themselves segment-
ally at the sides of the cord, some of them (neuroblasts) take on a fusiform
shape with a process extending from each end of the spindle, and one of
these processes (the axon) grows inward, penetrating the marginal velum,
to come into contact with the dendrites of the neuroblasts of the mantle
layer. Thus the two nerve roots of the spinal nerves are developed, the
ventral ones by cell processes growing outward, the dorsal ones by processes
growing inward. Thus, also, a beginning is made with the differentiation
of the cord into a grey and a white part, but as yet there is no contrast in
colour, for the medullary sheaths of the nerves do not appear till the fifth
month of antenatal life.

So far has the development of the cord progressed by the end of the
fourth week that twenty-nine spinal nerves (cervical 8, dorsal 12, lumbar
5, and sacral 4) can be counted (Mall's 26 day embryo), each with a large
dorsal ganglion and a ventral root. The ganglia are largest in the cervical
region, and the eight cervical nerves are united by anastomoses, the future
cervical and brachial plexuses. Posterior to the fourth sacral nerve, the
dorsal ganglia are not fully separated from the spinal cord.

During this (fourth) week there are as yet no sympathetic ganglia to
be recognised ; but, from the first six dorsal nerves, branches extend towards
the chorda dorsalis ; and three of these lie in front of the cceliac axis and
three behind it. From these branches arise the solar plexus and the
splanchnic ; at any rate this view commends itself to embryologists, for
it brings the sympathetic into line with the cerebro-spinal system as
derivatives of the epiblast or ectoderm. During development the cceliac
axis comes to lie nearer the caudal extremity, and so the sympathetic twigs
which surround it also lengthen.

The brain during the fourth week shows a marked increase in com-
plexity, and requires careful study. Five parts of the neural canal in the
cephalic end of the embryo can now be recognised : there is first the end-
brain, telencephalon, or hemisphere-brain; behind it (above it topographic-
ally) is the inter-brain, 'tween-brain, diencephalon or thalamencephalon ;
behind it is the mid-brain or mesencephalon ; and then follows the hind-
brain or rhombencephalon, consisting of the two parts, the secondary
hind-brain or metencephalon, and the after-brain or myelencephalon. Each
of these parts contains a cavity. At the beginning of the fourth week the
cavity of the telencephalon is single and communicates by a wide opening
with the diencephalon ; but towards the end of the week the hemisphere-
brain is formed by two oval projections whose cavities are the future lateral
ventricles, and they communicate with the rest of the cavity of the telen-

44

ANTENATAL PATHOLOGY AND HYGIENE

cephalon, which, together with that of the diencephalon, forms the third
ventricle. The openings between the lateral ventricles and the third are at
this time very wide; later they become the foramina of Monro. There is a thin
strip of tissue between the foramina of Monro, which is really the anterior

Fig. 15. — Reconstruction of Mall's 20 day embryo. Enlarged about 18 times. III.
IV., V., etc., cranial nerves; A.V., auditory vesicle ; 1, 2, 3, and 4, branchial
pockets ; T., thyroid gland ; B., bronchus ; L., liver ; K., kidney. The dotted
lines mark the extremities ; the spinal nerves and the blood vessels are indicated.
{Vide Mall's article in the Journal of Morphology, vol. v., 1891.)

end of the neural tube ; it is railed the lamina terminalis. The cavity of
the hind-brain or rhombencephalon is represented in after development by
the fourth ventricle ; and the cavity of the mesencephalon, although now
capacious, becomes the narrow iter, or aqueduct of Sylvius, of later dates.

DEVELOPMENT OF NERVOUS SYSTEM 45

The mesencephalon becomes continuous with the metencephalon in a con-
stricted portion, named the isthmus rhombencephali. Three flexures can
now be seen in this region : the cephalic flexure in the neighbourhood of
the mesencephalon, already noted and described in the preceding chapter ;
the cervical or nuchal bend, of which only an indication existed at the third
week ; and the pontine flexure in the region of the metencephalon, which is
a flexure simply of the ventral side of the neural tube, and does not involve
the whole head as the other two bends do. In the fourth week the
cephalic and cervical bends are very marked, but the pontine one is only
beginning to be indicated.

The walls of the neural tube in the region of the brain vary in thickness.
Those of the mesencephalon and rhombencephalon are thicker than the
parts anterior to them. In the myelencephalon the ventral wall alone
thickens, the dorsal remaining very thin. The walls have the same general
arrangement as in the region of the spinal cord : there is a roof-plate and a
floor-plate, and between them are the thicker lateral walls which soon show
the indications of a subdivision into a dorsal part or alar lamina, and a
ventral part or basal lamina. These fundamental divisions of the neural
tube are, before long, lost sight of in the great complexity of parts produced
in the region of the brain ; but they are there, although hidden, and have
much to do with the architecture of this all-important part of the body.

The ganglia of some of the cranial nerves can now be recognised. At
the beginning of the fourth week traces of the ganglia of the trigeminal or
fifth nerve, of the auditory-facial, of the glossopharyngeal or ninth, and of
the vagus or pneumogastric can be identified, although not very clearly ;
but by the end of the week their positions and relations, along with those
of other of the cranial nerve ganglia, can be definitely ascertained. To take
the nerves in their order, it has to be noted that the cells on the concave
(oral) side of the nasal pit are pyramidal in shape, and have their bases
directed towards the outside of the body ; they may be regarded as an
indication of the beginning of the olfactory or first nerve. There is as yet
no trace of an optic nerve ; the primary optic vesicle is still in communica-
tion with the cavity of the fore-brain, and in the secondary optic vesicle
(optic cup) two layers of cells can be recognised — a proximal or pigment
layer, and a distal or rods-and-cones layer (with a hyaline and a cellular
zone). Even at this date a vessel, the arteria centralis retinae, can be seen
(Mall's 26 day embryo) ; it perforates the hyaline zone of the layer of future
rods and cones. A possible indication of the origin of the third or oculo-
motor cranial nerve is found in the floor (basal lamina) of the mesencephalon ;
while there is a well-marked group of cells in the ventral wall (basal lamina)
of the isthmus rhombencephali, from which the fourth or trochlear nerve
arises.

The Gasserian (or semilunar) ganglion of the fifth or trigeminal nerve
is quite recognisable, as is also a small group of cells upon its ophthalmic
branch which constitutes the ciliary ganglion. Fibres forming the large
sensory root (dorsal) pass from the ganglion into the hind-brain ; the motor
root arises more ventrally from the basal lamina of the hind-brain, and
passes into the inferior maxillary division of the nerve. A group of cells in
the basal lamina of the upper part of the hind-brain represents the sixth or
abducent nerve. It is difficult at this age to separate the ganglia of the
auditory (eighth) and facial (seventh) nerves ; both these nerves arise from
the alar lamina of the hind-brain. The acoustic ganglion is adherent to the
auditory vesicle, which is pear-shaped, and has a prolongation from its
dorsal side, which indicates the beginning of the aqueductus vestibuli.

46

ANTENATAL PATHOLOGY AND HYGIENE

Between the auditory vesicle and the myelencephalon or after-brain lies
the upper ganglion of the glosso-pharyngeal or ninth nerve, which arises
from cells in the alar lamina j on the ventral side is a second ganglion, the

Fig. 16.— Deeper view of reconstruction of Mall's 26 day embryo. Enlarged about
18 times. II., hypophysis ; J/., mouth, mesentery ; 1, 2, 3, and 4, branchial
pockets; B., bronchus; P., pancreas; £., liver; IV. B., Wolffian body; W
/>., Wolffian duct ; A'., kidney ; C, cloaca ; 0., openings by which the pleurc

peritoneal cavities communicate; P., papilliform projection into the" lower
opening. {Fide Mall's article in Journal of Morphology, vol. v., 1891.)

ganglion petrosum. The vagus nerve is represented by two large ganglia,
the future ganglion nodosum and ganglion jugulare ; these are united by a
band of cells, and from the ganglion nodosum a branch passes to the fourth
branchial arch. The spinal accessory arises in a row of bundles which lies

DEVELOPMENT OF ALIMENTARY TRACT 47

between the vagus and the first cervical nerve, and emerges from the
myelencephalon midway between its dorsal and ventral walls. In Mall's
26 day embryo (upon which this description is largely based) there was no
trace of the rudimentary ganglion of the accessory, which has been found
by His in the human subject ; this is perhaps to be explained by the fact
that it is an organ in process of degeneration, and so may be expected to be
occasionally absent. The hypoglossal or twelfth nerve arises from a group
of fibres parallel to but more ventral than those of the spinal accessory. It
is evident, therefore, that already at the fourth week very considerable pro-
gress has been made with the ontogenesis of the complicated part of the
mechanism of the nervous system known as the cranial nerves. It may be
noted in passing that the trigeminus may be regarded as the nerve of the
first or mandibular branchial arch and groove; the facial as that of the
second or hyoid arch and of the groove in front of it ; the glossopharyngeal
as that of the third or thyrodiyoid arch and groove in front of it ; and the
superior and inferior laryngeal branches of the vagus as the nerves of the
fourth and fifth branchial arches respectively.

Like the nervous system, the alimentary tract has likewise been increas-
ing in complexity during the third week. The mouth cavity is still not
clearly delimited from the pharynx. The tuberculum impar which becomes
the buccal part of the tongue was noted in the third week ; it arises from
the first or mandibular branchial arch, and from the first interbranchial
space ; it now increases in size, and is marked off by the two alveolo-lingual
grooves ; and behind it is a V-shaped groove with a deep depression at its
apex, the future foramen caecum. The pharyngeal or posterior part of the
tongue is formed from a thickening of the ventral ends of the second
branchial arches, and into the angle between them the buccal part, so to
say, fits. Behind the milage1 of the tongue is an inverted U-shaped ridge,
the furcula, derived probably from the ventral parts of the third or fourth
branchial arches, which later becomes the epiglottis. Behind it, at the end
of the fourth week, is the narrow opening into the pulmonary diverticulum
from the ventral aspect of the pharynx ; from this diverticulum two lung
buds have been evaginated, and these push their way into the isthmus of
the ccelom (the neck of communication between the pericardial and peri-
toneal sacs), and form the pleural parts of the coelom. The median part of
the diverticulum forms the larynx and trachea. Rathke's pouch, an in-
vagination found in the roof of the oro-pharyngeal cavity, has not yet come
into contact with the infundibular prolongation of the telencephalon to form
the hypophysis ; this occurs in the fifth week. In the depression (foramen
caecum) behind the tuberculum impar, a bilobed body develops, which
later constitutes the median lobe of the thyroid gland, and takes up a
position lower in the neck. Thickenings of the tissues in the posterior and
anterior walls of the fourth and third branchial furrows develop later into
the lateral thyroids and the parathyroids; they are scarcely recognisable
even as thickenings at this age.

The oesophagus is comparatively short on account of the high level of
the diaphragm (septum transversum) at this age. The intestinal tube below
the oesophagus is dilated slightly in spindle fashion to form the anlage of
the stomach. In connection with the duodenum the anlage of the liver has
now taken the form of two lobes, a right and a left ; and it is said that the
hepatic cylinders (future bile-ducts) possess a lumen. Another bud from
the duodenum is recognisable at the end of this week : it is the small

1 Anlage, meaning the first outline or indication of a part or organ, is a useful
German word with no exact English equivalent.

48 ANTENATAL PATHOLOGY AND HYGIENE

group of cells constituting the milage of the pancreas, and it lies in the
mesogastrium (the future great omentum). Below this level the intestine
makes a loop toward the ventral aspect, and to this the yolk-stalk or
vitelline duct is attached. The part of the loop ahove the vitelline duct,
along with a portion of the part below, becomes the small intestine ; the
rest of the tube forms the large intestine, winch at this age is chiefly con-
stituted by the entodermal cloaca. The cloaca has a pyramidal form, the
apex pointing tailward ; there is, through the presence of the cloacal mem-
brane, no opening of the cloaca on the surface of the body. The small tube
known as the allantois forms a projection from the entodermal cloaca; and
on each side of it and dorsally is the opening of a Wolffian duct. It is to be
noted that the entodermal cloaca does not form the extreme caudal part of
the intestine ; that is constituted by the end-gut, which begins to wither
during the fourth week. For the end-gut Berry Hart has proposed the
name pars ultima, and for the entodermal cloaca that of pars penultima
(Trans. Edirib. Obst. Sue, xxvi. 259, 1901), and the nomenclature is concise
and descriptive. About the end of the fourth week, or a little later,
the penultimate part (entodermal cloaca) shows signs of division into an
anterior and a posterior cavity by a coronal septum ; the anterior cavity
forms the bladder (according to the view of Keibel and others, strongly
supported by Berry Hart, Brit. Med. Journ., ii. for 1902, p. 773), the
allantois only playing a subordinate part in its formation. The rest of the
anterior cavity constitutes the urino-genital sinus, and the posterior part
develops into the rectum.

The consideration of the urinary organs at this date of embryonic life
may be suitably taken up here in connection with the description of the
entodermal cloaca, for, as has been stated, the Wolffian ducts open into this
part of the primitive intestine. The Wolffian duct extends throughout the
Wolffian body or mesonephros, on the dorsal side of it in the lumbar region,
and on the ventral in the cervical. The Wolffian bodies are larcje lobulated
structures, situated one on each side of the intestine from the cloaca to the
sixth cervical nerve ; glomeruli can be recognised in them from the sixth
cervical to the fifth lumbar nerve, and there are about three glomeruli to a
segment (in Mall's 26 day embryo). Just before the Wolffian duct enters
the cloaca, a small blind tube arises from it ; in process of time this is con-
verted into the ureter and kidney (metanephros). This blind tube appears
during the fourth week ; it is not present at the beginning of it (e.g. in
His' embryo a).

The circulatory apparatus, like the other systems of organs, shows
marked developmental advances during this week (the fourth) of intra-
uterine life. The separation of the single atrium into two auricles by
means of a ridge growing from its dorsal and cephalic walls begins at this
date ; the separation, however, is incomplete, the opening between the
auricles constituting the foramen ovale, or rather the ostium primum. The
right auricle is larger than the left, and into it (the former) opens the sinus
venosus or sinus reuniens, into which in turn open the vitelline and um-
bilical (allantoic) veins and the ducts of Cuvier. The opening of the sinus
reuniens is guarded by a valve. The common atrio-ventricular canal is now
divided by an incomplete septum into two auriculo-ventricular openings, by
means of which the right and left auricular cavities communicate with the
right and left ventricular cavities respectively, for by this time the common
ventricular chamber is partly divided into two cavities. The two ven-
tricular cavities, however, still freely communicate with each other, for the
ridge or septum on the inside which corresponds to the sulcus inter-

DEVELOPMENT OF CIRCULATORY SYSTEM 49

ventricularis on the outside does not yet completely divide the ventricular
space. The truncus or bulbus arteriosus is by the end of the fourth week
nearly completely divided into two tubes. Already the symmetrical arrange-
ment of the five aortic arches has broken down and been replaced by a new
order. The third, fourth, and fifth arches of both sides persist, and these
unite to form the two aorta?, which in their turn unite, between the sixth
and seventh cervical nerves (in Mall's 26 day embryo), to form the single
aorta. The aorta is much larger in the dorsal and lumbar regions than in
the cervical, and in the lumbar region it divides abruptly into the two
umbilical (allantoic) arteries. The pulmonary artery on each side can be
seen arising from the fifth aortic arch as a comparatively small branch.
The first and second aortic arches have by the end of this week (the fourth)
apparently disappeared. The internal carotid artery with some branches
can be recognised arising from the third arch. On each side of the dorsal
aorta are (in Mall's 26 day embryo) twenty-one segmental arteries, the first
being in front of the first cervical nerve, and the last behind the tAvelfth
dorsal. The second segmental artery communicates with the vertebral, a
large branch which extends forward to the region of the otic vesicle and
gives off two twigs, the future anterior and posterior cerebellar arteries.
The subclavians grow out from the dorsal aortse, or from the segmentals
opposite the fourth arches. Between the lateral and ventral sides of the
aorta there are fourteen pairs of segmental branches which supply the
Wolffian bodies. From the ventral side of the aorta the cceliac axis and
the omphalo-mesenteric artery arise. Through the growth tailwards of
the parts in the neck, including the heart and diaphragm, the origins of all
these vessels are constantly moving backwards, as it were, in relation to the
segments of the body.

The same general arrangement of the veins persists, with the exception
that the left or sometimes the right omphalo-mesenteric or vitelline vein
has now disappeared ; the remaining vein (as portal vein) unites with the
umbilical vein in the region of the liver, but before this happens it receives
an inferior mesenteric branch. The liver is developed around the umbilical
and omphalo-mesenteric veins. The cardinal vein (which receives the
blood from the Wolffian body) joins with the jugular of the same side to
form the ductus Cuvieri which opens into the sinus reuniens. Veins from
the upper limb-bud (subclavian) also open into the duct of Cuvier or into
the jugular vein. The jugular and cardinal veins also receive segmental
branches. The right umbilical vein has usually disappeared by this time.

With regard to the supporting tissues of the embryo in the fourth week
certain facts are well established. The notochord is still recognisable, and
stretches from the caudal extremity to a point below the hind-brain. On
each side of it are the mesoblastic segments (consisting of myotomes, sclero-
tomes, and cutis plates or dermatomes), and in its anterior part the vertebral
arteries are also situated laterally. It is usually stated that cartilage does
not begin to form in the sclerotomes till the second month, but Mall
describes it already in his 26 day embryo. These sclerotomes grow round
the notochord, and are transformed at a later date into the spinal column,
the basi-occiput, and part of the basi-sphenoid. From the myotomes are
formed the muscles of the body and limbs.

Like the weeks that precede it, the fourth is full of organogenetic

activities. Construction is rapidly going on ; it is true we have not

to chronicle developments so astonishing as those of the third week,

that liebdomada mirabilis to which reference has lately been made,

VOL. ii. — 4

50 ANTENATAL PATHOLOGY AND HYGIENE

yet those we have to deal with are wonderful enough, and in all
respects more than difficult enough to explain or attempt to explain.
We see the embryo beginning to assert itself even in the matter of
bulk, while the umbilical vesicle has already reached its maximum
size, and has more than reached its maximum importance. The
curious dorsal flexure of the embryo has suddenly disappeared, and
our visual image of the new organism now is a C instead of an
inverted S ; this C shape the embryo never quite loses again, and in
the foetus we recognise it in the familiar attitude of flexion ; in a
sense, there is even a reminiscent and instinctive assumption of it by
the adult when in fear of bodily injury or hurt.

In this fourth week, also, there are seen for the first time the
indications of limbs. They are nothing more than buds, it is true ;
but their advent is very striking, for we now realise what it was
that gave to the limbless embryo part at least of its grotesque and
non-human appearance. The embryo is still far from human in its
aspect, the presence of the branchial arches and grooves quite prevents
that. Not yet have these gill-like structures begun to disappear,
buried from sight, in the cervical sinus ; that fate awaits them in the
next week. The first trace of a nose is now visible in the very
unnose-like nasal pits, too shallow almost to deserve the name of
pits ; stalked vesicles represent the eyes ; and the otocysts, by their
retreat from the surface, have for the time removed even the
suggestion of ears.

It is difficult to recognise the simple neural tube of the early
days of development in the complicated series of brain vesicles of
the fourth week ; and yet the nervous system is only beginning to
assume its labyrinthine form with its sinuous or tortuous passages,
with the intricate ramifications of its cavities, and with its endless
irregularities of contour, its invaginations and evaginations. We can
still identify the central cavity of its various parts, for it is through-
out fairly wide and canal-like, but soon this character disappears,
and it is difficult to see in the narrow iter of later weeks the wide
tract of the mesencephalon of the fourth week. Already the lateral
walls are thickening irregularly, while the roof and floor in most
parts remain thin and membrane-like. Quite at its anterior
extremity this series of vesicles has begun to expand into that
magnum opus of ontogenesis and organogenesis, the cerebral
hemispheres : there is as yet little to be seen of that crowning
superstructure of developmental activity; but its foundations are
there, firmly laid, and even beginning to show above the surface.
In the spinal cord, also, histological and structural differentiation
is going on : spongioblasts are forming that neuroglia-network, in
the meshes of which neuroblasts, potential neurons with potential
axons and dendrites, begin to appear ; and, having appeared, begin to
stretch forth in bewildering fashion, and with far-reaching results
so far as the later structure of this part of the nervous system is
concerned.

Hardly less remarkable are the developments going forth in the
great body cavity of the embryo, where we find an intestine with an

FOURTH WEEK AFTER IMPREGNATION 51

end-gut or pars ultima and an entodermal cloaca (2Jars 2)enultima),
the anlage of a liver and pancreas, the indication of a trachea and
lungs, and relatively large and well-formed Wolffian bodies. The
last-named organs have, indeed, already reached their acme of
structural differentiation and the culminating point of their develop-
mental activity. The heart, also, is rapidly approaching the high
degree of constructive detail which it maintains during the remainder
of antenatal life ; for, unlike the Wolffian bodies, it does not then
start upon a degenerative progress, but remains permanently efficient
and physiologically of great importance. Very soon its chambers,
right and left, will be divided off from each other by septa, complete
but for the presence of the foramen ovale, which serves as a com-
munication between the auricles until the occurrence of birth and
the replacement of the foetal with the adult type of circulation.

CHAPTER V

Chronology of Embryonic Life (continued) : Conditions found in the Fifth

Week after Impregnation.

Extra-embryonic Parts in the Fifth Week.

In the fifth week after impregnation, the decidual membranes bear much
the same relations to each other and to the chorionic vesicle as in the
fourth week. Minot (Human Embryology, p. 13, 1892) describes the
decidua serotina in a specimen of about this age (fifth week) as consisting
of a compact and a spongy layer, the former constituting one quarter of the
whole thickness of the serotina. The surface was without any trace of
epithelium, but was covered with a thin fibrous and granular coagulum.
The compact layer consisted almost exclusively of young large decidual
cells and a clear homogeneous matrix ; the cells were irregular in shape,
but more or less rounded, with processes which occasionally united two cells
together. In the deeper or spongy (cavernous) layer the decidual cells
were not so large ; they were more fusiform in shape, and had longer, less
round nuclei. The spaces of the cavernous layer were glandular, and in
many of them the shed epithelium (often degenerated) could be seen lying.
In the smaller spaces the epithelium (columnar in type) was well preserved
and still attached to the Avails. The blood vessels lay between the glands,
and Minot found nothing corresponding to the " colossal capillaries " of
Turner.

The chorionic vesicle is still covered all over with villi, but these are
most marked in the region corresponding to the decidua serotina. The
minute anatomy of the parts is the same as in the fourth week. The space
between the amniotic sac and the inner aspect of the chorionic vesicle — the
extra-embryonic coelom- — is still large, but not so large as in the preceding
week ; at the end of the fifth week it may be stated roughly that the space
between the embryo and the chorion is divided equally between the
amniotic cavity and the ccelom (Mall, Journ. Morphol., xii. 375, 1897).
The amniotic membrane is still not far removed from the body of the
embryo, and possesses the same histological appearances as formerly. The
umbilical vesicle is pear-shaped ; its stalk has lengthened considerably, and
for the first part of its course is enclosed within the Bauchstiel ; and the
vesicle is no longer increasing in size.

The Embryo in the Fifth Week.

Various human embryos of the age of the fifth week have been
described, several by His (Pi', M, Hi1, Kg, Ko, Sch, etc.), some by Minot
and Coste, and others by Rabl, Phisalix, R. Dorello, and Hochstetter. In

FIFTH WEEK AFTER IMPREGNATION 53

length the embryo now measures from 8 to 14 mm. ; probably, then,
1 cm. may be taken as representing its average length about the middle of the
fifth week. The distinct C shape of the fourth week is beginning to be
partly lost : the body of the embryo shows some straightening, and so the
head seems as if it formed a right angle with it. Between the region of
the hind-brain and the back opposite the origin of the anterior limb-bud,
there is a slight concavity, called Narkengrube by His ; this persists till
the eighth week (vide this Manual, vol. i. p. 81, 1902). Towards the
end of the fifth week the head, as seen from the side, has an area about
equal to that of the rest of the body ; the cephalic and cervical flexures or
bends are well marked, the latter reaching its maximum at this date. Both
the Bauchstiel and the stalk of the umbilical vesicle have lengthened ; they
are combined in part of their extent to form the single structure, which
may now be called the umbilical cord : towards the end of the week the
cord shows one or two spiral turns, and contains one or more coils of
intestine. The stalk of the umbilical vesicle is longer than the cord, and
is disposed between the chorion and the amnion.

The limb-buds show a marked development during this period of seven
days : at first they are unsegmented • then they exhibit two segments ; then
the tripartite division ; and finally the digits are roughly indicated in out-
line, being still webbed. The upper limbs are generally more advanced in
formation than the lower ones ; for instance, they show digitation earlier.
Along with their differentiation into three parts or segments, the limbs
have undergone increase in size, and in embryos of about thirty-five days
they project beyond the outline of the body in profile views. When the
limbs are in the two-segment stage, the distal part is flattened and the
proximal cylindrical, and there is a constriction between ; later, a second
furrow or constriction appears in the cylindrical part, dividing it into two
parts. So the foot, leg, and thigh, and the hand, forearm, and arm are
marked out.

The tail of the embryo is now very marked ; in fact, in this (the fifth)
week it reaches its maximum development, and is evidently a true tail
(Fig. 17). At the other end of the embryo the cervical or precervical
sinus has greatly deepened, so that the fourth, third, and even the second
branchial grooves have disappeared from the surface and lie within it.
The first branchial groove is still seen ; in fact it never enters into the
cervical sinus, but persists externally as the external auditory meatus.
Around it the six tubercles, Avhich unite later to form the pinna or external
ear, are quite recognisable. In the adjacent facial region marked changes
can also be seen. The nasal pits are in process of demarcation by the
growth of processes in the following manner : from the naso-frontal process
its two lateral edges, the two globular processes as they are called, become
thick and rounded, and form the inner boundaries of the nasal pits ; and
from the sides of the base of the naso-frontal process the two lateral nasal or
frontal processes grow downwards and outwards, and bound the nasal pits
externally, shutting them off from the depressions for the eyes. The nasal
pits, however, are still in communication below with the primitive oral
cavity. The lateral nasal process on each side is at first separated from the
maxillary process of the same side by a groove (the naso-optic furrow or
lachrymal groove), but later it unites with it and grows inward towards
the middle line below the nasal pit ; in the sixth week, as we shall see, it
joins the nasofrontal process, and so separates the nasal pit from the oral
cavity. Towards the end of the fifth week the mandibular processes unite
in the middle line. About this time the optic vesicle comes into contact

54

ANTENATAL PATHOLOGY AND HYGIENE

with the lens vesicle and is invaginated hy it, becoming the secondary
optic cup. The under surface of the vesicle is also invaginated, and so is
that of its tubular stalk, so that an aperture (choroidal fissure) and a trough
are thus formed. Mesodermic tissue grows into the optic cup through the
choroidal fissure, and it also grows into the trough of the stalk (future

ft

t "

X

'"%

. as***'

V **

•". .

FlG. 17.— Embryo of the fifth week. Enlarged 15 times.
Embryo Pr. (After His. )

optic nerve) ; in these places it gives origin later to the vitreous body and
the central artery of the retina.

The only other external feature of note at this stage of development is
the forward projection of the abdomen, due to the large size which the
liver has now attained. It may be added that the ectoderm (or epiblast)
covering the body consists of two layers : an outer one, the epitrichium,

DEVELOPMENT OF THE NERVOUS SYSTEM 55

made up of slightly flattened cells which later become dome-shaped, and an
inner layer of larger cells which give rise to the epidermis.

The internal arrangements may now he described, the same order of
description being followed as was adopted for the fourth week.

The spinal cord, in the fifth week, is divisible into three layers : an
outer neuroglia layer or marginal velum, a middle or mantle layer, and an
inner or ependymal layer. These have increased in thickness, and the
cells of the ependyma have become ciliated. The sympathetic system
shows a further advance in formation. Two ganglionic masses are found
near the omphalo-mesenteric artery, one above and the other below the
vessel ; these represent the ganglia of the solar plexus, and the upper
ganglion is joined to the ganglionated cord by fibres representing the
greater and lesser splanchnic nerves. Near the origin of the umbilical
arteries is another enlargement of the ganglionated cord, which represents
the pelvic plexus (inferior mesenteric and hypogastric ganglia). Cells, also,
are found in the neighbourhood of the stomach, and these are connected '
with a plexiis formed from fibres from the vagus nerves. In the cervical
region a plexus is being formed, composed of ganglia of cells which have
wandered from the cell column and of fibres closely related with the
descending branches of the vagus.

In the brain, changes are manifest. A longitudinal ridge-like thickening
appears in the roof or dorsal wall of the mid-brain vesicle or mesen-
cephalon ; later, in the ninth week, this is replaced by a furrow, at the
sides of which are developed the corpora quadrigemina, but as yet there is
no trace of these bodies. In the hind-brain or rhombencephalon, further
developments are visible now both in the floor and in the roof. The
roof-plate is wide and thin ; over the posterior half of the fourth ventricle
it forms the inferior medullary velum, while over the anterior half the
superior medullary velum develops as well as the rudiment of the cere-
bellum. Out of the alar and basal lamina? are formed the rudiments of
the pons and medulla ; the attachments of the roof-plate or velum to the
alar lamina? is marked by the obex and ligula, which are thickenings on the
margins of the lower anyle of the fourth ventricle. The rudiment of the
restiform body is found in the upper margin of the alar lamina. If we
now look at the parts in front of the mesencephalon, we find that the roof-
plate of the dienceplialon or inter-brain is thin-walled and somewhat folded,
while in the floor-plate is a well-marked ventral groove ; the lateral walls
are divided into dorsal and ventral zones by the grooves known as the
sulci of Monro, which extend forward towards the point of origin of the
optic evaginations. At the posterior end of the folded roof-plate is an
elevation which later becomes a hollow evagination, the pineal process, and
still later the pineal body or epiphysis. The rest of the roof-plate in front of
the pineal elevation represents the velum interpositum of adult anatomy ; it
forms a ridge at first, but soon becomes a thin membrane upon which at a
later date blood vessels arrange themselves, and the whole structure is invagin-
ated into the third ventricle as the choroid plexus. The dorsal zones of the
lateral walls thicken, and towards the end of the fifth week begin to project
inwards into the cavity of the ventricle as the optic thalami. Behind the
optic thalami are other thickenings giving rise to the pulvinares and the ex-
ternal geniculate bodies. The ventral zones become the subthalamic regions,
and in them or perhaps in the floor-plate are developed the corpora albicantia
(mammillary tubercles). In front of the dienceplialon is the telencephalon
or hemisphere-brain. It has, as Ave have already seen in the end of the
fourth week, a median portion which contains the anterior part of the third

56 ANTENATAL PATHOLOGY AND HYGIENE

ventricle, and two lateral outgrowths, the, cerehral hemispheres. The roof
of the median portion is converted into the anterior part of the velum
interpositum. From its dorsal zones arise the optic evaginations, as has
already been pointed out ; while* the ventral zones form the anterior part of
the subthalamic regions. In the floor-plate is the evagination of the hypo-
physis which during this week comes into contact with the invagination
of the roof of the oral cavity to form with it the pituitary body. The
cerebral hemispheres arise from the sides of the dorsal zones, and also get
a prolongation of the roof-plate or velum interpositum. In the ventral
part of the wall of each a thickening is to be noted, the future corpus
striatum, and in the more dorsal part is the mantle or pallial portion of the
hemisphere. The cavities of the hemispheres are, of course, the lateral
ventricles, and into them project the choroidal folds which do not at this
date possess blood vessels. In this relation are to be observed the arcuate
and choroidal Assures. In the floor of the telencephalon the tuber
cinereum appears. The first indication of the falx cerebri is found lying in
the longitudinal fissure between the two cerebral hemispheres. There are
as yet no traces of the corpus callosum or fornix. The olfactory lobes can
now be recognised as areas on the ventral surface of the cerebral hemi-
spheres ; they are at first hollow and later become solid, and finally (third
month) give rise to the olfactory bulbs and stalks and to the olfactory
tracts, trigone, and anterior perforated spaces.

The origins of the cranial nerves as they exist in the fourth week have
been already sketched ; but some further developments may here be noted.
During the fifth week the olfactory nerves are much more distinctly in-
dicated than in the preceding week ; the olfactory ganglion is produced,
its cells assume the bipolar form, and by the elongation of their poles on
the one side they come into relation with the brain, while on the other
side they join the olfactory epithelium. In His' embryo Ko (a fifth week
specimen), the ganglia and trunks of the third (oculo-motor), fourth (troch-
lear), fifth (trigeminus), seventh (facial), eighth (acoustic), ninth (glosso-
pharyngeal), tenth (vagus), eleventh (spinal-accessory), and twelfth (hypo-
glossal) nerves can all be much more clearly traced than in fourth week
embryos. The sixth (abducens) nerve is composed entirely of fibres
arising in the ventral or basal zone of the hind-brain ; it has no ganglion.
The eighth nerve has a vestibular and a cochlear branch ; at this time the
ear has advanced in development, the semicircular canals have commenced
to form, and the twisting of the cochlea has begun. The eleventh nerve
and its branches have practically their adult relations in this week of
embryonic life, and the twelfth (hypoglossal) nerve passes round the sinus
cervicalis and then curves forward into the tongue.

The alimentary system, in the fifth week, shows some increase in
complexity. The oral cavity is not yet shut off from the nasal. The
front of the tongue is marked off from the back by two oblique lines
which begin at the foramen caecum and form an open V ; the anterior part
is developed from the tuberculum impar, and the posterior from the region
of the second and third branchial arches (ventral ends). At the opening
(the future foramen caecum) is the orifice of the duct of the median part of
the thyroid gland (thyroglossal duct) ; it loses its lumen and begins to atrophy
at the fifth week. Towards the end of the week a longitudinal ridge
appears on each side in the floor of the mouth in the part known as the
alveolo-lingual groove ; this is the anlage of the submaxillary gland and
Wharton's duct. The pharynx is still relatively large, being equal to
about half the length of the alimentary canal. The pharyngeal pouches

DEVELOPMENT OF THE ALIMENTARY TRACT 57

are beginning to undergo the series of changes which transform them into
permanent structures ; for example, the first pouch, on each side, is being
shut oft' to form the tympanic cavity at one end and the future Eustachian
tube at the other, the " closing membrane " persisting as the tympanum ;
the median lobe of the thyroid gland is related to the anterior ends of the
second pair of pouches ; and from each of the two fourth pouches arises a
ventral prolongation or diverticulum, which becomes the anlage of one of
the two lateral lobes of the thyroid gland. At a later date (sixth week)
evaginations from the third pair of pockets give origin to the epithelial
parts of the thymus gland.

The opening of the trachea into the pharynx is slitdike ; in front is the
anlage of the epiglottis, and at the sides are the arytenoid ridges whose
thickened epithelium almost closes the opening ; in the ridges, on each side,
are two projections, the cuneiform and cornicular tubercles. There are as
yet no traces of the cartilages of the larynx ; these appear at the seventh
week, and the cartilages of the trachea a week or two later. Through an
increase in the number ami size of their lobes the lungs become much more
than simple buds. A pulmonary artery can be traced to each of them.

To return, now, to the description of the alimentary system, it may be
observed that during the fifth week the noteworthy flexure of the intestine
below the level of the stomach is established. In this way is formed a U-
shaped loop, with its closed end projecting towards the ventral body-wall ;
the dorsal mesentery at this point lengthens in order to permit of this
formation. The vitelline duct or yolk-stalk is attached to the apex of the
U-shaped loop, and may remain permanently as Meckel's diverticulum; the
vitelline artery (superior mesenteric) supplies the loop. At a point on the
lower border of the loop the intestine suddenly becomes larger ; this
indicates the caput caecum or commencement of the large intestine. All
the bowel above this point forms the small intestine, and thus it comes
about that Meckel's diverticulum, when present, is attached to the ileum
above the level of the ileo-csecal valve. At first the U-shaped loop is extra-
abdominal in position, being situated in a cavity formed by the somatopleure
at the umbilicus ; as a rule, it passes within the abdomen by the end of the
second month. The angle between the U-shaped loop and the hind-gut be-
comes the splenic flexure, although as yet, of course, there is no trace of the
anlage of the spleen. The hind-gut is supplied with a dorsal mesentery by
which it is suspended. The inner surface of the intestine is as yet quite
smooth, folds not making their appearance till the next week. It has been
stated that towards the end of the fifth week the mucous membrane of the
duodenum increases so much in thickness that the lumen is narrowed and
finally obliterated, to reappear again at the beginning of the sixth week
(Tandler). The pancreas lies between the layers of the dorsal meso-gastrium
at this age, and is parallel to the greater curvature of the stomach. The
part of the dorsal meso-gastrium which lies between the pancreas and the
stomach becomes at a later date much elongated, to form the great omentum.
The liver develops rapidly during this week, increasing greatly in size, and
causing the anterior abdominal Avail to project, as may be well seen in
Mall's reconstructed embryo No. ix. (Johns Hopkins Hosp. Bull., ix. p. 199,
1898). The gall-bladder is quite recognisable in this embryo. During this
week (the fifth) the end-gut (pars ultima of Berry Hart) is in course of dis-
appearance. The entodermal cloaca (pars penultima) is now divided into
an anterior and a posterior division, the former forming the bladder and
urinogenital sinus, and the latter the rectum. In the sixth week both the
bowel and urinogenital sinus communicate with a large space, the cloaca,

58 ANTENATAL PATHOLOGY AND HYGIENE

best known as the ectodermal cloaca. It may be conjectured, for we do not
know with certainty, that this cloaca has been formed during the fifth week
by the breaking down of an ectodermic pyramidal plug with its apex to-
wards the lower part of the urinogenital sinus meeting the coronal partition
of the primitive gut (Berry Hart, loc. cit.). Apparently the communication
i >f the cloaca with the exterior thus established becomes shut off again between
the seventh and eighth weeks by ReichePs anal tuberosities (Analliikher).

The urinogenital organs have advanced considerably in their ontogeny
since the fourth week. The mesonephros or Wolffian body is still well de-
veloped and large It extends as a ridge on each side of the spine from the
posterior cervical region to the pelvis. To its inner side lies the genital
ridge, and it is possible, according to Nagel (Arch. f. mikrosk. Anat., xxxiv.
269, 1889), even at this age (fifth week) to determine by microscopical exa-
mination whether the genital gland is male or female ; if it is to be an
ovary the large germinal cells (primitive ova) are numerous, and the
germinal or ccelomic epithelium forms a thick layer of several rows of cells,
while if it is to be a testicle the large germinal cells are fewer, and the cells
of the epithelium are arranged in groups rather than in a distinct band or
layer. The convoluted tubules and glomeruli of the Wolffian bodies open
into the Wolffian ducts, and these in their turn open into the entodermal
cloaca. As has been already pointed out, the ureter is present at the end of
the fourth week as a small bud attached to the Wolffian duct just above its
opening into the cloaca ; in the fifth week this bud develops a stalk and be-
comes the permanent kidney and duct (ureter) which lie in the lumbar
region behind both the Wolffian body and the peritoneum. The permanent
kidney or metanephros, then, is developed from this outgrowth from the
Wolffian duct ; at the same time it is believed that it obtains part of its
structure from a condensation of the mesenchyma which surrounds it, to
which the name of metanephric blastema has been given. Possibly the
blastema only contributes the connective tissue and vessels of the kidney.
About the end of the fifth week, or perhaps in the sixth week (the time is
not known with certainty), the Mullerian ducts appear as peritoneal evagina-
tions in the ridge or folds that lie on the ventral surface of the Wolffian
bodies. The Mullerian ducts come to open into the urinogenital canal
posteriorly, while anteriorly they have a free communication with the
peritoneal cavity. A trace of the external genitals is visible during the
fifth week in the shape of the genital tubercle, a small eminence on the
ventral wall of the urinogenital sinus. At this time, also, the suprarenal
capsules or bodies are quite recognisable, and each is found near the cephalic
end of the mesonephros or Wolffian body of the same side. Their relation
to the kidney is a later acquired one. They are probably derived from the
Wolffian bodies; but a part (medulla) has origin in the sympathetic nervous
system.

The heart and circulatory system are now well advanced in develop-
ment. The auricles and ventricles are not yet fully separated into right
and left chambers. The opening of the sinus venosus into the right auricle,
is guarded by right and left lateral valves, which prevent regurgitation of
blood when the auricle contracts. At a later date the right valve becomes
the Eustachian and Thebesian valves; probably the left forms part of the
auricular septum (primary). Even as early as the fifth week a trace of the
secondary septum (or septum spurium) of the auricles can be made out lying
to the right of the primary one. The auriculo-ventricular canal is divided
into right and left auriculo-ventricular orifices by two structures known as
the anterior and posterior endocardial cushions. The bulbus arteriosus is

FIFTH WEEK AFTER IMPREGNATION 59

divided into an anterior or pulmonary and a posterior or aortic part by
anterior and posterior ridges (anterior and posterior aortic septa), which fuse
together while their lower ends unite with the interventricular septum.
The resulting aortic part retains its connection with the fourth left aortic
arch, which becomes the ascending part of the arch of the aorta; the
pulmonary or anterior part is continuous with the fifth arch of the
left side and constitutes the ductus arteriosus (or ductus Botalli), with
branches to the two lungs. It is not known how this change in the
relations of the pulmonary arteries, so that both arise from the fifth
left arch, is brought about (Minot). The fifth arch on the right side
atrophies ; the right fourth arch persists as the right subclavian and
innominate arteries; the third arch on each side remains as the internal
carotid. Now, therefore, the third and fourth arches and the vessels repre-
senting the first and second arches are all connected with the left side of
the heart through the aorta, while the left fifth arch is brought into relation
Avith the right ventricle through the pulmonary trunk. The segmental
arteries and the vessels for the limbs doubtless undergo important modifica-
tions during this week, but these are not yet clearly known. The arrange-
ment of the veins is probably similar to that described for the fourth week ;
the origin of the vena cava inferior has not yet been clearly ascertained for
the human embryo. The blood at this age contains only nucleated red
blood corpuscles or erythrocytes.

The sustentacular parts of the embryo show further developments at the
fifth week. For instance, in the limbs the fibrous basis of some of the
future bones can be recognised, and here and there chondrification has
begun. In the pelvis, also, the three parts of the anlage of the os innomi-
natum (ilium, ischium, pubes) are present ; so are the membranous ribs and
clavicle, etc. According to Arthur Thomson (Cunningham's Text Book of
Anatomy, p. 180, 1902), ossification may be seen in the clavicle as early as
the fifth week. Meckel's cartilage is present in the mandibular arch.

It must be admitted that there is much that is uncertain about
human ontogenesis in the fifth week after impregnation. In this
period take place several complicated rearrangements of parts ; and
just how these rearrangements are effected we do not clearly under-
stand. This remark applies specially to the changes which occur in
the branchial arches and clefts, in the pharyngeal pouches, in the
great veins of the abdomen and liver, and in the pleuro-peritoneal
relations. We do not know how these changes take place, neither
do we know with any degree of certainty when they occur. Con-
sequently any attempt to place before the reader a chronological
account of the embryology of this period has to face the double
difficulty of uncertainty as to the facts and ignorance regarding the
dates. There is also the constant problem of the recognition of
parts; for when one, so to say, loses sight of a structure in the
embryo for a day or two it is not easy to recognise it again at a later
stage in a new form and with new relations. The whole problem,
therefore, becomes a very complex, involved, intricate, and perplexing-
one ; yet, in order to preserve unbroken the chronological account of
human embryonic life, I felt myself constrained to put before my
readers such facts as I could gather together from reliable authorities.
They are few in number and obscure, but they must serve till further

6 0 ANTENATAL PATHOLOGY ANT) HYGIENE

light has been thrown upon this feebly illumined part of ontogenesis.
In may be said, in parenthesis, that the nomenclature of embryology
does nothing bo render this obscure portion of the subject less dark;
in fact, the giving of two, three, or even four different names to the
same structure is irritatingly confusing.

Although there is much that is obscure about organogenesis in the
fifth week there is also not a little that is well known. The embryo
begins to lose something of the exaggerated C shape that it had in the
preceding week, and attains to the length of 1 cm., being now about
one-fiftieth of the length of the mature foetus. Much has yet to be
accomplished before the 1 cm. embryo is converted into the 51 cm.
foetus, but it may be doubted whether any of the superadded centi-
metres are accompanied by such a marvellous exhibition of develop-
mental activity as is this first one. Among the many phenomena of
the week we must note the assumption by the Bauchstiel and yolk-
stalk of a truly cord-like character, the transformation of the bud-
like extremities into evident and segmented limbs, and the presence
of a decided and indisputable tail. The face is now more face-like,
having a lower jaw united in the middle line, nasal pits which,
although incomplete, suggest a nose, and recognisable eyes but with
no eyelids or even anlages of eyelids. The embryo is beginning to be
dimly human in appearance, a change which is aided by the dis-
appearance of the second, third, and fourth branchial arches and
grooves into the sinus pnecervicalis whose margins close over and
entomb them.

In its internal arrangements the embryo is rapidly passing
towards a more mature distribution of structures and organs. The
origins of cranial nerves can be recognised; the pituitary body is
present, so are suprarenal capsules and a sympathetic system with a
solar plexus; the bowel has thrown itself into a U-shaped loop, and
given thus an indication of its coming division into small and large
intestine ; the thyroglossal duct is beginning to close ; the ureters
are present as little buds from the Wolffian ducts ; and in the genital
ridges histological evidence can be obtained of the sex to which the
embryo belongs. In the region of the brain, also, the anlages of
numbers of new parts and structures have appeared, including those
of the medullary velum, pons, velum interpositum, optic thalami,
corpora striata, tidier cinereum, and falx cerebri. Finally, the
surface of the whole body is covered by the epitrichium, that
mysterious layer whose fate and whose functions are alike unknown.

CHAPTER VI

Chronology of Embryonic Life {continued) : Conditions found in the Sixth Week

after Impregnation.

The sixth week after impregnation is the last of typically embryonic
life. It is not that there is no further organogenesis after this period,
as a matter of fact some organogenesis is going on up to the full term
of antenatal existence and even after it ; but the sixth week marks
the close of the epoch of almost purely constructive processes. By
this time all the great formative procedures have been initiated ; the
plan of the human embryo has been, as it were, fully indicated ; some
complicated rearrangements have also been effected ; and the embryo
may now be called the transition-organism, for although it is not yet
a foetus it is no longer simply an embryo. We may call it the neo-
foetus. There is still much to be done : many of the organogenetic
processes have to be carried to completion ; a great deal of histo-
logical differentiation has to be effected ; many parts have yet to
assume their mature relations with each other and with the frame-
work of the body ; the organism has to grow from an embryo measur-
ing little more than half an inch in length to a fcetus of twenty
inches ; and modifications have to occur in the manner in which its
organs carry on their functions, and in the relations which they bear
to the maternal economy. Nevertheless, for the organism as a whole,
truly embryonic life has nearly finished ; and what remains to be
done is no longer development but growth and histological and
physiological differentiation. I have already in the part of this
Manual dealing with the Fcetus described the characters of the
embryo at the close of the sixth week. It is not, therefore, necessary
for me to repeat the whole of that description here. I take the
opportunity, however, of giving in greater detail some obscure parts
of the subject, and of adding some information only recently made
available by the progress of embryological research. It has, for in-
stance, been found that the anlages of certain structures are present at
an earlier date than had been thought. These facts and others of the
same kind are noted here. Let us, with these words of explanation,
proceed to the study of the organogenetic processes of the sixth week.

Extra-embryonic Parts in the Sixth Week.

J. C. Webster gives a good and detailed account of the extra-
embryonic parts of the uterine contents at the sixth week (Human Placen-

61

62 ANTENATAL PATHOLOGY AND HYGIENE

tation, pp. 15, 28, 38, etc., 1901); upon this the following description is
based.

The decidua vera varies from 3 to 7 mm. in thickness, and can be
readily divided into a compact and a spongy layer. The spongy part can
be subdivided into an outer part with slightly branched glands, and a
deeper portion where there is much branching. The lining epithelium is
still to a great extent present, although somewhat altered : the columnar
cells are now cubical or flattened ; their nuclei are rounded or flattened in
shape, and some have degenerated ; and the cilia have disappeared. The
glands have increased in size, but there is no complete evidence of any new
formation of them ; their mouths (in the compact layer) are narrowed or
obliterated ; their deeper parts are much enlarged ; their epithelium is
generally cubical or flattened, very rarely columnar, and in many instances is
shed in masses or in individual cells. In the interglandular tissue the most
marked change is the development of the decidual cells : this change is
practically limited at present to the compact layer, and is most evident in
its outer portion ; the decidual cells are rounded, oval, polygonal, or spindle-
shaped, with large and somewhat rounded nuclei, and in most places they
are connected by broad or slender processes ; and here and there they are
seen with two nuclei, probably indicating the occurrence of cell-division.
In the compact layer the capillaries in some parts are enormously dilated, in
other parts there is only a moderate increase in size ; here and there are
small extravasations of blood in the decidual tissue.

The decidua reflexa (capsularis) has not yet come into contact with the
vera, so that a uterine cavity still exists, although it is diminished in size
by the growth of the embryo and its envelopes. The basal and polar part
of the reflexa can be distinguished : the former consists of an inner
compact and an outer spongy layer which contains gland spaces, not so
numerous as in the corresponding layer of the vera or serotina ; the latter
or polar portion contains very few or no glands. None of the glands open
on the inner surface of the reflexa. Blood spaces are to be seen throughout
the membrane ; in the basal part some communicate with the intervillous
spaces of the opposed chorion laeve ; and here and there masses of
syncytium may be traced into the vessels. At this age there are already
signs of degeneration in the reflexa, of the nature of coagulation-necrosis of
the decidual tissue, so that in the innermost part is found a layer of fibrin-
like material thickest at the pole. Possibly some of the fibrin may arise
from the blood. On the inner surface of the reflexa no trace of maternal
epithelial cells can be seen, and on the outer surface is an epithelium with
much the same characters as that on the vera (cubical or flattened, not
ciliated).

The decidua serotina is from 2 to 3 mm. in thickness, and the compact
layer measures about one-quarter of the whole. In some parts the whole
decidua looks as if made up of the compact layer, but as Webster points
out this may be a variation of no importance. The surface is irregular and
no surface epithelium is visible. The outer parts of the glands in the
compact layer are obliterated, and both in them and in the deeper parts
in the spongy layer the lining epithelium may be cubical or irregular,
attached or loose, and more or less degenerated. In the interglandular
tissue characteristic decidual cells are found in some parts, and in others
they show signs of degeneration. The superficial part of the compacta
shows an irregular layer of fibrin-like material ; this has to be distinguished
from the layer of fibrin on the surface which results from coagulation of
the blood. At intervals chorionic villi are fixed to the surface, and

SIXTH WEEK AFTER IMPREGNATION 63

between them flattened masses of protoplasm (syncytium or Plasmodium)
are attached. The syncytium forms a more broken layer than it did before.
There are no cell outlines, and the nuclei are arranged either in rows or
quite irregularly. Here and there the masses of syncytium are thickened
and resemble very large multinucleated giant cells, with prolongations
running into the intervillous spaces. It is to be noted that no structure
at all like this is to be found on the surface of the decidua vera, one
among several reasons which lead us to believe that it is of foetal origin.
Processes of it extend into the substance of the decidua as deep as the
muscular coat. In the spongy layer of the serotina are numerous small
tortuous arteries ; in the compact layer the arteries cannot be distinguished
from the veins ; there are numerous sinuses which may be dilated capillaries,
and some of them can be seen to open into intervillous spaces and to con-
tain masses of syncytium attached to the wall.

The surface of the chorion which is opposed to the decidua serotina is
thickly covered with much branched villi. They show a thick layer of
syncytium and a comparatively thin underlying cellular layer. Poth on
the general chorionic surface and on the villi are little projections of the
syncytium. The mesoblast of the chorion and that which forms the cores
of the villi has a denser and a more fibrillated appearance than in the
earlier weeks, and here and there it forms a sort of basement membrane
under the cellular or Langhans' layer. In the smaller villi the mesoblast
is of a mucoid type ; and capillaries exist in most of them, whether small
or large. Among the villi are irregularly shaped pieces of syncytium, and
clumps of decidual cells with pieces of Langhans' layer attached to them.
The decidual masses may show signs of fibrinous or hyaline degeneration.
The attachment of the villi to the serotina seems to be by means of the
proliferated mass of cells of Langhans' layer at the tips ; apparently the
syncytium has nothing to do with it. The villi are attached at no regular
intervals, by a main stem or by branches, and at right angles or obliquely
to the surface. From the chorionic surface which is opposed to the
decidua rerlexa not nearly so many villi spring ; they are less branched,
show very few bucWike projections, are poorly supplied with capillaries,
and sometimes contain connective tissue which is swollen or hyaline.

From the foregoing description it will be learned that the decidual
and chorionic investments of the embryo have not altered greatly from
what existed in the two preceding weeks. The space, however, between
the vera and reflexa is much smaller, and the rerlexa already has begun
to show retrogressive changes. The glands, also, of all the decidual mem-
branes are in a more or less marked state of defeneration ; this is true
even of the serotina, in which, however, the other parts are as yet in full
activity. There is a still further development of the chorion and its villi
opposed to the decidua serotina ; and, although there is not here a separate
and recognisable placenta, there is the physiological equivalent of that
organ. The important part played by the foetal tissues in the construction
of the placenta is also quite apparent.

The amniotic sac has now increased with the marked growth of the
embryo, so that the space (extra-embryonic ccelom) between it and the inner
surface of the chorionic vesicle has practically disappeared. The histo-
logical characters of the membrane scarcely alter, but the matrix between
its two layers becomes somewhat condensed and fibrillated. The yolk-
sac or umbilical vesicle has much the same size as in the fourth week ;
it is obviously being left behind by the embryo in the progress of
ontogenesis, for its useful part is played already. Consequently the

64 ANTENATAL PATHOLOGY AND HYGIENE

vitelline circulation is atrophying. The great characteristics of the
extra-embryonic structures in the sixth week are the growing importance
of the chorionic villi which face the decid.ua serotina, with the consequent
establishment ere long of a true and anatomically recognisable placenta,
and the dwindling of the decidua rellexa and umbilical vesicle. One of
the great rearrangements of ontogenesis is in progress. By its means
intrauterine existence is to be prolonged for seven months or more.

The Embryo in the Sixth Week.

The embryo at the sixth week measures from 14 to 17 or 18 mm. in
length. The increase in length during this and the preceding week is in
part due to the straightening of the body and the raising of the head.
Tbe changes in the external appearances of the embryo are also largely
caused by this alteration in attitude. The neck bend becomes in conse-
quence more rectangular, and the concavity at the back of the neck
(Naclcengrube) less marked. The mid-brain comes to lie more directly
above the hind-brain. The abdomen is still more prominent than it was
in the fifth week, with the result that the limbs do not in profile views
reach to the outline of it. Other external features at this age are : the
presence of a distinct tail, which, however, is relatively smaller than before ;
the indication towards the end of the week of slight folds surrounding the
conjunctival area, the anlages of the future eyelids ; the appearance of the
fingers as separate outgrowths ; the existence of a recognisable external
ear or concha ; the completion of the upper part of the face by the union
of the two maxillary processes ; and the direction of the long axes of the
limbs at right angles to that of the body. The umbilical cord is now quite
distinct in its whole length, and still evidently contains a loop of intestine.

If we look more closely at the face at this period of embryonic life, Ave
shall see that through the union of the maxillary processes with the lateral
nasal and fronto-nasal processes the external boundaries of the nostrils
have been marked out and the mouth cavity shut off from the nasal pits.
There is consequently a recognisable but very broad nose, the nasal pits
being the anterior nares. It is noteworthy, also, that through the down-
ward growth of the fronto-nasal process the mouth receives a new upper
boundary. Posterior to this process the oral and nasal cavities freely
communicate, for the two lateral halves of the palate do not begin to unite
till the eighth week. The oral surface of the upper jaw already (towards
the end of the sixth week) shows two parallel ridges, which afterwards
become the upper lip and the gum. At the posterior end of the embryo the
genital tubercle is now clearly recognisable ; behind it is the cloaca! fossa,
and encircling the fossa is a ridge (genital ridge of the external genitals) ;
no groove has yet appeared on the under surface of the genital tubercle.
The histological examination of the skin shows the same two layers as in
the fifth week with an indication of the dermis or cutis beneath them.

There are no very radical changes to be noted in the internal conditions
of the embryo in this week. As has been already stated, most of the
embryonic rearrangements have been initiated and many of them have been
almost carried through. Such changes as there are affect specially the
brain and spinal cord, the car and eye, the genito-urinary organs, and the
sustentacular framework of the body.

The alterations in the spinal cord are evident. The central canal has
changed from an oval running antero posteriorly into a somewhat rhom-
boidal form. The lateral angles of the lozenge-shaped space thus produced

DEVELOPMENT OF THE NEIl\rOUS SYSTEM 65

divide the lateral walls very distinctly into two zones or laminae, an alar
or dorsal and a basal or ventral. The roof- and floor-plates (mid-dorsal and
mid-ventral laminae) show no changes ; but the alar and basal laminae of
the lateral walls do so. The posterior nerve-roots, of which there were in-
dications at the fourth and fifth weeks, can now be recognised passing into
the alar lamina ; while the anterior or ventral roots take origin in the
ventral lamina. At present the cells of the ventral zone are much more
numerous than those of the dorsal, so that the rudiment of the anterior
horn of grey matter is much larger than that of the posterior horn. There
is also some sign of the developing white columns of the cord : the grey
matter of the anterior horn is separated from the ventral surface of the cord
by a zone in which longitudinal nerve fibres are found, the commencing
anterior white column ; in a less marked fashion the posterior white
columns have begun to be evident between the posterior horn and the sur-
face. There is, as yet, little or no trace of lateral columns, and there are no
signs of an anterior or a posterior fissure. About this time the two ventral
arteries unite into a single median vessel (arteria sulci). The whole of the
complicated arrangement of bundles of fibres in the cord comes into being
later than the sixth week, that is to say in the foetal and not in the
embryonic period of antenatal life.

The changes in the brain are of the nature of continuations of those
initiated in the fifth week, and the reader may refer to the preceding
chapter for the account of them. There are some slight indications of
further structural elaborations to which an allusion may be made. In the
hind-brain or rhombencephalon and in that part of it (myelencephalon)
which becomes the medulla oblongata, there is a broadening out of both
roof-plate and dorsal part of the brain cavity so that the margins of the
dorsal zone or alar lamina have a lateral position and are bent towards the
ventral aspect to form a reflected lip (Raidenlijrpe). In the sixth week
this lip continues to grow towards the ventral aspect of the myelencephalon
to which it is united, and it thus buries parts (e.g. tractus solitarius) which
primarily lay on the surface. Through this bending, the surfaces of the
dorsal zone come to be dorsal and ventral instead of internal and external ;
from it the future restiform body is formed. The ventral zones show
marked thickening, and so the floor of the cavity of the myelencephalon
(fourth ventricle) is flattened. The grey matter of the ventral zones forms
the formatio reticularis ; it is surrounded by great numbers of neuroblasts
(future olivary bodies, etc.). Probably somewhat similar changes occur in
the metencephalon and mesencephalon. The form and divisions of the
diencephalon, as described under the fifth week, are maintained ; the pineal
body or epiphysis, a product of the roof-plate, is now more evident ; there
is also an expansion of the subthalamic regions, which probably are the con-
tinuations forward of the formatio reticularis. In the telencephalon, the
lamina terminalis, to which reference has already been made and which
probably represents the roof-plate of this part of the brain, becomes
thickened and constitutes the anlage of the septum lucidum, the corpus
callosum, the fornix, and the anterior commissure. It is not till much
later that a cavity forms in the septum lucidum, to be known as the fifth
ventricle. The cerebral hemispheres are now growing rapidly and project
forwards and upwards in front of the diencephalon ; their communications
with the third ventricle (foramina of Monro) do not enlarge but their walls
thicken, and therefore the connecting structures enlarge ; a commencement
of the delimitation of the hemispheres into lobes is effected by the develop-
ment of the fossa of Sylvius which marks off a frontal lobe and a post-
vol. ii. — 5

66 ANTENATAL PATHOLOGY AND HYGIENE

Sylvian lobe (future temporal and occipital lobes), each of which contains
part of the lateral ventricle. About tbe sixth week the olfactory ganglion
on each side unites with the bulbus olfactorius of the olfactory lobe of that
side.

With regard to the eye, it may be noted that in this or in the succeed-
ing week (the seventh) the choroidal fissure disappears by a fusion of its
lips. The ocular tunics also begin to form; thus mesodermic tissue sur-
rounds the lens-vesicle and constitutes the tunica vasculosa lentis. In this
tunic, vessels are developed which are associated with the hyaloid artery of
the vitreous humour. The position of the eye changes : at first it was
lateral and high up; in the fifth week it descended somewhat; and, now,
in the sixth week it moves round more to the front ; but even at the end of
the second month the axes of the two eyes are far from parallel. In the
middle ear the malleus and incus and their relations with Meckel's cartilage
can be recognised ; the cartilage of the second branchial arch is also present
with the seventh nerve close behind its posterior end, and with a ring of
cartilage above it, representing the stapes. The internal ear shows a differ-
entiation into semicircular canals, cochlea, endolymphatic duct, sacculus,
and utricle. From six tubercles and a ridge lying posterior to them the
external ear (pinna or concha) is taking form. One tubercle becomes the
future tragus, another the antitragus, another the lobule, another the anti-
helix, and the two remaining ones along with the ridge become the
helix.

The alimentary system, like the nervous, shows no marked new de-
velopments during this week ; in fact, with the exception of the develop-
ment of the rectum, anus, and perineum, and the rearrangement of the
intestinal coils, this part of the body of the embryo may be said to have
taken on its permanent form. In the mouth the anlage of the submaxillary
gland is now more evident than it was in the fifth week ; and traces of the
milages of the parotid and sublingual glands may perhaps have appeared. The
dental shelf is visible as a horizontal outgrowth from the epithelial down-
growth which gives rise to the lip groove. In the third pair of pharyngeal
pouches the epithelial part of the thymus gland is beginning to form.
There is as yet no trace of the tonsils. The oesophagus is relatively longer
than before, and the stomach shows more distinctly its greater and lesser
curvatures. The duodenum is clearly marked off as the part of small
intestine which passes from the pyloric end of the stomach towards the
dorsal body -wall. Below this is the U-shaped loop already described with
the indication of the caecum. About this time the lower limb of the U so
alters its position that it comes to lie above the upper limb, and thus the
caecum, transverse colon, and descending colon come to occupy their adult
relations to the small intestines. The descent of the caecum and the de-
velopment of the ascending colon, however, have yet to take place. Five
other primary coils of the small intestine, in addition to the duodenal one,
can be recognised ; all these five lie within the umbilical cord at present
(Mall, Johns Hopkins II<></>. Bull., ix. 197, 1898). The ectodermal cloaca
is now present, but of this more must be said in the next paragraph.

Into the ectodermal cloaca open the urino-genital sinus and the bowel.
The bowel, however, which opens into the cloaca does not apparently con-
stitute the permanent anus. The cloaca, it would seem, is closed again,
and the perineum formed by the growth of two eminences (Reichel's
Analhucker) at the sides of it. In the present week it communicates with
the exterior, and into it opens the urino-genital sinus. The Wolffian ducts
in turn open into the urino-genital sinus. The two Mullerian ducts can be

SIXTH WEEK AFTER IMPREGNATION 67

recognised at this age ; they lie in the genital cord along with the Wolffian
ducts, and have apparently in part coalesced into one duct (Berry Hart,
Trans. Edinb. Obst. Soc, xxvi. 264, 1901); in the upper part of their
course they are separate. The kidneys and ureters are present ; the latter
have as yet no connection with the bladder. The Wolffian bodies may
even now show signs of regression.

Little need be said regarding the respiratory and circulatory systems in
this week of embryonic life. The larynx is recognisable as the dilated part
of the trachea which opens into the pharynx ; and the arytenoid ridges are
better marked than in the preceding week. In the heart the inter-
ventricular septum, the endocardial cushions, and the aortic ridges meet
and are united by a thin membrane, the pars membranacea septi ; thus
the ventricles are separated. The sustentacular system shows marked
progress ; if the clavicle has not yet begun to ossify, it may show
signs of doing so now ; ossification begins also in the lower jaw around
Meckel's cartilage; the ribs and basis cranii are undergoing chondrification ;
and cartilage is evident in the bodies of the vertebrae. According to some
authorities the anlage of the spleen may be seen even at the fifth week as a
thickening of the mesoderm (mesenchyma) on the dorsal surface of the
mesogastrium ; according to others it is not recognisable till the eighth week.

The sixth week is, as has been stated already, the closing period
of purely embryonic life. We expect, therefore, to find in it some
slackening in the rate of development, and some diminution in the
variety of organogenetic processes. There are fewer surprises of
evolution ; and there is a greater tendency for the constructive
procedures to follow in their progression the lines that have been
already indicated. The period of rapid transformations and of
bewildering rearrangements and alterations is past.

In the extra-embryonic parts it is evident that the typically
decidual environment has reached the culminating point of its
functional importance and usefulness ; already there are abundant
signs that in future it is to be one part only of the caducal mem-
branes (the decidua serotina) that is to pursue the path of elaboration
and structural differentiation, while the others follow retrogressive
ways. The preponderating importance of the chorionic villi of the
chorion frondosum is becoming more and more evident, while the
dwindling of the umbilical vesicle, its attachments, and its circula-
tion is clearly recognisable. The placenta looms big in the immediate
future of antenatal affairs. There has also been so marked an ex-
pansion of the amniotic sac and its contents (liquor amnii and
embryo) that its walls are now close to those of the chorionic vesicle
and the extra-embryonic coelom has disappeared.

The embryo has lost a little more of its exaggerated C shape ; its
abdomen has become more prominent and its tail less so ; it has re-
cognisably separate fingers, although its toes are not so far advanced ;
the mouth and nose are shut off from each other externally, and so
there is a face ; the eyes are coming round more to the front ; the
tubercles which go to form the external ear or pinna are grouping
themselves so as to shadow forth that structure ; and, at the other end
of the body, the genital tubercle, which is later to give origin to the

68 ANTENATAL PATHOLOGY AND HYGIENE

penis or the clitoris, is recognisable. By an organogenetic rearrange-
ment, the details of which are unknown to ns, the entodermal cloaca
has become transformed into the bladder and other parts, and an
ectodermal cloaca open to the exterior has appeared.

In the embryonic interior some further differentiations are in pro-
gress. The anlages of the salivary glands, the thymus, the larynx,
the kidneys, and perhaps the spleen have appeared ; the complicated
changes (possibly including ossification) which lead to the disappear-
ance of Meckel's cartilage and the formation of the auditory ossicles
have begun, and ossification has commenced in the clavicle. The
intestinal coils have made their great rotation with resulting com-
plexity of arrangement of themselves and of their mesentery. The
spinal cord begins to show traces of its mature division into grey and
white matter, or at least into the anlages of the grey horns and of
the white columns ; and the posterior roots of the spinal nerves are
forming. In the brain the signs of the great hemispherical expansion
are still more evident than in the preceding week; the fossa of
Sylvius with its adumbration of innumerable fissures and convolutions
yet to be evolved is to be recognised ; and the pineal body and the
anlagc of the corpus callosum are present.

Organogenetic Rearrangements.

Before I pass from this brief sketch of development during the
embryonic period (it is, as I have already pointed out, nothing
more than a sketch), it may be well if I recapitulate shortly some
of the more complicated organogenetic rearrangements. The chron-
ological method does not lend itself to the description of intricate
phenomena of ontogenesis extending over prolonged periods, hence
the necessity of recapitulation and grouping. The embryological
changes described in the following paragraphs are not all com-
pleted in the embryonic period of antenatal life, some of them
indeed are in progress late in the foetal epoch, but they are placed
in association for the sake of clearness and fulness.

The Branchial Grooves and Pharyngeal Pouches. — The
structures derived from these parts are :

1. From the first, the salivary glands, the external auditory
meatus, the tympanic cavity, and the Eustachian tube ;

2. From the second and the neighbouring parts, the tonsils, the
middle lobe of the thyroid gland, and the posterior third of the
tongue ;

3. From the third, the thymus gland and a parathyroid ;

4. From the fourth, the lateral lobes of the thyroid gland, and
another parathyroid ; and

5. From the fifth, the little known post-branchial bodies.

The Branchial Arches. — The osseous and cartilaginous' structures
derived from them are :

1. From the first, the maxillary process and its derivatives
(superior maxilla, zygoma of temporal, malar, internal pterygoid
process of sphenoid, and palate), and the mandibular process and its

ORGANOGENETIC REARRANGEMENTS 69

derivatives (lower jaw, and Meckel's cartilage with the malleus and
incus) ;

2. From the second, the stapes, the styloid process, the stylo-
hyoid ligament (which may ossify), the lesser cornu of the hyoid
hone, and probably part of the body of that bone ;

3. From the third arch, the greater cornu and the rest of the
body of the hyoid ;

4. From the fourth, probably the thyroid cartilage of the larynx ;
and

5. From the fifth, possibly a part of the thyroid cartilage.
The nerves of the arches are :

1. Of the first, the second and third divisions of the fifth ;

2. Of the second, the seventh and eighth ;

3. Of the third, the ninth or glossopharyngeal ;

4. Of the fourth, the superior laryngeal branch of the tenth
nerve or vagus ; and

5. Of the fifth, the inferior laryngeal branch of the vagus.

The arteries of the branchial arches, consisting of five 1 aortic
arches on each side, arising from a ventral stem and joining a dorsal
one, are transformed into the following permanent arteries : —

1. The first aortic arch disappears, unless the internal maxillary
artery represents it, and the ventral stem becomes part of the external
carotid, while the dorsal stem becomes part of the internal carotid;

2. The second aortic arch also degenerates, to be indicated
possibly by the future lingual artery ;

3. The third arch along with its dorsal stem and that of the
second and first arches becomes the internal carotid artery ;

4. The fourth arch on the right side becomes the first and second
parts of the subclavian artery and its lower part disappears, while
on the left side it forms the permanent aortic arch ;

5. The fifth arch on the right side in great part disappears, while
that of the left side remains as the ductus arteriosus.

The Cerebral Vesicles. — Each of the five cerebral vesicles
consists primarily of five parts, — a roof-plate, a Moor-plate, an alar
lamina or dorsal zone of the lateral wall on each side, a basal
lamina or ventral zone of the lateral wall on each side, and a
cavity. Each of these five parts may have a representative or
representatives in later development.

1. The MyelencepJialon. — From the myelencephalon, which, as a
whole, becomes the medulla oblongata, the following are derived : —

(a) From the roof -plate, the posterior velum.

(b) From the floor-plate, the median raphe.

(c) From the alar lamina?, the nuclei of the sensory roots of

some of the cranial nerves, the nuclei of Goll and Bur-
dach, and the olivary bodies.

(d) From the basal laminae, the nuclei of the motor roots of

some of the cranial nerves, and the formatio reticularis.

(e) From the cavity, the posterior part of the fourth ventricle.

2. The Metencephalon, as a whole, becomes the cerebellum and

1 Some embryologists maintain that there are six.

70 ANTENATAL PATHOLOGY AND HYGIENE

pons. Its connection with the mesencephalon constitutes the isthmus
rhombencephali. Both these parts are represented by certain
structures ; therefore those arising from the isthmus are placed
separately —

(a) From the roof-plate, part of the posterior velum and the

vermis of the cerebellum.

(b) From the floor-] date, the median raphe (?).

(c) From the alar laminae, the lobes of the cerebellum, the

flocculi, the nuclei of the sensory roots of some of the
cranial nerves, and the nuclei of the pons(?).

(d) From the basal laniime, the nuclei of the motor roots of

some of the cranial nerves and the formatio reti-
cularis (?).
(c) From the cavity, part of the fourth ventricle.
2a. The Isth m us Rhombencephali gives origin, in all probability, to
the following structures : —

(a) From the roof-plate, the anterior velum and the valve of

Vieussens.

(b) From the floor-plate, the median raphe.

(c) From the alar laniime, the superior peduncles of the cere-

bellum or brachia conjunctiva.

(d) From the basal lamina-, the posterior part of the crura

cerebri and the posterior part of the tegmentum (?).

(e) From the cavity, part of the fourth ventricle.

'■*>. The Mesencephalon, as a whole, gives rise to part of the crura
cerebri and to the corpora quadrigemina, but it is not exactly known
in what manner. The following is an incomplete and tentative
arrangement : — .

(a) From the roof-plate, some part not identified.

(b) From the floor-plate, the median raphe.

(c) From the alar lamina?, the corpora quadrigemina.

(d) From the basal laminae, the nuclei of the third and fourth

cranial nerves, the anterior part of the tegmentum, and
the anterior part of the crura cerebri.

(e) From the cavity, the iter or aqueduct of Sylvius.

4. The Dieneephalon or Thalamencephalon, which, as a whole,
constitutes the optic thalami, the corpora albicantia, and the pineal
body, gives rise in detail to the following parts : —

(a) From the roof-plate, the velum interpositum and the

pineal gland or epiphysis.

(b) From the floor-plate, the tissue of the mid- ventral line.

(c) From the alar laniime, the optic thalami, the pulvinares,

and the external geniculate bodies.

(d) From the basal lamina?, the subthalamic region, the corpora

albicantia (corpora manimillaria), and the tuber cinereum
(part).

(e) From the cavity, part of the third ventricle.

5. The Telencephalon, which, as a whole, gives rise to the cerebral
hemispheres, corpora striata, olfactory lobes, and infundibulum, is
converted into the following detailed structures : —

ORGANOGENETIC REARRANGEMENTS 71

(a) From the roof-plate, the anterior part of the velum inter-

positum (in the median part of the telencephalon) and
the floor of the choroidal fissure (in the hemisphere
part).

(b) From the floor-plate, the infundibulimi and part of the

pituitary body (hypophysis).

(c) From the alar laminae, the lamina terminalis and optic

evaginations (in the median part), and the pallium or
mantle, the corpora striata, and the olfactory lobes (in
the hemisphere part).

(d) From the basal lamina', the anterior part of the subthalamic

region and of the tuber cinereuin.

(e) From the cavity, the anterior part of the third ventricle,

the lateral ventricles, and the foramina of Monro.

CHAPTER VII

General Principles of the Pathology of the Embryo, or Teratology : (1) Physi-
ological Factor — Nature and Illustrations ; (2) Amniotic Factor — Nature,
Mode of Action, and Illustrations ; (3) Germinal Factor — Nature, Mode of
Action, and Illustrations.

From the perusal of the preceding chapters the reader will have
got some ideas of the chronology of embryonic life. No attempt has
been made to supply him with all the facts and theories of Embry-
ology ; for these he must look in the proper place, the standard
works on Embryology. In this volume it is Embryology in disorder,
or Teratology, with which we have to deal, and to devote more than
five chapters to Embryology in order would be to show a loss of all
sense pf proportion and a forgetf ulness of the main purpose of the
book. It would be as out of place as a full treatise on Physiology
in a work on Pathology. At the same time the writer has given
these chapters to the subject in order to emphasise the close
connection between Embryology and Teratology, and to leave
impressed on the mind of the reader the idea that his investigations
into teratological problems must be preceded by a knowledge of the
normal progress of development if they are to be in any degree
fruitful. Let us now pass from this excursus on Embryology and
return to the consideration of the general principles of Teratology
begun in Chapter I.

In the first section of this Manual, that dealing with the Path-
ology of the Foetus as distinct from that of the Embryo, I pointed out
that the peculiarities of foetal pathology could in large measure be
explained by the influence of three factors, — the environmental, the
placental, and the embryonic. In other words, I showed that the
physiology of the foetus, as exhibited in the special environment of
foetal life and in the placental economy, determined to a large
extent the characteristic manifestations of foetal diseases ; and I
indicated that such peculiarities as were not thus to be accounted
for might lie due to the effects of morbid conditions which had
arisen in the embryonic period of antenatal life, and been transmitted'
into the foetal. I hope now to show that it is reasonable to think
that in a somewhat similar way the peculiarities of the pathological
manifestations met with in the embryo may be determined by the
special physiological conditions of the embryonic period of life and
by the transmission into embryonic life of morbid states and tend-
encies acquired in the germinal epoch. In both cases I seek for the

72

PHYSIOLOGICAL FACTOR 73

explanation of the peculiarities of the pathological phenomena in the
peculiarities of the physiology of the period of life under considera-
tion and in the transmission into that period of morbid states
acquired in the preceding one. The pathology of each period of
antenatal life is influenced by the physiology of that period ; and it
in its turn influences the pathology of the succeeding period.

The Physiological Factor in Embryonic Pathology.

The peculiarities of embryonic pathology depend in the first place
upon the peculiarities of embryonic physiology, and embryonic physi-
ology, as I have already said, is simply Embryology, Ontogeny, or
Organogenesis. Embryonic life is concerned chiefly with the forma-
tion, elaboration, and specialisation of the materials which compose
the new organism. This is the fundamental fact in the physiology of
the embryo; the character of constructive activity pervades the
whole of embryonic life and gives to it its peculiar quality or
attribute. Where all the manifestations of life are of this formative
kind, it must of necessity follow that the disturbance of these mani-
festations by morbid causes will lead to malformations, or, if the dis-
turbance be great, to non-formations. Defects in the building of a
house show themselves nut in dry-rot but in architectural anomalies
and errors of construction. The pathology of the embryo has to do
not with diseases but with malformations.

Physiology underlies, pervades, and dominates pathology. To
understand embryonic pathology, then, it is necessary constantly to
keep in mind embryonic physiology. The more fully it is realised
that embryonic physiology means normal formation, the more easy it
becomes to concede that embryonic pathology must mean abnormal
formation. There can, I think, be no doubt that the distinctive
characters of embryonic physiology (Embryology) arc the chief
causes of the peculiarities of embryonic pathology (Teratology) ; in
this way embryonic physiology is the most important factor in em-
bryonic pathology. Of course it is not claimed that the embryo has
no functions apart from organ-formation ; some of its organs very
early begin to perform functions and so become liable to diseases as
distinguished from deformities ; but the main trend of its activities is
constructive in character. The physiology of the heart in embryonic
life consists in part in the rhythmical contractions which drive the
blood through the vessels both intra- and extra-embryonic; but it
also consists in the gradual elaboration of a simple sinuous tube, at
first made up of two cavities, through the intermediate stage of a
double-chambered organ, into a four-chambered heart, in which, how-
ever, the foramen ovale remains for the rest of antenatal life as one
of the indications of the structural changes through which the viscus
has passed. The pathology of the heart in embryonic life mainly
consists in an arrestment of this process of ontogenetic elaboration at
one stage or another, and in the effects upon the rest of the organ
which such a deviation from the normal produces. It may also, per-
haps, in some cases, consist in a hastening of some part of the process,

74 ANTENATAL PATHOLOGY AND HYGIENE

such as premature shutting off of the various chambers of the heart.
Since, however, the heart is also capable of contracting in such a way
as to propel the blood through the vessels, it follows that it has this
function interfered with and a true disease produced. I have taken
the heart as an illustration, for it shows clearly this combination of
functional activity with structural elaboration and the consequent
combination of characteristic foetal with characteristic embryonic life ;
but the heart is an exception to the general rule in embryonic physi-
ology. Most of the organs during the embryonic period are fully
occupied with one form of vital activity — formation — and have not
yet begun to functionate in the manner characteristic of fcetal or
postnatal existence.

To take another instance, physiological activity in the region of
the neck of the embryo consists in the formation of the branchial
arches and clefts, and later in their reorganisation and utilisation in
the construction of the cervical tissues, lower jaw, and inner and
middle ear. Pathological embryonic activity in this same cervical
region will take the form of disorderly development of these parts,
and in the persistence of certain structures or grouping of structures
which are normally quite transitory. Instances of such anomalies
are found in cervical fistula? and auricles ; while among monstrosities
thus formed may be mentioned hypoagnathus or defective develop-
ment of the mandible and its associated malformations. In the
posterior end of the embryo, physiological activity shuts oft* the uro-
genital sinus from the anus, and again interposes a septum between
the urethra and the vagina (in the female). Pathological activity re-
veals itself in an interference with this delimitation, and may cause a
persistence of the cloaca or of the sinus uro-genitalis.

These, then, are instances of the influence which the physiological
(embryological) factor has upon the pathological (teratological) mani-
festations of embryonic life. Let it be repeated that foetal life has
mostly to do with the growth of tissues and organs, and embryonic
life with the differentiation of tissues and organs. Diseases are the
characteristic morbid phenomena of foetal life, and deformities of em-
bryonic life. Teratology, in a word, is Embryology in disorder ; there
is defective Ontogeny ; the vital dynamics of the antenatal formative
processes is under the influence of morbid agencies. In a very
special way, therefore, the physiological factor dominates the path-
ology of the embryo, giving to it some at least of its outstanding
peculiarities.

The Amniotic Factor in Embryonic Pathology.

Just as disturbances in the physiology of the foetus make their
effect felt upon the pathology of the foetus chiefly through the inter-
medium of one organ (the placenta), so probably disturbances in the
physiology of the embryo modify the manifestations of pathology in
the embryo through the agency of one structure (the amnion). Mor-
bid causes in fcetal life chiefly act upon the foetus through the
placenta; so probably morbid causes in embryonic life act chiefly

AMNIOTIC FACTOR 75

through the amnion. The placental economy comes in between the
maternal and the foetal organisms ; so to some extent does the
amniotic economy come in between the maternal and the embryonic
organisms. The immediate environment of the embryo is amniotic ;
so it must be admitted is that of the foetus ; but in the latter the
placenta has greatly increased in physiological value, while the
amnion has diminished, and so from the point of view of functional
activity the placenta is the most important part of the fcetal environ-
ment. There is then a placental factor in foetal pathology, and an
amniotic factor in embryonic pathology. Disturbances in function or
diseases arise through the placenta, disturbances in construction
through the amnion.

This view, at any rate, is a feasible one, although it cannot be
claimed that it has been proved for the human embryo. In animals,
e.g. the chick, it seems perfectly clear that the normal evolution of
tlie embryo is very immediately dependent upon the normal evolu-
tion of the amniotic folds. The appearance of the cephalic, caudal,
and lateral folds in the extra-embryonic somatopleure, their regular
growth until the embryo is completely surrounded by them, their
entire separation from the body of the embryo by the interposition
of the liquor amnii, and the final closure of the amniotic sac, con-
stitute a series of complicated processes, the normal performance of
which is essential for the orderly formation of the new organism. It
is to Teratology, and more especially to Comparative and Experi-
mental Teratogeny, that science is indebted for the demonstration of
this important fact. I doubt whether the role of the amnion in the
normal formation of the chick could ever have been predicted from
the examination of healthy embryological specimens alone. Dareste
and other experimental teratologists have made numberless observa-
tions which seem to show that irregular development of the amnion
is an almost constant factor in the artificial production of monstrosi-
ties in the chick. Apparently these morbid effects are clue to the
pressure brought to bear upon the embryo by the. non-separation of
the amniotic membrane from various regions of its body or limbs.
This may be a transitory condition, and complete closure of the
amniotic sac and separation of the amniotic membrane may occur
later; but the removal of the pressure does not restore the status quo
ante, the embryo goes on developing along the morbid lines into which
it has been directed, and the pathological process continues although
the immediate cause be no longer evident. A good example of this
action of the amnion is found in the sympodial or sireniform foetus.
In its production there is defective development of the caudal fold of
the amnion and consequent pressure on the posterior end of the
embryo. This leads to rotation and partial fusion of the lower limbs
and of the two halves of the pelvis ; thereafter the development of
the amnion may be completed, but the pathological process initiated
remains, growth continues, and sympodia is found at the end of
embryonic life. Possibly the earlier the pressure is removed the
less complete will be the rotation and fusion of the lower limits.

It may, however, be urged, and with no little force, that it by no

76 ANTENATAL PATHOLOGY AND HYGIENE

means follows that the amnion plays the same part in human embryo-
genesis as it seems to do in that of the chick. This is perfectly true ;
and every effort must he made to avoid hasty generalisations regard-
ing human embryology derived from observations in comparative
embryology. If it were known, however, that the human amnion in
its development arose in the same manner as the corresponding
structure in the chick, there would be a strengthened presumption
that its al mormal development would produce similar teratological
results in the human subject and in the chick. Unfortunately this
information is not available. As has been shown in Chapter III., it
is precisely regarding the mode of development of the human amnion
that embryology is without knowledge. In the earliest known human
ovum the amnion is already present. Further, what is known of the
later stages of its formation rather suggests a manner of development
differing in essential details from that met wTith in the chick. Con-
sequently it cannot be safe to conclude that the amnion in human
embryogenesis plays a part similar to that which it does in avian
embryogenesis either under normal or under abnormal circumstances.

On the other hand there is evidence of a certain kind in favour of
the presence of an important amniotic factor in human teratogenesis.
There have been many cases of malformation and monstrosity in
which the amnion was adherent to the deformed part of the foetus,
or in which amniotic bands and threads coexisted with grave struc-
tural anomalies (vide Chapter XI.). I have met with several in-
stances of this : one was a feet us with exomphalus, facial fissures,
cleft palate, diaphragmatic hernia, a stunted condition of the right
arm, ocular anomalies, and a sacral spina bifida, and from the sacral
sac a band passed to the membranes in the neighbourhood of the
exomphalic sac ; two others were foetuses with exomphalos, facial
fissures, and digital defects ; another was a case of anencephalus and
spina bifida ; and another was an example of multiple malformations
of the digits. I have also examined cases in which the membranes
were adherent to the head or to other parts of the foetus. All the
large teratological collections may be expected to contain specimens
illustrating such bands and adhesions. Further, in cases of oligo-
hydramnion it has sometimes been found that the scantiness or
absence of liquor amnii was accompanied by the presence of various
fcetal malformations. It must be remembered, also, that the presence
of amniotic bands is not necessary for the theory of the amniotic
origin of human malformations. It is reasonable to expect that
amniotic pressure without actual adhesion may in some instances be
effective in teratogenesis. It is also quite conceivable that an
adhesion may exist at an early period of development, and, having
accomplished its pathological purpose, may then be absorbed or lost
to view in the morbid process which it has set up.

Evidence, therefore, is not lacking to support the view that the
amnion in the human subject as in the chick is an important factor
in teratogenesis, — evidence drawn from the study of human monstrosi-
ties themselves and not from experiments in avian embryogenesis.
At the same time it is quite possible that other parts of the environ-

GERMINAL FACTOR 77

ment of the embryo may also be effective factors in the production of
deformities. The chorion, the umbilical vesicle, and perhaps the
allantois may all exercise a distinct, although as yet unknown
and undefined, influence upon the morbid manifestations of em-
bryonic life.

In writing of the pathology of foetal life and its investigation, I
laid special stress upon the necessity of carefully examining the
placenta as well as the foetus in every case. Now, in reference to
the inquiry into the problems of teratology or embryonic pathology,
let me emphasise the importance of a thorough scrutiny of the ftetal
membranes and of the routine examination, microscopic as well as
macroscopic, of all abortion sacs and their contents thrown off in the
early months of pregnancy. What are most wanted at present are
careful descriptions of monstrous embryos from abortion sacs, obser-
vations upon teratological conditions while the organism is still in
the embryonic period of antenatal life. These are essential for the
further progress of a knowledge of human teratogenesis, and they are
at the present time the desiderata of embryonic pathology. Micro-
scopical human monstrosities are, as a matter of fact, almost un-
known.

Whether or not the amnion be the important factor in terato-
genesis, there can be no doubt that with the embryo just as with the
foetus it is the peculiarity of the intrauterine environment which so
powerfully modifies the morbid developments of antenatal existence.
This environmental factor in itself serves to explain nearly all the
apparently so inexplicable phenomena of teratology and foetal path-
ology. " Nearly all," for a very little thought will suffice to suggest
several problems which neither the factor of embryonic physiology
nor the role of the amnion serve to elucidate. This leads to the
consideration of the third factor in embryonic pathology, which I
have termed the germinal or pre-embryonic.

The Germinal Factor in Embryonic Pathology.

Just as there was found to be an embryonic factor in foetal path-
ology (Manual, Section L, p. 185), so in embryonic pathology there
are traces of the projection into it of the results of morbid causes
acting at a yet earlier period. The reader may have noticed that I
have made no reference to double monsters in what I have written
regarding the teratological manifestations of embryonic life. Yet as
every one knows, the united twins are very prominent and important
teratological productions. The reason of my silence hitherto is that
I believe that the double (or triple) nature of the organism is gener-
ally (if not always) decided before the period of the embryo is reached.
Let me try to make clear what is meant by this statement, and to
show the effect of the generalisation upon the study of the pathology
of embryonic life.

When there are twins in the uterus certain conditions may be
met with. In the first place, each twin may be separate having its
own placenta and membranes, and each of these separate twins may

78 ANTENATAL PATHOLOGY AND HYGIENE

be perfectly well formed and normal in every way. Again, one or
other twin or both twins may exhibit malformations ; one, for in-
stance, may be acephalic (placental parasite) or anencephalic (as in a
case reported by James Carmichael, Trans. Edinb. Obst. Soc, iv. 408,
1878), or both may be affected with hare-lip and cleft palate (as in
Underbill's observation, Trans. Edinb. Obst. Soc, vi. 2, 1881). In the
third place, the twins may be united together by a band but be in all
other respects normally formed, or the union may be so intimate as
necessarily to entail a considerable degree of deformity. In the
fourth place, the joined twins may be deformed in other parts of
their bodies than those concerned in the structural union ; they may,
for instance, exhibit spina bifida, imperforate rectum, talipes equinus,
and the like.

These being some of the conditions that may be met with in twin
pregnancies, I hold that they are not all developed in the embryonic
epoch. One twin may become deformed in this period of antenatal life,
both twins may have their organogenesis so interfered with that they
show malformations, and possibly two embryonic areas may become
so merged as to produce joined twins ; these things may conceivably
occur in the embryonic period. The fact, however, that there are
two organisms in the uterus has, I feel sure, been determined before
the appearance of the blastodermic layers and the commencement of
the life of the embryo (as that has been denned). The double nature
of the organism, but not necessarily its teratological character, has
been fixed in the pre-embryonic stage of antenatal life. Given the
presence of twins in the uterus, then one or other or both are subject
to the same influences which by their action produce monstrosities in
the single embryo and diseases in the single foetus, and may in con-
sequence become monstrous or diseased. I do not now refer to the
teratological nature of twins as twins, that is a matter which will be
dealt with later ; but I emphasise the statement that both the organ-
isms in the uterus in twinning must, in passing through the em-
bryonic period of their existence, be subject to the same morbid
influences as act upon the single embryo and produce in it tera-
tological results.

In addition, however, to this view of the subject, it must be borne
in mind that the fact that there are two organisms in the uterus
complicates the process of development by introducing the possibility
of the action of the one organism upon the other. It is a well-known
fact that if one twin die in utero it may, through the continued
growth of the living twin, become flattened and be converted into the
foetus papyraceus or compressus, so well known to obstetricians; and
recent observations have gone to show that this flattening may occur
at a very early period in intrauterine life (Loennberg, Monat*ch. f.
Geburtsk. n. Gyndk, xvi. 25, 1902). It is also believed by some
writers that one of the twins may not only influence the post-mortem
fortunes of the other, but may even affect its normal growth and
health. In the antenatal struggle for life and normal evolution,
which is thus supposed to be set up in utero, it is conceived that the
twin with greatest vitality and energy will gain an advantage over

TWINS 79

the other, will annex for his own benefit a larger area of the vas-
cnlarised chorion than is his by right of equal division, will obtain a
greater amount of nourishment, and will, in a phrase, become the
predominant partner. As a result, the other twin will be less well
nourished, will lag behind in its developmental processes, and may in
the end be reduced to the subordinate position of becoming a pen-
sioner upon his brother's bounty. A condition in which there were
two organisms in the uterus, each equally parasitic upon the maternal
system through the placenta, has then been replaced by a status in
which one of the organisms is a maternal parasite, while the other is
a parasite upon this parasite. In this way, therefore, twinning, a
pre-embryonically determined condition, comes to play a part in em-
bryonic pathology.

One need not, however, accept this explanation of the success of
one twin and the failure of the other. There is another possibility.
It may be necessary to look for the primary cause in germinal
or pre-embryonic existence. That there are to be twins has been
settled lief ore embryonic life begins; that one of these is to be
stronger and the other weaker while both are yet in the germinal
stage may also have been decided. One of the twins, if this view
be taken, may be so weak that its development cannot proceed
beyond an early and most rudimentary stage ; but through the cir-
cumstance of its twinship, and with the help of its co-twin, it is saved
from certain early death, and enabled to live on in utero, sometimes
without a heart, generally without any placenta or part of a placenta
to call its own.

Of course it must never be forgotten that it is going beyond the
bounds of strict accuracy to speak about such a thing as the struggle
for life in utero and success or failure in this struggle ; whether
we regard the action of one twin on the other as maleficent or bene-
ficent is not of so much importance as to bear in mind that we are
applying figurative terms to the results of processes in which, of
course, the idea of motives does not enter. The point upon which
special stress is laid is that in embryonic pathology there are peculi-
arities, for the explanation of which it is necessary to look in the
occurrences of the germinal period of antenatal life.

A still more cogent reason for believing in the existence of the
germinal factor in embryonic pathology is supplied by the frequently
observed heredity of some malformations. Sometimes it is found
that such an anomaly as Polydactyly, polymastia, perodactyly, cleft
palate, etc., recurs in one generation after another; sometimes several
members of the same family show the same structural anomaly ; and
occasionally a woman gives birth to several deformed children by one
husband and to several healthy ones by a second consort. In all
such cases it seems necessary to predicate the existence of morbid
tendencies prior to the embryonic epoch.

It is not claimed that the appreciation of the presence of these
three factors (physiological, amniotic, and germinal) will clear up all
the problems of embryonic pathology. Alas ! no. There is no such
easy accomplishment of our task. It is believed, however, that it

80 ANTENATAL PATHOLOGY AND HYGIENE

will throw some light upon teratological processes, enough light, per-
chance, to reveal that there are guiding principles and general laws
at work where all appears chaotic, disordered. It is something to
have realised that Teratology is Embryology in disorder ; it is more
to perceive that even this disorder is ruled by laws, is truly kept in
order by them. After all, let us check any feeling of pride in the
scientific achievements of these last years in the realm of Teratology
by listening to the definition of a monstrosity framed by Aristotle x
in the fourth century B.C. —

" The monstrosity is contrary to Nature, not contrary to Nature
taken absolutely, but contrary to the most usual course of Nature.
Nothing, in fact, can be produced contrary to that Nature, which is
both eternal and essential."

Have we advanced at all in our conception of the real nature of
teratological phenomena since the days of the great philosopher of
Stagira, who " grace a l'etonnante penetration cle son coup d'ceil, a
devine une foule de verites que les siecles posterieurs n'ont fait que
confirmer, apres une longue serie d'observations " ? But let us take
heart ; he also believed that there was order hidden behind the seem-
ing disorder. With our gaze focussed upon the things that are near
we see only the disorder, but with a wider view, taking in the whole
horizon and rising therefrom to the zenith, we may catch a glimpse of
the eternal purpose.

1 Aristotle, lie pi juiwp yeveaecos, lib. iv. ch. iii.

CHAPTER VIII

Teratogenesis or the Theories of the Causation of Monstrosities : Theories of the
Past ; General Survey ; Theory of the Supernatural, with the Gods, or the
Evil Spirits, or the Stars as Teratogenic Factors ; Theory of the Natural, with
Seminal and Menstrual Conditions and Hybridity as Teratogenic Factors.

In the preceding chapter I have given some account of what may be
called the modern view of the nature of monstrosities, and have
enumerated certain factors which, I believe, give to the pathological
phenomena of embryonic life some at least of their distinctive
characters. But it is now necessary to turn for a little while to the
consideration of the theories regarding the nature and causes of
teratological occurrences which were held in early times. Thereafter
I shall refer to some more modern views, and so bring the reader back
again to the embi yological theory of teratogenesis, which has been
already sketched in outline in Chapter VII. In dealing with the
theories of the past I shall not encumber the text with many biblio-
graphical references ; these may be found by those who are desirous
of consulting them in a series of articles on " Teratogenesis " which
appeared in the Transactions of the Edinburgh Obstetrical Society, vol.
xxl, 1896.

From the earliest times of which we possess any knowledge,
attempts have been made to explain the occurrence of teratological
phenomena. Many have been the theories ; great has been the
difference of opinion. Recently, however, real progress seems at
length to have been made ; and science now possesses a wider and
more accurate knowledge of the causes which lead to the production
of monstrosities and of the manner in which they act.

Some of the views held by the ancients regarding the cause of
monstrous births have long since been abandoned or exist only among
savage races of a low type ; others are still accepted by the popular
mind, but rejected by the scientific ; and yet others have found a
certain degree of justification in the results of the clinical and patho-
logical investigations of the present age. In addition to the ideas
handed down to us as a heritage from past times there are those
which have been brought into being or confirmed in existence by
modern scientific research. Experiment, that exact method so much
vaunted in these times, has been brought into play, with results that
have been regarded as of immense importance ; a new science has,
in a sense, been born, that of experimental teratogeny. With this
attempt to solve the problems of Teratology by means of the artificial

VOL. II. — 6

82 ANTENATAL PATHOLOGY AND HYGIENE

and experimental production of monstrosities I shall have to deal ere
long; but it maybe remarked in passing that the animals experi-
mented upon have either been low in the Vertebrate series or have
been among the Invertebrata. It follows, therefore, that we must not
hastily ascribe to these experiments upon chicks, sea-urchins, and the
like an overwhelming importance in the study of the monstrosities
which occur in the human subject. Neither, however, can we afford
to neglect the evidence regarding teratogenesis which they furnish.

In the meantime let us* try to reconstruct from such materials and
records as are available the teratogenetic theories of the past.

Theories of the Past.

I. THE SUPERNATURAL.

In the early ages of the world's history, dimly discernible now
through the mists that have gathered over them, it was customary to
ascribe all unusual phenomena of Nature — earthquakes, comets, solar
halos or corona?, eclipses, and the like — to supernatural causes.
Abundant evidence of this is to be found in the literature and legends
of the past, and in the customs and beliefs of savage or primitive
peoples existing at the present time, but representing in such matters
former times. Among the phenomena of Nature, justly regarded
as unusual, a place could hardly be refused to malformations and
monstrosities of the human foetus or of the young of the lower
animals ; so it happens that in the earliest records of Teratology are
to be found indications that such occurrences were regarded as due to
supernatural agencies.

In different parts of the world, and in the same part but at
different times, opinions differed as to the nature of the supernatural
teratogenic cause and the meaning of the teratological phenomenon.
When we read over these varied beliefs we are tempted to the con-
clusion that primitive man had a lively imagination, but a little
thought is sufficient to make it likely that he is not to be accused of
romancing and of inventing meaningless absurdities ; he studied
Nature and Life keenly no doubt, and read the meaning of their
phenomena to the best of his ability with the little knowledge that he
possessed. Various influences, geographical surroundings and the
like, gave a colour to the beliefs of each particular race, and so helped
to produce the diversity which we wonder at.

Some peoples ascribed monstrous births to the gods they wor-
shipped ; other races, who had divided their pantheon into good and
evil deities, put the blame sometimes upon the former and sometimes
upon the latter ; whilst yet others connected teratological states with
the positions and mode of combination of the stars and other heavenly
bodies. Polytheism in some countries gave place to monotheism ;
yet the same ideas lived on, but were altered in their details to suit
the newer conceptions of the supernatural. To the God of the Jew
and the Christian, to the Satan of the Old Testament and the evil
spirits of the New, and to the stars in the midnight sky, men still

THE SUPERNATURAL IN TERATOGENESIS 83

looked as the causes, direct or indirect, of the deformities with which
an infant might come into the world.

The meaning, also, of the teratological phenomenon was variously
conceived. The gods were amusing themselves, said some ; they
were exhibiting the extent of their creative powers, said others ; they
were angry with men, they were warning mankind, or they were
chastising individuals or nations, were the views of yet others. From
such beliefs by an easy transition came the idea that monstrosities
were portents of events in the future, that they foretold sometimes
good, but more commonly evil fortune. The Chaldeans read in the
starry heavens the secrets of the future, and rapidly learned to
transfer to the monstrous infant born under this or that astral com-
bination the meaning of the stellar message ; and so in ancient
Babylonia the birth of a babe of doubtful sex meant an approaching
calamity to the land, whilst the appearance of one witli an imper-
forate anus proclaimed a famine ! Similar astrological beliefs were in
vogue in Europe in the Middle Ages; but they did not reign alone,
for a transference of teratogenic powers had (it was thought) taken
place from the deity to the devil, and to the malign influence of the
tempter and his emissaries — the mediaeval succubi and incubi — were
ascribed monstrous and malformed births. Happy was the mother of
a deformed infant when there was found a preponderating amount of
evidence in favour of the stellar origin of her abnormal progeny ;
otherwise she might hardly escape a death at the stake, she and her
" devil's brat."

Such were the various ways in which the supernatural was
invoked to explain the origin of malformed fcetuses. Let us look,
now, more in detail at the different phases of this ancient belief.

A. The Gods as Teratogenic Causes.

It is possible that in the earliest ages of the world's history
monstrous infants were regarded as divine, and worshipped. They
might be the gods themselves or their progeny, or they might simply
be simulacra of the deity. This view may be called euhemeristic, and
for the following reason. Euhemerus was an ancient historian of
Sicily (he lived about 316 B.C.), who wrote a book (long since lost) in
which he maintained (as the New English Dictionary tells us) that the
deities of Hellenic mythology were deified men and women ; and so
euhemerism means " the method of mythological interpretation which
regards myths as traditional accounts of real incidents in human
history." On this euhemeristic hypothesis it is easy to account for
the teratological appearances of many of the heathen gods and demi-
gods. Of course, some of them are unlike the cases of monstrosity
met with at the present time, but then it must be remembered that
facts handed down by oral tradition from distant times and through
the agency of men unacquainted with anatomical details, must inevit-
ably be distorted. Doubtless the artificer of the idol was seldom, if
ever, an eye-witness of the appearances of the deformed infant, and
was consequently compelled to draw upon his imagination in order

84 ANTENATAL PATHOLOGY AND HYGIENE

to eke out the exiguous data handed down to him to work with.
Possibly, also, as Schatz has suggested in an interesting address (Die
griechischen Gutter und die menschlichen Missgeburten, Wiesbaden,
1001), each race would pick out those monstrosities which accorded
most nearly with its particular notions of what its gods should be.
The Greeks, for instance, in their deity-construction seem to have
made no use of such wonder-exciting monstrosities as the acephalic
placental parasites and the various types of united twins ; and this
fact may be due to the national taste which led to the selection of
monstrosities that were not in themselves ugly or inartistic. Schatz
goes on to point out that in the case of the cyclops foetus there are
two types, an ugly one and one which might in comparison be termed
pretty; the Greeks took the pretty (!) one for the construction of the
god Polyphemus ; further, the nose which in the monstrous infant lies
above the single eye was put in its proper (aesthetic) position below
the eye. Figs. 18 and 19 will give an idea of the two types of
cyclopia.

Perhaps, then, we may euhemerisejthe Siren into the sympodial
foetus (Fig. 20), the Centaur into the infant born with two pairs of
lower limbs or into a hydrocephalic calf, the Gorgon head into an
acormic placental parasite, Atlas into a case of occipital encephalocele,
Janus into the diprosopous monstrosity, and Prometheus with his
liver being torn by the vulture into an instance of foetal exomphalos.
At any rate the god Pthah of the Egyptians would seem clearly to
have been an example of achondroplasia. So it may be that terato-
logical specimens have served to supply the races of mankind with
many of the deities which they have worshipped. The gods then
after all did not create men, but men made the gods ! J Thus, in
a novel sense, we may say " scepe visa? format Dcorum."

To take up another line of thought and one that commended itself
more to the ancients than did the hypothesis of Euhemerus : the gods
were the creators of monstrous infants, and their purpose was amuse-
ment. Zrug vaifyi, Jupiter is playing, said Heraclitus ; and Pliny
(Nat. Hist., lib. vii. c. 2) only slightly altered the idea when he wrote
that " Nature creates monsters for the purpose of astonishing us
and amusing herself," 1 or as it has been quaintly rendered by his
translator (P. Holland, Translation of Pliny, vol. i. p. 157, London,
1601), " see how Nature is disposed for the nones to devise full
wittily in this and such like pastimes to play with mankind, thereby
not onely to make her selfe merrie, but to set us a wondering at such
strange miracles." Traces of this old belief are to be found at the
present day in our use of the word " sport " in Botany, in the German
"Spielart" and " Naturspiel," the French "jeu de la nature," and in
the Latin " lusus naturae," as synonyms of monstrosity. " Freak of
nature " conveys a somewhat similar idea.

Soon, however, the notion of the sportive, pleasure-loving deities
turning out monstrosities very much like children making mud-pies
gave place to another and a graver conception. The gods were
still the cause, and they were still friendly or at least not actively

1 " Ludibria sibi, miraeula nobis ingeniosa fecit natuva."

CYCLOPS FCETUS

85

Fig. 18. — Head and upper part of trunk of Cyclops Foetus with nasal proboscis,
without mouth (astomus) and with ears displaced (synotus). Specimen
No. 270.

86

ANTENATAL PATHOLOGY AND HYGIENE

unfriendly ; but now their purpose was to warn, admonish, or
threaten mankind. The belief in the monitory or minatory meaning
of teratological occurrences took a firm hold on the minds of men,
and the introduction of Christianity only meant the transference
to the one God of the power and purpose that had been previously
ascribed to the many deities of the Greek and Eoman pantheon. It
may be that the word monster itself is derived from monco, I warn ;
but another origin has been suggested for it, to which reference
will immediately be made. The birth of a deformed infant, then,
was a Divine warning ; it meant, further, that a deity must be
propitiated, else some calamity would happen. From this belief

Fig. 19. — Cyclops Fcetus, with nasal proboscis,
which occurred in the practice of Dr.
J. W. Eodgers of Bristol (Nov. 13, 1891).

to the destruction of the malformed infant is but a step, and thus
doubtless arose one at least of the reasons why such monstrosities
were, in Europe at any rate, almost invariably killed during the Early
and Middle Ages. The Greeks of Sparta may have thrown their
deformed infants into the abyss near Mount Taygetus in order to
preserve or foster the physical perfection of the race ; but in other
countries the killing was sacrificial and propitiatory, and fortunate
was the mother if she escaped her infant's fate. Julius Obsequens
(Procligiorum Liber, pp. 86, 103, Basilere, 1552), for instance, states
that in the year 120 B.C., "in foro Vessano androgynus natus in mare
delatus est " ; and again, in 92 B.C., " Androgynus Urbino natus, in
mare deportatus." Lucan (Pharsalia, lib. i. verse 589), also, relates

SIREN FCETUS

87

'«/'

W

Fig. 20. — Sympodial or Siren Foetus. Specimen No. 121.

88 ANTENATAL PATHOLOGY AND HYGIENE

how Arnus, the chief of the Etruscan diviners, ordered the slaughter
and burning of all monstrous infants at the time of the overthrow of
the Roman Republic.

Gradually it would appear that the idea of the deity as the cause
of the monstrosity became lost in the consideration of the calamity
which the malformed infant was supposed to predict, portend, or
presage. The supposed cause of the phenomenon was lost sight of
in the supposed effect. In this way it is believed that the word
monstrum originated, namely, from monstro, I declare or show ; and, as
Cicero puts it (Be Divinatione, lib. i. par. 42), "Monstra, Ostenta,
Portenta, Prodigia appellantur, quoniam monstrant, ostendunt, por-
tendunt, predicant." Numerous instances might, in fact, be given
of the pretended connection between monstrous births and national
or personal calamities and misfortunes; the works of Lycosthenes
(Prodigiorum ac ostentorum chronicon, quae prccter naturae ordinem,
motum, et operationem, et in supcrioribus et his inferioribus mundi
regionibus, ab exordio mundi usque ad hcec nostra tem/pora, acciderunt,
Basileae, 1557), Obsequens (pp. cit.), Boaistuau (Histoires prodigieuses,
Paris, 1578), and others, are full of them. Perhaps, however, the
most remarkable list of teratotogical occurrences with the events
which they prognosticated or heralded is that which was literally
unearthed by Assyriologists among the brick tablets of Asshurbani-
pal's library at Nineveh. I have elsewhere (Teratologia, i. 127,
1894) described at length this curious document with its cuneiform
characters and its startling contents ; startling indeed they are,
for who could have expected to find among the ruins of the ancient
civilisation of Babylonia a catalogue of monstrosities of the human
infant, many of them quite capable of being identified by the present-
day teratologist. The teratological tablet is reproduced in Fig. 21,
and the reader who may be curious regarding the art of Teratoscopy
as practised in Mesopotamia possibly 2000 years B.C. is referred to
my article above mentioned. Sometimes the event predicted by the
monstrous birth was of a fortunate nature, as when an infant with
three legs (tripod) was born there would be great prosperity in the
land, and when Caesar's horse with feet showing digits served as
an indication that the world would one day belong to his master
(Suetonius). Notwithstanding some active opposition on the part of
such writers as Licetus and Polydore Vergil, the idea that monstrous
births predicted disaster held its ground till the close of the seven-
teenth century. Who shall say that in certain minds it is even now
dead?

There is evidence to show that a malformed infant was sometimes
regarded as a punishment of sins committed. In early French history
we read that Robert n. (the Pious), who succeeded Hugues Capet,
married near the close of the tenth century his cousin (in the fourth
degree) Bertha, daughter of Conrad, King of Aries, and widow of
Eudes I., Count of Blois. For this reason the royal pair were excom-
municated by the Pope. The Queen gave birth to an infant having
the head and neck of a goose (" une tete et un col d'oie "). Sismondi
suggests that the monstrous infant was caused by the fright received

CHALDEAN TERATOSCOPY

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-Chaldean Cuneiform Tablets with descriptive list of Monstrous Births.
(Vide Teratologic^ i. 127, 1894.)

90 ANTENATAL PATHOLOGY AND HYGIENE

by the Queen during her pregnancy from the papal excommunication ;
but Segur (quoted by E. Martin in his Histoire de monstres, p. 78,
1880) states that it was a punishment from God (" un punition de
Dieu "). This display of what we may call the vicarious teratogenic
power of the Pope seems to have brought Eobert the Pious to his
knees ; at any rate Bertha was repudiated in 1006, and the King
married the haughty Constance, daughter of the Count of Toulouse.

Possibly the disciples of our Lord had some thought of the puni-
tive nature of congenital malformations when they put the question
concerning the man born blind : " Master, who did sin, this man, or
his parents, that he was born blind ?" The reply probably gave rise
to the view that monstrous births were for the purpose of revealing
the glory of God. Pare, in his treatise on monsters, enumerates
thirteen causes of such productions, of which the first is to proclaim
the glory of God; to quote his translator (T. Johnson, lib. xxv. c. 1,
p. 585, London, 1678): "There are reckoned up many causes of
Monsters, the first whereof is the glory of God, that His immense
power may be manifest to those which are ignorant of it, by the
sending of those things which happen contrary to Nature." Expres-
sion was again given to this view in the discussions which took place
towards the close of the seventeenth century on the pre-existence of
germs. If normal germs pre-exist, why not also monstrous ones ?
This, however, Swammerdam and Malebranche would not admit,
because that would be to confess that monstrous infants were the
immediate work of the Creator. Regis, in his Systtme de philosophic
(in., lib. viii. pt. 1, c. ix.), accepted this consequence, and explained
that whilst normal beings showed forth the infinite wisdom of the
Creator, monstrosities demonstrated His infinite power. Obviously
this question does not stand alone, but forms one of a group (which
contains the relation of the Creator to human disease and sin and
disaster) ; its discussion would obviously be out of place here.

B. Evil Spirits as Teratogenic Causes.

It was inevitable that a belief in the teratogenic power of the
deity should be followed sooner or later by the idea that this power
was specially the possession of an evil or malignant spirit. Indica-
tions of this belief are to be found everywhere, and even at the
present time the notion gets whispered about even if it be not openly
stated. The history of the effects of this notion is full of sad, revolt-
ing, repulsive, and almost incredible incidents, and no good purpose
can be served by lingering over it. Reference, however, may be made
in passing to a piece of evidence which shows that the belief in the
diabolical origin of monstrosities prevailed in Northern as well as
in Middle and Southern Europe. Du Chaillu, in his work on the
Vikings (The Viking Age, ii. 40), gives the following extract from
an ancient legal enactment of the Norsemen : " Every child which is
born into this world shall be reared, baptized, and carried to the
church ; except that only which is born so deformed that the mother
cannot give strength to it. . . . It shall be carried to a beach and

ASTROLOGY AND TERATOLOGY 91

buried where neither men nor cattle go ; that is, the beach of the evil
one." The exposure of the deformed infant was, however, one of the
lesser evils associated with this theory of the production of mon-
strosities ; but as I have said it is unprofitable to inquire further into
this darkest fancy of the Dark Ages.

C. The Stars and Moon as Teratogenic Causes.

The position, movements, and combinations of the heavenly bodies
have entered largely into the formation of the opinions and beliefs of
ancient races concerning the origin of terrestrial phenomena. The
Chaldeans were, as is well known, past-masters in the art of divina-
tion from all kinds of phenomena, stellar or terrestrial. That they
connected together teratological and astrological occurrences is also
beyond doubt. Lenormant, in his work on Chaldean divination (La
Divination et la science <h s jnrsaf/en chez les Chaldeens, p. 103, Paris,
1875), explains in a very reasonable way how this belief grew. The
passage may be rendered as follows : " The development that their
astrology had given to genethlialogy or the art of horoscopes of
births, had led them early to attribute great importance to all the
teratological facts which were there produced. They claimed that an
experience of 470,000 years of observations, all concordant, fully
justified their system, and that in nothing was the influence of the
stars marked in a more indubitable manner than in the fatal law
which determined the destiny of each individual, according to the
state of the sky at the moment when he came into the world. Cicero,
by the very terms which he uses to refute the Chaldeans, shows that
the result of these ideas was to consider all infirmities and mon-
strosities that new-born infants exhibited as the inevitable and
irremediable consequence of the action of these astral positions.
This being granted, the observation of similar monstrosities gave, as
it were, a reflection of the state of the sky, on which depended all
terrestrial things ; consequently one might read the future in them
with as much certainty as in the stars themselves."

Astrology and the casting of nativities survived by many centuries
the fall of the Babylonian empire and the special Chaldean genethli-
alogy ; indeed, the notion of stellar influence upon the foetus lives
still in the expression " born under a lucky star," and Zadkiel's
Almanac is a solid fact. In the popular mind there was all along a
sort of intermittent and sneaking regard for such beliefs, although
the learned might slight " the art of those foolish Astrologers, and
Genethliacall Ephemerists, that use to pry into the horoscope of
Nativities " 1 and write of the " Genethliack and Horoscopal fool."

Ptolemasus Claudius, an Alexandrian astrologer of the second
century, not only stated the astral conditions under which terata
were born, but also mentioned the nature of the malformation to be
expected (Tctrabiblon, lib. iii. c. viii. ; quoted by Taruffi, Storia delta
Teratologia, iv. 18, 1886). The position of Venus or of the moon
at the moment of conception had, according to the astrological doc-

1 Howell, Dodonds Grove, p. 191, 1640.

92 ANTENATAL PATHOLOGY AND HYGIENE

trine, a potent effect in determining whether the infant was to be
normal or deformed. About the year 1200, a cow gave birth to a
deformed calf, said to be half human, and the shepherd in charge
thereof, being suspected of an unnatural crime, was condemned to
be burned alive. Fortunately for him, however, Albertus Magnus
(1193-1280), that most voluminous writer upon so vast a number
of different subjects, came forward with the explanation that the
teratological phenomenon was due to a certain constellation ; so the
shepherd's life was saved. In Be animalibus, one of the works of
Albertus, it is accordingly stated that a monstrosity may be due
either to a fault in the " material " or to celestial influence ; when
certain heavenly bodies are found in certain places in the sky a man
cannot be generated, and so a child is born with the head of a bull
or a lamb. Thomas Aquinas (1225-1274), " the Angelic Doctor," a
disciple of Albertus, accepted these theories, and even expounded
them to some extent. Cardanus, a prolific writer of the sixteenth
century (1501-1576), notable for the extraordinary mixture of
sagacity and obtuseness in his works, moulded his beliefs upon those
of Ptolemy already referred to ; he even supplied his readers with
the representation of the constellation under which a double monster
was born. Septalius (Libr. de Nccvis, Mediolani, 1606) looked to the
stars for the explanation of moles and mevi seen on the fcetus ; and
even T. Bartholin (Be cometd consilium rnedicum, monstrorum nuper
in Bania natorum historia, Copenhagen, 1665) believed in the potency
of stellar influences upon the unborn infant. In a work entitled
Grande Encyclope'die Unlverselle, by Henricus Asteldius, published
about the beginning of the seventeenth century, it is stated that a
Danish astronomer, who was also a medical man, had discovered the
origin of monstrosities. He ascribed them to comets, and regarded
them as tumours scattered throughout the firmament, which when
they were precipitated upon the earth, took on there all kinds of
unusual and extraordinary forms (E. Martin, op. cit.). In the work by
Aldrovandus (Be monstrorum historia, p. 391, Bononiffi, 1642), edited
by Ambrosinus, credence is given to the foregoing beliefs ; at any rate
the views are stated, and their adherents named. Ambrose Pare,
however, is openly sceptical, and says (to quote again his English
translator, Th. Johnson) : " Yet astrologers (lest there should seem
to be anything which they are ignorant of) refer the causes of these
(i.e. monsters) to certain constellations and aspects of the planets and
stars." Licetus (Be monstris, lib. i. cap. vi., Patavii, 1668) altogether
omitted the stars from his list of teratological causes, and so the
theory died out, at least among the scientific and so to say the official
teratologists.

Among the heavenly bodies the moon has always held a high
place in so far as her effect upon mundane affairs was concerned, and
a trace of a belief in her teratogenic powers is preserved in the word
"moon-calf." This word has three meanings given to it in most
English dictionaries. In Webster's Unabridged (Goodrich and
Porter), for instance, it is defined as (1) a monster; false conception;
(2) a mole, or mass of fleshy matter generated in the uterus; and (3)

"MOON-CALF" 93

a dolt ; a stupid fellow. Ogilvie, in the Imperial Dictionary (New
Edit., iii., 1882), gives the same three meanings, and adds that it is
comparable to the German " Mond-Kalb," a person or conception in-
liuenced by the moon. In Foster's Illustrated Medical Dictionary (iv.
p. 2337), its German equivalents are stated to be " Mondkalb " or
"Mohnkalb," and it is defined as (1) a foetal monstrosity, and (2) the
translation of an old term for a fleshy mole. J. 0. Halliwell, in his
Dictionary of Archaic and Provincial Words (ii. p. 5G0, London,
1847), quotes Cotgrave's definition, "a moon-calfe, a hard swelling or
shapeless peece of flesh in the wombe, which makes women beleeve
they are with child when they are not," and goes on to state that the
term was often applied to a monster or a fool, and that in Somerset a
crying child is so called. As with so many other words, its earliest
use in any of these three meanings is not known. Some authors
refer back to Pliny, but, of course, the word used by him was niola.
In the Natural History (lib. vii. cap. 15) it is said, "solum autem
animal menstruale mulier est : inde unius utero, quas appellarunt
molas. Ea est caro informis, inamma5 ferri ictum et aciem respuens " ;
and again in lib. x. cap. 64 we read, " Molas, de quibus ante dixinms,
gigni putant, ubi mulier non ex mare, veruin ex semetipsa tantum
conceperit." Now, in Holland's translation of Pliny's works (1601),
the word mooncalf appears as the equivalent of " mola " : " of all
living creatures, a woman hath a ilux of bloud every moneth; and
hereupon it is that in her wombe only there aie found a false con-
ception called mola, i. a moonecalfe, that is to say, a lumpe of flesh
without shape, without life, and so hard withall, that uneth a knife
will enter and pierce it either with edge or point," "as for false
conceptions or moonecalves (whereof we spake before) some thinke
they are engendred of the woman's seed only ; namely when she is
not conceived by a man but by herselfe." It is quite evident, there-
fore, that in the beginning of the seventeenth century the word
"mooncalf" was regarded as a synoiiym of uterine mole; how much
earlier I cannot say. In this sense it may be taken that the term
" calf " meant not the young of the cow, but a swelling or lump (as
in " calf " of the leg). Mooncalf might then be freely rendered
" menstrual lump." In Viardel's Accouchemens (Paris, 1671) the
word " mole " is used to signify a round mass engendered in the
uterus; but it is very interesting to find in the German translation
(Franckfurt, 1676) the term Monkalb or Mutterkalb used constantly
as its equivalent. At the same time it is not clear that the term
"Mon" meant the "moon" in this combination; perhaps rather it
was allied to " Monch," a gelding, and the Latin " mancus," defective,
in reference to Pliny's idea that the woman who conceived the mass
did so without impregnation.

It is not certain, then, that in this meaning the word mooncalf
suggested a belief in lunar influence ; but as a synonym of monstrosity
it truly seems to have done so. This becomes apparent from several
passages in Shakespeare's Tempest In this play Caliban, the son of
the " foul witch, Sycorax," is on several occasions called " a moon-
calf," for example, " the siege of this mooncalf," and " the dead

94 ANTENATAL PATHOLOGY AND HYGIENE

mooncalf's gaberdine," Act ii. sc. 2. He is described as " a freckled
whelp, hag-born — not honour 'd with a human shape," " half a fish,
half a monster."

" This misshapen knave,
His mother was a witch, and one so strong
That could control the moon, make flows and ebbs,
And deal in her command, without her power."

Whether Shakespeare in this passage hinted at a connection between
the witch-mother's control of the moon and her mooncalf son or is
merely playing upon words is not clear, but the latter hypothesis is
the more probable, for elsewhere in the Tempest the diabolical origin
of Caliban is referred to. In its meaning of monster we may take it
that mooncalf is derived from " moon " and " calf," the latter word
signifying in Middle English, Anglo-Saxon, German, etc., the young
of the cow or other animal, and being related to the Greek fip'upog
(embryo), and to the Sanskrit garbha (foetus or embryo) ; while
" moon " may refer to this belief in the lunar influence on the unborn
foetus. So mooncalf may be defined as an embryo or foetus become
monstrous under the influence of the moon.

Such were the theories of the supernatural which our ancestors
used to explain the occurrence of teratological phenomena, and while
they may bring a smile to the face of the modern scientist, it must
not be forgotten that their existence was a very real thing to the
people of the Middle Ages, and that even at the present day traces
of these early superstitions are to be found not only amongst savage
races, but also in our own land and in the popular mind.

II. THE NATURAL.

It must not be supposed that the only teratogenic theories in
vogue in past times were those which recognised supernatural
agencies. As a matter of fact the early Greek philosophers looked
for purely physical causes of monstrosities, and these they found in
disturbances of the natural phenomena of reproduction. At the
same time it must be borne in mind that the notions regarding
generation which prevailed in the early Greek age, although vastly
more correct than many of the fancies of the Middle Ages, were yet
in certain points defective and even erroneous. Consequently the
teratogenic theories founded upon them could not be free from
mistakes. Such as they were, however, they held their ground for
something like two thousand years, and even now their influence is
to be felt in many of the doctrines of the day.

In order to understand these theories it is necessary to remember
the views concerning reproduction which were held by the early
Greek writers ; these may be indicated in a few words. Since the
invention of the microscope was still in the distant future, nothing
was known of the existence of human ova and spermatozoa. The
evident facts of the generative process were the seminal discharge in
the male and the menses in the female. Some writers regarded the
semen as the sole, or at least the principal, factor in reproduction ;

THE NATURAL IN TERATOGENESIS 95

according to them the woman was nothing save a temporary abode
for the foetus. Others were of opinion that the menses supplied the
material for generation, and that the male semen was of compara-
tively little importance. Others, again, saw that both semen and
menses were essential factors ; and in words which have been ascribed
to Aristotle, " The blood of the menses is the marble, the semen is
the sculptor, and the foetus is the statue." Somewhat similar views
prevailed amongst the Eomans. Winy, for instance, in speaking of
the menses says — I use the words of Holland's translation: — "In
very deed it" (i.e. the menses) "is the materiall substance of genera-
tion : and the man's seed serveth instead of a runnet to gather it
round into a curd ; which afterwards in processe of time quickeneth
and groweth to the forme of a bodie."

The menstrual blood seems sometimes to have been called the
female semen, and the sex of the foetus was supposed to be due to
the greater strength of one of the two seeds. Another notion was
that boys were generated in the right testicle and girls in the left ;
and yet another was that when the semen was deposited in the left side
of the uterus a female embryo resulted, when in the right side a male.

Mention, also, must here be made of another belief concerning
generation which had its origin far back in the history of the world,
and in illustration of which many examples may be found in Greek
and Roman mythology. I refer to the supposed fertility of animals
of different kinds with one another, and even with man. Aristotle,
even, believed in this view to a certain but small extent, and later
authors accepted the theory in its widest sense, and with its most
ludicrous developments.

The theories of the Greeks passed in process of time to the
Romans. During the Dark Ages in Europe they were cherished by
the physicians of Arabia, and so in process of time they came to
be the views of the nations of Western Europe, when the revival
of learning put an end to the epoch of intellectual barrenness. In
many ways, however, the original lucidity of the classic theories
was obscured during the long period which elapsed between the
times of Hippocrates and Aristotle, and those of Pare and Licetus ;
and metaphysical notions abounded in the writings of the Middle
Ages, greatly to the detriment of the purely physical notions of the
Greeks.

The teratogenic theories of the past which looked for physical
causes of monstrosities were based upon the study of the phenomena
of generation as these were then understood ; they were, in fact,
deductions from them. Further, the various phases of the ideas con-
cerning reproduction were all reproduced in the theories of the causes
of teratological productions. Those writers, for instance, who re-
garded the male semen as the sole, or at any rate as the chief factor
in generation, ascribed teratogenic properties to alterations in its
quantity or quality. Others, considering the menstrual discharge as
of prime importance in reproduction, looked to it as the cause of
monstrous births. These two primary theories did not, however,
long exist separate, for it soon came to be recognised that both

96 ANTENATAL PATHOLOGY AND HYGIENE

parents might supply teratogenic factors ; thus a sort of coalition
hypothesis, in which now the seminal and again the menstrual
element would predominate, came into being and long persisted, —
persists in a certain sense still.

Alongside of these theories was constantly to be found that other
which regarded teratological productions as the result of intercourse
between animals of different kinds and between man and the brutes.
This, also, as will be at once seen, was the direct outcome of the old
notion of fertility between different genera.

A. Seminal and Menstrual Causes.

The theory that monstrous infants are the result of unusual con-
ditions of the paternal element in generation — the male semen — is a
very old one ; it seems first to have been expressed by Empedocles of
Agrigentum, in Sicily, a physicist living between the years 495 and
435 B.C. He has been called the "father of the evolution idea"
(Osborn, From the Greeks to Darwin, p. 37, 1894), and seems to have
had some knowledge of embryology and teratology. Empedocles,
like Lucretius some four centuries later, recognised what may be
called a teratological stage in the world's history when animals
appeared, spontaneously generated, as incomplete individuals —
" heads without necks, arms without shoulders, eyes without their
sockets " — and when through the fortuitous union of these parts with
one another all sorts of monstrous forms came into being, soon, how-
ever, to become extinct through the absence of reproductive powers.
But it is rather with the views that this philosopher advanced to ex-
plain the origin of monstrosities, after Nature had succeeded in pro-
ducing normal permanent animal types, that we have here to do. As
Plutarch (De pleicit. philos., lib. v. cap. 8) relates, Empedocles thought
that monsters happened either from abundance or from defect of the
semen, from slowness of movement, from division of it into several
parts, or from aberration of the movement. Although five states are
here enumerated, only three ideas really exist, these being the notions
of excess, of defect, and of alteration of the semen. Curiously enough,
these are precisely the ideas which underlie recent views, not, indeed,
of the causation of teratological products, but of their classification.
For the primary division of monstrosities into monstra per execssum,
monstra per defectum, and ■monstra. per fabricam alienam, which
Forster employed, or into monstra deficientia, monstra. abundantia,
and monstra sensu strictiore deformia, as Otto recommended, cannot
yet be said to have been improved upon. So in a certain sense it
may be said that the views advanced so long ago by the Sicilian
physicist live still ; and there can be no doubt that they were widely
accepted by Greek, Roman, and mediaeval authors.

The teratogenic theories of Democritus of Abdera, another physi-
cist of the same age as Empedocles, did not take a very firm hold
upon the philosophic mind. As we are told by Albertus Magnus
{De animalibus, lib. xviii. c. 6), Democritus thought that double
monsters were generated when the semen belonging to one or to two

THE NATURAL IN TERATOGENESIS 97

species was introduced into the uterus repeatedly at intervals; to
begin with, the first semen reached the womb and sufficed to form
the new being ; soon thereafter the second semen arrived, mixed with
the first, and began to operate in its turn ; and so the members came
to be duplicated. The abuse of coitus was thus made out to be a
teratogenic factor.

Neither Empedocles nor Democritus ascribed to the female any
formative power in the matter of generation ; Aristotle, on the other
hand, did so ; and, in consequence of this, his theory of teratogenesis
included faults in the female element in reproduction as well as in
the semen. The Aristotelian hypothesis, therefore, was one which
recognised a dual cause, and as such it will fall to be discussed im-
mediately ; in the meantime it may be said that through its weighty
authority the case of the theories which looked for solely a seminal
or solely a menstrual teratogenic factor was greatly weakened.
Nevertheless Galen, living in the second century of the Christian
era, seems to have believed in a purely seminal theory, for he thought
that hermaphrodites were due to the entrance into the uterus of
spermatic fluid from both the testicles.

The Arabian physicians during the Dark Ages in Europe served
to perpetuate the doctrines of the past, and prominently those of
Aristotle ; but Avicenna, like Galen, sought for an explanation of the
origin of hermaphrodites along the lines of the older theories, for he
believed that whilst semen deposited in the left side of the uterus
resulted in the birth of a girl, and in the right side in that of a boy,
the male element when placed in the middle of the cavity gave rise
to the procreation of a hermaphrodite.

In Europe in the thirteenth and fourteenth centuries certain con-
tributions were made to the study of the causes of monstrosities ; but
the question was greatly complicated by the introduction of the terms
" materia " or " material " instead of semen, and by the supposition
that this materia had a formative virtue or faculty. In this way
confusion was the result, for " materia " came to be applied to the
menses as well as the semen, and the " formative faculty " was an
idea which encouraged all sorts of profitless theorisings. To some of
these hypotheses allusion will be made immediately when the causa-
tion of monstrosities through faults in both male and female elements
in generation is under consideration. Here it may simply be said
that the theory of Empedocles never again came to be accepted fully,
although the discovery by Leuwenhcek (in 1677) of spermatic
animalcules in the semen gave a temporary support to the notion
that after all the male element might be the chief, if not the sole
teratogenic factor. With the discussion of the influence of the
spermatozoa in producing monstrosities we have not at present to do ;
that is a matter to be considered along with the theories of the
present, not with those of the past.

The idea that the menses play an important part in the generation

of monsters seems largely to have been the result of the Hebrew

legislation regarding coitus during or immediately after the flow. In

a non-canonical book (Esdras, Book iv. c. 5, verse 8), the following

vol. n. — 7

98 ANTENATAL PATHOLOGY AND HYGIENE

statement occurs : — " Menstruous women shall bring forth monsters "
(mulieres menstruates parient monstra); and in the Talmud the infant
conceived during the period of impurity was regarded as certain to be
an epileptic, a cretin, a drunkard, or insane. At the same time in-
dications of a similar belief are to be found in the records of classic
mythology, for it is said of Vulcan, who was so deformed at his birth
that his mother in disgust threw him into the sea, that he was be-
gotten by Jupiter when Juno was menstruating. Witkowski {Histoirc
des Accouche mi iits, p. 335, Paris, 1887) refers also to this belief, which
he regards as a very common one, and quotes the following lines in
which it is embodied in verse : —

" Femmes qui desirez de la progeniture,
Durant le cours des mois respectez la nature ;
Le fils de Jupiter, Vulcain, ainsi concu,
Vint an monde impotent, cul-de-jatte et -liossu."

Aristotle and his followers did not admit the pre-eminence of the
menses in teratogenesis ; the menstrual blood or materia was only
one factor in normal generation, and therefore also in abnormal
generation. Nevertheless there were some who regarded the men-
strual blood as of greater importance than the semen in the causation
of monstrosities; thus Henricus of Saxony (Dc sccrctis mulierum,c. 6,
1478), a pupil of Albertus Magnus, regarded teratological phenomena
as due to deficiency or excess of the materia or menses. Pare {op. cit.),
also, amongst the numerous causes of monsters, places intercourse
between parents at such times as they ought to forbear by the
command of God and the Church. Licetus, writing in 1616,
mentions the menstrual flow at the time of conception as the seventh
cause of the origin of shapeless monstrosities, and founds his belief
on the passage in Esdras already referred to, and on a quotation from
Solinus.

When it is borne in mind that Pliny and many of the writers of
the Middle Ages attributed to menstrual blood the most maleficent
powers as regards vegetables, metals, glass, trees, and even animals, it
is not to be wondered at that numbers of authors found in it one at
least of the causes of deformed births. Towards the close of the
seventeenth century, however, discoveries were made which revealed
the hollowness of such beliefs ; and it was from the investigations of
Harvey and de Graaf, and from the light which they threw upon the
true nature of the female factor in reproduction, that the menstrual
theory of teratogenesis received its deathblow. We know now that
anomalies in the female factor are frequently causes of monstrosities,
but that factor is no longer the menstrual blood but the ovum.

B. Seminal-Menstrual Causes.

The theory which regarded alterations in both the male and female
elements in generation as the cause of monstrous forms may rightly be
called the Aristotelian. Aristotle (384 to 322 B.C.), by means of his
great intellect and strictly scientific methods of research by experi-

ARISTOTLE'S VIEWS 99

ment and induction, placed all the workers who followed him in the
position of debtors. Even in the subject of teratology his views were
far in advance of his time. His definition of a monstrosity (Be genera-
tionc animalium, lib. iv. c. 3 and 4) has already been stated (p. 80) ;
it revealed an extraordinary degree of critical insight. Aristotle, also,
refuted the opinion of Democritus concerning the origin of monstrosi-
ties ; neither did he adhere to the theory of Empedocles, for he
believed in the conjoint action of perverted states of both sperm and
germ. He recognised the teratogenic power of faults in the materia
which exists in the menstrual blood, and pointed out the more fre-
quent occurrence of terata in the animals that produce many young
ones. He was truly the founder of both human and comparative
teratology, and as regards the latter subject his knowledge of the
embryonic origin of double monsters in the hen's egg was really
wonderful. Had he known that man also originates in an ovum, it
is more than probable that he would have applied the facts of terato-
genesis in the chick to human teratology, and so have discovered in
the fourth century B.C. what has been reserved for the savants of
the nineteenth century a.d.

The doctrines of Aristotle were handed down by succeeding
writers, Roman and Arabian, to the times of the Middle Ages in
Europe, when Albertus Magnus, the Dominican monk (1193 to 1280
a.d.), accepted, but" with modifications, the views of the Greeks con-
cerning the production of monstrosities. The modifications were
most unfortunate, and were destined greatly to obscure the original
theories. Thus, Albertus admitted the teratogenic powers of the
semen, but he used the word materia as a synonym; those who
followed him lost sight of the real meaning of the term, and thus great
confusion was introduced. Again, Albertus ascribed to the stars the
same powers in the production of monstrosities as he gave to the
materia, and so lent his sanction to the supernatural notions of his
time. He also believed with Aristotle that terata were less frequent
in uniparous than in pluriparous animals. His disciple, Henricus
of Saxony, held views to which reference has been already made.
He seems to have regarded the materia as the menses, and he
thought that all the causes of monstrosities might be reduced to
two : disobedience of the materia, and its insufficiency. He in-
troduced, also, such secondary causes as faults in the uterus pre-
venting the retention of the semen, and the position of the body
during coitus.

Thomas Aquinas, another Dominican of about the same period
(1225 to 1274 a.d.), introduced the idea of a formative power or
faculty which acted upon the semen ; and, like Albertus Magnus, he
ascribed teratogenic properties to the heavenly bodies. With him
indisposition of the materia was the immediate, and the effect of the
stars the remote factor in teratology. Pietro di Argelata added the
notion of a formative power or faculty to the doctrine of the Greeks,
for in writing concerning supernumerary and large and small digits,
he admitted that they were caused by excess or defect of the materia,
but only if these were combined with a strong or a weak formative

100 ANTENATAL PATHOLOGY AND HYGIENE

power. The last-named factor was the more important; indeed, with
only a small amount of materia, a large part might be produced if the
formative faculty were sufficiently great. Possibly in such a case the
formative power made the materia more obedient to movement and
more capable of extension.

In the early years of the sixteenth century ample evidence was
forthcoming in the writings of the time to show how confused the
original Greek theories had become. Bonacioli (Enneas muliebris, c.
ix.), an author of this age, seems to have returned to the views of
Empedocles with regard to the formation of fcetuses with super-
numerary parts, hermaphrodites, and monstrosities showing defects ;
he also complicated matters by referring to a materia supplied by the
woman as well as by the man. About the middle of this century
Savonarola {Practica major, tr. vi. c. xxi. f. 269, Venetiis, 1559) wrote
at some length on the generation of moles from the semen and
menses, reproducing largely the opinions of Avicenna ; he stated that
some women, from impurity of the blood, gave birth not only to a
true fcetus, but also to a piece of flesh resembling an animal, e.g., a
toad, falcon, lizard, etc. ; this was due to an error in the second
semen, and this error was the result of stellar influence. Varchi
(Lczioni sopra la generazione dei mostri, p. 101, Firenze, 1560), whilst
he believed that the two principal causes of monstrosities were faults
(qualitative and quantitative) in the male semen and in the menses
of the woman, also recognised others, such as the state of the uterus
and the secundines, the imagination, and the effect of the heavenly
bodies. The last-named cause was introduced so as not to contradict
the theologians.

Towards the close of the sixteenth and beginning of the seven-
teenth century the tendency, already seen in Varchi's writings,
towards a multiplication of the causes of monstrosities became very
evident. Pare, for instance, enumerated eleven different teratogenic
factors, and Licetus gave even a larger number for . selection ; but
both these writers gave a very prominent place to faults in the
material ; thus the third and fourth causes of Pare were, " abundance
of seed and overflowing matter, and the same in too little quantity
and deficient " ; whilst Licetus referred to such conditions as " defect
of proper material," " inaptitude of the material," " feebleness of the
formative or separative faculty," and the like.

It is unnecessary to quote further from the writers of this cen-
tury (the seventeenth), for in all of them the same ideas are repro-
duced,— often, however, with a great deal of obscurity, and not
uncommonly in language which now has to us little or no meaning
at all. The embryological discoveries of the second half of the
century {e.g. those of Harvey) so altered the notions of generation
which had prevailed that the old teratogenic theories founded upon
them fell to the ground. As we shall see later, their place was taken
by new hypotheses, which in not a few instances embodied the
notions of the ancients ; and it came to pass that instead of talking
of excess or defect or alteration of the semen, the menses, and the
materia, teratologists began to theorise regarding faults in the

HYBRIDITY IN TERATOGENESIS 101

spermatozoa, in the ovum, or in the product of their union, the
embryo.

C. Hybridity as a Teratogenic Cause.

The theory which has now to be considered might quite well have
been discussed with the preceding, for it has to do with conditions
affecting the male and female elements in generation ; but it has at
the same time very characteristic features of its own, which serve
specially to differentiate it. It may be termed the hybridity theory
of teratogenesis, and it is founded upon the notion that animals of
different kinds may be fertile with one another ; the products of such
unions are, however, monstrous. It was only an expansion of this
doctrine to believe that deformed infants resulted from the cohabita-
tion of the human subject (male or female) with one of the lower
animals.

" That which followeth is a horrid thing to be spoken ; but the
chaste mind of the Reader will give me pardon, and conceive that,
which not onely the Stoicks, but all Philosophers, who are busied
about the search of the causes of things must hold, That there is
nothing obscene or filthy to be spoken." Thus wrote Pare (Johnson's
Translation, p. 599, London, 1678) some three hundred years ago in
his chapter entitled, " Of monsters by the confusion of Seed of divers
kinds " ; and although we must agree with him in theory, yet in
practice it will be wise to skim lightly over the surface of the quag-
mire of offensive and unprofitable imaginings which has collected
round the central notion of the teratogenic fertility of different kinds
of animals. We do not wish to be miserably quagmired among these
teratogenic theories of the past ; we desire to reach as soon as possible
the comparatively solid land on which have been reared the theories
of the present. Let us skirt the quaking bog.

The finding of an anencephalic human foetus (Geoffroy Saint-
Hilaire, Ann. d. sc, nat., vii. 357, 1826) preserved as a mummy in a
sarcophagus reserved for sacred animals at Hermopolis, on the Nile,
proves that in ancient Egypt the bestial origin of monstrosities was a
common belief. The anencephalic foetus had evidently been regarded
as a monkey, an animal sacred in the eyes of the Egyptians, and
having been the offspring of a woman, its conception had, doubtless,
been ascribed to an act of unnatural intercourse. Possibly the same
views held also in ancient Chaldea ; at any rate, the cuneiform
tablets of Nineveh speak of a ewe giving birth to a lion, etc. (Terato-
logia, i. 139, 1894). It is easy to understand how such beliefs arose
when it is remembered that, at the time, all kinds of animals, in-
cluding man, were supposed to be fertile with one another, and that
the male was regarded as the sole factor in generation.

In the mythology of nearly all the races of mankind traces are to
be found of similar notions. In the legends of the Greeks and
Romans, for instance, mention is often made of the Minotaur,
" semibovemque virum, semivirumque bovem " ; of Echidna and
Chimaera, offspring of Medusa ; of Chiron the Centaur, half a man and

102 ANTENATAL PATHOLOGY AND HYGIENE

half a horse ; of Castor and Pollux ; and of many others. But the
conception of half-human monstrosities was not an attribute of the
gods of Olympus only ; it was a faculty shared in by the humblest of
mortals, as is witnessed by the tale which Plutarch (Berger de
Xivrey, Traditions Uratologigues, p. 34, Paris, 1836) tells of Periander's
shepherd and the centaur-like offspring of a mare. This same writer
attributed to Aristotle the story of the beautiful daughter of Aristo-
nymus of Ephesus, whose name was Onoscellis, which name suggests
clearly her asinine origin.

This reference leads us naturally to the consideration of Aristotle's
views upon the subject of hybridism. He (De generatione animcdium,
lib. ii. c. 12) held that animals were only fertile with each other
when the duration of their gestation-period was the same and when
their size was similar. The offspring would resemble either of the
parents. The case of the fox and the dog was cited, and Aristotle
elsewhere (De animalibus hist., lib. viii. c. 27) spoke of the products
of the intercourse of heterogeneous animals in Libya, Cyrene, and
Laconia. In this relation, as in so many others, Aristotle's more
correct views were soon lost; and Pliny (Holland's Translation, i.
p. 157, London, 1601), for instance, speaks of a bondwoman who
brought forth a serpent, and of Alcippe, who was delivered of an
elephant, " marie that was a monstrous and prodigious token, and
foreshewed some heavie fortune that followed after."

It is easy to imagine how such beliefs as those mentioned above
grew and flourished in the period of intellectual darkness in Europe.
Many traces of this are to be found in the writings of Vincent de
Beauvais, of Bartholin, of Lycosthenes, of Rueff, of Sir Thomas
Browne (Religio medici), and others. C. Stalpart vander Wiel, for
instance, in his Hondert seldzame Aanmerkingcn, pp. 234 and 248,
Amsterdam, 1682, not only relates how a woman gave birth to a
little dog (" Hondeken van een Vrouw geboren "), but even figures the
prodigy, which seems really to have been a human otocephalic foetus
without a lower jaw.

With the revival of learning came attempts, not very active at
first, to controvert the doctrine of the bestial origin of monstrosities,
and the ancient views of Aristotle came again to the front, that
animals could only be fertile with those of the same size whose
period of utero-gestation was approximately of equal length. Martin
del Rio (Disquisit. magicarum, lib. ii. qusest. 14, p. 331, Lovani,
1598-1600), a writer of the early part of the seventeenth century,
while declaring that physically a cow could not give birth to a
human foetus, was yet of opinion that something of this kind could
happen through a diabolic agency. Licetus (De monstris, lib. ii.
c. 68 ct seq. pp. 213-231, 1668), however, in five chapters in the
second book of his work, opposed the doctrines of Del Eio,
and endeavoured by copious references to cases, and by analogy
with the vegetable kingdom, to prove that such unnatural unions
may not only result in the birth of a human foetus or an animal, but
also in the production of monstrous forms partaking of the characters
of both. Neither the legal nor the theological side of this matter

HYBRIDITY IN TERATOGEXESIS 103

were neglected by the writers of this century, and the question
whether such offspring ought to be baptized caused much discussion.

Even near the close of the seventeenth century evidence is not
wanting to show how persistent wTas the belief in this mode of origin
of monstrosities ; for instance, Paullinus (Miscell. curios, mcd. phys.,
Dec. ii. Ann. 6 ; Appendix, obs. xli. p. 48, Norimbergse, 1688), in
1688, gravely writes of a"Monstrum canino-humanum cum flamma
et fragore natum " ; and Bartholin tells of a " puella ex cane nata "
(Hixt. anat, Cent. V., p. 166, 1661). It is true that Ambrosinus,
in his edition of Aldrovandus' Historia Monstrorum (p. 385, Bononiae,
1642), had brought in other factors, such as the influence of the
maternal imagination, to explain such births ; but his suggestions do
not seem to have been taken up very widely.

It is not till the beginning of the eighteenth century that we find
a strong protest being made against the belief in the hybridity theory
of teratogenesis. Bianchi (Dc naturali in hurnano corpore, vitiosa
morbosaque (jcncrationc historia, pt. ii. p. 238, 1741), of Turin, was
amongst the first to point out that when the product of human
gestation had a rough resemblance to an animal-form this resem-
blance might be due to a foetal disease, or the product itself might be
a mole or retained portion of the placenta. Even at the present day,
however, there still exists a strong popular belief in the old theory,
and even in the ranks of the profession I have met with its adherents.

Now, there can be no doubt that the cases which gave rise to this
belief and kept it in existence were those in which disease or de-
formity gave to the human foetus an animal appearance, or in which
the young of one of the lower animals, through the existence of such
a condition as hydrocephalus, came to bear a rough resemblance to the
human form. At the same time, the theory has a modern and
scientific side, of which a great deal may yet be heard. To quote
from such a skilled teratologist as Mathias Duval (in Bouchard's
TraitS dc pathologie gdnSrale, i. 159, 1895), writing as recently as 1895,
hybridity is a phenomenon of which little is known in the higher
forms of animal life, but some day its discussion may form an
important chapter in teratology ; for it is quite possible that sterility
between animals of different species may be due, not to an absence of
impregnation, but to such an abnormal development subsequent to
impregnation as to cause arrest by its very abnormality. In support
of this it has to be noted that Guyer, in an article on the " sperma-
togenesis of normal and hybrid pigeons" {University of Cincinnati
Bulletin, No. 22, s. 2, iii. p. 1, 1900), states that he found marked
abnormalities in the spermatozoa and germ cells in the pigeons that
were crosses between very divergent species ; these birds were sterile ;
in the fertile birds the only changes found were anomalies in mitosis
in the cells.

Such, then, were the theories of the past, which regarded purely
physical conditions as the causes of monstrosities ; and with regard to
them all it must be noted that the ideas underlying them exist still,
but in an altered form, and expressed in different words. It will
have been noticed also that there exists a much closer connection

104 ANTENATAL PATHOLOGY AND HYGIENE

between these theories of the past in their earliest form and the views
of the present, than between the latter and the notions of the Middle
Ages. The theories of Empedocles and Aristotle are, in other words,
more nearly allied to those now in vogue than are the latter to those
of Lycosthenes, Licetus, and Aldrovandus. I have regarded the dis-
covery by means of the microscope of the human ova and spermatozoa
as the boundary line between what are called theories of the past and
those which I shall have soon to describe and designate theories of
the present ; but there can be no hard-and-fast dividing line in this
matter, and it becomes an interesting subject for speculation to
consider in what way the knowledge obtained from the use of the
microscope might have influenced the teratogenic views of the early
Greek philosophers.

I have now passed in review both the supernatural and the
physical teratogenic theories of the past ; and, before proceeding to the
discussion of another old yet ever new theory, — that, namely, of the
effect of the imagination of the parents upon the infant in utero,— it
must be pointed out that these hypotheses have not followed one
another in chronological order, but have existed, more or less side by
side, and have even overlapped. For example, a belief in the terato-
genic powers of the stars has been seen combating the notion of the
hybrid origin of malformed infants, and this in its turn has been
confronted with the view that the powers of evil are the potent
factors in teratogenesis. At the same time, it is easy to understand
how in certain epochs of the world's history certain opinions have
been more widely held and more strongly insisted upon than in
others.

CHAPTER IX

Teratogenesis (cont.) : Theories of the Past (font.) : Mental Impressions as a
Cause of Monstrosities : History of the Theory among the Hehrews, the
Ancient Greeks, the Eomans, Jews, and Christian Fathers ; in the Middle
Ages ; in the Sixteenth Century ; in the Seventeenth, Eighteenth, and Nine-
teenth Centuries ; at the Present Time : Summary.

Theories of the Past {continued).

III. THE MENTAL.

Doubtless some modern writers may be inclined to cavil at the
position which is here assigned to the theory of the influence of the
maternal imagination in teratogenesis, for it is placed with the notions
of the past, and not with those of the present. But I think that the
arrangement is warranted, for it must be admitted that the theory of
maternal (or paternal) mental influence as originally enunciated is
indefensible. At the same time, the belief has a modern side, in-
finitely less repulsive to the scientific sense of inquiry ; this side has,
I believe, a just claim to credit. The hypothesis as originally stated
is, I humbly think, untenable ; it is truly a theory of the past. In its
modern form, and with certain limitations to be referred to later, it is
both tenable and credible ; in this sense it can be properly termed a
theory of the present. Still, when reference is made to the maternal
impression theory in teratogenesis, probably there is scarcely any one
who thinks of it in any sense save in that old and extravagant one
which demands belief in an absolute similarity between the thing
producing the impression and the defect or anomaly resulting there-
from. In its generally accepted and classic sense, therefore, the
hypothesis is of the past ; but, like others of these ancient doctrines,
it has its less widely known but vastly more reasonable modern
development. In accordance with these circumstances, it has been
placed among the theories of the past, but in juxtaposition with those
of the present.

As will be made abundantly evident, when the history of the
belief in maternal impressions comes under consideration, the notion
is one of great antiquity ; but it may here be pointed out that it is
also one of practically world-wide distribution. Ploss (Das Weib,
2nd edit., i. 505, 1887) has succeeded in gathering together indica-
tions of its existence in such far apart lands as India, China, South
America, Western Asia, and East Africa: and I have elsewhere (The

105

106 ANTENATAL PATHOLOGY AND HYGIENE

Diseases of the Foetus, i. pp. 30, 31, 41, 1892) quoted from various
works to show that maternal impressions are not unknown among
the Esquimaux, the Loango negroes, and the old Japanese. It is
also, of course, the common possession of the nations of Europe. In
fact, it may safely be postulated that the belief in the potency of
maternal impressions has a geographical distribution corresponding
with that of the human race, whilst in the matter of antiquity it
is coeval with it.

It will now be well to trace so far as is possible the origin and
growth of this ancient and well-nigh universal doctrine. In this way
it will be learned that in its earliest beginnings it was not specially a
teratogenic theory, but rather one used to explain how the young of
men and of animals came to be of special colours, and especially of a
tint differing from that of their parents ; then it was accepted as
the solution of the origin of mother's marks or nsevi ; then as a
possible explanation of the birth of infants with hypertrichosis ;
and, finally, it came to be applied, and in most ingenious ways,
to account for almost every kind of anomaly, malformation, and
monstrosity. The story will be found to be one of no little
interest, and at times amusing.

Among the Hebrews.

Writers have generally commenced the history of maternal
impressions with a reference to the patriarch Jacob and his triple
artifice, by means of which he acquired a large number of speckled
cattle of a vigorous breed. The sacred narrative tells (Genesis xxx.)
how Jacob first placed " rods of fresh poplar, and of the almond, and
of the plane tree," in which he had " peeled white strakes," before the
flocks when they conceived at the water-troughs, " and the flocks
brought forth ring-straked, speckled, and spotted"; having thus
obtained some parti-coloured lambs, he put them (instead of the rods)
before the flocks, and thus increased the number of such animals still
more ; and, finally, in order to get as vigorous as possible a breed
of the parti-coloured, he put the peeled rods before only the stronger
of the flock. " And the man increased exceedingly, and had much
cattle, and maid-servants, and men-servants, and camels, and asses."
From this it may be learned that Jacob was an expert stock-raiser
well versed in all the notions of the age, which included a belief in the
efficacy of maternal impressions upon the offspring at the time of
conception. To attempt, as many have done, to use the narrative as
a proof of the correctness of this ancient theory is manifestly unfair,
for throughout the whole passage there is the thinly veiled suggestion
of an overruling Providence acting favourably to Jacob's plans.

In Ancient Greece.

Traces of this belief are also to be found in certain of the Greek
writers, and Plutarch (De placit. philos., lib. v. c. 12; cited by
T. Fienus in his De virions imaginationis, p. 217, Lugd. Batav., 1635)

MATERNAL IMPRESSIONS 107

states that Empedocles had remarked that women gave birth to
infants resembling the statues which they had found pleasure in
regarding during pregnancy. Allusions to the effect of pictures and
statues seen by women at the time of conception or during gestation
are to be found also in the law of Lycurgua, which required the
Spartan wives to look upon the representations of the strong and
beautiful, e.g. statues of Castor and Pollux; and in the story of
Dionysius of Syracuse, who hung the picture of Jason in full view of
his pregnant spouse (T. Fienus, loc. tit.). There is, however, no
absolute proof that Hippocrates shared in these beliefs, for the story
which St. Jerome and others tell concerning the use he made of this
notion in the defence of a woman accused of adultery is not to lie
found in the Hippocratic writings (Ballantyne, " Antenatal Pathology
and Heredity in the Hippocratic "Writings," Teratologic/,, ii. 279,1895).
Further, the statement that " if a pregnant woman has a longing
to eat earth and coals, and eats of them, the infant which is born
carries on its head the mark" of these things," occurs in the treatise
De Superfcetatione, which practically all commentators regard as
not the work of the Father of Medicine himself.

Among the Eomans, Jews, and Christian Fathers.

The following quotation from Pliirvjs Natural History (Holland's
translation, p. 161) demonstrates Tahat among the Eomans a belief
in the efficacy of both the maternal and the paternal mind in the
moulding of the foetus was prevalent. The writer speaks of the
black child of a white mother, and then goes on to say : " Certes
the cogitations and discourses of the mind make much for these
similitudes and resemblances whereof wee speake : and so likewise
many other accidents and occurrent objects are thought to bee very
strong and effectual therein, whether they come by sight, hearing,
and calling to remembrance ; or imaginations onely received, and
deeply apprehended in the very act of generation, or the instant of
conception. The wandering cogitation also and quicke spirit either
of father or mother, Hying too and fro all on a suddaine, from one
thing to another, at the same time, is supposed to bee one cause of
this impression, that maketh either the foresaid uniforme likenesse,
■or confusion and varietie." For this reason Pliny thinks that men
are more unlike one another than other creatures : " For the nimble
motions of the spirit, the quicke thoughts, the agilitie of the mind,
the varietie of discourse in our wits, imprinteth diverse formes, and
many markes of sundrie cogitations ; whereas the imaginate facultie
of other living creatures is unmoveable, and alwaies continueth in
one, in all it is alike, and the same still in everyone, which causeth
them alwaies to engender like to themselves, each one in their severall
kind."

Galen (De theriaca ad Pisonem liber, c. xi.), also, believed in the
power of the maternal imagination when influenced by pictures, etc. ;
and So.ran.us of Ephesus (Usp! yuwizsiw <raduv, c. x.) spoke of the ape-
like children which were born to women who had looked at monkeys

108 ANTENATAL PATHOLOGY AND HYGIENE

at the time of conception. Oppian (Kvusyzrixw, lib. i. v. 358), in his
Cyncgcticon, gave some interesting details concerning the application
of the belief in the breeding of horses and doves of particular colours.
Such statements, however, were not confined to what may be called
the scientific literature of the age; for in the well-known romance of
Heliodorus (sEfhiopica, lib. iv. c. x.), the belief is adduced to explain
the colour of Chariclea, the white daughter of the black king and
queen of Ethiopia, the queen having regarded a statue of Andromeda
at the time of impregnation. It may be mentioned that Quintilian,
in his Inztitutiones Oratoricce, referred to a similar tale, only in this
instance the parents were white and the infant black, and the cause
was the picture of a Moor.

In the Talmud (for Talmudic references, vide J. Preuss, Berlin.
Klinik, Hft. 51, 1892), also, such stories are repeated, and there is an
indication of a belief in the potency of impressions made in pregnancy
after conception. The early Fathers of the Christian Church held
similar opinions. St. Jerome thought there was nothing strange in
the infant bearing the impress of things seen or imagined at the
moment of conception ; and St. Augustine, in his Quccstiones in
Genesim, referred to the biblical narrative of Jacob and his parti-
coloured lambs.

In the Middle Ages.

Between the fifth and the fifteenth centuries little is recorded of
the belief in maternal impressions, although it cannot be doubted
that it existed. It would seem that Marcus Damascenus gave origin
to the oft-repeated tale of the birth of a hirsute infant consequent
upon the mother regarding the picture of John the Baptist in his
hairy garment. St. Isidore, also, seems to have warned women against
looking at monkeys, cynocephali, etc., during pregnancy. The Arabian
physicians had little to say on the subject, but Avicenna laid stress
upon the belief that infants resembled in colour, etc., the things seen
by the parents at the time of conception ; and gave credence to the
ridiculous story of a hen sitting upon her eggs being frightened by a
kite, with the result that the chicks had heads like falcons.

From what has been said, it will have been gathered that prior to
the fifteenth century the notion of maternal impressions was a well-
established one ; but it is noteworthy that it does not seem to have
been much employed to explain monstrosities, unless indeed we
regard the abnormal colour of the skin, nrevi, and hypertrichosis as
teratological. Further, its efficacy, save at the moment of conception,
was not widely recognised ; indeed, there is only slight evidence to
prove that it was ever supposed to be active in later pregnancy. Its
existence, however, was admitted both in the human species and in
some of the lower animals, and we read over and over again of the
great influence of pictures and statues. The mental impression seems
most often to have been one of admiration ; rarely is the idea of fear
or disgust hinted at.

With the Eevival of Learning in Europe came a great extension

IMAGINATION IN TERATOGENESIS 109

of the doctrine of the influence of the maternal fancy (phantasia), or,
as it was often called, " imaginatio gravidarum," " Einbildungskraft,"
and (more popularly) " Versehen."

In the Sixteenth Century.

In a work on the " imagination," by Francisco Pico of Mirandola
{Liber de imagination*' , Venetiis, 1505), published in the early years
of the sixteenth century, no mention is made of any peculiar pro-
perties of the fancy in pregnant women ; and Taruffi {Storia dclla
Teratologia, i. 231, 1881) is of opinion that the omission indicated
that the author discredited the belief, not that he was unaware of it.
Whether this be so or not, Martin Luther, in his work on Genesis,
regarded the notion as a " res certa et conveniens cum doctrina medi-
corum " ; he also told a story about a pregnant woman who was
frightened by a mouse. Lycosthenes {Prodigiorum ac Ostcntorum
Chronicon, p. 445, Basilew, 1557) indicated the popular belief when
he related how in the year 1282 an infant was born with hair and
claws like a bear, and how the Pope of the time straightway ordered
the destruction of all pictures of bears in Rome. The child belonged
to the well-known family of the Ursini. It may be noted that this
story was frequently repeated by subsequent writers, who, however,
did not all see in it an instance of such an innocent cause as the
mother's imagination. Both Cardan {Opera omnia, "De sanitate
tuenda," lib. i. c. 9, 1663) and Porta (Magice natures, lib. ii. c. 23,
Neapoli, 1558) were firm believers in the theory that, if a pregnant
woman longed for anything and touched a part of her body, the foetus
would show a mark of the thing desired on the part corresponding
to that touched by the mother {chirapsy). They also ventured an
explanation to the effect that the spirit of the mother is united with
that of the infant, and the heat, which is the instrument of the spirit,
moves the blood (which is at once disturbed by the desire of the
mother) into the corresponding part in the foetus. Luiz Mercado
{De mulierum affectionibus, lib. iii. c. vii., Valladolid, 1579), a Spaniard,
had another theory of the modus operandi, for he thought that the
imagination disturbed the action of the semen of one of the parents,
and so led to the mark on the infant.

Lemnius {De miraculis occultis natural., lib. iv. c. 18, 1564), in his
interesting work, stated his preference for the doctrine of the maternal
imagination over that of stellar influence ; and Ambrose Pare {De
monstrcs, Paris, 1573), in 1573, gave it as the fifth cause of mon-
strosities, mentioning the illustrative instances of black and hairy
children already so often referred to. The latter writer also figured
an infant with the face of a frog, which Bellanger, " a man of an
acute wit," regarded as occasioned by the mother's holding a frog in
her hand just before conception. Pare went on to say (I quote from
Johnson's translation, p. 596) : " There are some who think the infant
once formed in the Womb, which is done at the utmost within two
and forty days after the conception, is in no clanger of the Mother's
imagination, neither of the seed of the Father which is cast into the

110 ANTENATAL PATHOLOGY AND HYGIENE

Womb, because when it hath got a perfect figure it cannot be altered
with any external form of things ; which whether it be true or no, is
not here to be inquired of : truly I think it best to keep the woman
all the time she goeth with child, from the sight of such shapes and
figures." Cautious Pare !

It is scarcely necessary to do more than mention some of the
other references to maternal impressions found in the literature of
the sixteenth century. Sebastian Munster (Cosmographie univcrscllc,
lib. iii., Paris, 1575), for instance, tells how two women, one of them
being pregnant, got their heads knocked together, and how in due
time twins united by the vertices were born to one of them. J. Rueff
(Dc conccptu ct gcncrationc Iwminis, lib. v. c. 3, p. 46, Frankforti ad M.,
1580) speaks of the grievous effects of terror induced by the sight of
hares and other animals, and says : "Ex appetitu rursum et terrori-
bus multi nascuntur, qui varias maculas et figuras corpori impressas
habeant, puta, crinium, murium, variorum colorum, racemorum,
flammarum, fructuum, rerumque aliarum." Jean Wier (Dc praistigli*
dcemonum, lib. iv. c. 18, Basilea;, 1563) and Cornelius Gemma (Cosmo-
critica, Antwerpke, 1575), and several others, participated in the
popular belief, which even found its way into the non-medical litera-
ture of the time : witness the story of black parents and a white
child in the twelfth canto of Tasso's " Jerusalem Delivered." This
was indeed the age of the wonderful, and nothing seems to have been
regarded as incredible. Profound indeed were the discussions regard-
ing the cutting of a golden tooth by an infant in Silesia, in which
Horstius (Dc aureo dcntc maxillari pueri Silcsii, 1595), Ingolstetter
(Dc aureo dente Silcsii pucri, 1596), and Euland (Dc dente aureo pueri
Silcsii, 1597) took part; and in Weinrichius' quaint work entitled
Dc ortu monstrorum commcntarius (c. 17, p. 158, Breske, 1595) we
read at great length concerning " to <pavraar/.ov " and its supposed
effects, about " fcetus rame facies," about " infans facie cadaverosa,"
and so on.

In the Seventeenth Centuky.

In the succeeding century (the seventeenth) the belief in the tera-
togenic powers of maternal impressions reigned supreme. Scarcely a
writer ventured to throw a doubt upon its accuracy, and the most
far-fetched explanations of monstrosities and deformities were eagerly
accepted by both the public and the profession. Credulity would
indeed seem to have reached a maximum when it began to be asserted
and believed that conception might occur through the imagination
alone. Of course, little was at this time known of the physiology of
generation and of intrauterine life, else such assertions could scarcely
have been entertained. Interesting instances illustrating this extra-
ordinary development of the imagination theory were reported by
Thomas Bartholin (Hist. anat. ct mcd. rar., Cent. V. and VI., hist., 61,
p. 296, Hafnise, 1661), G. C. Petrus (Miseett. curios, p>hys.-mcd., Ann.
ii. obs. ciii. p. 303, Jenae, 1671), and E. S. Grass (" Conceptus prodi-
giosus," Misccll. curios, phys.-med., Dec. ii. Ann. x. obs. lvi. p. 102,

IMAGINATION IN TERATOGENESIS 111

Norimbergae, 1692). In the first of these we read how Magdalena of
Auvermont gave birth to an infant although her husband had been
absent from her for four years ; and how the occurrence was ex-
plained by the fact that the lady had in her dreams imagined her
husband to be present ; and so the child (Emanuel) was conceived
" sola imaginationis virtute." After matrons and medical men had
given their evidence upon this matter, the Parliament of Grenoble
in 1637 declared the child to be the legitimate son and heir of
Hieronymus Augustus of Montleon, the husband of the aforesaid
Magdalena.

Even in this credulous age, however, reports of cases like the
above were rarities. Most authors were content to narrate how an
already existing foetus was altered ; few had the courage to describe
the creation of an embryo through the force of the imagination alone.
Certain of the records of the time may here be briefly referred to.

In 1605 Prolan (Be monstro nato Lull tin- anno Domini, 1605, "Dis-
put. philos.," Parisiis, 1605), in connection with a case of double foetus,
referred to the imagination theory, stating his preference for it over
the competitive notion of Satanic influence ; the birth of a child
resembling a demon was not due to the activity of an incubus, but
to the influence upon the mother of pictures of diabolic creatures.
Doubtless the women of that day were, or ought to have been, glad
to welcome this change in the teratogenic doctrines of the time, for
the substitution of the maternal-fancy theory for that of Satanic or
bestial intercourse rendered the birth of a malformed foetus a mis-
fortune, but not any longer a crime. Eiolan also believed that while
the imagination could alter the properties of the uterine contents,
it could not change the species. Fienus (Be virions imaginationis
tractatus, Qua'Stiones xiii. to xxiii., Louvain, 1608), in his treatise
on the powers of the imagination, devoted more than 150 pages to
the discussion of maternal impressions and the various questions
arising therefrom ; but that he did much to clear up these matters
can hardly be asserted. He related the story told by Philippus
Meurs about the child with a mussel for a head, who nevertheless
lived for eleven years, receiving licpiid nourishment from a spoon
into the gaping bivalve ; of course the mother had longed for sea-
mussels in her pregnancy. Concerning this extraordinary maid and
her still more wonderful death (through the breaking of one of the
shells), Fienus said " dico me non credere"; but he did not hesitate
to accept many other marvellous tales. Sir Thomas Browne, it may
be noted, does not discuss maternal impressions in his Vulgar
Errors.

Septalius (Be nccvis, Dordrechti, 1650) and Hildanus (Observ. et
cur. chirurg., Cent. III., obs. 56, Francofurti, 1646) also wrote upon
maternal impressions ; and Schenkius (Monstrorum historic/, memora-
bilis, Francofurti, 1609) and Bauhinus (Be herma/phroditorum mon-
strorumque partium natura, 1614) frequently adduced them to account
for various monstrosities. Licetus (Be monstrorum natura, etc., lib.
ii. c. ix., xi., xix., etc., Patavii, 1616), although holding more advanced
views on antenatal pathology, yet clung firmly to the theory of the

112 ANTENATAL PATHOLOGY AND HYGIENE

mother's imagination in teratogenesis ; but he restricted its power in
certain directions, and did not believe that mutilated monstrosities
and those showing excess of parts could be thus produced. He ad-
mitted the efficacy of the paternal imagination also (lib. ii. c. xlii.) ;
but this could not be so powerful as the maternal, for the time of its
action was limited to the venereal act.

It has been pointed out by Tarufri (Storta delta Teratologia, i. p.
234) that Zacchia (Qucestiones medico-legales, in quibus, etc., i. lib. vii.,
Eomse, 1621), writing in 1621, had indicated weak points in the
doctrine of the maternal imagination when he stated that if such
things were true, then there would be no infants without marks, and
some children would be spotted all over like leopards; but these
arguments seem to have fallen on deaf ears, a fate which also met
Santorelli's (Antepraxis medica, lib. ii. c. xiii. par. 35, Neapoli, 1651)
statement, made thirty years later, that monstrosities were born to
women who had experienced no impression whatever. Whilst, how-
ever, one is willing to give to these Italian observers credit for critical
views regarding the popular theory of their time, it must be confessed
that their notions found little or no support till many years had
passed. The writers who immediately followed held an almost un-
questioning faith in the potency of the mother's imagination ; and
reference may here be made to Aldrovandi's work (Monstrorum
historia, p. 384, Bononise, 1642) and to that of Stengelius (Be monstris
ct monstrosis quam mirabilis, etc., p. 179, Ingolstadii, 1647), in both of
which the matter is dealt with. Of course these authors recognised
not only this but also many other theories of teratogenesis.

Towards the close of the seventeenth century, observations on the
supposed results of the mother's fancy multiplied enormously. Thus
Kerkring (" Monstrum cacodiemonis picture, quam -humame figurae
similius," Spicilegium anatomicum, obs. xxiii. p. 56, 1670) puts on
record the birth of what was evidently an anencephalic foetus, but
whose appearance, taken in conjunction with the mother's history,
led the midwives to call it a devil — "diabolum non hominem esse
conclamitant muliercukc." He also told of a woman whose occiput
was fractured in her pregnancy ; the infant's head had no occiput.
Swammerdam (Uteri muliebris fabrica, p. 29, Leidae, 1672) opened up
new ground when he narrated how a clever woman counteracted her
own impression : she saw a negro in her pregnancy, and to prevent
her child being black she washed herself in warm water ; the matter
turned out well, for the infant (who, by the way, was born with teeth)
was white save between the fingers and toes and in the grooves on
the face, places which the mother in her washing had evidently
neglected.

One of the most frequently quoted stories of maternal influence
upon the foetus in utero is that taken from Malebranche's work
(Recherche etc la verity lib. ii. c 7, Paris, 1674), in which it is stated
that a pregnant woman after having seen a criminal broken on the
wheel, gave birth to an idiot boy, whose limbs were fractured. He
also wrote of a woman who from much gazing at a picture of St. Pius
was delivered of an infant closely resembling the saint. " 'Tis what

IMAGINATION IN TERATOGENESIS 113

all Paris may have seen as well as I, since it has been for a long time
preserved in spirits of wine." With regard to the first of these tales,
Malebranche had no difficulty in explaining all the phenomena to his
own satisfaction. " Children," said he (to quote from Blondel's trans-
lation), " see what their Mothers see, they hear the same Cries, they
receive the same Impressions of the Objects, and are moved by the
same Passions. All the Blows given to the Malefactor did violently
strike the Mother's Imagination, and, by a Counter-blow, the tender
and soft Brain of the Child. The Fibres of the Child's Brain, not
being able to resist the Torrent of the Spirits, were broken : That's
the Beason why he came into the World without Understanding.
The violent Course of the Mother's animal Spirits went, with Force,
from her Brains to the several Parts of her Body, which answered
to the Parts of the Malefactor. 'Tvvas the same in the Child, but
because the Bones of the Mother were capable to resist the Violence
of the Spirits, they were not wounded. Perhaps she did not feel the
least Pain ; but this rapid Stream of the Spirits was capable to carry
away the soft and tender Parts of the Bones of the Child." This sad
result would have been avoided had the mother known and practised
Malebranche's instructions — " si cette mere eut determine le mouve-
nient de ses esprits vers quelqu' autre partie de son corps en se
chatouillant au ce force, son infant n'auroit point eu les os rompus."

Doheus {Encyclopedia ch irurgica, Francofurti, 1689) attempted a
more physiological explanation of the modus operandi of maternal
impressions, and held that the image in the mind was involuntarily
communicated to the animal spirits, which impressed it upon the
foetus by means of the nerves of the uterus. The movements of the
heart and intestines proved that the animal spirits were capable of
involuntary motion. This theory was accepted by other writers,
among whom was Bandiera (" Delle voglie che s'imprimono nel feto,"
Galleria di Minerva, iii. pt. 4, p. 101, Venezia, 1695), who, however,
by bringing in the paternal imagination, which, of course, can only
act at the time of conception, rather stultified the notion, for there is
no nervous connection at that epoch.

The remaining contributions of this century may now be sum-
marised. Bayle (Dissertationes mcdiccc, Diss. iii. p. 116, 1678) dealt
with the matter of maternal impressions, and regarded it as an
endless task; Boyle {Exper. Philos., p. 151) repeated a tale about a
speckled child whose mother had (before pregnancy !) gazed long
and earnestly at some red pebble-stones near St. Winifred's Well, —
and yet his mind conceived Boyle's Law of the relation between
volume and pressure ! Diemerbroeck {Opera omnia, Anat., lib. i.
c. 28, p. 165&, Utrecht, 1685) was so sure of the theory that he
employed it in his scheme of heredity ; and Fidelis {De relationibus
medicorum, lib. iii. c. 4, p. 405, 1674) used it to explain why de-
formed parents had not always deformed children. In the pages of a
periodical called Zodiacus mcdico-gallicus, Nicolas de Blegny (i. pp.
61, 62, Geneva, 1680) and Cuchotius (v. p. 249, Geneva, 1685)
recorded further instances of maternal impressions. Finally, the
volumes of the Miscellanea euriosa are full of cases of foetal mon-

VOL. II. — 8

114 ANTENATAL PATHOLOGY AND HYGIENE

strosities ascribed to the influence of the mother's mind and ima-
gination. Amongst these may be mentioned the contributions of
Ludovici (Ann. iv. and v. obs. 208, 1676), Segerus (Ann. iv. and v.
obs. 147, 1676), Mercklinus (Ann. viii. obs. 46, 1678), Sommerus
(Dec. ii. Ann. i. p. 113, 1683), Albrecht (Dec. ii. Ann. vi. obs. 14,
1688), Fehrius (Dec. ii. Ann. vi. obs. 30, 1688) Gockelius (Dec. ii.
Ann. vi. obs. 126, 1688), Hoffmannus (Dec. ii. Ann. viii. p. 483,
1690), Lentilius (Dec. hi. Ann. ii. obs. 117, 1695), and Eeiselius
(Dec. ii. Ann. ii. p. 272, 1684).

In the Eighteenth Century.

In the seventeenth century the attitude of the profession towards
the doctrine of maternal impressions was one of blind credulity ;
but in the eighteenth this was quickly changed for one of sceptical
criticism. Of course, there were still many who in the latter cen-
tury believed in the efficacy of the mother's mind in moulding the
unborn infant ; but the prevailing opinion was one of grave doubt.
It would have been strange indeed if this had not been so, for the
discoveries of the close of the seventeenth century had done much
to clear up many of the vexed questions of reproduction and ante-
natal physiology.

In the early years of the century, criticism was not yet rife.
Diittel {vide Teratologic/,, i. p. 41, 1894), for instance, in his work on
foetal diseases, clearly stated his views : " So great," said he, " is the
sympathy between mothers and the foetuses which they carry in the
uterus that they suffer closely with them, to such an extent that not
only are the delicate fibrilla1 of the infants excited to anomalous
movements by every severe affection of the mind of the mother (e.g.
sorrow and anger), but, what is more, the formation of parts is alto-
gether prevented, disturbed, or vitiated by the strong imagination of
the mind." In the Miscellanea curiosa cases of maternal impression
continued to be reported ; and in England Daniel Turner (A Treatise
on Diseases incident to the Skin, pt. 1, c. xii., London, 1714) stated
very confidently his belief in such occurrences, for after enumerating
most of the cases to which reference has been made above, he said :
" Thus is it made apparent by a Multitude of Examples, how manifest
and great an Empire the Fantasy of a pregnant Woman lias over the
Blood and Humours together with the Spirits of her Body, and how
by their Ministry she is able to give not only monstrous Shapes and
Figures to that of the more tender Ftetus, but to communicate
Diseases also." He also related cases from his own practice of a
raspberry mark near the eyebrow, of a currant-like excrescence - on
the internal canthus of the eye, etc. In the year 1726 the matter of
maternal impressions was brought still more prominently before the
profession and the public in England in connection with the notorious
case of an " Extraordinary Delivery of Eabbets," which was alleged
to have occurred in the case of Maria Tofts of Godlyman (Godalming)
in Surrey ; she had had a great longing for " Eabbets " in early
pregnancy. Those who are interested in this case will find the

MENTAL IMPRESSIONS 115

details in the books by Sir E. Manningham, T. Braithwaite, C. Ahlers,
and K St. Andre (London, 1726, 1727).

It is probable that the " Godlyman " case, in conjunction with the
appearance of Turner's appreciation of the doctrine of maternal im-
pressions, stirred up Blondel {The Strength of Imagination in Pregnant
Women examin'd, London, 1727) to write his famous criticism there-
upon ; but already in the year 1712 Nigrisoli (Intorno alia generazione
dei viventi, p. 5, Ferrara, 1712) had argued against the popular
belief, and had pointed out that the foetus was entirely separated
from the mother, that monstrosities occurred in animals in whom
they could scarcely be ascribed to imagination, and that in the
human subject deformities were met with for which no disturbing
impression could be alleged. To Blondel, however, belongs the credit
of dealing to the maternal impression theory the most deadly blow it
had yet received. Very skilfully did this writer marshal his facts ;
and with no small degree of humour, and now and then some biting
sarcasm, did he state his arguments and refute the assertions of those
whom he delighted to dub the " imaginationists." In the first in-
stance, his satire was specially directed against Mr. Daniel Turner ;
but he did not spare other, older, and more widely known exponents
of the imagination theory. The first edition of Blondel's work was
published anonymously in 1727, at least on the title-page the author
was simply described as " a member of the College of Physicians " ;
but in the second issue of the book in 1729, in which were many
additions and amplifications of the argument, the writer openly
avowed himself, not " out of ostentation," as he said, but to show
" that I dare face the enemy."

Blondel arranged his facts and arguments with intent to prove
that the current theory was unsupported by experience, by reason,
and by anatomy. With regard to experience, he showed that im-
pressions often occurred which were not followed by marks or
anomalies in the foetus, and that deformities were met with in cases
in which no impression was alleged. Further, he analysed in detail
the exceptional cases in which it had been maintained that a definite
impression had resulted in the production of a similar mark on the
infant ; and under such pithily descriptive epithets as " Parey's
Frog," " Miss Muscle and the Grenadier," " the Baker bit," " Sir
Kenelm Digby's Patch," etc., he subjected to much ridicule the illus-
trative instances gathered (mostly from Fienus) with great care and
apparently with almost perfect faith by Daniel Turner. Having dis-
missed these, Blondel turned his attention to the story of Jacob and
his rods, which curiously enough seems greatly to have exercised him.
Most of his readers will agree that he was at altogether unnecessary
pains to explain away this particular case. The author concluded
that there " are so many odds against imagination that the cases
related in its favour can never overbalance those which are against
it," and in his opinion they " may. be compared to an accidental hit of
a dream or the predictions of a Fortune-teller, which, now and then,
are accomplished."

Blondel, then, having " entirely beat the Imaginationists out of

116 ANTENATAL PATHOLOGY AND HYGIENE

their Entrenchments of Experience," attempted to show that reason
also was against the popular belief. Conception, the growth of the
embryo, and the determination of its sex were all outside the power
of the mother's will. " How," he said, " can anybody believe, without
reflecting upon the Wisdom of God, that it is left to her to disfigure
the child, and to spoil the regular Work of Nature ? " Again, since
the woman cannot by her imagination mark her own body, it does
not seem probable that she should be able to act thus on her foetus.
Other arguments were advanced, and then the writer pointed out that
there exists no communication of nerves between the mother and
foetus, and so there could be no communication of thoughts, as alleged
by Malebranche and others. In his efforts to absolutely extinguish
any flickering flames of belief in the imagination theory, Blondel
adopted the erroneous doctrine of the pre-existence of all the parts of
the foetus somewhere before conception. By what means, then, can
the mother's imagination obliterate the lineaments of the foetus,
which were pre-existent to conception and subsisting ever since the
creation of the world ? Finally, anatomical reasons are adduced to
show that there is no direct circulation of blood between mother and
foetus, and Blondel refers to the much more probable natural causes of
foetal marks, such as intrauterine diseases or traumatism and heredity.

Blondel's work, which I have thus attempted to epitomise, pro-
duced a great effect upon medical opinion all over Europe, and soon
numbered adherents in nearly every civilised country. In France,
for instance, not only did translations of the English physician's work
appear, but Belief also wrote a book to combat the popular belief in
impressions (Lcttres sur Icpouvoir dc I 'imagination dcsfcmmes enceintes,
Paris, 1745). In Germany, also, Haller (Opuscula sua anatomica,
p. 150, Gottingce, 1751) to a large extent threw the weight of his
authority into the scale against the popular theory ; and, in Eussia,
Eoederer (Dc vi imaginationis in foitum ncgato, 1756) did likewise,
though with less effect. In Scotland, Alexander Monro (Med. Essays
and Observ., ii. 238, Edinburgh, 1734), in his essays on the nutrition
of foetuses, showed clearly that he was an opponent of the maternal
imagination theory, for he wrote (in 1734) as follows: — "The Liquors
sent into the Foetus by the umbilical Vein not having their propelling
Force communicated from the Mother, the State of the Mother's
Pulse cannot affect the Child otherwise than by occasioning Abortion,
or vitiating the Fluids that are to be absorbed ; and therefore we may
be convinced, how vain it is to pretend to account in a physical Way
for the Impressions said to be made on Children by the Imaginations
of the Mothers." Monro's correct views on foetal physiology, along
with their bearing on impressions, seem, however, to have been over-
looked by succeeding writers. Vari (Bag ion amenta in aggiunta alia
Dissertazionc del Signor G. Blondel, p. 175, Ferrara, 1760), in Italy,
was at first a supporter of Blondel's position, but afterwards through
a personal experience came to change his views. Plancus, another
Italian, entirely sided with Blondel.

Whilst, however, the spirit of criticism was thus infused into the
subject, there was still in the minds of many authorities a firm belief

MENTAL IMPRESSIONS 117

in the potency of maternal impressions as teratogenic factors. Super-
ville (Philos. Trans., xli. (for 1740), pt. 1, p. 306, London, 1744), for
instance, after relating how seven pigs taken from a freshly
slaughtered sow all showed the bloody mark of the knife about their
necks, proceeded to state his adherence to the theory of the influence
of the " disturbed and disordered imagination of females." He re-
ferred to certain objections to the belief, and then said : " I own I do
not comprehend it neither. It does not follow from thence, that we
ought to reject as false all that our lieason cannot penetrate into."
Gregory (Philos. Trans., xli. (for 1741), pt. 2, p. 764, London, 1744),
writing soon after, seems to have had even less doubt, for he told how
a pregnant woman " took prodigious notice " of a monkey with a hood
turning on a stick, and how the foBtus in utero turned over and over
in like manner (for were there not twists in the umbilical cord ?), and
when born was seen to resemble closely a hooded monkey. Nicolai
(Gedancken von der Erzeugung des Kindes, Halle, 1746) tried to refute
Blondel's opinions ; but had to admit that monstrosities did not
resemble the subjects of the impression (1749).

It must also be borne in mind in estimating the influence of the
writings of Blondel and his followers that on the Continent the great
weight of Boerhaave s authority (Prcelectiones academicte, iv. pt. 2, p.
261, Taurini, 1742-45) had been in favour of the old theory, with
certain reservations regarding the sort of impression and its mode of
incidence. Boerhaave's commentator, Van Swieten (Commentaria in
H. Boerhaave Aphorismos, iii. pt. 1, par. 1075, Vienme, 1743), seems
to have been much struck by the lifelike representation of a cater-
pillar on the neck of a young girl whose mother had in her pregnancy
been impressed by a real caterpillar crawling on her neck. Another
defender of the imagination theory was Krause (Qua- nam sit causa
proxima mutans corpus foetus, St. Petersburg, 1756), whose dissertation
obtained special honour at the Imperial Academy of Science in St.
Petersburg ; he alleged the existence of a nervous communication be-
tween the mother's uterus, the placenta, and the fetus, and had no
difficulty in understanding how disturbances of the mother could
deform the infant, especially since the organs of the latter were so
tender.

Boerhaave, however, was not always so fortunate as to have a
commentator willing to accept all his views on the subject of maternal
impressions. Albert von Haller (Elementa Physiologic, viii. lib. 29,
p. 129, Bern, 1766), in fact, did much to support Blondel's criticism,
whilst he also established it on more scientiflc arguments. For in-
stance, he maintained that if there existed a nervous communication
between mother and foetus, then the gathering together of the
placental nerves in the umbilical cord would constitute so evident a
strand of nerve-tissue that no observer could possibly overlook it.
If, again, the communication was a vascular one, — and this might be
admitted, — how could images of things be transmitted by means of a
column of liquid in a tube. The birth of twins of different colour
was explicable in a more prosaic manner than by the invocation of
the mother's imagination. Naevi, according to Haller, were due to

118 ANTENATAL PATHOLOGY AND HYGIENE

skin disease, and monstrosities were the result of foetal maladies. It
does not appear that this author was altogether opposed to the
doctrine of impressions, for he seems to have been struck by the fact
that children resemble their parents, and to have regarded it as an
argument in favour of the theory. Smellie {Treatise on Midwifery,
ii., cases 89 and 90, London, 1754), who lived about the same time,
was, doubtless, sceptical also ; for, after noting two cases, he wrote :
" Notwithstanding these examples, I have delivered many women of
children who retained no marks, although the mothers had been
frightened and surprised by disagreeable objects, and were extremely
apprehensive of such consequences."

Morgagni (Dc sedibus ct causis morborum, Epist. 48, Venetiis,
1701) was yet another well-known writer of this period who
hesitated about throwing overboard the theory of impressions ; he
believed that while most cases in which a fcetal defect resembled
a maternal impression were accidental coincidences, yet some con-
tained another element which he freely confessed that he did not
comprehend.

Between the years 1760 and 1780 it ought to be noted that several
popular statements of the anti-imaginationist side of the matter were
put before the British public in the form of " letters " or " essays "
from anonymous authors, addressed to " the ladies " or to " a married
lady." In these works the arguments used by the writers to convince
their fair readers are often interesting and even amusing. In one,
published in 1765, the author says, "a mark which has been foretold
has once in 1000 times happened to answer its description; another
effect of chance, which may serve to support the prejudice of those
who do not reason, but can have no influence on those who, like you,
madam, are only convinced by truth." Another author, writing in
1772, sums up as follows : " The same chain of ideas that shows the
impropriety of believing the cure of a toothache by a charm ; the
destruction of warts by turning thief, and stealing raw meat ; the
cure of the cramp by wearing rosemary, or placing the soals of our
shoes uppermost when we go to bed : I say, the same chain of ideas
that shows the impropriety of believing these absurdities, will, when
applied with a real desire to be informed, whether the imagination in
pregnant women possesses the powers ascribed, convince the enquirer
that the tales told of the mother's imagination exerted on the fcetus,
are a collection of falsities formed by superstition and ignorance, and
continued by prejudice and credulity."

The last years of the eighteenth century did little to settle the
question of the potency of the mother's imagination on the foetal
form. We find, in fact, that the minds of the writers of the time
were greatly exercised with the side question — a theological one-
concerning the result which an acceptance of the imagination theory
would have upon the idea formed of the Creator of the Universe.
To the discussion of this matter Bickmann, Arnold, Engelhardt, and
others devoted themselves ; but into their arguments it is unnecessary
to enter. Other writers, some for and some against the popular
theory, were Eller, Kooy, Schonwald, Schenk, Jennet, Bablot, Hoff-

MENTAL IMPRESSIONS 119

maun, Fielitz, and Schumann ; but the enumeration of their names
must suffice for the purposes of this sketch.

It may be of interest to the curious to know that near the close of
the eighteenth century the money value of a maternal impression (of
a patriotic kind) was fixed at 400 francs per annum ; for Geoffroy
Saint-Hilaire (Histoire des Anomalies, i. p. 332, 1832) records that in
the third year of the French Republic an infant was born with the
representation of a Phrygian cap of liberty on the left breast, and to
the mother the Government awarded the above sum, presumably for
her patriotic thoughts !

In the Nineteenth Century.

The nineteenth century opened with the question of maternal im-
pressions still unsolved. Medical opinion took one of three lines :
complete belief in the potency of a mother's imagination to mark her
fojtus in exact resemblance with the object acting upon her mind ; or
complete disbelief in any such power ; or an intermediate line of partial
acceptance of the doctrine of maternal mental influence over the
infant, — an attempt to reconcile the traditional belief with the spirit
of modern scientific inquiry. Popular opinion took a line which is
well exemplified by the accompanying illustration (Fig. 22) and the
legend annexed thereto. Doubtless the supposed words on the eyes
were irregularities in the iris such as have been seen in more recent
times by A. Glaus (Flandre mcJ., i. p. 65, 1894) and others.

It now became increasingly difficult for the trained physician to
accept the old doctrine, for the researches of the eighteenth century
had demonstrated that there was no direct communication between
the circulatory system of the mother and that of her foetus, and in
the early part of the nineteenth it became evident that no demonstra-
tion of a structural nervous union could be given. On the other
hand, the effect of these anatomical discoveries concerning the con-
nection between mother and unborn infant was to some degree
discounted by the enunciation of animal magnetism by Mesmer, and
its partial acceptance by the medical profession ; for, if its tenets were
correct, there was no longer any need for the demonstration of nerves
in the umbilical cord.

In the first year of the century Desgranges (Rcc. d. actes Soc. de
Saute" de Lyon, ii. p. 83, 1801) published his communication in sup-
port of the maternal imagination theory, specially in reference to na?vi
materni, and he has been followed by a long series of writers, some
favourable, some inimical, to the doctrine. Besides the authors who
have made special contributions to this subject, nearly every one who
has written on midwifery, surgery, diseases of children, and dermat-
ology, has ranged himself amongst the believers, the unbelievers, or
the neutrals. An enormous literature has, therefore, gathered around
the question of the mother and her influence upon her unborn child,
and it becomes impossible to do more than point out certain of the
more important articles which have dealt with the matter.

If we consider first what the teratologists of the nineteenth century

120 ANTENATAL PATHOLOGY AND HYGIENE

- fwffMb

n

A FULL and PARTICULAR ACCOUNT of
that most wonderful CULLD from Gallowayj
now exhibiting in this City, for the inspection
of the curious ; to which is added, a correct
likeness, drawn from the life.

A vodltG woman in Galloway having proved with child, laid the same to «
respectable mun of tlie name, of John Woods, who denied being the lather
of the name, and persisted in his denial saving that he would never acknow-
ledge the child, unless Ida name tccu «*{£*» <U fuiUene;th on its face ; and
be accordingly gave his solemn oath before the Court to that effect. This
made so much impression on the mind of the young woman, who was pre-.
taut, that his name and person remained constandy in her wind's eye. and
when the child was boru, the name of the lather appeared iu legible tetters
in tie child's eyes, the name of " Jou.v Woods," «J the right eye, and
** Bo»n 1817," on the left eye. When John Woods, die alleged father, came
to know this circumstance, he instantly absconded and has not since been
beard of.

This woederful child has now arrived in this city, and has Iteen inspected
by the Professors, ami ulhcr learned Faculties of this city, and pronounced
to be a moat wonderful phenomenon of nature, and an astonishing dispen-
sation of Providence ill pointing out the truth against the wicked and per-
jured ways of men.

An inap<-ction of this child will, il is hoped, tx* a salutary warning to all
• young persons of both sexes, first u> Ix-ware of all such doings, and second

to b*w*r« of peijury in their attempts u» conceal their shame.
t ..- ■ '"'■

Fie-. 22. — A "Maternal Impression" story of the beginning of the
nineteenth century.

MATERNAL IMPRESSIONS 121

have said on the subject, we shall find a very marked leaning towards
the total condemnation of the theory of maternal impressions. Jens
Bang (Abhandlung ilber cine Missgeburt, Kopenhagen und Leipzig,
1801), it is true, supported the old doctrine, and Jouard (Des mon-
struosites et bizarreries de la Nature, Paris, 1806) took up an inter-
mediate position; but nearly every one else has showed great
scepticism, some even passing the subject over in complete silence.
Tiedemann (Anatomie der ■ Kopflosen Missgeburten, par. 88, p. 100,
Landshut, 1813), in his work on acephalic fcetuses, pointed out that
only in one case was an impression recorded ; further, he insisted
that the fact that one twin was commonly normal whilst the other
was so deformed was a strong argument against the theory ; and,
finally, he propounded the question, What imagination of the mother
could be at work to produce in the infant a one-horned uterus or an
intestinal diverticulum ? Zimmer (Physiologisehe Untcrsuchungen
ilber Missgeburten, p. 35, Kudolstadt, 1806) and Clesius (Etwas fur
Eheleute, etc., p. 40, 1812) also opposed the imagination theory, and
Herholdt (Betragtninger over Misfostere, 1828) entered at great length
into its history. We find Etienne G. Saint-Hilaire (" Monstre " in
Diet, classique d'histoire naturelle, xi. p. 40, 1827) taking an inter-
mediate position ; but he pointed out that prolonged moral im-
pressions could have little or no teratogenic effect, as shown by
the very small number of monstrosities born in such a large city as
Paris. He also noted three cases in which a sudden and sharp im-
pression was the cause of anencephaly, not through the imagination
alone, but on account of the sudden uterine contraction thereby pro-
duced, and the consequent pressure on the ovum or embryo. He
thought that prolonged emotions might weaken the foetus without
making it actually monstrous. Isidore G. Saint-Hilaire {Histoire des
Anomalies, iii. p. 540, Paris, 1836) adopted largely the views of his
father in this matter; but he more clearly defined the sphere of
action of an impression. Violent and sudden impressions may cause
monstrosities in the manner above indicated ; moderate or feeble, but
long-continued impressions may also, it cannot be denied, produce
malformed and even monstrous infants ; but it cannot be believed
that slight and transient desires, feelings of disgust or of fear, etc.,
have any influence upon the form of the unborn infant. " II est
surtout contraire a toutes les donnees de la science et de la raison, de
croire qu'un objet vu, craint ou desire par la mere, puisse venir, pour
ainsi dire, se peindre sur le corps de l'enfant qu'elle porte dans son
sein ; et la saine physiologie ne peut voir dans cette ancienne croy-
ance qu'un prejuge aussi absurde, et quelquefois aussi dangereux qu'il
est ancien."

Vrolik, another well-known teratologist, was more clearly an un-
believer than were the Saint-Hilaires, for he admitted nothing further
than that a nutritive disturbance in the mother following a severe
shock might lead to a defective development of the foetus. Fbrster
(Die Missbildungen des Mensclien, 2nd edit., p. 4, Jena, 1865) enumerated
eight arguments against the impression theory, including the state-
ment that most malformations develop in the first month, nay, even

122 ANTENATAL PATHOLOGY AND HYGIENE

in the first week of pregnancy, and therefore at a time when the
mother commonly does not know that she is pregnant. He also
noted that impressions were common while monstrosities were rare,
that there was no direct nervous communication between mother and
foetus, and that one twin might be normal and the other malformed.
Wonderful stories impressed Forster not at all : " die Literatur ist
voll von solchen Fallen, noch mehr aber die Kopfe der Hebammen
und alten Weiber weiblichen und mannliehen Geschlechtes."

Of the more recent teratologists nearly all have treated the
subject of the influence of the maternal imagination as a silly fable ;
and in the works of Ahlfeld and Hirst and Piersol there is no refer-
ence to it, whilst in those of Taruffi, Guinard, and Blanc it is simply
considered from a historical standpoint. E. Martin (Histoirc des
monstres, p. 266, Paris, 1880), alone, has argued in favour of a
modified form of the old belief. He has stated that imagination plays
an undoubted part in the procreation of monstrous forms ; but he has
also insisted that this part is a mechanical one, — that through the
nervous system the uterus is thrown into contraction, and thus undue
pressure is brought to bear on the embryo. The occasional occur-
rence of foetal malformations which resemble the objects causing
the maternal nervous disturbance is purely coincidental. Martin
seems also to regard the mother's mental state during pregnancy
as in some degree modified so as to be more susceptible to im-
pressions.

Alongside of this almost total abandonment of the imagination
theory by the teratologists of the nineteenth century, we have to place
the extraordinary rejuvenation of the ancient doctrine among the
leaders of the medical profession in the United States of America.
Between 1839 (the year in which the report of the " Snake-man,"
Bobert H. Copeland, whose mother, when pregnant, had been struck by
a rattlesnake, appeared) and 1900, papers dealing with maternal
impressions, to the number of 170, were published in American
journals. Not only have the contributions been many, but they have
also, with few exceptions, been favourable to the imagination theory.
Further, amongst those who have most warmly supported the old
doctrine were such acute thinkers and weighty authorities as Fordyce
Barker (Trans. Amer. Gynec. Soc, xi. 152, 1887), Samuel C. Busey
{ibid., p. 176), and William Goodell (Amer. Journ. Obst., iv. 131,
1871). Fordyce Barker thought " that the weight of authority must
be conceded to be in favour of the doctrine that maternal impressions
may affect the development, form, and character of the foetus," and
even went so far as to believe that " these causes may act effectively
on the ovules before fecundation," alleging in proof the famous " Dun-
dreary Case." Barker also included telegony, or the influence of a
previous sire, in the sphere of maternal impressions. Goodell was,
" to a certain extent," converted from a sceptic into a believer by
a case of pre-natal " circumcision " which occurred in his own practice.

It must not, however, be thought that the theory of maternal
impressions was unanimously accepted by the profession in the
States. On the contrary, J. G. Fisher (Amer. Journ. Insan., xxvi.

MATERNAL IMPRESSIONS 123

241, 18G9-70) wrote at length against the popular opinion, but did
not add any novel arguments; and Dugas (South. Med. and Surg.
Journ., 3 s. i. p. 317, 1866-67), Norman Bridge (Chicago Med. Journ.
and Exam., xxxii. 577, 1875), and Conant (Trans. N. York Acad. Med.,
ii. 267, 1857-63) were all strong in their opposition. Still, it is evi-
dent that within recent times the theory has been held strongly by a
great many medical men of note on the other side of the Atlantic ;
for Hubert Work (Med. News, lxv. 451, Philadelphia, 1894), writing
in October 1894, was able to report that out of thirteen physicians
(including Penrose, Goodell, Matthew D. Mann, Hatfield, Cooke,
Hirst, W. A. Edwards, and A. T. King) whose opinion he asked, only
one (Hatfield) was emphatic in his disbelief of the statement that
" pronounced impressions made upon the mind of a pregnant woman
predispose to bodily defects and birth-marks in the child."

The trend of opinion in America is also learned from the study of
Dabney's contribution to Keating's Cyclopaedia of the Diseases of Chil-
dren (i. p. 191, 1889), and from that of Edward P. Davis to Norris'
Text-book of Obstetrics (p. 213, 1896). Finally, to quote from popular
literature, Oliver Wendell Plolmes had not a little to say about the
result of an "impression" in his fascinating novel Elsie Venner.
This is by no means the only instance of the belief entering into the
fiction of past and present times ; for allusions to it are found in
Shakespeare's Merchant of Venice (Act i. sc. 3), Scott's Fortunes of
Nigel, and Sterne's Tristram Shandy, while from a casual survey of
modern literature I have noted references thereto in Blackmore's
Lorna Boone, in Henty's Rujub the Juggler, in Merriman's From one
Generation to Another, in Egerton's Keynotes, and in Cobban's Red
Sultan.

Whilst the teratologists of the nineteenth century practically
abandoned the imagination theory, and whilst American writers in
large degree resuscitated the doctrine, surgeons, obstetricians, physi-
ologists, and dermatologists in Europe held very various opinions
thereupon. In Germany, for instance, in the first half of the century
there were many who wrote on the power of maternal impressions,
among whom were Harting, Merle, Klein, Gittermann, Hirsch,
Wesener, Schneider, Keyler, Braun, Spath, Koehler, Pauli, Rubner,
Solbrig, Droste, Hoffmann, Straube, and Leopold. All did not
support the old belief; but many did so, and some even went to
ridiculous lengths, such as to affirm that the infant's feet were de-
formed by the mother wearing tight shoes in her pregnancy (Schneider,
Ztschr. f. d. Staatsarznk, ix. p. 37, Erlangen, 1825), and to say that
the sight by a woman of a one-armed man at the eighth month of her
pregnancy could cause the birth of a foetus with one arm wanting.
Others, such as Jorg and Meissner, argued against the theory, the
latter bringing forward some strong clinical evidence. J. Miiller, the
physiologist, also, was a strong opponent of the theory ; but Burdach,
another physiologist, supported it, and thought that the relation
between mother and foetus was an occult one, like animal magnetism.
In the second half of this century a comparatively small number of
German writers wrote specially on the subject (including Brach,

124 ANTENATAL PATHOLOGY AND HYGIENE

Wagner, Hitter, van Praag, Tassius, Willige, Adler, Santlus, Heidel-
berg, Upmann, Clemens, Roth, Wolff, Preuss, and the text-book
writers); but save in the period 1850 to 1860 the general feeling has
been strongly opposed to the ancient doctrine.

In France and Belgium the general trend of opinion during the
century was much the same as in Germany. Amongst the special
writers on the subject must be named Desgranges, Martin, Clarae-
Faget, Girard, Leveque-Lasource, Demangeon, Be'rard, Burggraeve,
Steinbrenner, Guislain, Schoenfeld, Pigeolet, van Camp, Bayard,
Turck, Schrevens, Liebrecht, Loin, Liegey, Mijnlieff, Delassus, and
Variot. Burggraeve (Ann. de gynic. et pediat, 2 s. i. p. 115,
Bruxelles, 1840-41) attempted to give a more scientific aspect to the
old theory by combining it with the known facts of embryology : he
related how a woman at the third month of pregnancy saw a pig witli
its throat cut, and feared that her infant might be affected ; the child
showed a cleft in the throat which the author regarded as due to the
want of union of the branchial arches which were separate at the
time of the impression. Guislain reported (amongst others) a curious
case of aural deformity following a " longing," and Bourgeois con-
cluded that " les impressions profondes ou vivaces de l'imagination
occasionnent quelquefois chez l'enfant des vices de conformation."
In 1876 Liebrecht wrote in support of the theory of impressions ;
and Delassus (Arch, de tocol., xviii. p. 923, Paris, 1891) related two
cases (one of hare-lip and one of anencephaly) which seemed to have
confirmed him in his belief in the old doctrine.

In Italy, also, the power of the imagination of the mother in
teratogenesis has found defenders and opponents and critics, to some
of whom Tarufn (Storia delta teratologia, i. p. 242) refers. For in-
stance, Monteggia put on record a case of intrauterine amputation
alleged to be due to the mother seeing many cases of amputations ;
Barbieri argued against the belief, and suggested more natural ex-
planations of foetal states, and Vannoni hinted that possibly a pre-
disposing condition was needed in mother and fcetus before an
impression could become efficient. Fenoglio, Viparelli, and others
contributed further to the literature of the subject in Italy. In
Poland, Neugebauer was a believer, as Drzewiecki (Med. Bee., xl. p.
529, New York, 1891) tells us.

With regard to Great Britain, it would seem that the influence of
Blondel, Monro, and Smellie was largely effective in preventing a
wide acceptance of the imagination theory in the ranks of the pro-
fession. Alleged cases from time to time appeared in the Lancet (e.g.
those reported by Hoare, Canton, Rankin, Wansbrough, O'Shea,
Dunn, Beale, Child, Daly, and others) ; the British Medical Journal
(cy. those by Curran, Houghton, Graham, Thompson, Clapperton,
Huntley, R. J. Lee, Sandham, Jenkyns, Jones, and others) ; the
Medical Times, the Dublin Medical Brcss, and the Glasgow Medical
Journal ; but although these contributions were fairly numerous, the
feeling amongst obstetrical teachers was inimical to the notion of
maternal influence. Ryan (Loud. Med. and Surg. Journ., iv. 170,
1833-34), for instance, was strongly opposed to it, and so have been

I

MATERNAL IMPRESSIONS 125

many others. Montgomery (Signs and Sym^jtoms of Pregnancy, 2nd
edit., p. 17, London, 1856) was an exception, for he wrote, " I cannot
help thinking it quite consistent with reason and the present state of
our knowledge to believe that a very powerful impression on the
mother's mind or nervous system may injuriously affect the foetus,
still lodged in her womb, actually a part of herself, and deriving its
supply of life from her blood ; and this, the more especially, as many
instances have been witnessed in which the child after birth has
suffered seriously by being suckled by a woman labouring under some
strong mental impression."

The matter was brought up by myself on two occasions (Edirib.
Med, Journ., xxxvi. p. 624, 1890-91 ; xxxvii. p. 1025, 1891-92) at
meetings of the Edinburgh Obstetrical Society, with the general
result that no decisive proof for or against was forthcoming, and that
the question was still to be regarded as sub judice. Barnes was con-
tent to leave the problem unsolved ; but Ashburton Thompson (Trans.
Obst. Soc. Lond., xix. p. 94, 1878) would seem to have been a believer.
Much more might be said concerning British contributions to this
vexed question, but it must suffice to note that Everard Home (Phil.
Trans., p. 66, London, 1825) was a supporter of the doctrine.

At the Present Time.

What may be the fate of the theory of maternal impressions in
teratogenesis in the twentieth century it is impossible to foretell ;
but it is safe to say that the diffusion of the views that group them-
selves around " Christian Science " and " Mind Cures " will strengthen
the belief in the power of the mother's imagination to alter the
appearance of her unborn infant and so tend still further to raise the
idea in popular estimation. A glance at current medical literature
will show that the subject is still before the medical profession,
although I fancy there are signs that scientific practitioners are
becoming more and more sceptical regarding it and more exacting in
their investigation of the details of recorded cases. At any rate the
articles of H. F. Lewis (Amer. Journ. Obst., xl. 84, 1899) and J. G.
Kiernan (Medicine, vi. 450, 1901) suggest this conclusion.

Summary.

A survey of the wdiole history of the belief in maternal im-
pressions leads me to say about it what Sir Thomas Browne said
long ago about the common opinion that crystal is nothing else
but ice or snow concreted (Pseuelodoxia Epidemiea, bk. ii. c. i., 1646):
" Of which assertion, if prescription of time and numerosity of
assertors were a sufficient demonstration, we might sit down herein,
as an unquestionable truth, nor should there need ulterior dis-
quisition; for few opinions there are which have found so many
friends, or been so popularly received, through all professions and
ages." But the scientific antenatal pathologist refuses to " sit down
herein " !

126 ANTENATAL PATHOLOGY AND HYGIENE

Such is, in outline, the history of this teratogenetic theory, extra-
ordinary alike whether its truth be affirmed or denied; wonderful
alike in its wide extension in space and time, and its firm hold upon
the minds of both profession and laity. To those who wish to see it
dead it is most disappointingly vital ; to those who wish to demon-
strate its truth most strikingly destitute of scientific proof. In its
support is a marvellous mass of evidence of the post hoc ergo propter
hoc kind ; arrayed against it are the scientific theories of biology
which have been gradually and laboriously built up by the savants of
this and of other ages.

From the standpoint of the student of antenatal pathology the
theory of maternal impressions must be regarded as a great mis-
fortune. Many instructive cases of fo3tal disease and deformity
have been practically lost to science through the absorption of the
observer's mind in ideas of the mental process in the mother which
could have caused the state of her infant, and through his desire to
trace in the morbid foetal condition a resemblance, often far-fetched
indeed, to some animal, vegetable, or mineral. In this sense the
theory has been the grave in which a great deal of fcetal pathology
has been buried ; for instead of a case being entitled one of anence-
phaly, or of fcetal ichthyosis, or of hirsuties or hairy mevus, it has
been simply labelled " a curious instance of maternal impressions,"
" a striking case of the influence of the mother's mind on the foetus,"
etc. Further, the material thus hidden away is in most cases per-
manently lost ; for a reference to the report usually gives us no
anatomical or pathological details to enable us to identify the malady
or the malformation, but simply tells us that the infant had a head
like a cat or a rat, or was hairy like a bear, or had scales like an
alligator or a snake.

The theory has had a maleficent effect on the progress of the
study of antenatal pathology. Of that there can be no doubt. But
the question whether there is or is not any truth in the theory still
remains to be settled. In the historical sketch which has been given
I have indicated most of the arguments which the supporters and the
opponents of the doctrine have adduced ; these I neecl not again refer
to, but may simply state briefly what I regard as the correct view to
take of " maternal impressions." In order to centralise our know-
ledge on the subject, I may ask myself two questions : (1) Does a
definite impression upon a pregnant woman's mind often or ever
cause a defect in the foetus closely resembling the thing producing
the impression ? and (2) Has the state of the mother's mind during
gestation any effect upon her unborn infant's development ?

The first question I answer in the negative, and the second
affirmatively. Since I wrote upon the subject some years ago I have
very carefully examined the evidence upon which several alleged
cases have been founded, I have had opportunities of attending
confinements in which all the elements regarded as suitable for the
production of the results of an impression were present, and in which
the children were free from anything at all resembling the impres-
sion, and I have very fully acquainted myself with the voluminous

MATERNAL IMPRESSIONS 127

literature of the subject. The conclusion drawn is, that the cases
which have been advanced to prove the potency of maternal im-
pressions have been accidental coincidences, and not the effects of the
alleged causes. Their great number may be adduced to prove that
the doctrine must contain an element of truth ; but it has to be
pointed out that, numerous as they are, the cases in which nothing of
the kind happened are vastly more numerous. Further, although I
do not wish to lay too much stress upon this, no adequate scientific
explanation of the modus operandi of such impressions has ever been
advanced. Whilst I am willing to admit that the mother's nervous
system is often in a peculiar state during pregnancy (Neumann,
Centrlbl. f. Gynak., xix. 201, 1895), and whilst I fully recognise the
possibility of the mother affecting the foetus with a disease, and vice
versd, I regard it as impossible for (e.g.) the sight of a mutilated
individual to be so transmitted mentally by the mother as to cause a
foetal amputation resulting in a similar deformity in utero. At the
same time, I must concede that perhaps one case in a hundred
presents phenomena which it is very difficult entirely to explain
away. One such case I have myself met with ; but it seemed to me
reasonable enough to think that a more rational explanation of it
existed, although as yet unknown, than the influence of the mother's
imagination.

With regard to the second question, I think there can lie no
doubt that prolonged or strongly marked mental states of the mother
may affect the development of the foetus in her uterus. That such
can produce abortion none, I fancy, will deny ; and that short of
producing miscarriage they can also lead, through vascular and
nutritive disturbances, to irregularities in embryogenesis is eminently
probable. In Paris, it is said, the children that were conceived
during the disastrous sie<i;e of the Franco-Prussian war can be
recognised by bodily and mental stigmata so marked that they have
been called " enfants du sioge." In reference to this phenomenon
Fere (La Famille Neuropathique, p. 22, 1894) says " les enfants qui
ont etc' con^.us et pork's a certaines epoques troublees offrent en
grand noinbre des troubles cle nutrition, des malformations et en
particulier des alterations des fonctions du systeme nerveux." Of
course it must be admitted that the poor or bad food and the de-
fective hygiene of such seasons were also operative, still it is reason-
able to believe that in part the results were due to the continued and
severe terror and constant anxiety incident to a time of siege in
modern warfare. In the same way the results are not always
teratological, but may, as Fere has also shown (Teratologia, ii. 245,
1895), be of the nature of sterility, abortion, premature labour,
mortinatality, congenital debility, and retarded growth and develop-
ment. There are then many causes at work and many resulting
phenomena ; but amongst the former it is fair to reckon mental
emotion, and among the latter monstrosities. To this extent I
believe in the old doctrine of maternal impressions; this is, I think,
the one grain of truth in an immense mass of fiction and accidental
coincidence.

128 ANTENATAL PATHOLOGY AND HYGIENE

In order that more light may be thrown upon this matter, cases
in which mental impressions in pregnancy were followed by the
birth of infants in any way abnormal ought to be put on record. For
instance, Fere, recounts how a woman, pregnant four or five weeks,
was greatly impressed by the sight of a child with a hare-lip ; at the
full time all her friends came together to see an infant born similarly
affected, but the offspring showed not a hare-lip but a large naevus on
the neck (" Un fait pour servir a l'histoire des bouffees de chaleur et
des rougeurs morbides," Compt. rend. Soe. de biol., 10 s. i. 643, 1894). It
is by no means certain that this was an instance of cause and effect, but
such cases are far Worthier of record than are those in which an
impression is said to reproduce itself, as regards form and situation,
on the infant's body.

CHAPTER X

Teratogenesis (emit.) : Theories of the Present ; Traumatism and Mechanical
Pressure as a Cause of Monstrosities ; Extra-genital Maternal Traumatism ;
Injuries affecting the Mother's Abdomen ; Intra-abdominal Pressure ; Intra-
uterine Pressure ; Funic Pressure, with Literature.

While, as has been shown in the preceding two chapters, some of
the teratogenic theories of the past are represented by modern
developments, so also some of the present views spring from notions
with which the ancients were acquainted. The division, therefore, of
the theories of the causes of monstrosities into past and present is
artificial and somewhat inaccurate ; but some sort of classification is
necessary, and the one employed has the merit of being at least
convenient. Thus it comes about that the last of the beliefs of the
past (maternal impressions) in a certain sense still exists, while the
first of the beliefs of the present (traumatism) has its roots in
antiquity.

The idea that antenatal deformities might be due to causes acting
mechanically upon the foetus was present in the mind of Hippocrates
when he wrote in his treatise De Gentium the passage which may
thus be rendered : " As to the infant crippled in the womb, I say that
it is crippled in consequence of a contusion, the mother having been
struck on the place corresponding to the foetus, or having had a fall,
or having sustained some other form of violence. If the infant
experience a contusion, it becomes crippled in the part contused ; if
the contusion be greater, the membrane which surrounds it ruptures,
and the woman aborts. Or yet again, infants become crippled in the
following way : when in the womb there is a narrowness at the part
where in fact the crippling is produced, it is inevitable that the body
moving in a narrow place shall be crippled in that part. It is thus
that trees which in the earth have not enough space, and are hindered
by a stone or other thing, become bent during growth, or rather
become large in one part and small in another. The infant experi-
ences the same thing when, in the womb, a portion is relatively too
narrow for the corresponding part of the infant."

This quotation does not indeed refer to monstrosities, but only to
such deformities as club-foot and congenital dislocations ; but it is still
of great interest, being the earliest expression of a belief in the
moulding power of the uterine environment. It will be specially
noted that Hippocrates referred both to the sudden and temporary
action upon the foetus as seen in a maternal traumatism, and to the

VOL. II. — Q

130 ANTENATAL PATHOLOGY AND HYGIENE

gradual and more permanent effect produced by narrowness or strait-
ness of a part of the uterus.

Aristotle went a great deal farther than the Hippocratic writers,
for he suggested the manner in which true monstrosities were
produced, and perceived that the cause must act during the early
period of embryonic life. In the fourth book of his Generation of
Animals, and in the third chapter, where he is writing of the eggs of
birds, Aristotle makes this statement (I translate from the masterly
rendering into French by Barthelemy Saint-Hilaire, Traite tie la
(j i titration des animaux d'Aristote, ii. 277, 1887): "When the germs
are very close together they fuse, as very often happens with fruits in
the vegetable world. When the vitelli (' les jaunes ') are separated
by the membrane, two chicks are produced showing nothing abnormal ;
but when the vitelli are in contact and nothing isolates them,
monstrous chicks are produced which, while they have only one head
and one body, possess four feet and four wings, for the upper parts are
formed from the white, and the nourishment coming from it (this
white) has been portioned out at once to them, while the lower part
has only appeared later, the nourishment being single and in-
divisible." The latter part of the passage is obscure, but in the first
part the idea of pressure acting as a teratogenic factor in embryonic
or germinal life is clearly indicated, even if not expressly stated in so
many words. Further, the absence of juxtaposition of the ova is
afterwards adduced to explain why double monsters are less common
in serpents, and why they do not occur in bees and wasps where the
ova are in separate alveoli. Monstrosities, Aristotle continued, are
more common in the animals that have several young ones, and
herein lies the reason why they are less often met with in the human
subject. The idea of pressure acting at any rate as a secondary
factor in the production of monstrosities is, therefore, at least as
old as Aristotle ; and it is impossible to read the passages quoted
without again wondering at and admiring the extraordinary insight
into the deepest mysteries of Nature which the peripatetic philosopher
possessed so many centuries ago.

Aristotle's writings indicated the high-water mark of teratological
knowledge, both human and comparative, for more than two thousand
years ; and even at the present day and with all modern methods of
research, including the microscope, at our command, we have not,
after all, advanced the subject very far beyond the point at which it
was then left. Observations have indeed been multiplied until it has
become a task of some magnitude to collect together all the recorded
cases of any one kind of monstrosity, but I doubt whether we
really see much farther into the very heart of the matter than did
Aristotle.

In the Hippocratic and Aristotelian writings are found the roots,
so to speak, of the belief in the teratogenic power of traumatism and
pressure acting upon the organism yet in the womb ; but more than
two thousand years had to pass by before the tree blossomed out into
the full growth of the modern theory of the production of malforma-
tions by mechanical influences. Pare (Dcs monstrcs tant tcrrcstres que

PRESSURE IN TERATOGENESIS 131

marins, Paris, 1573. Translation by Th. Johnson, p. 597, London,
1678), among the many causes of monstrosity which he enumerated,
placed narrowness of the womb and external violence : in writing of
the sixth cause he says, " We are constrained to confess by the event
of things, that Monsters are bred and caused by the straitness of the
Womb ; for so Apples growing upon the Trees, if before they come to
just ripeness they be put into strait Vessels, their growth is hindred.
So some Whelps which Women take delight in, are hindred from any
further growth by the littleness of the place -where they are kept."
Allusion is then made to the distortion of the roots of trees by a
" Flint or any other solid body," and it is affirmed that since " by the
opinion of Naturalists, the place is the form of the thing placed, it is
necessary that those things that are shut up in straiter spaces,
prohibited of free motion, should be lessened, depraved, and lamed."
To continue, Fare's seventh cause is the " ill-placing " of the pregnant
woman : " They which sit idlely at home at the time of their being
with Child, as cross-legged, those which holding their heads down, do
sew or work with the needle, or do any other labour, which press
the belly too hard with cloaths, breeches, and swathes, do produce
children wry-necked, stooping, crooked, and disfigured in their feet,
hands, and the rest of their joints." In confirmation hereof Pare
adds the illustration of an infant with crooked hands and feet so
produced. The eighth cause is injury to the mother " by reason of a
stroke, fall from on high, or the like occasion " through which the
fcetal bones are affected or a vein opened, with the result that the
infant loses nourishment and is small or altogether monstrous.

In the views of Pare will be recognised those of Hippocrates, with
some picturesque amplifications but with a loss in scientific exactness.
In them, as in the Hippocratic conception, the underlying theory is the
action of pressure either temporary and violent, or long-continued but
less severe, upon the uterine contents ; the cause of the pressure may
be the uterus itself, the parts surrounding the uterus, or extra-
corporeal agencies. Pare's opinions are closely allied to those of
Lemnius (Be miraculis occultis natures, lib. i. c. viii. p. 34 ; lib. iv.
c. vii. p. 380, Prancofurti, 1628), for he enumerated (along with
faults in the materia, the influence of the mother's imagination, and
the effect of the stars) defects in the constitution of the uterus ; and
he compared the growth of the foetus in utero to the casting of a
metal into a particular form, when faults in the mould as well as in
the material moulded would lead to deformities. M. Weinrichius (Be
orta monstrorum commentarius, c. xiv. 127, 132, Breshe, 1597), also,
among the causes which he named, placed uterine pressure ; for we
read that if the uterus cannot distend suitably to accommodate the
foetus (" quod si igitur uterus in figuram f cetui convenientem dilatare
se non potest ") then a monstrosity results. The comparison with
apples in a bottle is adduced as proof ; and reference is made to the
Hippocratic doctrine of the mutilating effect upon the foetus of
violence happening to the mother. In the work of Ulysses Aldro-
, vandus (Monstrorum historia, p. 380, Bononia?, 1642), prepared by
Ambrosinus, the narrowness of the uterus is spoken of as one among

132 ANTENATAL PATHOLOGY AND HYGIENE

several teratogenic causes — " ratione angustiae receptaculi foetus,
monstra nascuntur."

F. Licetus (Be monstris, lib. ii. c. v. p. 67 ; c. xvi. p. 100 ; c. xx.
p. 108; c. xxiv. p. 121; c. xxxi., xxxii., and xxxiii. pp. 136-138;
c. xxxix. p. 144; c. xli. p. 145, Patavii, 1668), writing about a
hundred years after the time of Pare, dealt fully with the various
ways in which pressure may act upon the foetus ; at this we need
show no surprise, for he enumerated most carefully every known or
suggested factor in teratogenesis. The uterus may be too narrow to
allow a perfect infant or perfect twins to be formed ; and the narrow-
ness may be due to a tumour occupying part of the cavity and
attached to the uterus, or to the membranes, or to the placenta ; or it
may be caused by an extrauterine growth ; or, again, it may result
from tight-lacing. Through violence affecting the mother the parts
of two foetuses may be driven together and caused to unite. The
presence of a " mole " in the uterus may also have a deforming effect
upon the foetus. In view of recent discoveries it is specially note-
worthy to find in the teratogenic system of Licetus a prominent place
given to tightness of the membranes enveloping the foetus.

Here is an interesting observation from the close of the seven-
teenth century. In 1680 united twins were born in the town of
Ebeleben, and B. Scharfius (Miscell. Acad. not. curios. (1683), Dec. ii.
Ann. ii. obs. cii. p. 254, Norimb., 1684) unhesitatingly ascribed their
production to abdominal constriction applied on the part of the
mother to conceal her pregnancy. He entitled his observation
" Monstrum a constrictione," and warned pregnant women against
such practices lest they should, like the unfortunate of Ebeleben,
bring forth monsters. L. Schrock, however, in a Scholium following
Scharfs paper, threw grave doubts upon the explanation, and pointed
out that many women illegitimately pregnant tight-laced, and yet
brought forth normal infants, and that in cases like the twins of
Ebeleben the history of abdominal compression was generally want-
ing. Further, he enforced his argument by referring to the absence
of any cicatrix indicating the point of fusion, to the presence of
normally formed parts in the conjoined twins, and to the want of any
sign of such hemorrhage as ought to have occurred when the two
hearts violently became one (!). Schrock himself regarded the pheno-
menon as due to the presence of two cicatriculre or " treads " in the
ovum.

Early in the next century (the eighteenth), the doctrine of
monstrosity by mechanical causes came prominently to the front
in connection with the famous series of discussions in the Parisian
Academy of Sciences. In 1706 Du Verney (Mthn. de V Acad. roy. des
sciences, Ann. 1706, p. 431, Paris, 1731) described an interesting case
of double monster (recognisably an instance of diccplicdus lecanopagus,
as we may call it at the present day), and stated that he did not
regard such a phenomenon as the result of chance or of a blind
formative power, but as the exhibition of a divine intelligence making
plain the constructive resources of the Creator. Eighteen years
later Lemery (ibid,, Ann. 1724, p. 44, Paris, 1726), also in connection

PRESSURE IN TERATOGENESIS 133

with a case of joined twins (classifiable by us now as dicephalus
somatopagus), argued against the idea of germs originally and essen-
tially monstrous as propounded by the philosopher P. S. Eegis (in
1690) and repeated by Du Verney; with considerable skill he stated
the case for the pressure-theory without at all clearly indicating the
kind of pressure referred to. He found an explanation of the
structural peculiarities of double terata in the action of a moderate
or a strong pressure acting in this or that direction. A moderate
compressing force, he thought, caused the fusion of external parts,
while a stronger one was needed to unite the internal organs of the
two foetuses. In Italy, also, Vallisneri (Istoria delta generazione, p.
250, 1721) had spoken of an external compressing force in connection
with absent limbs or parts.

Some years later Winslow (Mim. de I' Acad., Ann. 1733, p. 366,
1735 ; Ann. 1734, p. 453, 1736) appeared as a supporter of I)u
Verney ;s notion of original monstrosity, and consequently as an
opponent of the accidental theory of origin of Lemery. This writer
discussed in order a large number of reported cases of malformation
and monstrosity, pointing out that in them all there existed grave
technical difficulties preventing the acceptance of a mechanical and
accidental teratogenic cause. In a very special way did these diffi-
culties exist in the cases of transposition of the viscera, double
uterus, Polydactyly, supernumerary ribs and vertebra?, and extra-
ordinary arrangements of muscles. Stimulated doubtless by Win-
slow's criticism, Lemery (ibid., Ann. 1738, pp. 260, 305, Paris, 1740 ;
Ann. 1740, pp. 109, 210, 324, 433, 517, and 607, Paris 1742) now pub-
lished a series of exhaustive memoirs on the two competitive theories
of the causation of monstrosities ; in these he endeavoured, inter alia,
to discredit by theological arguments the idea of germs originally
monstrous, while he supported his own opinions as to their origin from
mechanical influences acting upon germs originally normal by an
appeal to many anatomical peculiarities found especially in double
terata. He wrote much about pressure, but did not very clearly
define what he meant thereby, although in one passage at least he
appears to have been referring to that produced by uterine contrac-
tions. Winslow (ibid., Ann. 1740, p. 586, Paris, 1742), in connection
with the description of an acephalic twin, took up the matter again,
reiterated his views, and maintained that, far from depreciating the
attributes of the Creator of the Universe, they rather rendered
homage to His all-powerful sovereign liberty (" a sa toute-puissante
liberte souveraine "). He contributed two other papers (ibid., Ann.
1742, p. 91; Ann. 1743, p. 335, Paris, 1745) on the subject; and
doubtless Lemery would again have replied had not death seized
him and so closed in an emphatic and certain manner this long
controversy.

A perusal of the various memoirs (a work, by the way, of no little
magnitude, yet of intense interest to those who are concerned about
such matters) makes it clear that, on the whole, Lemery had the best
of the argument ; for Winslow certainly resiled from certain positions
originally occupied by him, and this too notwithstanding the fact

134 ANTENATAL PATHOLOGY AND HYGIENE

that he had that highly coveted advantage, the last word. It was a
memorable controversy, " nne longue et solennelle discussion " Saint-
Hilaire called it, and not without cause ; it showed now and again a
tendency to wander away from teratology into teleology, and it was
not altogether free from the odium theologicum ; and it is interesting
to us now chiefly from the light that it throws upon the theory of
monstrosity by pressure and traumatism as it was held in the first
half of the eighteenth century. It was useful because it stimulated
the gathering together and preservation of many records of individual
cases of malformation ; but it ended in a somewhat disappointing
fashion, as indeed it could only be expected to do, the state of know-
ledge upon Embryology being at that time what it was. For they
who took part in or listened to the discussions were hampered by
their ignorance of the possibility that monstrosities might be due to
accidental causes acting before impregnation or, at any rate, before
the rudiments of the embryo were laid down. It was not till 1759,
or sixteen years after the death of Lemery, that the idea of the pre-
existence of germs, normal as well as monstrous, was discredited
through the labours of Caspar F. Wolff and the establishment of the
theory of epigenesis. At the same time, had Lemery and Winslow
explored the writings of Harvey, and especially his Exercitationes de
Generatione Animalium, they would have been equipped, long before
Wolff's time, with the knowledge of the great law that the complex
animal arises from a relatively homogeneous germ by gradual dif-
ferentiation or epigenesis ; to both Lemery and Winslow this know-
ledge would have been of no little value, and might, also, have greatly
clarified some of the dark parts in their disagreements. (If my
readers have doubts about Harvey's claims in these embryological
matters they ought to peruse W. K. Brooks' convincing arguments
as set forth in the Johns Hopkins Hospital Bulletin (vol. viii. p. 167)
in 1897.)

While it is true that the Lemery -Winslow discussion in the
French Academy of Sciences as well by its magnitude as by the
personnel of the contestants dwarfed all contemporaneous writing on
the pressure-theory of teratogenesis, the existence of such writings
must not on that account be forgotten. The authors of these contri-
butions took sides in the great controversy, and one of them (A. von
Haller) even intervened in the debates. Haller at first supported
Winslow ; but some years later, when the controversy had passed
from France into Germany, he admitted that there was much force
in several of the arguments of Lemery, and conceded that anence-
phaly might arise from some external injury ("ab aliqua injuria
externa "). Haller 's views are to be found in several scattered papers
published between 1735 and 1745 ; they are gathered together along
with his later opinions in his treatise "DeMonstris" (Opuscvla sua
anatomica, 1751). A passing reference is all that need be made to
the writings of other contemporary scientists. Vallisneri (Istoria
delta generazione dell' uomo e degli animali, p. 249, Venezia, 1721),
for instance, affirmed that double terata resulted from the union of
the originally separate ova ; E. Sandifort (" Anatome infantis cerebro

PRESSURE-THEORY 135

destituti," p. 66, in his Opuscula anatomica, Lugd. Batav., 1784)
ascribed foetal anencephaly, in a case known to him, to the falling of the
mother backward downstairs during her pregnancy ; and Fabri (quoted
by Taruffi, Storia <ldl<t teratologia, i. 254, 1881) instanced spasmodic
uterine contractions due to violent mental emotion or hysteria as
possible compressing and deforming forces. There were others,
however, who showed a less complete attachment to the mechanical
theory of teratogenesis: Riviera (quoted by Taruffi, op. tit.), in
describing a cyclops foetus, opposed the pressure-theory, arguing that
the regularity of the arrangement of parts and the absence of marks
of compression militated against it ; and S. T. Sommerring (Abbil-
dungen und Beschrcibangen ciniger Misgeburtcn, p. 35, Mainz, 1791)
did not agree with Siebold in his belief that tight-lacing was a
sufficient cause for foetal encephalocele.

In the closing years of the eighteenth century there was a distinct
diminution in the popularity of the pressure-theory among medical men,
a change in opinion to which Morgagni in great measure contributed
by his promulgation of the view that f oetal diseases (e.g. accumulation of
Huid in the body cavities) are the direct causes of monstrosities. The
nineteenth century, however, witnessed a great revival and develop-
ment of the idea that antenatal mechanical action had an important
influence in teratogenesis, and in its scientific form the doctrine is
to-day regarded by many teratologists as adequate and satisfactory,
bid, let it be observed, only in its scientific form. It did not by any
means gain acceptance at once, for J. F. Meckel (Handbuch der
pathologischen Anatomic, Bd. i. pp. 21, 29, etc., Leipzig, 1812) denied
the teratogenic efficacy of mechanical causes, among which he placed
irregular position, the pressure of one foetus on another, and abnor-
malities in the form of the uterus ; he repeated many of the argu-
ments of Winslow, instanced the cases of twins, one of which was a
foetus compressus but otherwise normal; and he pointed out that
mechanical agencies afforded no explanation whatever of hereditarily
transmitted structural anomalies. Other writers followed the
example of Meckel; nevertheless the theory grew in importance,
became more and more elaborated, and constantly added to the
number of its adherents (vide Hans Koch's Dissertation, Ucbcr die
auf mcchanischer Ursache beruhendcn congenitalen Dcformitaten,
Koln, 1895).

In order, however, that we may understand the history of this
teratogenic theory in the nineteenth century, it is necessary clearly
to recognise certain preliminary facts. It must, for instance, be
noted that the pressure-theory as stated by one observer differed
widely from that enunciated by another : there is, in one sense, all
the difference in the world between the idea of tightly laced corsets
causing foetal malformations and the doctrine of the production of
monstrosities by the constricting force of the umbilical cord, of bands
of lymph, or of amniotic membrane. One may hold the latter
theory without swearing allegiance to the former. The idea of
pressure is present in all these hypotheses, but the kind of pressure
meant and its mode of action differ widely. Again, it has to be

136 ANTENATAL PATHOLOGY AND HYGIENE

borne in mind that while some have regarded pressure as the
ultimate and only cause of teratological formations, others have
rather looked upon it as one among many causes, or as the inci-
dental circumstance which brings into play other factors, such
as intrauterine inflammations, arrested developments, and the like.
For these and for other reasons it will be necessary to consider the
various pressure - theories separately, each one on its own merits.
For this purpose I have attempted to classify them provisionally
into two primary groups, according as the pressure is supposed to act
upon the foetus from without or from the inside of the mother's
abdomen. In each group there are two or three sub-groups, corre-
sponding to the exact nature and sphere of action of the pressure in
operation. Thus, in the first group are placed injuries to the
mother's body in its various parts, the continuous pressure exerted
by tightly laced stays, and blows upon or wounds of the abdomen ;
while in the third group are found the constricting pressure of the
umbilical cord, etc., the compression of one fcetus by another foetus
or by one of its own limbs, and tightness of the amniotic membrane.
Here is the arrangement in a tabular form.

Pressure ^Theories in Teratogenesis.

I. External Pressure — ■

(a) General Maternal Traumatism, e.g. falls and blows.

(b) Injuries to and Pressure affecting the Maternal Abdomen,

e.g. corsets.

II. Internal Pressure —

(a) Intra-abdominal and Intra-pelvic.

1. Abdominal and Pelvic Tumours.

2. Pelvic Contraction.

3. Narrowness and Malformation of the Uterus.

(b) Intrauterine —

1. Uterine Tumours.

2. Twin Foetus.

3. Foetal Limb.

4. Umbilical Cord.

5. Amniotic Bands and Amnion.

Some space may be devoted to each of these variants upon the
pressure-theory in teratogenesis.

Maternal Traumatism (Extra-genital).

The first of the pressure-theories which I shall describe regards
it as possible that a maternal traumatism, a blow or other injury,
occurring outside the genital sphere, may produce a monstrosity or
at least a malformation. For instance, in the year 1895, I was

1 It is to be observed that the word " pressure " is used in the wide sense which
includes the notion of transient but excessive pressure as seen in a blow or fall.

EXTRA-GENITAL TRAUMATISM 137

examining a child, three years of age, who had a congenital contracture
of the index ringer of hoth hands and talipes calcaneus of the left
foot ; the mother at once volunteered as an explanation of her child's
deformities the information that three weeks before labour (which
occurred at the seventh month) she had fallen downstairs. Again,
some years ago, a medical man sent to me for dissection an anence-
phalic fcetus, and stated that the mother had knocked her head against
a gas bracket during the early part of her pregnancy ; he wished to
know if there could be any connection between the maternal trauma-
tism and the condition of the foetal head.

Eeports, such as the two cited above, are far from uncommon in
medical literature, but the medical man does not always or indeed
often accept the explanation advanced by the mother of the mal-
formed infant. He commonly records the statement without
expressing an opinion as to its worth. Let me here briefly allude
to some of these alleged teratogenic causal conditions.

In the first place, several such statements are to be found in the
Transactions of the Edinburgh Obstetrical Society. In 1852, in con-
nection with Fenton of Alyth's case of anencephalus (Edinb. Month. J.
Med. Sc., xv. 170, 1852), it was noted that in early pregnancy the
mother had strained herself in lifting a box ; in 1882, A. K. Simpson
in a Maternity Hospital lieport (Trans. Edinh. Obst. Soc, vii. 115,
1882) referred to an instance, also of anencephaly, in which there was
much lifting of heavyweights in pregnancy ; and in a paper which
I myself communicated in 1892 (Trans. Edinb. Obst. Soc, xvii. 99,
1892) were recorded cases in which a sprain of the mother's ankle
was the alleged cause of talipes varus in the infant, in which a dog
bite on the mother's leg was looked to as the origin of a scar on the leg
of the foetus, and in which a blow on the mother's chest was followed
by the birth of a child with a nsevoid patch in the corresponding
region.

Some cases from other sources may also be noted to illustrate
different types of this belief. J. S. Beale (Lancet, ii. for 1863, p. 27)
saw an infant born with a black eye whose mother had been struck
over the eye eight hours previous to her confinement. G. E.
Jeaffreson (Lancet, ii. for 1866, p. 568) met with an example of
anencephaly in which the mother at the second month of her
pregnancy had suffered from a severe injury to the head. E. A.
Martin (Uebcr Sclbstamputation bcim Fotus, Jena, 1850) recorded
the birth of a child whose left arm was represented by a short
stump and whose mother had had a fall in pregnancy ; the separated
part of the limb was expelled with the placenta. F. Ormrod (Lancet,
i. for 1875, p. 393) gave the history of a pregnancy in which the
mother had the fingers of her right hand crushed in a door and her
child showed defective development of the fingers of the right hand.
At a meeting of the Eoyal Academy of Medicine in Ireland (Novem-
ber 25, 1882) M'Ardle described a case of spina bifida and other
malformations in a foetus, ascribed to the fact that the mother when
three months pregnant had been in a railway accident and had got
her back injured.

138 ANTENATAL PATHOLOGY AND HYGIENE

Such records might be multiplied, but with this small fasciculus
of illustrative instances the reader may well he satisfied. If he
will scrutinise them it will soon be apparent to him that here
after all we have no new theory of teratogenesis but only an old
one masquerading in a new garb ; it is simply the doctrine of
maternal impressions in a slightly veiled form. The impression,
it is true, is physical as well as mental, but the idea of its
modus operandi is much the same ; the scientific mind finds it
scarcely less difficult to accept the notion of a physical than of
a purely mental influence. Nevertheless, the theory is of interest,
for it serves as a link joining together the theories of the past
with those of the present. Save for this purpose it might be
safely allowed to pass into the oblivion of the immemorabilia of
our subject.

At the same time there is one sense in which traumatism outside
the genital sphere may possibly be potent for the production of
deformity. If it be accepted that falls and blows may by a process of
contre-coup lead to the occurrence of foetal fractures, wounds, and
dislocations, then it may also be believed that the injured parts may
during healing become distorted and even malformed. Dieterich
{Med. Cor.-Bl. d. vmrttemb. arztl. Ver., Stuttgart, viii. 5, 1838), at
any rate, reports a case in which a fall by the mother upon her
back was apparently the cause of wounds of the foetal head and
fracture of the forearm. It Avould seem that this result would be
more likely to follow if there were little or no liquor amnii in
utero, although in Dieterich's case the membranes were unruptured
at the time of the accident. The effect of foetal fractures in the
production of malformations falls, however, more naturally to be
considered with the next theory, that, namely, which regards pres-
sure applied directly to the maternal abdomen as a teratogenic
agency.

What Morgagni, writing in the eighteenth century, thought
about the theory of traumatism outside the genital sphere may be
gathered from the following extract from his great work (Be Scdibus
et Causis Morborum). I quote from Alexander's translation (vol. i.
p. 272, Letter xii. article 16, London, 1769). Morgagni is speaking
of a child with spina bifida. " But to return to our boy : not being
ignorant of the excessive credulity of other people, at some times,
I ask'd, however, from the mother, whether, as she was in the flower
of her life, and both she and her husband healthy, she had ever
fall'n on her back, while she was pregnant with this child, which was
her first-born, or had received a blow in that part by any means, or
had dreaded, or been frighten'd at any thing ; or, in fine, whether
she had long'd for any thing : all which she immediately and ex-
pressly answer'd in the negative ; although afterwards, as the custom
is with these weak women, it came into her mind, that she had
wish'd for a fig." Morgagni, it is evident, was no believer in impres-
sions, mental or physical.

MATERNAL TRAUMATISM 139

Injuries affecting the Mother's Abdomen.

A less incredible theory than that just considered is founded
upon the action of external pressure upon the mother's abdomen
during pregnancy. Such pressure, it may be reasonably supposed,
bearing as it does more directly upon the organ which contains the
young foetus, will be more likely to have injurious effects upon it
than injuries to the head or limbs of the mother. The abdominal
pressure may be long-continued and not excessive, or it may be
short, sharp, and severe.

The practice of tight-lacing for the concealment of pregnancy
suggests itself as a form of abdominal compression likely to have
teratogenic effects. It appealed, as we have seen, very strongly to
Scharfius (loc. cit.), and in the nineteenth century also it had its
advocates. Etienne Geoffroy Saint-Hilaire (" Sur un foetus no a terme,
blesse dans le troisieme mois de son age, et devenu monstrueux a la
suite, d'une tentative d'avortement," Mem. Sue. mid. d'emtdat. de Paris,
ix. 65, 1826) described very graphically the case of " Julie," who,
finding herself pregnant, and desiring to conceal the fact from her
sister with whom she lived, endeavoured to bring her pregnancy to
an end by wearing very tight corsets. " Elle se compose un corset
larde de buses epais ; elle se plastrone le ventre de maniere a placer
au dehors une force vive, ri'agissante et destructive des developpemens
interieurs." Nevertheless she arrived at the full term and gave
birth to an infant with a monstrosity of the head, to which Saint-
Hilaire gave the name thlipscnc<:p]i<dc (" cerveau ecrase "). Isidore
Geoffroy Saint-Hilaire (ffistoire des Anomalies, iii. 534, Paris, 1836)
evidently accepted his father's views concerning the teratogenic
power of tightly laced stays ; but he dwelt more upon the effects of
blows and falls upon the abdomen, and to this subject I shall refer
immediately. Hohl (Die Geburten missgcstalteter, kranker und tocltcr
Kinder, p. 102, Halle, 1850) had much difficulty in finding arguments
in support of the maleficent effect of the corset : he reported a case
of tight-lacing in which the foetus was born with deformed extremities ;
but there was also hypospadias, and he was puzzled to account for
the latter malformation on the corset hypothesis.

It is somewhat striking that the pathologists and teratologists
who followed the Saint-Hilaires do not seem to have been specially
impressed with the idea of corset-pressure in teratogenesis. Bouvier
{Bull. Acad, de mt!d,, Paris, xviii. 355, 389, 1852-53 ; ibid., xx. 1106,
1854-55 ; Diet, encyclop. d. se. mid., Paris, xx. 745, 1877), for instance,
in his several articles upon the medical aspects of the corset, refers
to its effects in producing abortion and causing imperfect develop-
ment of the foetus, but he gives no details of cases, and indeed states
that he had watched three instances of tight-lacing in pregnant
women, and in all of them there were no ill consequences at all.
It is also a striking fact that the writers who have so emphatically
condemned the corset as " the evil of the age," and the active cause
of pelvic congestion, of defective secretion of bile, of dyspepsia and

140 ANTENATAL PATHOLOGY AND HYGIENE

the like, have little or nothing to say of its teratogenic influence
upon the unborn infant. One is driven to the conclusion that its ill
effects, in this direction at least, are few ; perhaps the presence of
the liquor amnii is the great safeguard of the foetus.

There is, however, another form of abdominal pressure which
must be referred to ; I mean that produced by falls or blows, by
direct contusion or by contrc-coup. Several kinds of malformation
and monstrosity have been freely ascribed to injuries affecting the
mother's abdomen in the early months of pregnancy, and many
instances from medical literature might be cited as proof. Let me
here simply present a small fasciculus of illustrative cases ; it will
suffice. Roux (in Breschet's article, Arch. gcsn. dc med., xxvi. 38,
1831) described a case of occipital encephalocele in an infant whose
mother had fallen downstairs at the seventh month of pregnancy ;
Bayer (Compf. rend. Soc. de biol., 2 s., ii. 103, 1856) reported how a
mulatto woman at the sixth week of pregnancy had been ill treated
by her husband and had given birth to an anencephalic foetus whose
malformed head was adherent to the amnion covering the placenta ;
C. C. Werthheim (Monatsschr. f. Geburtsh, ix. 127, 1857) met with a
case of twins, one of which had died early in pregnancy, while the
other showed a large spina bifida with absence of the sacral and
coccygeal vertebra?, and the mother had received a severe kick in
the left inguinal region from her husband some weeks before the
mid-term of her gestation ; instances of exstrophy of the bladder
following upon a fall and a blow by a cow's horn on the abdomen
respectively were reported by A. Freund (Arch. f. Gynaek., iii. 381,
1872) and James Mowat (Med. Essays and Observ. Soc. in Edinburgh,
iii. 276, 1735); an example of anterior encephalocele was attributed
by R. Hein (Ztschr.f. Geburtsh., vi. 352, 1881) to a jump which the
mother made from a carriage when she was three weeks pregnant ;
and W. Graeffner (Deutsche Arch, f. Idin. Med., xxxiii. 95, 1883)
related how a woman, a few days before her confinement, fell on a
rock, and her infant was born with a deformity of the sternum
(sternal cyphosis or Trichtcrbrust). Histories somewhat similar to
these given above were related by T. G. A. Eoose (Diss, inaug.,
Gottingas, 1793), by P. Vannoni (Gazz. med, ital.fedcr. Tosc,, 2 s., i.
394, 1850), and by 0. Werler (Diss, inaug., Berlin, 1881). The Saint-
Hilaires, father and son, expanded this theory of abdominal trau-
matism, and endeavoured to give to it a degree of scientific exactness.
They maintained (Histoire des Anomalies, iii. p. 534, 1836) that
two varieties of deformity of the head (nosencephaly and thlipsen-
cephaly) resulted from it, and that when the violence occurred at
the fourth month the former would follow, while if it took place
at the third month the latter monstrosity would be developed ; the
earlier in pregnancy the traumatism took place the further removed
from the normal would the infant be. Vrolik (Todd's Cyclopaedia of
Anatomy, iv. 943, 1849-52), also, and Forster (Missbildungen des
Menschcn, p. 5, Jena, 1865) have admitted the possibility of the
teratogenic potency of injuries to the mother's abdomen in pregnancy.
In a case published by Kriwsky (Monatschr. f. Geburtsh. u. Gynaek.,

INTRA-ABDOMINAL PRESSURE 141

vi. 895, 1900) the mother of an infant with exoniphalos and
deformed limbs had to spend twelve hours daily in a squatting
posture at her work.

It must, I think, be admitted that abdominal pressure and trau-
matism have some influence in the production of monstrosities and
malformations ; at the same time I do not regard it as great. What I
have said in the first part of this Manual (pp. 177, 393) regarding fa?tal
fractures, dislocations, and wounds, and their origin from external
violence, may, I believe, be repeated concerning the genesis of
monstrosities. There is good evidence that traumatism in early
pregnancy is sometimes followed by the birth of an abnormal infant,
just as there is good evidence that blows and falls in later pregnancy
may be followed by the birth of a foetus showing fractures or
dislocations, or both, — E. Gurlt's article (Monatsschr. f. Geburtsk.,
ix. 321, 401, 1857) with its long list of literature is sufficient to
demonstrate this, — but in many cases no such history of injury is
forthcoming, and in many others there is a record of injury but no de-
velopment of morbid results. Doubtless the amount of the liquor amnii
present in the uterus, the existence of abnormal fragility or abnormal
resistance of the foetal bones and tissues, the relaxed or contracted
condition of the abdominal muscles at the time of the injury, and
many other modifying or determining circumstances have an effect
upon the result, and ought, if we had knowledge of them, to be taken
into account. It has to be borne in mind, also, that when we have
admitted the teratogenic power of abdominal traumatism we have
by no manner of means defined or explained its mode of action.
Does it act directly upon the embryo, crushing or wounding it in
any part, with the result that the malformed part is the immediate
consequence thereof, modified and masked by the subsequent growth
of that part and of the surrounding structures ? Does it not rather
cause a morbid state of the uterine walls, placenta, or amnion, which
in process of time reacts upon the developing embryo or foetus ?
Both are possible, but the latter seems the more probable.

Intra-abdominal Pressure.

Intra-abdominal pressure may conceivably act upon the foetus in
utero, and it may be of two kinds. It may consist in a compression
of the whole uterus in virtue of a pelvic contraction (due to
deformity of the pelvis or to neoplasms inside it) or of an abdominal
tumour, or it may lie a purely uterine pressure caused by narrow-
ness or by malformation of the uterus itself. We have little or no
evidence that pelvic contractions or abdominal and pelvic tumours
tend to produce malformations of the foetus in utero (P. Vannoni,
loc. cit.), although there are some indications that these morbid
states may lead to fractures of some of the bones of the unborn
infant (J. Vilcoq, " Fractures Intra-uterines," These, p. 19, Paris,
1888), and so perhaps to deformities resulting therefrom (e.g.
amputations, vide J. Y. Simpson, Dublin J. Med. and Chem. Se., x. 239,
1836). Jules Platon (Marseille mid., xxxiv. 785, 1897), however,

142 ANTENATAL PATHOLOGY AND HYGIENE

seems to ascribe pseudencephaly to the pressure of two uterine
fibroids, but the evidence is not convincing.

That uterine narrowness may be the cause of foetal anomalies
is a firmly established belief ; and the narrowness may be the result
of contractions of the uterine muscle, or it may represent a real
diminution of the size of the cavity of the womb from the presence
of a malformation, such as is seen in the uterus duplex or the
uterus septus. Here, again, in the first of these suppositions, the
teratologist finds himself in the presence of that almost ubiquitous
theory of the maternal imagination, for those who desired to give a
scientific aspect to the " impression " doctrine did so by suggesting
that the perturbation of the mother's mind translated itself into
powerful contractions of the mother's uterus, and that these caused
a temporary narrowness of the uterine cavity, with teratogenic
results. But, apart from the " maternal impression " theory, many
writers hold that persistent or oft-recurring contractions of the uterus
in pregnancy have malforming effects upon the foetus. 0. Hourlier
("Mortdu foetus," These, Paris, p. 45, 1880), for instance, believed
that they might be the cause of foetal deformities, dislocations, curva-
tures, and fractures ; and many others have looked to them as the
determining factors in the production of club-foot and other anomalies
of the extremities (L. H. Ormsby, Deformities of the Human Body,
p. 22, 1875 ; E. W. Parker and S. G. Shattock, Trans. Path, Soc.
Lond., xxxv. 423, 1884; H. W. Berg, Arch. Med., viii. 226, 1882,
and Med. Bee, xxxii. 509, 1887; A. Schanz, Ztschr.f. ortliopdd. Chir.,
v. 9, 1897 ; and others whose names are in the Text-Books of
Surgery and Orthopedics and in Literature Lists). Some of these
writers {e.g. Parker and Shattock) are not, it is true, at all explicit
as to the meaning of the term " uterine pressure " which they use so
freely ; but it may be inferred that pressure due to a diminution in
the amount of space inside the uterus from contractions of its
muscular wTalls has been most prominent in their thoughts. It is
supposed that the special variety of club-foot produced may be
explained by the date in antenatal life when the pressure became
a constraining force ; the foot is fixed in the position then natural
to it, and the pressure being maintained, the attitude becomes per-
manent ; and thus talipes varus is due to an earlier compression
than is talipes calcaneus. Opitz {Ztschr.f. Gclmrtsh. u, Gynak., xl.
520, 1899), also, has described a case of hyperextension of the knee
in a foetus which was the offspring of a woman with a uterus
duplex, and has traced back the deformity to narrowness of the
pregnant side of the uterine cavity; E. Zuckerkandl {Allg. Wien.
med. Ztg., xxiii. 247, 1878) attributed the small size of the head,
the condition of the brain, and the deformed state of the feet of a
foetus to uterine pressure compressing the head against the chest;
P. L. Gardini {Ann. di ostct. e ginec., xxi. 705, 1899) has recorded a
case of pregnancy in a bilocular 'uterus in which the foetus was
anencephalic, but without suggesting any relation of cause and effect
between the two states; and F. Winckel {Milneln n. med. WcJtnschr.,
xliii. p. 390, 1890) has very clearly stated his belief that a narrow

UTERINE PRESSURE IN TERATOGENESIS 143

uterine horn (in a case of uterus bicornis bicollis) was the cause of
double pes varus in the foetus.

There are, however, not a few difficulties in the way of a full
acceptance of the theory of the effect of uterine pressure in the pro-
duction of even the minor malformations. E. J. Chance (Bodily
Deformities, p. 139, 1862), under the chapter-heading " Can Position
in utero produce Deformity ? " referred to many of these difficulties,
and dwelt specially upon the admitted coexistence of deformities
and excess of liquor amnii as being a very strong argument against
the pressure-theory ; while Keller {Arch. f. Gfynaek., lxvii. 477,
1902), among recent authors, has been compelled to recognise the
serious problem raised by the occurrence of hydramnios in association
with monstrosities. It is practically inconceivable that the pressure
of the uterine walls can have a deforming effect upon a foetus
surrounded by an excess of liquor amnii. Further, the same
deformities have been met with in cases of oligohydramnion (vide
the first section of this Manual, p. 407) as in those of hydramnios.
Of course, it may be argued that the state of scarcity of liquor
amnii preceded that of excess, and that the foetal deformities were
produced by uterine contractions in action during the former con-
dition; but there is no actual proof of this, although E. Porro's case
(Gazz. med. di Lombard., s. 6, vi. 393, 1873), in which the mother's
abdomen was first too small and later too large, and in which there
were malformed twins, might seem to lend some support thereto.

It is doubtful whether the authors who have written with such
facile pens concerning uterine pressure, and its effects on the uterine
contents, have seriously considered the problem in biological physics
thereby raised. The conception present in their minds seems to have
been that of a rigid case with easily moulded contents ; but, as a
matter of fact, the uterus is not a rigid case but an organ which
adapts itself both to its external surroundings and to the organism
within it. Frozen sections have shown it moulded to the vertebral
column, and clinical observations have demonstrated how it changes
in shape with changes in the presentation of the foetus (e.g. in shoulder
cases). The presence of the liquor amnii also introduces a factor
into the problem which increases its complexity, and appears greatly
to weaken the theory of uterine pressure in teratogenesis ; for the
existence of this intrauterine fluid cannot but lead to the equable
diffusion of pressure, and so, it would seem, to the prevention of
localised deformities. For my own part, I believe that there is some
truth in the theory, more especially in regard to the minor malfor-
mations which may arise during the foetal period of antenatal life.
I have met with a case of genu recurvation in a female infant, the
child of a woman who in her previous pregnancy had a miscarriage,
followed by a pelvic abscess and consequent adhesions and cicatrisa-
tions ; and I think it possible that in this instance the uterus was
impeded in its proper gestational expansion and the contents com-
pressed. But I do not nail this theory to the mast, for while I
believe that there may be some morsel of truth in it, I am inclined
rather to consider that as a rule the uterine walls, even when con-

144 ANTENATAL PATHOLOGY AND HYGIENE

tracted, protect the foetus from malforming influences. Thus, in a
case which I reported some years ago {Trans. Edinb. Obst. Soc., xvii.
"24:2, 1892), a foetus was found partly expelled into the peritoneal
cavity through the rent in a ruptured tubal gestation sac, and it
showed multiple deformities (anchylosis of joints, etc.), which I
regarded as due to the adhesions which had formed between it and
the intestines of the mother. Actual adhesions between the intes-
tines and an extrauterine foetus are shown by Vrolik (Tab. ad illustr.
Embryogen., PL xvi., Amsterdam, 1849), in a plate taken from Cru-
veilhier's Anatomic pathologique du corps humain. H. M. Lufkin
(Amcr. Journ. Obst.,x\i. 289, 1900) also has recorded a case in which a
foetus from a tubal pregnancy seems to have lain and grown for six
months free in the mother's peritoneal cavity ; it showed malforma-
tions of the feet, hands, and head, which were looked upon as due to
" the pressure of the bony and soft structures of the mother upon
the unprotected babe."

Since I have referred to cases of tubal pregnancy, it may be
interesting to keep in mind that apparently the foetuses from such
ectopic gestations are not infrequently malformed. J. C. Webster
(Ectopic Pregnancy, p. 112, 1895) states that various malformations
may be found, and particularises club-foot, dislocation of limbs,
exomphalos, and encephalocele ; he also noted instances of flattening
of the head, of compression of the face, and of amniotic bands
extending from the foetus to the sac wall. F. von Winckel, in a
beautifully illustrated monograph (Uber die Missbildungen von ch-
topisch entwickelten Erilchten unci deren Ursachen, Wiesbaden, 1902),
has given details of eighty-seven cases, thirteen of which were seen
by himself, in which the foetus in tubal pregnancy exhibited malfor-
mations. Cragin (Amcr. Journ. Obst., xli. 740, 1900), in an article
on the question whether a viable ectopic foetus is worth saving, gives
notes of a case in which the infant had a small umbilical hernia,
asymmetry of the head, and a dislocation of the left hip ; there was
very little liquor amnii in the sac. It may, perhaps, be concluded
that the walls of an ectopic gestation sac are more likely to exercise
injurious pressure on the foetus than are the uterine parietes. On
the other hand, it may be that the cases in which the foetus has
shown malformations have been those in which rupture of the sac had
occurred, and the infant passed into the abdominal cavity, where it
would be more subject to compression by the maternal structures.
In support of this opinion I note that J. W. Taylor (Extra- Uterine
Pregnancy, p. 81, 1899) states that " malformations are most com-
monly met with in children of abdominal or ventral pregnancies."
After all, therefore, we return to> the general conclusion that the
teratogenic effects of the pressure, either of the uterine walls or of
those of an extrauterine sac, are not clearly proven.

Intrauterine Pressure.

It is conceivable that the foetus may be acted upon by various
structures lying inside the uterus, and that the result of such action

GEMINOUS PRESSURE 145

may be the production of malformations or monstrosities. • For in-
stance, it has been supposed that tumours, e.g. polypi, might have
such a teratogenic effect. C. Taruffi (Storia delta Teratologic/,, i. 257,
1881) refers to a case published by C. M. Billard, in which a woman
suffering from a uterine polypus gave birth to an infant with a
cerebral hernia ; the pressure exercised by the polypus was supposed
to have been the cause of the localised destruction of the scalp, and
of the defect in the left temporal bone which permitted the hernia.
On turning to Billard's work (Traite des malddies des enfans, pp. 51
and 346, Bruxelles, 1835) I found, however, that there was no evi-
dence of the existence of a uterine polypus in the case referred to
(that of " Marianne Masse," obs. 79), and that it was merely a sup-
position of the writer that such a condition had been present.
Further, Billard added, as an alternative supposition, that the pelvis
may have been deformed, and that the growth of the uterus may
thus have been hindered. It does not seem to me, from a study of
the literature of the subject of fibroids in pregnancy, and from a
personal acquaintance with six cases in which this complication
existed, that there is proof that the foetus becomes malformed from
the pressure exerted upon it by the neoplasm. This form of intra-
uterine pressure may, I consider, be neglected as a factor in tera-
togenesis.

It is well known that in twin births one of the foetuses is not
uncommonly malformed, and the idea that his malformations have
been caused by the pressure of his companion in utero has com-
mended itself to some writers (e.g. Osiander and Zacchias, as cited by
A. Klautsch in his Inaugural Dissertation (Ueber ungleich entwickelte
Zivillitige, Halle a. S., 1892), and A. Forster in his Die Missbildungen
des Menschen, p. 5, 1865). In the first part of this Manual (p. 424)
I have referred to the fcetus com/pressus, by which is commonly under-
stood a twin that has died between the third and fifth months of
intrauterine existence, and has been flattened out and mummified
by the pressure exercised on it by the living and growing co-twin.
No one doubts that in these cases the flattening of the dead twin is
produced by the compression to which it is subjected by the living
one, although it is possible that the flattening may have preceded
the death and been in some measure the cause of it, — possible, but
not probable. It is not doubted, I repeat, that the flattening is due
to pressure ; but the question arises at once, Is the foetus compressus
teratological ? If it be not a teratological phenomenon, then its value
in connection with the pressure-theory in teratogenesis is largely
lost. If, in other words, it be not an instance of disordered embry-
ology, but merely a peculiarity in the pathology of foetal death, then,
from our present standpoint, it proves little or nothing. Now,
Ahlfeld (Die Missbildungen des Menschen, p. 34, 1880) evidently
regarded the foetus papyraceus (s. compressus) as teratological, and
indeed placed it in a special group among the double terata, and
Schatz (Arch. f. Gynael:, xxx. 367, 1887) has very fully described
the manner of its teratogenesis ; but a careful scrutiny of the facts

VOL. II. — IO

14G ANTENATAL PATHOLOGY AND HYGIENE

seems to me to show that it is the cause of the death of the foetus
that is teratological, while the flattening is the result of pressure
Drought to bear upon the dead foetus. The cause of the foetal death
would appear to be, in some instances at least, the asymmetry of the
placental circulations of the twins, an asymmetry which is to the
detriment of the one twin and to the advantage of the other. Of
course this can only be the case in uniovular (monochorionic) twins
with a common placenta and an anastomosis between the two circu-
lations. Now, as a matter of fact, the foetus papyraceus may be one
of binovular (dichorionic) twins (as in the three cases reported by
me, 159, 180, 190), and then this teratological mode of causation
cannot be accepted. Under these circumstances we must look for
the cause of foetal death in compression or torsion of the umbilical
cord or in placental haemorrhages, conditions which can hardly be
called teratological. We come, therefore, to the conclusion that in
some cases neither the flattened state of the twin foetus, nor the
cause that led to the death that preceded the flattening, is terato-
logical in the true sense of the word.

More light, however, has been thrown upon this subject by the
discovery of true compressed embryos (cmbryones papyracei, or, to
use a more classical form, embryo, papyracea). Until recent years the
compressed product of gestation had always been a foetus, i.e. had had
an intrauterine age of more than two months ; but lately Sundberg
(Upsala Ldharef. Forhandl, n. F. iii. 560, 1897-98) has described a
papyraceous embryo measuring only 29 mm. in length, and A.
Loennberg (Monatsschr. f. Gclmrtsh. u. GynaeJc, xvi. 25, 1902) has
recorded a true embryon papyraceum, for in addition to being
flattened in characteristic fashion it also exhibited some branchial
clefts and had only bud-like limbs. It is evident, therefore, that as
early as the embryonic period of antenatal life the pressure of one
twin may cause flattening of the other. Now, it is in the embryonic
epoch that monstrosities arise, and we are therefore led to inquire
whether there is any evidence that this geminous pressure might
directly lead to their production. The fact that Sundberg's papy-
raceous embryo {he. cit.) was also an amorphous acardiac embryo
seems to support this conclusion, although, of course, it is open for
us to believe that the acardiac condition was primary and led to the
early death of the embryo and so to its flattening. On the other
hand, there is evidence that the living (and compressing) twin may
be the monstrous one, the compressed co-twin being normal save for
the flattening; for Curatulo (Ann. di ostct. e ginec, xvii. 287, 1895)
has reported the birth of an anencephalic foetus along with a foetus
papyraceus. There is not, therefore, sufficient evidence to enable us
to decide whether or not the pressure of one twin may be the cause
of teratological phenomena in the other ; but a very evident necessity
exists for the recognition and examination of all small papyraceous
foetuses and embryos, since these are no doubt often overlooked
or regarded simply as little blood-clots or minute succenturiate
placentae.

I am inclined to think that the mechanical pressure of one twin

TWINS IN TERATOGENESIS 147

upon the other is not a cause of true teratological developments. I
do not doubt that the one twin has an influence upon the other
which may lead to the production of malformations in it, but I do
not recognise pressure as the modus operandi. I think rather that
Ahlfeld (op. cit.), Schatz (he. tit), and others have put the clue in our
hands when they ascribe the teratological effects to the vascular re-
lations existing between the twins in their common placenta. Of
this I shall write more in detail later. At the same time, it is
possible, especially if the liquor amnii be in small amount, that one
twin may so press upon, encumber, and embarrass the other that it
exhibits at birth the indications of compression in such deformities as
unilateral atrophies, depressed areas of the body, club-feet, scoliosis
(E. Perdu, La Seoliose, p. 18, Paris, 1902), and the like. These de-
formities, it is to be noted, may quite well originate in the foetal
period of antenatal life (p. 6), and are not, therefore, so distinctively
teratological as are those which arise in the embryonic epoch.

To summarise this somewhat extended argument: there is evidence
that a dead twin in utero may have its form altered by the pressure
of the surviving co-twin and be converted into a foetus compressus ;
it would seem that this may occur also in the embryonic period of
antenatal life and lead to the production of an embryon papyraceum ;
there is some support for the view that in the foetal period one twin
may cause surface-alterations (e.g. depressions and dislocations of
parts) in its co-twin, even if the latter be still alive ; but, finally,
there is little or no proof that simple mechanical pressure between
twins in the embryonic epoch is effective in the development of
teratological states. There is a pressure between twins which has
teratological results, but it is the pressure of competition for the best
or largest part of the placental circulation, and so scarcely falls
within the scope of our present argument.

Another form of intrauterine pressure which may possibly have
deforming effects is that exerted by the parts of the embryo or foetus
upon each other. Like the other varieties of the pressure-theory in
teratogenesis, this contains an element of truth combined with a large
amount of speculation ; the data are few, but the qucesita are many.
It has also been pushed to absurd lengths, as when it was suggested
by Jourdain (Journ. cle mfcl., chir., pharm., xxxix. 163, 1773) that
hare-lip might be caused by the foetus lacerating its upper lip with
its nails, and when it was thought that cleft palate might be due to
intrauterine thumb-sucking (Wynn Williams, Lancet, ii. for 1862,
p. 619) or to the pressure of the tip of the infant's tongue. J.
Jackson Clarke (Physician and Surgeon, i. 980, 1900) also has sug-
gested that, in a case of club-hand, macrostoma, and pre-auricular
appendages, the pressure of the hand upon the face of the foetus had
led to the deformities which were present ; and Schenk (Prag. mcd.
Wchnschr., xxv. 6, 1900) thought that a congenital lateral ventral
hernia was due to strong pressure of the flexed knee against the loin.
A less violent hypothesis is found in the assertion that the heel of
the foetus (in the intrauterine and antenatal attitude of flexion) may

148 ANTENATAL PATHOLOGY AND HYGIENE

cause a depression in the sternum. N. Hagmann (Jahrb.f. Kinder-*
hlk., n. F. xv. 455, 1880), for instance, reported a case in which a
new-born infant showed such a condition of sternal cyphosis ; the
mother noticed when she was washing the child that the bath water
collected (to the extent of a few teaspoonfuls) in a little depression
on the anterior aspect of the chest ; and the writer hazarded the
supposition that the hollow in the breast-bone was due to the pres-
sure of the heel ("Druck der Fersen auf das Brustbein"). The
potency of calcaneal pressure, however, is doubted by many, includ-
ing Taruffi (Storia della Teratologia, vii. 181, 1894) and G. D'Ajutolo
(Delia Cifosi e della Lordosi stcrnale, Bologna, 1898), and there are,
of course, other possible explanations of these sternal defects. In
fact the imbutiform or funnel-shaped thorax (Triehtcrbrust of the
Germans) has been ascribed to the pressure of the chin of the foetus
in an attitude of marked flexion (Zuckerkandl, Wien. med. Blatt., iii.,
No. 50, 1880; H. Bibbert, Deutsche vied. Wchnsehr., x. 533, 1888), as
well as to retraction of the diaphragm, rickets, heredity, external
violence, and defective development of the sternum (A. Griinenthal,
Diss, inaug., Berlin, 1888).

There is a large amount of evidence in favour of the view that
some malformations of the chest-wall may be due to the pressure of
the arm of the foetus in antenatal life. These malformations are
generally unilateral, and consist in the defective development of
some of the ribs and costal cartilages, in the absence of the pectorals
and adjacent muscles, in the dwarfing or complete absence of the
mammary gland and nipple, and in the want of the normal sub-
cutaneous fat and hair of the affected region ; the hand and arm of
the affected side may also show malformations. Froriep (Neue Noti-
zen. aus dem Gebiete, etc., x. 9, 1839) put on record the case of a
woman with absence of the right breast and defective development
of the pectoral muscles and the ribs of the same side, and expressed
the opinion that in the early stages of her antenatal life the right
arm had been pressed against the chest-wall and had so prevented
the growth of the underlying parts. G. Bitter (Jahrb. f. Kinderhlk.,
n. F. xi. 334, 1877; Oesferr. Jcthrb. f. PadiaL, vii. 101, 1876) also
described a new-born infant the left side of whose chest was very
defective ; the upper limb of the same side was poorly developed and
ended in a single finger ; and it was thought that the malformed
state of both these parts was due to the pressure which they had
exercised on each other. John Thomson (Tercdolugia, ii. 1, 1895)
reported three instances of congenital thoracic deformity in which
the pectoral muscles, the mamma, and one or more ribs were defec-
tive ; and gathered together from medical journals and books eighty-
six somewhat similar cases. In eleven of these eighty-six cases the
hand of the affected side was more or less malformed, and in a few
instances the forearm also was abnormal ; the malformation of the
hand was usually of the nature of webbing of the fingers (syn-
dactylism). In one of Greif's cases (Inaug. Diss., p. 25, Greifswald,
1891) the hand of the side corresponding to the thoracic defect (left)
was smaller and more slender than the other. Thomson constructed

LIMB-PRESSURE IN TERATOGENESIS 149

two plates representing in diagrammatic fashion the thoracic and
manual deformities in some of the recorded cases, and showing very
suggestively that the level and area of the chest defect was such that
part of the upper limb of the foetus might quite possibly have lain
over it. He pointed out that the defect did not correspond to any-
thing in the normal course of development, and could not, therefore,
be regarded as an arrest of the same nature as mesial fissure of the
thorax and abdomen. Not only the muscles but nearly all the
structures in the lower pectoral region were prone to be affected ;
and they were affected in a manner having no relation at all to their
vascular or nervous supply. Thomson was led, therefore, to the
conclusion that the foetus was so acted on in utero that its arm was
kept for a long time pressed firmly against its side, and that in con-
sequence of this continued pressure not only was the growth of the
parts retarded but the absorption of some of them was brought about.
From this statement we may conclude that Thomson did not agree
with some other writers in regarding the pressure of the arm as in
itself sufficient to account for the results, but looked for a cause
outside the foetus which would press the arm against the side. He
did not think that amniotic bands could be invoked in this case, but
his reasons for holding this view are hardly sufficient. Apert {France
med.,x\\i. 326, 1899), in connection with a case of bilateral depression
in the chest-wall with a congenital cardiac anomaly, advanced the
pressure of the elbows of the foetus as a possible cause, and in order
to explain their compressing effects suggested deficiency of the liquor
amnii.

Before I pass from the consideration of this particular variety
of intrauterine pressure, I must refer briefly to the curious cases
reported by Strassmann {Arch. f. Gynaek., xlvii. 463, 1894), in
which the penis of the foetus was caught and compressed between
two of the toes of one of the feet. The foetuses were otherwise
malformed, exhibiting pes varus, Polydactyly, cystic kidneys, etc. ;
and in one of them the penis, evidently on account of the pressure
brought to bear on it by the toes, was in a state of paraphimosis.
In F. von Winckel's case of congenital hypertrophy of the penis,
this curious cause does not seem to have been present {Mihnchen.
vied. Wchnschr., xliii. 428, 1896), although the foetus resembled in
many ways that described by Strassmann. I may refer here in
passing to the cases recorded by Lannelongue in which hare-lip
and cleft palate are ascribed to the pressure of tumours of the jaws
and tongue.

From a survey of the facts that have been narrated in the preced-
ing pages, there is evidence that the pressure exercised by one twin
foetus upon another or by the parts of a foetus upon itself may
produce teratological results ; but it is, at the same time, generally
admitted that there is the necessity for the presence of some force
which shall press the compressing and compressed parts together, if
the theory is to be firmly established on reasonable premises. This
naturally leads me to the consideration of the question of the pressure
of the umbilical cord and of the amnion.

150 ANTENATAL PATHOLOGY AND HYGIENE

Funic Pressure.

There are at least two ways in which it may he supposed that the
pressure of the umbilical cord may produce teratogenic effects. It
may he so disposed around the foetus as to cause one part of the body
to press upon another, or it may itself encircle and constrict one of
the parts of the unborn infant. As instances of the former mode of
action may be cited G. Barbieri's case (referred to by Taruffi, Storia
dclla Teratoloijia, v. 204, 1889), in which the right hand of the fcetus
was kept in apposition to the cheek of the same side by a coil of the
cord, with the effect that the ear, the zygoma, and the whole side of
the face were deformed; C. M. Daubert's case {De funiculo umbilicali
humano fcctui circumvoluto, Diss, inaug., Gottingen, 1808), in which
the sole of the right foot was brought against the lower jaw by the
umbilical cord ; and the illustrative examples of L. Heusner {Arch. f.
klin. Chir., lix. 206, 1899). It is unnecessary to discuss further this
variety of funic pressure, for it is quite conceivable and rational ; but
the second mode of action of the cord calls for more extended con-
sideration.

May the umbilical cord when coiled round the body or limbs of
the embryo or foetus in utero cause constrictions, arrested develop-
ments, and amputations of the parts so encircled by it ? This is the
question which has for many years called for an answer and led to
much discussion and controversy. So great has been the difference of
opinion and so large (in consequence) has been the amount of writing
upon the subject, that I am compelled to pass over many articles
and to collect together the titles of those to which I do refer at the
end of this chapter.

The question first arose prominently in connection with the
explanation of the curious cases in which a child was born with
partial absence of the limbs or with defective development of »the
fingers and toes. Such cases were well known to writers before
the nineteenth century; for Bartholin in 1675, Acoluth in 1740,
Maternus de Cilano in 1752, J. U. T. Schaeffer in 1775, Hinze in
1797, and many others put on record instances of different kinds of
mutilation of the extremities which had evidently arisen before birth.
Maternus de Cilano, to whom I have referred, described an infant
born at the seventh month with the right arm defective ; he main-
tained that the case proved that formed parts might be destroyed and
absorbed " per energiam autocraticam " ; and he ascribed the result to
the impression made upon the mother by the sight of a begging
soldier without a right arm : the infant lived for eleven weeks, and
then gently passed away (" blande expiravit "). The cases reported
by Bartholin and Acoluth were also regarded as caused by maternal
impressions. Schaeffer's observation was a most interesting one : the
infant, a female, born at the seventh month, showed webbing of the
fingers of the right hand, and defective formation of those of the left ;
the right foot had a short hallux, and there was a membranous cord
attached to the second toe ; the left leg seemed as if it had been

FUNIC PRESSURE

151

amputated below the gastrocnemii, the stump was covered with
newly formed cuticle, and from it was suspended by a fibrous cord a
small body representing a tiny foot (of the size seen at the third
month of intrauterine life) with five toes (Fig. 23). This observa-
tion was a most important one, for it answered Haller's objection that
a separated hand or foot had never been found in such cases. None
of the writers referred to seem to have considered that the pressure
of the umbilical cord might have had to do with the congenital
mutilations met with. Morgagni, indeed, appears to have regarded
funic pressure as capable of fracturing a foetal limb ; but he is silent
about its effect in producing antenatal amputations.

Even after the commencement of the nineteenth century, the

Fig. 23. — Schaeffer's case of Spontaneous Amputation.
a, membranous cord attached to second toe of
right foot ; b, ends of tibia and fibula of left leg ;
c, membranous cord with d, foot suspended by it.

pathogenesis of congenital defects of the limbs did not include funic
pressure as a possibility. F. Chaussier, indeed, found the lower
segment of an arm in the foetal membranes and observed the cicatrised
stump, and yet failed to regard the condition as due to amputation,
ascribing it to mortification instead. Meckel, however, hinted some-
what vaguely at this possible teratogenic power of the funis um-
bilicalis in his description of a three months' foetus, the cord of which
had made a groove in the skin over the right scapula, and was twisted
round the limbs, which were without fingers and toes. In Jameson's
case the death of the foetus in utero was ascribed to the constriction
of the trunk with the umbilical cord; the writer noted the swelling
of the upper part of the foetus and the atrophy of the lower.
Watkinson's observation, published in 1825, was in many respects

152

ANTENATAL PATHOLOGY AND HYGIENE

most remarkable. It referred to an infant, born at the seventh
month, without a left foot. " I discovered," wrote the author, " that
the left foot had been amputated a little above the ankle, and the
part nearly, but not quite, healed, in consequence, perhaps, of the
bones protruding." He goes on to say : " On examination after the
birth, I discovered the foot in the vagina, which I brought away : it
also was nearly healed, but here likewise the bones protruded. There
did not appear to have been any extravasation of blood from the
limb, as far as I was able to judge. This foot (the left) was much
smaller than the other, which was rather turned inward ; it showed
no marks of putrefaction, and, from comparing the two, I am led
to suppose that it must have been two months separated from the

Fig. 24. — Watkinson's case of Spontaneous Amputation of Foot.

body." The editors of the Journal in which Watkinson's paper
appeared added a rough sketch of the affected parts (Fig. 24). The
author gives no explanation of the mode of occurrence of the
" amputation " beyond stating that the mother had not been frightened
during her pregnancy. It was left for Barzellotti in 1827 to suggest
that the umbilical cord, especially if it were unusually long, might
become twisted round a limb at an early period in development, might
constrict it, cause atrophy of its tissues, and finally cut it through.
He was led to this conclusion by the examination of an infant whose
limbs consisted of stumps representing about one-third of the humerus
or femur ; skin of a cicatricial kind covered the ends of the stumps.
I have not been able to consult Barzellotti's article in the original,

FUNIC PRESSURE 153

but I quote from Taruffi's abstract of it (Storia delta Teratologia, i.
260, 1881 ; iv. 89, 1886), from which it appears that Tarufri is justified
in claiming for his fellow-countryman the honour of suggesting the
teratogenic powers of the funis.

In 1832-33 Montgomery published his short but most valuable
papers on spontaneous amputations and circular depressions on the
limbs of the foetus ; he, however, ascribed these morbid states to the
constricting effects of fine threads or bands of lymph, and not of the
umbilical cord. He could hazard no conjecture as to the origin of
these threads, but he clearly stated that the circular depressions
were intermediate stages which preceded the complete separation of
the affected limb. We need not further consider these constricting
threads of Montgomery now, for they will be dealt with in the
succeeding chapter.

The next incident worthy of note in this relation was the
publication, in the United States, of Fitch's case of spontaneous
amputation of the right leg ; the foot was first expelled, and then
two weeks later a foetus lacking the corresponding foot was born.
J. Y. Simpson in 1836 touched upon the action of the umbilical cord
in connection with spontaneous amputations ; he maintained that it
could not produce these effects unless it became fixed in one position
round the limbs, but had evidently no difficulty in believing in its
constricting power when it was adherent to the underlying parts, and
he refers to a case published by Wrisberg in support of this con-
clusion. In 1837, Montgomery, in his work on Pregnancy, returned
to the subject of spontaneous amputation in utero, and freely admitted
the power of the umbilical cord to produce it ; he described two
cases, in each of which a three months' foetus showed indentations in
the leg due to the presence of a tight coil of the funis round it. In
the succeeding year Schwabe recorded a similar case in Germany ; in
1839 Buchanan reported another in America; and Beatty another in
Ireland in 1846. Terstesse's observation was not, in all probability, of
the nature of a spontaneous amputation.

In 1848 J. Y. Simpson touched upon a very interesting question
when he pointed out that there sometimes existed, on the ends of the
stumps of these spontaneously amputated limits, little nodules or
projections occasionally bearing nails. He suggested that these
indicated an attempt, of a rudimentary kind, to reproduce the
amputated extremity, similar to what is seen in many of the lower
animals. In 1850 E. A. Martin described a case illustrating Simpson's
third possible cause of intrauterine amputation — by fracture : the
mother fell from a ladder eight weeks before delivery, and the child
was born with the left arm amputated near the shoulder, and the
wound still not entirely closed ; the separated but nearly full-sized
arm was expelled with the placenta.

Thus, by the middle of the nineteenth century, three new
theories had come into existence to explain the origin of congenital
amputations : by the umbilical cord, by bands of lymph, and by
external violence causing foetal fractures ; and the theory with
which we are now specially concerned had begun to encounter

154 ANTENATAL PATHOLOGY AND HYGIENE

criticism. As the years went by the criticism was to increase, as we
shall see.

The idea that the funis could cut through the tissues of a limb
was evidently open to criticism. Martin, as has been stated, doubted
its power to act thus unless a fracture of the bone were present ; and
Gatty pointed out that while the cord might constrict a limb it
could not amputate it, because the pressure on the vessels in the
cord itself would be so great as to stop the circulation and so kill
the fcetus. This objection, however, was somewhat weakened by the
publication of J. B. Hillairet's case in which the cord was twisted
round the neck of a three months' fcetus ; the head was almost
amputated, the tissues of the neck being reduced to a pedicle only
1| mm. in thickness. Weidemann, writing in the same year as
Hillairet, apparently did not doubt the power of the cord to cause
constrictions, amputations, and death of the foetus ; but Barkow, in
1859, inclined rather to the idea of amputation taking place by
means of foetal gangrene (gangnena fcetalis) comparable to the
senile process (gangrama senilis), and Kristeller advanced the idea
of an inflammation of the foetal skin. Chance, also, was a believer
in the teratogenic potency of the funis ; so was Nebinger ; and
W. B. Owen related a case in which a foetus of four months had the
legs bound down by the cord in front of the pelvis so closely that
they had become embedded in the soft parts of the wall of the
abdomen, and one leg was in process of being amputated by the
funic pressure.

In 1869, P. Keuss made a valuable contribution to the literature
of spontaneous amputations ; but, while admitting the teratogenic
power of amniotic bands and of pathological processes going on in
the skin, he thought that the pressure of the umbilical cord rather
tended to cause the death of the fcetus than to produce an amputation.
L. Fiirst held somewhat similar views ; and Barkow, in the same year
(1871), examined again, but more in detail, the whole subject of spon-
taneous amputations, and enumerated four possible causes, namely,
external violence, funic pressure, amniotic bands, and foetal gangrene.
Kaschkow, in 1872, published a case in which one limb showed the
process of amputation going on, the muscles having disappeared and
the bone showing atrophy ; he endeavoured to meet the objections of
Keuss and others by pointing out the rapid growth of the encircled
parts (in early foetal Jife) and the presence of pulsating vessels in
the encircling cord. Gilliam's case appeared to demonstrate the
deforming power of the cord. Menzel attempted to explain the
spontaneous amputation of fingers and toes by a morbid change in
the epidermis consisting in a proliferation and downgrowth of the
surface epithelium ("epithelial dactylolysis"), a process comparable
to that seen in the disease known as ainhum. This view or modifica-
tions of it have been advanced by several other writers, including
Jeannel (who regarded the amputations as trophic lesions of the
nature of scleroderma), Raynaud (ainhum), Longuet (trophic nervous
lesion), and Lancereaux (autocopic trophoneurosis). Miram admitted
that congenital amputations might be produced in several differ-

PRESSURE THEORIES IN TERATOGENESIS 155

ent ways, but thought that funic pressure was seldom the active
factor.

Within recent years the theory of the deforming effects of con-
volutions of the umbilical cord cannot be said to have gained
strength, although J. Cleland, E. Viehofer, E. Pestalozza, F. Merkel,
R. Lhomme, Turgard, C. Tournier, M. Duval, J. Koubinovitch, and
many others admit its possibility, and would give it a place in the
pathogenesis of deformities of the limbs if not of actual amputations.
On the other hand, B. Thompson Lowne, C. Dareste, and the
supporters of the theories of nerve influence, etc., are sceptical. It
must be confessed that there are obvious difficulties in the way,
which make it difficult to understand how a structure like the
umbilical cord can cut through a part of the body like an arm or a
leg ; at the same time it must be borne in mind that the possibilities
of pathological activity and of physiological repair in the early
periods of antenatal life are not fully known to us and may be
much greater than is commonly supposed. Again, if we admit the
power of bands or threads of amniotic tissue to perform amputa-
tions in utero (as most teratologists do), it is only a step further to
ascribe a similar potentiality to the umbilical cord. Possibly it may
be necessary, for the full exercise of this morbid action of the cord,
that the underlying skin develop pathological changes; but this
only diminishes the amount of responsibility, so to say, that the cord
has in the results produced, and does not entirely free it from
participation. Finally, it may be said that the power of funic
pressure to produce grooves and scars on the limbs and body of the
fcetus and to interfere with the nutrition of distally situated parts
can hardly be doubted, and that, in the absence of exact knowledge
regarding the possibilities of pathological processes in embryonic
life, it cannot be definitely stated that the cord is incapable of
actually producing the amputation of a part.

I have, in this chapter, given a sketch of the pressure-theories
of teratogenesis, and have shown how there has been a progressive
tendency to minimise the causal factor involved, as more and more
light has been thrown upon antenatal problems. I have not, it is
true, yet considered the theory of amniotic pressure in teratogenesis,
for that is more conveniently and correctly discussed with the
theories of disease as a cause of monstrosity and malformation, but
it also has of late years been thrown somewhat into the shade by
the inquiry into the causes which produce the amniotic tightness
and the amniotic bands. This will become evident to the reader
after he has perused the following chapter. For all these reasons
I have placed the theory of traumatism and pressure first among
the teratogenic theories of the present ; to my mind it comes nearest
to the views of the past and is furthest removed from the most
advanced conceptions of the day. This does not by any means
imply that it contains no truth, neither does it exclude it as a
probable explanation of some at least of the monstrosities which
are met with.

156 ANTENATAL PATHOLOGY AND HYGIENE

LITERATURE BEARING UPON FUNIC PRESSURE.

J. C. Acoluth, Acta Acad. not. curios., v. obs. 48, p. 181, Norimb.,
1740; H. C. L. Barkow, Beitrdge zur pathologischen Enttoickelungsge-
schichte, iii. 9, 1859 ; iv. 14, 1871 ; T. Bartholin, Acta med. et phil. 1673,
Hafn., ii. 1, 1G75; G. Barzellotti, Ann. univ. di med., xlv. 574, 1828;
Beatty, Dublin Quart. Journ, Med. Sc, ii. 532, 1846 ; A. H. Buchanan,
Trans. Tennessee Med. Soc, Columbia, x. 41, 1839; E. J. Chance, Bodily
Deformities, i. 130, 1862 ; F. Chaussier, " Discours prononce a l'hospice de
la Maternite, juin 1812," Diet. d. sc. med., xvi. 70, 1816; J. Cleland,
Memoirs and Memoranda in Anatomy, i. p. 133, 1889; J. R. Cormack,
Lond. Med. Gaz., i. 410, 1838; D'Argo Natale, L'osservatore medico,
Palermo, iii. 392, 1855; C. Dareste, Production des Monstruosites, p. 419,
1891 ; E. J. de Nonancourt, "Des amputations spontanees intrauterines,"
These, Strasbourg, 1864 ; M. Duval, in Bouchard's Traits de pathologie
generate, i. p. 245, 1895 ; F. P. Fitch, Amer. Journ, Med. Sc, xviii. 90,
1836; L. Furst, Arch. f Gynaek., ii. 315, 1871; W. H. Gatty, Lond.
Med. Gaz., n.s., xii. 627, 1851 ; D. T. Gililam, Clinic, Ciucin., iii. 230,
1872; Gurlt, Med. Ztg., Berlin, ii. 13, 1833; Haller, Element a Physi-
ologic, viii. 140, Berne, 1766 ; J. B. Hillairet, Mem. Soc, de biol,, Par., 2
s., iii. 117, 1856; Hinze, Journ. d. prakt. Arznk. u. Wund-arznk., iv. 763,
1797 ; A. F. Hohl, Die Geburten missgestalteter, kranker und todter Kinder,
p. 135, 1850; H. G. Jameson, Amer. Med. Recorder, Pliila., vi. 292, 1823;
Jeannel, Arch, de tocol,, xiii. 774, 1886; Kristeller, Monatschr. f
Geliurtsk. u. Frauenkr., xiv. 87, 1859 ; Lancereaux, Semaine med., Ann. xiv.
429, 1894; R. Lhomme, "Amputations congenitales," These, Paris, 1893 ;
M. Longuet, Comjit. rend. Soc. de biol., 6 s., iii. 110, 1877; B. Thompson
Lowne, Teratological Series in the Museum of the Royal College of Surgeons
of England, p. 108, 1893; E. A. Martin, Jenaische Ann. f. Physiol, u.
Med., i. 333, 1849-50; G. C. Maternus de Cilano, Acta Acad, nat.
curios., ix. 360, 1752 ; J. F. Meckel, Llandbuch der pathologischen Ana-
tomic, ii. 137, 1816; A. Menzel, " Dattilolisi spontanea (strozzatura spon-
tanea delle dita)," Gazz. med. ifed. lomb., xxxiii. 241, 1873 ; F. Merkel,
Centrlbl.f. Gynak., xiii. 289, 1889 ; J. E. Miram, " Zur Casuistik der spon-
tanen Amputationen und ihrer Folgezustande," Diss, inaug., Dorpat, 1877 ;
W. F. Montgomery, Dublin Journ. Med. and Chem. Sc, i. 140, 1832; ii.
49, 1833; W. F. Montgomery, Signs and Symptoms of Pregnancy, p. 321,
1837; G. B. Morgagni, The Seats and Causes of Disease (Alexander's
translation), ii. pp. 724, 757, Lond., 1769; A. Nebinger, Amer. Journ.
Med. Sc, Phila., liv. 129, 1867; W. B. Owen, Trans. Lond. Obst. Soc,
viii. 4, 1867; E. Pestalozza, Riv. di ostet. e ginecol, i. 209, 225, 1890;
Raschkow, Beitr. z, Geburtsh. u. Gynak., ii. 177, 1872-73; L. Raynaud,
Journ. mal. cutan. et syph., vii. 193, 1895; P. Reuss, " Ueber Spontan-
amputation beim Fcetus," Diss, inaug., Wurzburg, 1869 ; Beitr. z. Geburtsk.
u. Gynaek., vi. 1, 1869; J. Roubinovitch, Compt. rend. Soc. de biol,, 10 s.,
v. 819, 1898; J. Rouget, "Amputations congenitales et ainhum," These,
Paris, 1889 ; J. U. T. Schaeffer, " Foetus cum matre per nervos commer-
cium solemniter ventilatum," Diss, inaug. Erlangce, 1775 ; Schwabe, Journ.
f. Geburtsli., xvii. 270, 1838 ; Simonart, Arch, de la med. beige, 112, 1845 ;
J. Y. Simpson, Dublin Journ. Med. and Chem, Sc, x. 220, 1836 ; J. Y.
Simpson, Month. Journ. Med, Sc, viii. 890, 1848; J. Y. Simpson, Memoirs
and Contributions, i. 355, 374, 1856; H. Terstesse, " De funiculi umbili-

FUNIC PRESSURE 157

calis circumvolutione, et de rariori quodam spontanea^ amputationis intra-
uterinae casu," Diss, inaug. Bounce, 1848; C. Tourniek, Rev. d'orthop., ii.
272, 1891; Turgard, Bull med. du nord, Lille, xxvii. 257, 302, 1888;
E. Viehofeu, " Ueber intrauterine Amputationen," Diss, inaug., Bonn,
1887 ; Watkinson, Land. Med. and Phys. Journ., liv. 38, 1825 ; *H. W. H.
\Yeidemann, " Bemerkungen iiber die Umscblingungen des Nabelstrangs
um die Frucht," Diss, inaug., Marburg, 1856.

CHAPTER XI

The Nosological Theory in Teratogenesis : Foetal Diseases as Causes of Monstrosi-
ties, the Theory and its Critics ; Disease of the Fcetal Annexa as a Cause of
Monstrosities ; Amniotic Disease in Teratogenesis ; the Amniotic Theory
and its Developments ; the Facts and the " Fancies," the Adherents and the
Critics of the Theory ; Congenital Amputations and Fcetal Skin Diseases ;
Eegeneration of lost Parts ; Conclusions ; Literature.

The idea that the diseases of the foetus or of its investing membranes
may be the cause of monstrosities is not to be reckoned among the
theories of the past ; it is indeed a theory of comparatively modern
times without even roots in the past ; it may in some respects be
called the teratogenic theory of the nineteenth century. It is not,
however, on that account an accredited view at the present time, and
several teratologists, including Tarum and M. Duval, have declared
against it. In this respect the older authors and the most modern
are in accord ; but it does not on that account follow that the former
reached their conclusion by the same process of reasoning as the latter.
Probably the older writers on monstrosities were so impressed with
the altogether special and peculiar features of teratological pheno-
mena that it never entered their mind that they could possibly be
due to diseases, and so they kept silence on that point.

The idea that diseases are the causes of deformities may be called
the nosological or pathological theory of teratogenesis ; the former
name more correctly expresses what is meant, although the latter
has a less unfamiliar appearance. There are two views contained
within this one theory : according to the first, the foetus itself is the
subject of diseases which produce the malformations ; according to
the second, it is through disease of the foetal membranes and more
particularly of the amnion that anomalous conditions are set up in
the foetus or embryo. It will be convenient to consider these two
aspects of the nosological theory separately ; since the notion that
regards foetal maladies as the causes of monstrosities is older than
that which ascribes teratogenic powers to the diseased amnion, it will
be dealt with first.

Foetal Disease in Teratogenesis.

Very little is heard of the nosological theory in teratogenesis
before the middle of the eighteenth century. Licetus, however, who
wrote in 1G16 (De Monstris, lib. ii. cap. viii.), had a short chapter on

158

DISEASE IN TERATOGENESIS 159

the subject. In it he pointed out how in adults certain diseases such
as syphilis might have deforming effects due to destruction of tissues
{e.g. in the case of the nose and penis) ; and he suggested that, in the
more tender foetus, inflammations and corroding humours might lead
to the consumption of a hand or a foot. If the vis medicatrix naturae
were effective, the mutilated part would cicatrise and so a monster
would be produced. " Sic igitur ex vi morbi oriuntur pleraque mon-
stra mutila," he said in conclusion.

In 1741 J. B. Bianchi published a noteworthy work (De Naturali
in Hmnano Corpore, Vitiosa Morbosaquc Generation e Historia, Augustre
Taurinorum, 1711); in it he discussed the possible causes of mon-
strosities (" causae autem," he wrote, " eheu iiimise ! " recognising that
perplexing plurality of etiological factors which my readers already
know so well), and referred specially (on p. 242) to diseases of the
foetus and their deforming effects, such as the production of infants
with tails, beaks, and claws, or covered with hair. Bianchi's con-
ception of how these deformities arose was not, however, very clear,
and it was reserved for Morgagni to frame a rational and scientific
explanation of the possible modus operandi of diseases as causes of
deformities. In his great work, De sedibus et causis morborum, pub-
lished in 1761, Morgagni dealt with many matters in an epoch-making
manner, and among these was the theme of monstrosities. " What I
shall say here," he wrote (I quote from B. Alexander's translation of
1769, vol. ii. p. 751), " will be rather what relates to the observation.,
themselves than to the controversy which is agitated in this age " (he
refers to the Parisian discussion, vide p. 133), " among very learned
men, in regard to the origin of these monsters " ; nevertheless he
treated of their origin also and in more luminous fashion than the
" very learned men." Here is his account of the manner in which he
believed that anencephaly was produced from hydrocephalus (vol. i.
p. 251): "Doubtless the force of the water, gradually collected in a
hydrocephalus, especially if the foetus be tender, is very urgent and
manifest, and the more so in proportion as it begins the sooner to
act on the less resisting parts of the cranium. And hence the
approach of the bones to each other is not only impeded, but also the
very growth of them, sometimes in a few, and sometimes in the
greatest parts ; so that they seem to be entirely wanting." In an-
other passage, Morgagni, in writing of an anencephalic foetus, further
defined his theory : " Here then you have a description of no common
monster ; but of a hydrocephalus monster : so that after you have
set down as many things as you please, to the account of the
monstrous condition ; yet still more circumstances will remain, that
you must attribute to the impelling force of the water, such as the
obstructed growth of some of the bones of the cranium, the depression
of some, the impelling of some to one side, and the drawing asunder
of others."

Dareste (Production artificielle des MonstruosiUs, p. 17, 1891)
claims for Marcot of Montpellier the honour of having been the first
to suggest this theory of causation of anencephaly, and quotes his
article published in 1716 (" Memoire sur un enfant monstrueux,"

160 ANTENATAL PATHOLOGY AND HYGIENE

Mem. de VAcacl. roy. ties sc. tie I'anne'e mdccxvl, p. 329, Paris, 1741).
On referring to Marcot's article, I find that he does indeed very
clearly state that he regarded the disappearance of the parts of
the brain in anencephaly as due to hydrocephalus ; he thought that
the pressure of the serous fluid upon the blood vessels going to the
brain hindered the nourishment of that organ, and so led to its
diminution. The pressure acted most upon the internal carotid
arteries, and so an excess of blood found its way into the external
carotids and led to the lividity of the neck and face and to the
swelling of the eyelids. What really puzzled Marcot was how the
foetus could have lived in utero without the brain which furnished
the animal spirits — " c'est ici veritablement ou se trouve le noeud de
la difficult^ " ; he was forced to leave this difficulty unanswered. It
would seem at first sight as if Dareste were right in giving Marcot
the honour of first proposing this theory of the origin of anencephaly,
inasmuch as the year 1716 certainly preceded 1761 ; but the question
of precedence is not thus easily settled, for I find that Taruffi {Storia
della Teratolotjia, vi. 150, 1891) cites a much earlier work of Morgagni
{Adversaria Anatomica, Bononiae, 1706) as that which contained the
first statement of his views on the causation of anencephaly. So the
honour returns to Morgagni after all.

The idea of diseases as the causes of monstrosities, once enunciated,
rapidly gained supporters. Before referring to other kinds of disease
»vhich have been believed to be teratogenic, I may rapidly sketch the
history of the theory that hydrocephalus produces anencephaly, for it
was, as I have shown, that with which Morgagni's name was specially
associated. In the early part of the nineteenth century, C. W.
Schlegel and K. A. Eudolphi (cited by Ahlfeld, Die Missblldungen cles
Mensehen, p. 284, 1882) reported instances in human embryos (about
six weeks old) in which a dropsical vesicle was found in place of a
brain ; these were regarded as representing the stage of development
of anencephaly prior to the rupture of the hydrocephalic sac. Tiede-
mann {Ztschr.f. Physiol, Hi. 36, 1829), also cited by Ahlfeld, described
the case of a foetus with a dropsical brain covered only by a mem-
branous sac ; this observation might perhaps be adduced in support
of Morgagni's view. Eyan {Load. Med, and Sure/. Journ,, vii. 77,
1835) wrote as follows : " It is easy to understand that the serosity or
water, as it is improperly called, may distend the brain, rupture its
membranes, separate the bones of the cranium, burst the scalp, and
finally escape into the amniotic fluid. The whole brain, membranes,
bones, and scalp are destroyed by absorption, and the base of the
skull alone remains, forming the acephaly." In another place (p.
175) he stated that " hydrocephalus may advance rapidly, burst the
cranium, and produce anencephaly or microcephaly." Eyan's views
on this subject are, therefore, quite clear. V. Portal's record of a
twin labour in which one fetus was hydrocephalic and the other
anencephalic (as cited by Taruffi, Storia della Teratolotjia, vi. 151,
1891) forms an interesting although indirect piece of evidence in
favour of Morgagni's opinions. Otto {Monstrorum Scxcentorum Dc-
scriptio, Introduct. pp. xv-xviii, Vratislavin.*, 1841) discussed the

HYDROCEPHALUS AND ANENCEPHALUS 161

effects of various degrees of distension of the cerebral ventricles, and
pointed out that the nervous system was not only very fragile but
was also the first part to appear in the embryo ; it was therefore very
apt to be injuriously acted upon and to have its evolution retarded or
misdirected ; most monstrosities except those characterised by excess
of parts were due to diseases. Fbrster (Die Missbildungen des Men-
schen, 2 edit., p. 77, 1865) arranged the results of the accumulation
of fluid in the cerebro-spinal canal into three groups (1, hydro-
cephalus and hydrorrhachis ; 2, hydrencephalocele and hydromyelo-
cele ; 3, cranioschisis and rhachischisis), according to the degree of
distension and destruction of parts.

At a meeting of the Edinburgh Obstetrical Society in 1847
(Month. J. Med, Sc, n.s., ii. 215, 1847 x) J. Y. Simpson stated that,
in his opinion, the malformation in anencephalous monsters arises
from intrauterine disease, namely, from the bursting of the head when
hydrocephalic. In another place (Obstetric Memoirs and Contributions,
ii. 199, 1856) he wrote : " Though assuredly this doctrine has been
carried far beyond its just limits by some of the authors here alluded
to (Morgagni, Penada, Tiedemann, Beclard, Duges), there is still,
however, as we believe, much reason to suppose that one or two
varieties of anencephalous malformations are truly referable to the
purely physical effects of hydrocephalic inflammation and effusion."
But to Simpson's contributions to the nosological theory of terato-
genesis I shall refer again immediately.

F. Ahlfeld (Die Missbildungen des Mcnschen, pp. 284-290, 1882)
thought that while some few cases of anencephaly might be due to
amniotic adhesions, most were caused by hydrocephalus, and he
added that the bursting of the hydrocephalic sac could not occur
before the fourth week of embryonic life; the probable reason for
this reservation is (as Taruffi points out, op. cit., vi. 151, 1891) that
before the fourth week the eyes, which are often well formed in
anencephaly, are not independent of the part of the brain which is
destroyed. Alfred W. Hughes, in an article on the central nervous
system and axial skeleton in anencephalous monsters (Lancet, ii. for
1887, p. 1212), was struck with this difficulty, for he found well-
developed eyes with optic nerves, although only the parts originating
in the posterior cerebral vesicle seemed to be represented ; he
thought, therefore, that all the vesicles at one time underwent a
certain degree of development which afterwards became aborted,
and gave place to retrogressive changes. Cleland (Journ. of Anat.
and Physiol., xvii. 257, 1883), in a well-considered contribution to
the study of spina bifida, encephalocele, and anencephalus, came to
the conclusion that while the open condition of the cerebro-spinal
canal might be due to non-closure of its walls, it was probable
that anencephalus was due to rupture after the primary optic
vesicles were cut off from the cerebral vesicles. Many other
writers (e.g., Doran, Trans. Obst. Soc, Lond,, xxxi. 52, 1890) have

1 This reference is erroneously stated in Simpson's Obstetric Memoirs, both the name
of the journal and the number of the page being wrong ; defective references are as
" pin-pricks'' to the earnest student, and he is often thus maltreated.

VOL. II. — II

162 ANTENATAL PATHOLOGY AND HYGIENE

supported Morgagni's views of the origin of anencephaly and allied
states; and I myself, in referring some years ago to a case in
which a man begat a hydrocephalic child by his first wife and two
anencephalic foetuses by his second, stated that I thought the
occurrence might have some bearing upon the theory that anen-
cephaly is due to early hydrocephalus {Trans. Eclinb. Obst. Soc, xvii.
228, 1892). It must, however, be admitted that there are many
difficulties in the way of the full acceptance of the theory, and
to these the attention of the reader is now turned. It may at
the same time be noted that H. Eibbert's observation of two cases
of rupture of the cranium in embryos of the goat and cow is
evidence in support of Morgagni's contention (Arch. f. path. Anat.,
xciii. 396, 1883).

The criticism to which Morgagni's theory of the causation of
anencephaly was subjected was no doubt in large measure due to
the unjustifiable extension of the view to the explanation of many
other, in fact of most other, monstrosities and malformations, with the
exception of those in which there was excessive development. It
was the expansion of the theory that suggested its defects and
excited criticism, not without cause, as every one must admit. Let
us consider some aspects of this expansion.

Beclard (Bull. Fac. de mid. de Par., v. 488, 1816-17) insisted
that dropsy was one of the most frequent diseases of the foetus,
being produced by twisting of the cord, obliteration of one of the
umbilical arteries, etc. ; it most commonly affected the head and
brain, and might then give rise to a hydrencephalic hernia, to spina
bifida, or to anencephaly, according as it occurred late or early in
intrauterine life. Thus far Beclard simply repeated the views of
others, but he carried his opinions much further : he thought that
destruction of the olfactory nerve would cause the atrophy of the
ethmoid bone and the union of the two eyes, that destruction of the
medulla oblongata and its nerves would lead to loss of the face, that
the diaphragm would be wanting if its nerve were involved in the
common destruction, and that in like manner the arms, the thorax,
the abdominal walls, and even parts of the feet would disappear as
the dropsical ruin of the nervous system extended downwards. In
this way Beclard explained the origin of cyclopia, of otocephaly, of
paracephaly, and even of acephaly ; and in this view he was fully
supported by A. Duges (Ephem. mdd. de MontpeL, ii. 132, 275, 1826;
Rev. mid. f rang, et Strang., iv. 407, 1827). Another development of
the nosological theory was seen when A. Velpeau (Me"m. Acad. roy.
de mid., Par., hi. 90, 1833) attempted to explain exstrophy of the
bladder and its associated malformations by the occurrence -of a
slight erosion of the anterior abdominal walls in the hypogastric
region which gradually deepened and finally involved the bladder.
B. Phillips {Cyclopaedia of Anatomy and Physiology, i. 392, 1835-36)
defined the process and its steps more particularly when he stated
that the disease began as an ulceration or perforation of the penis
or of the hypogastrium,and that the bladder was secondarily involved.
The same sort of explanation was given for the so-called spontaneous

FCETAL PERITONITIS AND MALFORMATIONS 163

amputations of limbs by Chaussier (Discours prononcc en 1812 a la
distribution des prix de la maternit£) and others.

Yet another development of the theory that foetal diseases are
the causes of monstrosities took place in connection with foetal
peritonitis. J. Y. Simpson, in an article on peritonitis in the foetus,
published in 1838 {Edirib.Med. and Surg. Journ., vol. 1. p. 390, 1838),
hinted that " to these foetal diseases, and more particularly to certain
degrees of inflammation, might yet be traced the origin of various
morbid states, the true nature of which is at present little suspected."
In the following year he returned to the subject, and brought
forward evidence to prove that various congenital hernial (umbilical,
diaphragmatic, and inguinal) are due to foetal peritonitis ; that
various malformations, both in the way of displacement and of
arrested development, in the digestive, urinary, and genital organs,
are indirectly caused by the same malady ; and that inflammatory
action in the other serous cavities (pericarditis and pleuritis) may
lead in the same manner to malformations among their visceral
contents (Edinb. Med. and Surg. Journ., vol. Hi. p. 17, 1839). The
word " indirectly " in the above paragraph must be specially noted,
for Simpson was too perspicacious and keen-sighted not to realise
that the nosological theory was insufficient in itself to explain
several of the malformations with which he was dealing. He
referred to the " happy idea that was first suggested by the master
mind of Harvey, relative to certain malformations consisting, not in
the substitution of an entirely new and anomalous type of structure
in the malformed part, but only in the simple permanence of some
of its foetal types," and then proceeded to work into Harvey's " happy
idea " his own no less happy idea that " the state itself of impeded
development might be traced to the anterior influence of different
inflammatory and other casual pathological conditions of the early
embryo." It seemed probable to him also that " a limited series of
those malformations that are now looked upon as the decided results
of arrested development may come again to be regarded, as they
were formerly, not as instances of absolute want of development of
the defective part, but as cases in which this part had been, in the
first instance, more or less fully evolved, and then subsequently
destroyed by morbid action." It will be seen, therefore, that Simpson
greatly strengthened the nosological theory of teratogenesis by
including in it that of arrested development ; in this way he gave to
it a more scientific aspect, and answered several of the objections
which had been brought forward against it. It does not, however,
appear to have entered into his mind that the arrestments could
have arisen otherwise than through preceding diseases either of the
foetus or of its membranes. In this respect only did he fall short of
holding the most advanced views on teratogenesis which prevail at
the present clay.

Before I pass to the consideration of the criticisms which have
been advanced against the nosological theory, I must devote a para-
graph to a notice of the opinions of Jules Guorin ; these were stated
on various occasions between the years 1838 and 1880, and summarised

164 ANTENATAL PATHOLOGY AND HYGIENE

in his large work (Bcchcrchcs sur lea difformiUs congtnitales chcz les
monstrcs, le fmtus, et V enfant) in 1880. He brought together a great
mass of facts from various sources along with not a few personal
observations ; and he argued from these, not without the suspicion of
special pleading, that the disease of the foetus which is the cause
both of monstrosities and of the minor malformations which accom-
pany them is a general or local affection of the nervous system. He
also laid great stress upon the occurrence of convulsive muscular
retraction or contracture as one of the steps in the process which
ended in the production of teratological phenomena, major and
minor. According to his theory, a large number of monstrosities
and malformations, including suppressions, arrestments, and per-
versions of development, were due to one great cause, an affection
of the cerebro-spinal system. He certainly lost no opportunity
during a long series of years of bringing his opinions prominently
forward, and he claimed that he had converted that famous teratologist,
Geoffroy Saint-Hilaire, to his way of thinking. This conversion took
place, as Guerin tells us, at the meeting of the Parisian Academy of
Sciences in 1840, on the occasion of his reading there his contribution
entitled ." Essai d'une thcorie gencrale des difformites articulaires
chez les monstres, le foetus, et l'enfant." The incident I give in
Guerin's own words : " AussitAt ma lecture termince, il vint me
trouver a mon banc et me dit a haute voix : J'avais tort et vous avez
raison " (ojx cit., p. 174). All this and much more in the way of the
application and extension of the doctrine referred to will be found
in Guerin's large book already named. It may be added that
P. E. F. Delplanque, in his Etudes Teratologiqucs (Lille, 1885), looked
to an intrauterine disease of the foetal nervous system (of the nature
of tetanus) as the cause of the curious bovine malformation known
as the calf with a dog's head.

It is now time to consider some of the criticisms which were
provoked by the nosological theory in teratogenesis and its expan-
sions, ramifications, and ultimate developments. P. Vannoni {Gaz.
med. ital.fcdcr. tosc, 2 s., i. 218, 1850), for instance, pointed out that
changes in the cerebro-spinal system failed to account for the
variety of forms of monstrosity, and that many of the alleged causal
agents were obviously incapable of producing any kind of monstrosity
at all. He did not, however, deny that the morbid state and its
results might suspend, alter, or divert the organic processes so as to
lead to fissions, to asymmetry, and to other deformities. Vannoni,
therefore, did not criticise so much the theory that hydrocephalic
distension of the cerebro-spinal canal produced rupture and resulting
anencephaly and spina bifida, etc., as its wide and somewhat jash
enlargements and amplifications. Perls, however, did not hesitate
to animadvert upon the theory as it dealt with anencephaly itself,
and thus to attack the view in what had been considered its
strongest position. He pointed out {Lclirhueh der cdlgemcinen jEtio-
Jogie und der Missbildungen, 282, 1879), from the examination of
twenty -seven specimens, that the basis cranii in anencephaly shows
a convexity and not a concavity, or at least a flattening such as is

CAUSES OF ANENCEPHALUS 165

found in hydrocephalus. If hydrocephalus is the cause of anen-
cephaly we should expect to find not a convexity but a concavity
of the cranial base ; but, as a matter of fact, we discover, according
to Perls, signs not of an internal expanding force but of a pressure
acting from without inwards. A much older criticism than that of
Perls was brought forward by Gall and Spurzheim, and repeated by
Etienne Geoffroy Saint-Hilaire (Philosophie anatomique des mon-
struosites humaines, p. 152, Paris, 1822), who pointed out that the
perfect state in which one often found the delicate structures lying
on the base of the skull in anencephaly (e.g., the auditory, olfactory,
and optic nerves) was an argument against the production of that
monstrous condition by internal pressure. " Comment eussent-ils pu
register a Paction d'un fiuide qui aurait dissous des os si durs, des
membranes si tenaces ? " Another argument was found in the well-
nourished state of anencephalic foetuses at the time of birth —
surely if in the early weeks of intrauterine life the foetus were
the subject of such a serious disease as hydrocephalus it should be
born weak and puny ! The younger Geoffroy Saint-Hilaire (Isidore)
in his Histoirc des Anomalies (vol. iii. p. 518, 1836) gave other
reasons which led him to reject the theory of hydrocephalus as the
cause of anencephaly, among which were the facts that the bones of
the cranial vault were equally reduced in all their dimensions and
not truncated and incomplete, that the bones of the basis cranii
were complete but badly formed, and that the vertebral lamina
were not simply partly destroyed but were also displaced laterally
and spread out : these characters, he thought, pointed to an arrest
of development and not to the destructive action of hydrocephalic
fluid within the cranium. We have it, however, on the authority of
G-uerm (vide supra) that Saint-Hilaire resiled from this position in
after years. At the same time it must be admitted that the argu-
ments against the hydrocephalic theory of origin of anencephaly
possess considerable weight, and that the weight is not greatly
lessened by supposing that the rupture of the cranium would so
relieve the pressure upon the cranial base as to cause a convexity to
take the place of a concavity (Virchow, Untersuchungen ilhcr die
Enhviekelung des Schcidelgr uncles, p. 102, 1857 ; Ahlfeld, Die Missbil-
dungen des Menschen, p. 291, 1882). Among recent writers who
have opposed the nosological theory of teratogenesis, both in its
wide application and its special relation to anencephaly and spina
bifida, are Dareste and Duval. The former (Production artifieielh
des monstruosiUs, p. 54, 1891) insists that both the pressure-theory
as commonly held and the theory of diseases as the causes of
monstrosities are founded upon erroneous conceptions of embryo-
genesis ; those who have held these views have sought for the
explanation of teratological occurrences in the embryo without
knowing how the embryo itself is formed. In regard to the special
and so oft-quoted case, anencephaly, Dareste (op. cit., p. 385) ascribes
its development to an arrest in ontogenesis ; the medullary groove
either does not close at all or does so too late, with the result that
the upper wall of the cerebro-spinal canal is composed of an

166 ANTENATAL PATHOLOGY AND HYGIENE

ectoderm which does not give origin to nervous tissues ; serum
continues to accumulate in the tube, and so a state of dropsical
distension in the embryological but not in the pathological sense
of the word is produced. Duval (Bouchard's TraiU de pathologic
geuerale,\. 181, 1895) holds similar views; he admits that in exen-
cephaly and pseudencephaly there is an accumulation of fluid in the
cerebral ventricles, but asks pointedly whether this circumstance is
to be regarded as a cause or a consequence of the arrest of develop-
ment of the walls of the cerebral vesicles ; he himself has evidently
no doubt that it is a consequence, and not a cause. Vaschide and
Vurpas (Nouv. iconogr. de la Salpetrierc, xiv. 388, 1901), in their
admirable article on the histology of the nervous system of an
anencephalic fcetus, indicate their belief that there had been cerebral
hemispheres in this case at an early period of embryonic life, and
that these had disappeared, being converted into the vascular new
formation found on the basis cranii ; there were signs of a very
active and intense inflammation affecting the nerve tissues, the
meninges, and the vessels. These authors are evidently impressed
by their discoveries, but they do not comment upon them, promising
to return to the subject of their bearing upon teratogenesis in
another article.

My views on this subject of the relation of diseases to mon-
strosities I have already indicated in Chapter VII. ; but I shall
return to the matter more in detail after I have sketched the
second- aspect of the nosological theory, that which looks to diseases
not- of the fcetus itself but of its membranes for the causal factors in
teratogenesis.

Disease of the Foetal Annexa in Teratogenesis.

There is no very clearly marked line dividing the one aspect of
the nosological theory in teratogenesis from the other; the transition
from the idea of foetal diseases as causes of monstrosity to that of
diseases of the fcetal annexa in the same relation is an easy one.
Authors are not always careful to separate the two notions, and so
they are sometimes found side by side in the explanation of the
origin of a malformation or monstrosity. The annexa of the embryo
and foetus are the amnion and chorion, the umbilical vesicle, and
(perhaps) the allantois. Doubtless, diseases of any of these struc-
tures may exist, but in relation to teratogenesis little has been
advanced regarding any of them save the amnion. It is true that
J. Y. Simpson, in 1836, wrote of the malforming effects of placental
inflammation (placentitis) upon the foetus ; but he referred not so
much to disease of the chorionic villi and decidua serotina, which go
to form the placenta, as to inflammation of the amnion covering its
fcetal aspect; it was really amniotitis, and not placentitis, that
Simpson described and meant. There is no need, therefore, for us to
delay our consideration of the theory of disease of the amnion as the
cause of monstrosities. It is practically the only theory that we
require to deal with.

DISEASE OF THE AMNION 167

Amniotic Disease in Teratogenesis.

In the pathology of the embryo, the amnion is believed to occupy
the same dominant position that the placenta does in connection with
the pathology of the foetus. It is, or rather it is held to be, the
chief cause, direct or indirect, of all those malformations or mon-
strosities that are characterised by defective development. At the
same time, all teratologists do not by any means view its mode of
action in the same way ; as a matter of fact, they look at it in a
number of very different ways. The idea of disease of the amnion
as the cause of teratological results is not the only amniotic theory
of teratogenesis ; there is the idea of pressure of the amnion as the
cause of malformations ; there is also the notion of arrested develop-
ment of the amnion as the chief factor in teratogenesis. There are,
then, three amniotic theories, and not one. Further, these three
views of disease, pressure, and arrested development of the amnion
have become so combined, coupled, connected, and as it were con-
federated together, that their disentanglement has come to be an
almost impossible process. In the description which follows I shall
not indeed try rigidly to dissociate them from one another, for to do
so would lead to unnecessary repetition, and would not tend to make
a complicated subject any less complicated. At the same time I
shall endeavour to keep the notion of amniotic disease rather than
that of pressure or of arrested development prominently before the
reader.

There are two parts of this theory, as of all theories, namely, the
records of the cases which have been observed and the reflections
that the observers have made upon them ; the facts and, one had
almost said, the fancies. About the former there can be no reason-
able doubt, always granting the knowledge and good faith of the
fact-recorder ; with regard to the latter there may be the most varied
opinions and estimates according to the acumen and discriminative
penetration of the critic. Theories, also, which seemed at one time
to fit the facts may, with the discovery of fresh facts and with
advances in the method of regarding them, cease in any degree to
explain them satisfactorily. This has happened not once but several
times with the amniotic theory in teratogenesis, progress in the
allied subjects of Embryology, Biology, and Pathology having called
for a revision of current views. Let us, in the first place, consider
the facts, and thereafter deal with the theories.

The facts are of different kinds, and to be intelligible they must
be stated with some degree of fulness. The reader must not be
impatient of details at this stage in his study of Antenatal Path-
ology, for he is now approaching some of its deepest problems and
most hidden mysteries; he is entering into the penetralia or inmost
recesses of teratogenesis. Let him be resigned while the facts are
being marshalled.

The first detachment of facts contains the observations that have
been made upon adhesions between the cranium or face of the foetus

168

ANTENATAL PATHOLOGY AND HYGIENE

and the amnion. Since in many of these cases the adherent part of
the amnion has been that covering the fcetal surface of the placenta,
the observers have described them as instances of adhesion of the
head to the placenta ; no objection need be taken to the nomen-
clature so long as it is borne in mind what the actual facts are.
More than fifty cases of this kind have been collected together by
Taruffi (Sforia delta teratologic, i. 270, 1881 ; v. 298, 1889 ; viii. 59,
1894), and I have found some additional records. Further, I have
been able^ to examine two foetuses showing various malformations

Fir;. 25.— Foetus showing amniotic adhesion to forehead, double facial fissure, and

exomphalos. Specimen No. 300.

with adhesion of the head to the amnion covering the
m one of these instances the specimen came into my

associated
placenta ;

possession with the afterbirth still attached to the fcctus, leaving
therefore no room for doubt as to the exact nature of the case. For
this interesting monstrosity (Fig. 25) I am indebted to Dr. S. H.
Richards, of North Ormesby, Middlesbrough, from whom I received
it in March 1903. It was the product of a five months' pregnancy;
the mother, a II-para whose first pregnancy ended in the birth of a
normal infant fell off a table and badly bruised her side when two

AMNIOTIC ADHESIONS TO HEAD 169

months advanced in gestation; there was at the labour a large amount
of liquor amnii, and it was stated that before the quickening the
abdomen had been larger than usual and had thereafter diminished ;
the shoulder of the foetus and the placenta presented together, and
the latter and the whole foetus (a female) came away at the same
time. There was no osseous cranial vault, and the brain projected
as a bilobed mass covered only by the membranes ; there was a
double facial fissure, and the eyes were malformed ; most of the
abdominal contents were outside the abdominal cavity (exomphalos) ;
the umbilical cord, five inches in length, was inserted as usual into
the foetal aspect of the placenta ; the foetal membranes were some-
what torn and disarranged ; and from the amnion over the placenta
a distinct band, showing some twisting, passed to the cerebral mem-
branes above the left eye. There was a circular groove round the
right upper arm, and both feet were clubbed. By a curious coinci-
dence I had received from Dr. T. J. Thomson, of Edinburgh, an
almost exactly similar monstrosity not many days before Dr. Eichard's
specimen arrived ; this (Fig. 26) was an eight months' foetus with
exomphalos, a single facial fissure (right side), absence of the cranial
vault, and malformation of the right hand ; unfortunately the
placenta was not available for examination, but the head and upper
part of the foetus were surrounded by strands of membrane continu-
ous with the cerebral membranes and peritoneum, and it is probable,
therefore, that in this case also there had been extensive amniotic
adhesions.

Cases resembling the two above described have been reported, as
has been said, in considerable numbers. One of the oldest observa-
tions was that made by Paul Portal1 in 1671 and published in 1685.
He was called to a labour case, and found the infant presenting by
the back of the neck ; he turned and delivered, and discovered that
the foetus was monstrous. It was a female, and had attached to the
head a large sac from which a band of membrane passed to the
foetal surface of the placenta; there was a hare-lip (median in
position according to the illustration) ; the right hand lacked the
ring finger and the left arm was shortened ; the feet were clubbed.
The umbilicus, however, was normally closed. To his record the
author adds the reflection that one often sees strange things at con-
finements, and " on pent dire que la Nature se joue dans la genera-
tion." Another early observation was that of Adam von Lebenwaldt,
published in 1690. His record was entitled " Singularis Amnii cum
foetu coha?sio " ; it was so brief that I may give it here verbatim :
" Scriptum mini est a Viro fide digno in Dioecesi Salisburgensi natum
fuisse infantem, cui membrana amnios per totum caput, faciem usq ;
ad pectus arete con juncta fuit ; dum circa os et labia secaretur, infans
linguam exporrexit et per unum diem vixit." Von Lebenwaldt is
truly so laconic that he leaves us in doubt as to the state of the
foetus, although he finds room to tell us that it put out its tongue !
Possibly the " man worthy of trust " who saw the case did not know
the details.

1 Vide bibliographical list at the end of this chapter for this and other references.

170

ANTENATAL PATHOLOGY AND HYGIENE

Fig. 26. — Foetus with exomphalos, absence of cranial vault, facial fissure, deformed
right hand, and amniotic adhesions. Specimen No. 299.

CEPHALIC ADHESIONS OF AMNION 171

A few cases were reported in the eighteenth century, and in
1800, T. Pole, Surgeon and Lecturer upon Midwifery, London, de-
scribed a typical instance. The infant had been born as a footling,
and had lived for thirty-six hours. The bones of the cranial vault
were absent, a large sac (cerebral hernia) occupying the summit of
the head ; there was a facial fissure and a split nose ; and the middle
ringer of the right hand was defective. The placenta was attached
to the upper part of the child's head, the largest portion lying over
the occiput and scapulae. Pole had not before seen a case of placental
adhesion to the head, but he reflected that " Nature is, however, so
frequently regardless of her common laws, as to deviate in such an
endless variety, that the best informed man can have no adequate
idea of the diversity of her productions." He seems to have ascribed
the condition of the cranium and placenta to inflammation of two
surfaces in contact with each other, followed by adhesions.

It is unnecessary to describe in detail any further cases, for they
all have a certain family resemblance. Among the more noteworthy
of them may be named those reported by T. C. Cam, E. Geoffroy
Saint-Hilaire, J. F. Meckel, Cerutti, Rudolphi, J. Y. Simpson, 1-Jobert
Lee, A. G. Otto, Pies, llayer, Houel, Heyfelder, Barkow, Forster,
Virchow, Jensen, Charvet, Walker, Hager, C. E. Stein, Hein, Bassett,
Bogatsch, Fromm, Baacke, Broca, Gueniot, Jester, Krumrey, Debierre
and Lambert, Fronhiifer, H. N. Eobertson, Fleischer, Jacobs, and
Veith. From these cases we may draw certain general conclusions
regarding cephalic adhesion of the amnion. The bones of the cranial
vault are usually absent, and most commonly there is no proper scalp,
the brain being covered only by its membranes ; in many instances
there is also encephalocele or hydrencephalocele as well ; in a few
cases there is absence of the brain (auencephaly). The amnion on
the placenta or bands proceeding from it are usually attached to the
membranes covering the more or less altered brain ; but sometimes
the amniotic connection is formed with the face, which then shows
malformations. The facial deformity generally takes the form of
hare-lip or facial fissures ; in the latter case the fissure, which may
be unilateral or bilateral, passes from the mouth to the orbit, and
sometimes has the amnion attached to its margins. The nose and
eyes may also exhibit various deformities. The malformations are
not necessarily limited to the region of the head, for the anterior
abdominal and thoracic walls may be defective and allow the pro-
trusion of several of the viscera ; further, the extremities not un-
frequently show such anomalies as absence of digits or parts of
digits, so-called congenital amputations, club-feet, and circular or
oblique grooves. A limb may also be fixed to some part of the
body, even to the head, by a band. The umbilical cord is usually
shorter than normal.

A second detachment of the facts regarding the amniotic factor,
which I am now marshalling, contains the cases in which the
abdomen of the foetus is the part most obviously affected. In these
cases the amnion is generally attached to the margins of a more or
less large opening or defect in the anterior or lateral abdominal wall.

172

ANTENATAL PATHOLOGY AND HYGIENE

I have been able to examine several specimens of this type of mon-
strosity. One of the most typical of them (Fig. 27) was the six
months' female foetus given to me for examination by Dr. Ballantyne,
of Dalkeith, and briefly described by me in the Transactions of the
Edinburgh Obstetrical Society (vol. xvii. p. 241, 1892). The case was
a footling, and, as there was some haemorrhage going on, the labour
was quickly terminated by traction on the feet; during the extrac-
tion of the fcetus a sac was ruptured, and this sac, which had been
taken for the bag of membranes of a second child, was found to be

Fig. 27. — Case of Exomphalos, Anencephaly, and adhesion of amnion to umbilical

aperture. Specimen No. 24.

made up of amnion adherent to an opening in the anterior abdominal
wall and continuous with the peritoneum. The sac contained several
of the , abdominal viscera (liver, spleen, stomach, large and small
intestine) as well as the heart, for the diaphragm was defective ;
there was no separate umbilical cord, for the vessels ran in the wall
of the sac directly to the membranes to be inserted near the centre
of the placenta, and had a length of not more than twelve centimetres.
In addition to the exomphalos thus produced, the fcetus showed
anencephaly, spina bifida affecting the whole spine, and marked

ABDOMINAL ADHESIONS OF AMNION

173

retroflexion of the vertebral column (Fig. 28). The mother had
given birth on previous occasions to four healthy children.

Another somewhat similar fo3tus was given to me by Dr. C. B. Ker
in 1894. The mother had previously had three children, all of them
normal ; during the present confinement she had suffered much from
pain in the back and left iliac fossa unlike anything she had had in

Fig. 28. — Frozen section of Specimen shown in Fig. 27, showing anencephaly,
retroflexion of spine, and exomphalos.

her former gestations. There was some hemorrhage during the
confinement, and the head, although descending easily on to the
perineum, had to be delivered with forceps. The foetus and placenta
were born together. There was no excess of liquor amnii. The
mother had never been strong, and the father was crippled with
sciatica and was alcoholic. The foetus, the sex of which was not
apparent, had an exomphalic protrusion continuous with the mem-

174

ANTENATAL PATHOLOGY AND HYGIENE

branes and placenta ; it had also a big sacral meningocele, clubbed
feet, and an imperforate anus (Fig. 29). I exhibited the monstrosity to

Fig. 29. — Fa'tus showing exomphalos, the sac being continuous with the amnion,
and sacral sjiina bifida. Specimen No. 124.

the Edinburgh Obstetrical Society (Transactions, vol. xix. 106, 1894).
I have examined two or three other foetuses in which the abdominal

DORSAL ADHESIONS OF AMNION 175

protrusion was almost certainly continuous with the amnion on the
surface of the placenta ; but as I did not obtain the afterbirth with the
foetus I shall not cite them as instances of amniotic adhesions.

Cases resembling more or less closely the above monstrosities
have been put upon record in considerable numbers. Taruffi
gathered together over forty of them (Storia delict teratologia, i. 276,
1881 ; viii. 63, 1894), including one personal observation, and many
others have been observed in the past ten years. Some of the
authors of the articles in which these monstrosities are described
may be named : they are Littre, Saxtorph, Breschet, Meckel,
Heusinger, Bosenbaum, Henot, Simpson, Otto, G. Braun, Calori,
Gusserow, Dehn, Jensen, Johnson, Philipeaux, Oswald, Buschan,
Leith Napier, Bischpler, and B. Marchese. In fact almost every
writer who has reported a case of exomphalos has had something to
say regarding the attachment of the amnion or of the placenta to
the abdominal aperture.

The general characters of this type of monstrosity have been
indicated in connection with the two cases that I have described.
The chief character is the attachment of the placenta to the defective
anterior abdominal wall of the foetus by the instrumentality of the
amnion, which, as a rule, passes from the margins of the opening to
the surface of the placenta, leaving thus an enclosed space which
contains the abdominal viscera. The viscera, therefore, come into
contact with the chorion covering the placental surface at this point,
as has been diagrammatically shown in connection with Leith
Napier's specimen (q.v.). The umbilical vessels are not usually
gathered together into a cord, but run on the inner aspect of the
amnion to the placenta; they are consequently rather short. The
intestinal coils may show adhesions to each other and to the amnion.
Not uncommonly the defect in the abdominal wall extends low
enough to affect the bladder (vesical exstrophy) and symphysis pubis;
then the deformity has been called by the Germans BauclMascn-
schambeinspalte. The vessels, especially the umbilical arteries and
vein, are often anomalous. The genitals may be affected ; there may
be little or no indication of sex, or there may be a cloacal condition ;
the anus may be imperforate. The vertebral column is nearly
always deformed (scoliosis), and the limbs may be clubbed or grooved
or lacking in digits, or they may show dislocations. Some of the
recorded cases have also had malformations of the head resembling
those described in the first series of observations.

A third group of facts may now be referred to, namely those in
which the amnion is attached to the back of the body of the foetus.
I have in my collection two foetuses which show this amniotic
anomaly. In one of them, which I got from Professor Simpson in
1889, there was well-marked exomphalos, a double facial fissure
(Fig. 30), a cleft palate, a diaphragmatic hernia, a stunted condition of
the right arm, irregularities of the digits, grooves round the limbs, a
rudimentary penis, and an imperforate anus. In addition to these de-
formities, however, there was a curious conical projection near the lower
end of the vertebral column, which seemed to contain the lower end

176

ANTENATAL PATHOLOGY AND HYGIENE

of the spine in it, and from its tip a narrow band or filament passed
round the right side of the foetus to join the membrane (amnion ?)
in connection with the exomphalos (Fig. 31). During the pregnancy
(seventh), or rather since the middle of it, the mother had suffered
from pain in the left iliac and left lumbar regions, growing in
intensity and becoming very severe during labour. The sac contain-
ing the abdominal viscera presented, and there was haemorrhage

Fig. 30. — Fcetus showing exomphalos, facial fissures, and unilateral atrophy
of right side. Specimen No. 23.

during the confinement, calling for delivery by traction upon a foot.
The placenta and membranes were not available for examination.
My second case was that of a female anencephalic foetus (Fig. 32)
given to me by Dr. Lundie, and briefly described in the Transactions
of the Edinburgh Obstetrical Society (vol. xx. 89, 1895). In addition
to the anencephaly, there was talipes calcaneus, a malformed right
thumb, and spina bifida in the lumbar region. From the margin of
the spina bifida a band passed to the membranes, which were wrapped

AMNIOTIC BANDS

177

round the lower limbs when the specimen came into my possession
(Fig. 33). The pregnancy had lasted eleven months ; and the mother

Fig. 31. — Posterior view of Foetus (Specimen No. 23), showing sacral projection
with band passing from it to exomphalic sac.

had already given birth to a hydrocephalic and otherwise malformed
infant as the result of a former pregnancy.

Cases such as the above would appear to be rare, but Strassmann

VOL. II. 12

178

ANTENATAL PATHOLOGY AND HYGIENE

Fk;. 32. — Fcetus with Anenceplialy, Deformed Right Thumb, and Adherent
Membranes. Specimen No. 148.

AMNIOTIC BANDS

179

Fig. 33.— Posterior view of Foetus (Specimen No. 148), with lumbar spina bitida
and amniotic thread attached to it. Also shows anencephaly.

180 ANTENATAL PATHOLOGY AND HYGIENE

has described a specimen in which there was a spina bifida in the
neck (spina bifida atlantica), and from its apex a band passed to the
amnion. Ahlfeld, also, in 1894, figured a somewhat similar instance
in which, however, the amnion was attached a little above the level
of the neck ; this case was apparently the one observed by Pinard
and Varnier in 1892. There are, nevertheless, enough of such
observations {e.g. those of Pies and Klotz) to prove that the amnion
may be adherent to the back of the foetus as well as to its head and
to the anterior aspect of its abdomen.

In a, fourth detachment of the facts which I am attempting in
some sort to classify and marshal are the cases in which the
adhesion is between some part of the foetus and the umbilical cord,
or rather the covering of the cord. I have not myself met with an
example of this variety of amniotic adhesion, but many such have
been reported by careful observers, so that Tarufri ' (Storici delta
teratologic/,, i. 280, 1881 ; v. 303, 1889 ; viii. G6, 1894) was able to
collect more than twelve of them, including those by Portal, Burggrav,
Sandifort, Walter, Meckel, Nicati, Haan, Simpson, Tessier, Klotz,
Hein, Hager, Garnett, and Leopold. Within recent years several
interesting cases have been added to the list, and I may specially
refer to those of F. von Winckel and of Couvelaire. In the former
an amnio-amniotic band arose by a broad basis of attachment from
the placenta near to the origin of the umbilical cord, and formed a
figure of 8 loop round the cord and round the right upper arm of
the foetus ; a distinct circular groove was thus produced in the arm ;
a torn band was also found attached to the index finger of the left
hand. In Couvelaire's specimen the foetus had died suddenly during
labour ; it was a male and was not malformed. The placenta was
circular ; the membranous sac was incomplete, the half only of the
placental border giving attachment to a large membranous piece,
which included the amnion, chorion, and decidua. From one border
of this piece a thin strip, formed by amnion, passed to the umbilical
cord around which it was coiled three times and knotted as well ;
then it passed on to a separate piece consisting of chorion, amnion,
and decidua. The umbilical cord was 51 cms. long, and was very
thick and ©edematous save at the point of constriction, where its
diameter was only 7 nuns. The amniotic or inter-amniotic band
was more than 23 cms. in length. In the cases of F. von Winckel
and Couvelaire the foetus was not specially malformed, but in
some of the other reported instances (e.g. Haan's) it was clearly
monstrous.

A fifth group contains the cases in which a band lias been
observed passing from one part of the foetus to another or from
one part of the amniotic covering of the placenta to another. I
have, in my collection, three well-marked instances of this type of
malformation. In one of them, a foetus with placenta which I
received from Dr. Maclagan, of Sleaford, Lincolnshire, in October
1901, there was marked exomphalos, and the right arm was fixed to
the margin of the aperture in the abdominal wall by a band of skin
inserted into the wrist (Fig. 34). The mother had always been very

AMNIOTIC BANDS

181

>

i

V

Fig. 34. — foetus with Exomphalos and Cutaneous Band from wrist to margin of
opening in abdominothoracic wall. Specimen No. 268.

182

ANTENATAL PATHOLOGY AND HYGIENE

delicate and subject to fainting fits ; this foetus was the product of
her second pregnancy. Another specimen illustrating the adhesion
of different parts of the foetus to each other was that of a small twin
born along with a normal infant ; there was a single placenta, by far
the larger part of which belonged to the normal infant ; the small
foetus had its own amniotic sac, and showed membranous union of the
lower limbs with partial rotation of them, so that the right heel
looked forwards (Fig. 35). The membrane between the legs seemed

FlG. 35. — Twin Fcetus, showing early stage of synipodia and fused placenta.

Specimen No. 209.

to be continuous with the sheath of the umbilical cord. It is
probable that there was here the first stage of sympodia, and that
the membranous adhesion had to do with its origin. The mother
was healthy, but had had two miscarriages as well as five earlier
normal labours. The specimen was given to me by Dr. Lundie in
1898. The third specimen to which I shall here refer has been
mentioned already ; it was the case of exoniphalos, facial fissure, and
adhesion of the amnion to the head sent to me by Dr. Richards, of

PATAGIUM 183

North Ormesby (Fig. 25); and in addition to the above deformities
the foetal aspect of the placenta showed a filiform band (amniotic)
fixed to it at both ends and forming a loop ; probably some part of
the foetus had lain in this loop during intrauterine life, but there
was nothing to indicate what part it had been.

Several cases illustrating the above type of adhesion are to be
found in the literature of the subject. The first of the two foetuses
described by C. L. Klotz, for instance, is a good example ; in it the
amniotic surface of the placenta was adherent to the back of the
infant, there was an amniotic adhesion to the face, and in addition
there was a cutaneous bridge in the lower part of the back. These
adhesions of bands may be called foeto-foetal or amnio-amniotic,
according as they pass between two parts of the fcetus or of the
amnion. Possibly the weblike structure (Flughauthildung of the
Germans) which is in rare cases found passing between various parts
of the body may be of the same nature as the foeto-foetal bands
referred to above. The weblike expansion, to which the name
patagium may be conveniently given, may pass from the neck to the
shoulder on one or both sides, as in 0. Kobylinski's observation
(Arch. f. Anthrop., xiv. 343, 1883); or it may stretch from the
posterior aspect of the thigh to the back of the leg, as in the cases of
J. Wolff (Arch. f. klin. Chir., xxxviii. 66, 1889) and K. Basch
(Ztschr.f. Heilk., xii. 499, 1891; Prog. med. Wchnschr., xvii. 289,
1892) ; or it may reach like a wing from the upper arm to the chest-
wall, as in the instances reported by L. Brims and L. Kredel
(Fortschr. d. Med., viii. 1, 1890), by J. Benario (Berlin, klin. Wchnschr.,
xxvii. 225, 1890), by J. Thomson (Teratologia, ii. 1, 1895), and
others. The patagium may not be amniotic in origin, but it is some-
times found associated with malformations which are generally
regarded as such.

A sixth group of facts concerning this complicated and perplexing
amniotic problem contains the observations which have been made
upon the relation of bands of the amnion to malformations of the
limbs. The number of cases in which deformities of the extremities
of almost all kinds have been ascribed to the action of the amnion is
very large ; but the instances in which bands or filaments or adhesions
have been actually recognised are comparatively few. Of course only
the cases in the latter category can be looked upon as facts in the
strict sense of the word. To this sixth group of facts belong the
so-called spontaneous or intrauterine amputations ; to these interest-
ing deformities I have already alluded in the first part of this Manual
(p. 396), and also in the preceding chapter in connection with the
possible teratogenic influence of the umbilical cord. I need not,
therefore, repeat here what has been previously stated regarding
them.

The deformities of the limbs which have been ascribed to amniotic
bands and filaments are very various, and in only a few of them have
the connections with the amnion been actually found in situ. I have
not myself had an opportunity of examining a case in which the bands
were to be seen attached to the deformed part of the limb, but others

184

ANTENATAL PATHOLOGY AND HYGIENE

have observed this, and there is no reason to doubt its occurrence ;
probably the process of birth is a great means of tearing and
separating these connections. F. von Winckel was fortunate in being
able to record and figure a case in which an amniotic band arose from
the placental surface by a broad basis, passed to encircle the left
upper limb, then formed a loop round the umbilical cord, and finally
returned to the placenta ; in its course it made a figure of 8 con-
volution ; there was marked smallness of the limb above the con-
striction ; and there was a second short band attached to the index
finger of the left hand, which doubtless represented a torn-across
filament. J. Wolffs observation (Fig. 36) was even more demon-
strative of the direct connection between the deformity of an
extremity and an amniotic band : in it a stout thread took origin

Fig. 36. — J. Wolffs case of Amniotic Adhesions to the Fingers (vide Arch. f. Oynaek.,

lx. 281, 1900).

from the foetal surface of the placenta, and passed to the tip of
the middle finger of the right hand of the foetus, the nail of which
was deformed ; it then was continued to be attached to the ring
finger, the terminal phalanx of which was fixed to the hand only
by a thin band ; the whole length of the band was 10 cms..; the
left hand showed syndactyly of the ring and middle fingers, and
their terminal phalanges showed constrictions and defects, which
Wolff regarded as probably due to a second amniotic band which
had disappeared.

In other cases, malformations of the extremities are present and
amniotic bands are present, but the two are not connected together ;
in sucli instances, therefore, the evidence of structural continuity
between the bands and the deformities (grooves, amputations, con-
tortions) is not flawless, but it is strong enough to merit a general

AMNIOTIC AMPUTATIONS 185

acceptance. In Moreau's case, for instance, the foetus showed amputa-
tions of several digits, and the amniotic surface of the placenta showed
threadlike bands, some of which hung free at one end while others
were attached at both ends. It is reasonable to connect together the
two abnormal states. In yet other cases various malformations of the
extremities are to be noted, and bands may be found adhering to
them ; but there are either no traces of anomalies of the amnion, or,
what is more common, no information is forthcoming about the
amnion, for the sufficient reason that it has not been preserved or
examined at the time of birth. Many examples of these imperfectly
observed and recorded cases are to be met with in obstetrical litera-
ture, and to some of them I must now direct the reader's attention.
I may add that I have had occasion in my own experience twice
to lament the loss of such specimens : in one instance through my
absence from Edinburgh on University work in Aberdeen I missed
seeing a case of multiform deformities of the digits of a premature
infant, and I lost the chance of securing the membranes if not also the
foetus for examination ; in the other instance a medical friend told me
of the birth in his practice of an infant whose fingers showed all the
stages of intrauterine amputations, one of them having a raw surface
at the tip ; in this case also no post-mortem examination was allowed,
and the placenta and membranes were not secured for inspection.

Perhaps the most interesting of all these imperfectly recorded
cases are the two referred to by J. U. T. Schaeffer in 1775 (vide
Bibliography at the end of Chapter X.) and by P. Zagorsky in 1834.
Unfortunately I have not been able to consult these records in
the original. The following notes of them, however, are given
by J. Y. Simpson in his article published in 1836. In Schaeffer's
specimen the fingers of the right hand showed syndactyly, and the
thumb was large and without a nail ; the thumb of the left hand also
had no nail, and the index and middle fingers wanted their second
and third phalanges ; the second toe of the right foot had a sort of
membranous cord attached to it, and the great toe was short, de-
formed, and had a cicatrix where the nail ought to have been ; all the
left leg below the gastrocnemii seemed, as it were, absent, and from
the uncicatrised central part of the stump a fibrous cord took its
origin, to which was appended a small body which " on minute
examination proved to be a foot of the size of one belonging to a
fcetus of the third month, witli divisions on its inferior edge repre-
senting the five toes" (Fig. 23). The infant was at the eighth month.
Zagorsky's specimen was a five months' male foetus : the head and
face were deformed, in much the same manner as in the cases of
amniotic adhesion to the head to which I have referred (vide p. 169) ;
the right hand was slightly malformed, and the left hand greatly so,
the fingers being absent or represented only by cutaneous nodules ;
the right thigh ended in a rounded stump, from the centre of which a
slender, threadlike membrane ran across to below the middle of the
left leg, which it encircled, causing a groove ; from about the middle of
the threadlike membrane joining the two legs, a small oblong body
was suspended, which on inspection proved to be the right foot, of the

186 ANTENATAL PATHOLOGY AND HYGIENE

size of the foot of a fcetus of the tenth or twelfth week. The im-
portance of these two eases can hardly be exaggerated ; for, if they
were accurately described and figured, they prove almost to demon-
stration that threadlike bands can lead to the amputation of foetal
parts. J. Y. Simpson, however, refers to the defective representation
of the structures in Zagorsky's original drawing ; and it is a striking
fact that no such clearly teratogenic bands (with the possible exception
of the case reported by Fieux) have been recognised in recently
published observations. While, then, we must regard Schaeffer's and
Zagorsky's cases as facts, it is perhaps not so certain that the little
filipendulous foot represented in the accompanying illustrations was
also an indubitable fact. Besides the cases of Schaeffer and Zagorsky,
instances of deformed limbs with bands adhering to them have been
put on record by Montgomery (1837), Frickhoeffer (1856), Crede
(1858, 1869), Barkow (1859), Kottmeier (1864), Eeuss (1869), Fiirst
(1871), and many others. In the recent case of Fieux (1902) there
was a filipendulous hand instead of a foot (" tme petite main en minia-
ture ").

Finally, in this sixth detachment of facts must be placed the
cases in which malformations of the limbs have been met with, which,
although no traces of bands or amniotic connections have been dis-
covered, have yet been ascribed to such agencies simply because they
have resembled the malformations in which amniotic adhesions were
present. I have seen several instances of this kind. In 1891, for
example, I saw, with Dr. T. B. Darling, a baby of a few days old
whose right arm looked as if it had been amputated at the wrist
(Fig. 37); there was a well-formed conical stump with five little
nodules at the end of it which Dr. Darling regarded as rudimentary
fingers. The child was otherwise well formed, and no convolutions of
the cord or bands from the amnion had been observed at birth. This
case was shown to the Edinburgh Obstetrical Society in March 1891
{Transactions, xvi. 89, 1891). In 1898, I had the opportunity of
examining Dr. Gemmill Thomson's case of an armless male infant ;
the arms looked as if they had been amputated at the shoulder, and
the lower limbs were stunted ; the child was shown at a meeting
of the Glasgow Obstetrical Society (Transactions, i. 73, 1898), and its
anatomy was described by Dr. Lindsay in 1899 (Transactions, ii. 16,
1901). In 1900, 1 published (Interstate Med. Joiirn,, vii. 367, 1900) an
account of a kitten born without the right forelimb ; the specimen
had been given to me for examination by Dr. J. K. Drysdale. Cases,
like these which I have met with, have been reported in large numbers
in medical literature ; and, on account of the dexterity which armless
individuals have gained in using their feet instead of their hands, the
subjects of these deformities have forced themselves upon the attention
of the general public as well as upon that of the teratologist. Con-
necting links between the so-called spontaneous amputations and the
normal are found in the cases in which there exists a more or less
circular and a more or less deep groove round an arm, a leg, or a
digit. F. Potier, for instance, reported a case in 1898 in which an
infant, born at term, showed radial paralysis of the right arm ; on the

SPONTANEOUS AMPUTATIONS 187

external surface of the limb at the point where the radial nerve
traverses the humeral groove there was an irregular rather deep
depression of the skin, regarded by the writer as a sort of cicatrix
attributable to the pressure of an amniotic band; the histological
examination of the skin of the affected area showed an increase in
thickness and density of the dermis, a thickening of the rete Malpighii,
and a marked development of sebaceous and sudoriparous glands; the
radial nerve was found to exhibit an increase in its connective tissue
elements, and changes in its nerve elements consisting in disappear-
ance of nuclei and axis-cylinders and retention of vestiges only of
myeline ; the affected muscles had fibres smaller than normal and less
distinctly striated. Potier's record differs from most of the others
that are to be found in literature, in the fact that the histological

Fig. 37. — Infant with Spontaneous Amputation of
Right Hand. Case No. 41.

characters of the skin and underlying structures at the site of
the groove are described; too often these important details are
lacking.

A seventh detachment of facts regarding the amniotic theory must
now be drawn up in orderly array before the reader ; if indeed that
sorely tried individual has not already grown wearied of the whole
subject and turned his attention to some other part of this volume.
Several cases have been observed, and the number is constantly being
added to, in which there is good evidence to show that the foetus,
if not the embryo, has developed outside the amniotic cavity. Both
Hamard and Osmont have described a case of this kind which occurred
in the hospital practice of Dr. Paul Bar; Bar also put on record
a second somewhat similar case ; other instances are those of Lebedeff
(1878), of Maygrier {L'ObsUtrique, iv. 303, 1899), of M. V. Cathala

188 ANTENATAL PATHOLOGY AND HYGIENE

(L'ObsUtrique, vii. 309, 1902), and of Ferret (L'ObsUtrique, vii. 316,
1902); and E. Glaize dealt with the whole subject of La grossesse
extra-membrane vse in a Paris Thesis in 1899. In Bar's specimen the
chorion contained a foetus which lay free in it ; the amnion consisted
of a small sac from which the root of the umbilical cord emerged ; and
attached to the outer surface of the amnion were several threadlike
bands or amniotic fringes. The foetus showed malformations of the
nature of grooves round the left leg, the toes, and the fingers ; there
was also syndactyly, some of the digits were defective in the number
of the phalanges which they contained, and to the left hand a
membranous band was attached. Crouzat and Payrau (L'ObsUtrique,
vi. 235, 1901) met with a case of twin pregnancy in which the first-
born infant was normal ; the second foetus had exencephaly and
various facial and digital malformations (fissures, grooves, adherent
bands), was attached to the afterbirth by a very short cord (about
4 inches in length) and by two broad membranous bands, and had
been expelled from its proper amniotic sac and acquired adhesions
with the chorion. The foetus in the second case reported by Bar also
showed congenital grooves on the digits ; but these malformations are
not constantly associated with an extra-membranous foetus, for in
Cathala's five months' specimen there were no deformities at all,
neither were there any in Dubrisay's case exhibited at the meeting of
the Paris Obstetrical Society in March 1901 (L'ObsUtrique, vi. 274,
1901). Further, in a very curious specimen met with by Tarnier
(1896), as well as in one somewhat resembling it described by Bar
(1898), the foetus must have developed outside both the chorion and
the amnion, between these membranes and the uterine wall ; yet in
these two instances the foetus was not malformed. The observations
of C. Giacomini on emigration of the human embryo in early abortion
sacs (Sidle anomalie di sviluppo dell' cmbrionc umano, Torino, 1895) are
similar to the cases which have been met with by Bar and others at
a later date in pregnancy ; in some of Giacomini's specimens the
embryo had wandered into the outer ccelomic cavity and in others
it had passed through all the ovular membranes. H. Knoop's
2 mm. embryo was attached by its back to the chorion (Bcitr. z.
Geburtsk. u. Gynaek., vii. 284, 1903). These extra-amniotic and extra-
membranous foetuses form an interesting group of facts which must
in the future find a place in all theories of amniotic influence in
teratogenesis.

An eighth and final series of facts contains the observations which
have been made upon amniotic bands, adhesions, and pressure in the
lower animals. Apparently, amniotic adhesions are very rare in Com-
parative Pathology, for Taruffi (Storia dclla teratologia, i. 283, 1881-; v.
301, 1889) has only been able to find three cases, two of which were
reported by Gurlt ( Ueber thierische Missgcburten, plate ix. fig. 57 ;
plate xiv. fig. 75, Berlin, 1877), and one by Millot (Journ. de nUd.
titer., v. 53, 1834). One of Gurlt's specimens was a lamb with a
wide buccal aperture (megalostomus) and the amnion adherent to
a hydrocephalic sac ; the other was a calf-foetus with cyclops, megalo-
stomus, and the membranous amnion attached to a hydrence-

A MNIOTIC ADHESIONS IN ANIMALS 189

phalocele. Millot's specimen was also a calf, and the amnion was fixed
to the left cheek below the eye. In strong contrast with the scarcity
of observations of amniotic adhesions in the lower animals is the large
number of cases in which it has been found possible artificially to
arrest the development of the amnion in the hen's egg during in-
cubation. C. Dareste, in his long, laborious, and epoch-making series
of experiments upon the chick during development {Production
artijicielle des monstruosiUs, Paris, 1877; 2nd edit., 1891), was early
struck by the frequency of the coexistence of various malformations
and monstrosities with arrested development of the amnion. Some-
times, although rarely, the amnion was not developed at all, and then
the embryo usually died early or was more or less gravely altered in
its form ; more commonly certain parts of the amnion, e.g. the cephalic
or caudal folds, were delayed in their evolution, and then the embryo
was monstrous in the part corresponding to the defect of development
of the surrounding membrane. Dareste ascribed these embryonic
defects to pressure produced by the non-separation of the amnion
from the body or head of the embryo, and believed that in this way
it was possible to account for most of the simple monstrosities,
such as anencephaly, cyclopia, and sympodia, and for many other
malformations.

Such, then, are some of the facts connected with this great
amniotic theory of teratogenesis. I have endeavoured to pass them
before the reader's mental vision in some sort of regular order ; I
have tried to show that after all there is a certain, if obscure, arrange-
ment of them, and that they are not unrelated to one another. I have
enumerated illustrative cases in which the amnion has been found at
birth to be adherent to the head, face, abdomen, back, and limbs of
the foetus ; I have referred to instances in which amniotic bands have
been seen stretching from one part of the general amniotic surface
to another, and to others in which cutaneous bands have passed from
one part of the foetus to another ; I have cited examples of so-called
spontaneous amputations, in some at least of which amniotic bands
seemed undoubtedly to have been instrumental in their production ;
the curious cases have been alluded to in which the foetus seems to
have developed entirely outside the amnion if not also outside the
chorion ; and reference has been made to defects in the amnion
discovered in association with experimentally induced monstrosities
in the chick. The survey has been a long one, for the field of facts
has been a large one ; but an apology is hardly called for, facts of all
kinds being of no little value and the time expended in mastering
them being well spent. Facts are indeed the data of experience and
observation and have a prime importance ; factlessness is a quality
most fatal to a subject appealing to the scientific mind, as we trust
Antenatal Pathology does appeal ; but we must not be content with
the accumulation of facts, we must pass sooner or later (the fashion
of the day is later rather than sooner) to contemplation of the facts,
to reflection upon them, and to the drawing of conclusions from them.
What do the facts mean ? What can we learn from them ? Have
they anything to teach us ? Thus are we brought naturally enough

190 ANTENATAL PATHOLOGY AND HYGIENE

to the second part of the amniotic theory, to the reflections based
upon the facts, to the interpretation of the phenomena, to the
thinker's thoughts about the observer's data. The data are the
things given to us, not simply to be labelled, catalogued, and placed
safely on the shelf, but to be absorbed, intellectually assimilated, and
given back again to Science in the form of conclusions, inferences,
deductions, and theories. Let us now consider the theories or hypo-
theses that have grown up around the facts.

Etienne Geoffroy Saint-Hilaire {Philosophic anatomique, pp. 237,
530, 539, 1822) was glad to seize upon the notion of adhesions to the
amnion as a reasonable and sufficient cause for the various mon-
strosities which he had met with. At first, he wrote, the spectacle
of monstrosities so numerous and so extravagantly bizarre led him
to think of the constructive forces in their saturnalian days (" dans
ses jours de saturnales "), tired out with the weary task of formation
and seeking relaxation in capricious developments. To him amniotic
adhesions offered a scientific explanation of what had seemed inex-
plicable, fantastic, a saturnalia of bizarrerie. He saw in bands
passing from the membranes to the foetus the single and satisfactory
explanation of all monstrosities. The foetus was like the lung in the
pleural sac ; its skin, if not surrounded by liquor amnii, might con-
tract adhesions with the enveloping membranes ; these adhesions
were vascular in their nature ; and the viscera of the foetus, if not
shut off from the exterior, might become attached by such vascular
connections to the placenta. " Je crois," he wrote, " qu'il n'est
qu'une cause unique, gonc'rale et exterieure" de monstruosites, qu'il
n'existe qu'un seul mode pour faire devier les formations organiques
de l'ordre commun ; c'est quand le f ictus contracte des adherences
avec ses membranes ambiantes." Meckel, however, thought that
Saint-Hilaire's explanation could only hold good in a few cases, for
in all his specimens there had been no adhesions. C. E. Eudolph, in
1829, went a step further and maintained that the adhesions were
not the cause but the effects of the monstrosity of the foetus. He was
of opinion that if the foetus were brought near to the placenta it
might compress and irritate it and so lead to adhesions ; or that, if the
skin of the unborn infant were the seat of inflammation, a plastic
lymph might be thrown out which would form adhesive bands glue-
ing it to the membranes. He thought also that if the foetus had the
monstrosity known as anencephaly, pointing to antecedent hydro-
cephalus {i.e. inflammation of the brain), the inflammatory changes
would occur with all the greater probability.

Isidore Geoffroy Saint-Hilaire, influenced perhaps by the criticisms
to which his father's theory had been subjected, stated that he did
not regard it as applicable to all monstrosities and anomalies, but
only to those characterised by defect, by displacement, and by want
of union of contiguous surfaces ; when no adhesions were to be seen
he supposed that they had been torn through {Histoire des anomalies,
iii. 523, 1836). While, however, these criticisms and their conse-
quences were in process of elaboration on the Continent, Montgomery
and J. Y. Simpson were engaged in the study of what may be called

AMNIOTIC THEORIES 191

the liner varieties of adhesions and bands. The Saint-Hilaires had
dealt only with the gross lesions due to widespread union between
the f(ctus and the amnion covering the placenta ; but Montgomery
got the help of a newly observed series of facts, the cases in which
the limbs are surrounded by threadlike ligatures and show various
stages in the production of amputations and other malformations.
In 1832 Montgomery wrote of these ligatures as " distinct threads
of, I presume, organised lymph," and explained their mode of action
as follows : " From the condition of the limbs thus produced, and the
impossibility of the parts below the ligatures continuing their growth
under such circumstances, it seems exceedingly probable that, as the
child continued to live and grow, the parts of the legs below the
ligatures would have been separated and thus undergone spontaneous
amputation ; the formation of these threads, and particularly their
application so as to stricture the limbs, are circumstances in explana-
tion of which I do not feel prepared even to hazard a conjecture."
J. Y. Simpson accepted Montgomery's view that the bands were of
the nature of organised lymph ; he proceeded to expand the theory
in the following words : " That this lymph has been effused by
inflammatory action is, I am inclined to think, in the highest degree
probable. No one would now attribute such morbid bands or cords,
if they were found in other parts of the body, as in the cavities of
the chest or abdomen, to any other morbid action than that of
inflammation ; and that the cutaneous texture of the foetus in utero
is liable to inflammation attended with the effusion of organisable
lymph is known from the circumstance of different parts of the sur-
face of the foetus being occasionally found adhering, either directly
or by the medium of false membranes, to the amnion covering the
placenta, ... to some part of the umbilical cord, ... or to other
parts of its own body. Further, that organisable lymph, when effused
by inflammatory action between two contiguous parts of the surface
of the foetus, is capable of being changed into pseudo-membranous
bands or cords, similar to those observed in Dr. Montgomery's first
case, and in the cases described by Schaeffer and Zagorsky, appears
from the circumstance of lymph occasionally assuming this form,
when effused between the contiguous points of such surfaces as have
a free motion on one another, as is sometimes seen in the bands of
false membrane found stretching from one point to another of the
cavities of the pleura, pericardium, and peritoneum." It is quite
clear, from these quotations, that Simpson regarded Montgomery's
bands as similar to those found in cases of pleuritis and peritonitis,
and as due to an inflamed condition of the two apposed surfaces
of the foetal skin and the amniotic membrane during intrauterine
life. Here, then, was the theory of disease of the amnion as the
cause of monstrosities stated fully and freely ; and other writers
were not slow in applying it to the explanation of many other
deformities besides those specially considered by Montgomery and
Simpson.

The amniotic theory, in its strictly nosological aspect, met with
a good deal of criticism. Simonart (1846), while generally accepting

192 ANTENATAL PATHOLOGY AND HYGIENE

the conclusions reached by Simpson, gave to the bands the unfortunate
name of " ligaments," and did not exclude the possibility of the ad-
hesions being due to cutaneous ulcers. Kristeller, in 1859, also
admitted the existence of inflammation of the skin. Scanzoni, from
a consideration of the anatomical characters of the amnion, doubted
whether it could produce an inflammatory exudation, and was inclined
to think that the lymph transuded through the membrane from the
wall of the uterus. Houel was rather inclined to adduce shortness of
the umbilical cord as an important causal agent ; it kept the foetus in
close contact with the membranes, and so favoured adhesion ; but he
did not take into account or explain the instances in which the cord
had been found of usual length. G. Braun advanced again the state-
ment which Etienne G. Saint-Hilaire had originally made, that the
liquor amnii must be scanty to permit of the formation of adhesions ;
when it is small in amount the amniotic membrane (or folds of it)
remain in contact with the fetus ; these folds are with growth drawn
out into bands, and by the traction they exert upon the foetal parts
lead to malformations of them. The coexistence of hydramnios (in-
stead of scarcity of the amniotic fluid) with monstrous conditions of
the fcetus was a difficulty in the way of the acceptance of this theory,
and no very satisfactory solution was forthcoming. Thus several
arguments were brought forward against the nosological theory of
amniotic influence in teratogenesis. To these must be added the
fact that the bands had been found to consist sometimes at least of
amnion and not of organised lymph, and the circumstance that while
such diseased states of the amnion might account for malformations
and monstrosities by defect, they could in no degree explain the
monstrosities by excess and the double terata.

About the middle of the nineteenth century a new factor came
into the consideration of the amniotic theory in teratogenesis : Em-
bryology by its discoveries began to throw light upon the nature and
mode of origin of all the fetal membranes and vesicles, including, of
course, the amnion. The thoughts of teratologists were thus turned
into a new channel, and this new channel led the current of the
theory rather away from than towards the nosological side of it. As
a matter of fact, the intrusion of the embryological factor distinctly
. weakened the hypothesis of disease of the fetal membranes as a cause
of deformity and monstrosity. As soon as it began to be recognised
that the amnion, in some of the lower animals at least, was formed by
the upgrowth of folds of the epiblast and mesoblast of the blasto-
dermic vesicle extending until they met all round the embryo and
walled it in, just so soon it began to be apparent that any arrest
in the development of these folds or any deviation from the normal
mode of their upgrowth would lead to the establishment of abnormal
relations between them and the embryo lying within them. For a
time the attempt continued to be made by some writers to reconcile
this developmental defect with the notion of disease either of the
amnion or of the foetus. The idea that commended itself seems to
have been that an inflammatory irritation of the amnion or of the
embryonic parts opposed to it served to prevent the regular and

AMNIOTIC THEORIES 193

complete separation of the two structures ; liquor amnii would, how-
ever, be poured out at certain places, and so an imperfect and irregular
separation would occur and the unseparated parts would remain as
bands or adhesions or filaments. Calori and G. Braun apparently
held views of this nature, and Jensen (in 1866) expanded them by
emphasising the fact that the amnion was developed in several folds
(cephalic, caudal, lateral) ; and that, as one or other of these parts was
affected, so the resulting deformity or monstrosity would vary in its
position and character. Perls (Zehrb. d. ally. Pathologie, ii. 255, 1879)
also gave a pathological or nosological bearing to the theory, by sup-
posing that substances passing from the mother to the foetus might
cause irritation of the parts and especially of the amnion in their
transit, and so set up localised inflammation and the formation of
bands and adhesions. In some instances, he thought that mechanical
compression also might cause inflammation.

Gradually, however, the nosological idea was allowed to drop out
of the amniotic theory of teratogenesis, and this change was largely
due to the results of the experimental production of monstrosities in
the case of the chick. Panum, Lombardini, and more particularly
Dareste have shown that by altering the environmental conditions
during the incubation of the egg — by varnishing the shell, by raising
or lowering the temperature in the incubator, by shaking the incu-
bating tray, etc. — we can produce malformations, monstrosities, or
defective developments in the embryo, although we can never foretell
which particular anomaly will be presented. Dareste found that in
most of these cases it was the development of the amnion that was
at fault, that there was usually a defective secretion of liquor amnii
in the amniotic cavity ; and that, in consequence thereof, the membrane
remained in contact with the surface of the embryo and exercised
pressure upon it. The reader will notice, therefore, that the effect of
embryological investigations and of experimental teratogenesis has
been to resuscitate the pressure-theory. When the amnion does not
separate over any part of the embryo, it leads to compression of that
part, perhaps also to the fusion of contiguous structures in that part
which ought to have remained separate, and so malformations of
that compressed region are produced ; when the whole amniotic
membrane is ill-developed, multiple and various deformities are the
results. It will be seen, therefore, that I have led the reader by a
detour through the nosological theory back again to the pressure-
theory in teratogenesis, as indeed I indicated I was going to do at
the end of the preceding chapter (p. 155). For some years back
amniotic pressure has been the most popular theory of teratogenesis
among teratologists, and with them it has largely supplanted any
notions of disease either of the foetus itself or of its surrounding-
membranes. The exact mode of action of the pressure has been
supposed to be by causing arrestment of the ontogenetic processes
going on in the developing embryo ; in this way ontogenetic phases,
which are normally transitory and passing, have been rendered per-
manent, and the results of their permanence are seen in monstrous
parts surrounded by normal structures. It has also been stated that

VOL. II. — 17

194 ANTENATAL PATHOLOGY AND HYGIENE

the embryo is not subject to diseases at all but only to monstrosities,
or that, to change slightly the wording of the conclusion, that the
manifestations of the pathology of the embryo are malformations and
not diseases, that the " diseases " of the embryo are monstrosities, that
embryonic pathology is teratology. Thus, what has been called the
embryological theory of teratogenesis has come to replace the noso-
logical or pathological, and in so doing has borrowed from the
traumatic theory the notion of long-continued pressure exercised by
an ill-developed and unseparated amnion.

Of recent years, however, there have been signs that the nosologi-
cal theory is not dead. These signs have been manifest chiefly in
connection with an offshoot from the nosological theory, with, in fact,
the question of the mode of production of the so-called spontaneous
or intrauterine amputation of parts. We have thus far traced the
main current of the nosological theory without looking particularly
at the side currents, the little eddies as it were, which swirl past
somewhat in the shade ; they, however, are not without importance.
In the preceding chapter I have already said something with regard
to spontaneous amputations brought about through the agency of the
umbilical cord, and it will be remembered that I then pointed out
that not funic pressure only but also amniotic pressure had been
invoked to explain the solutions in continuity of both the soft and
hard tissues in the foetal limbs. I also showed that there were diffi-
culties in believing that pressure alone could perform the amputations,
and that some writers had alleged the existence of morbid processes,
of disease in fact, in the skin of the affected parts. The reader will
recollect how Menzel (in 1873), in connection with a case of con-
genital sulci of the digits, found certain changes in the skin of the
affected parts which seemed to him to suggest commencing amputa-
tion by a downgrowth of epithelium. He thought that in the embryo
the normal digitation of the extremities was produced by such
downgrowths acting in the long axis of the hand or foot, and im-
agined that if these came to act transversely or circularly after the
original digitation had been carried out, then spontaneous amputations
would result. He called the process dactylolysis of an epithelial
type. Incidentally he explained syndactyly as due to the absence of
the normal separation of the digits by physiological dactylolysis.
Longuet, in 1877, also criticised the ordinarily accepted view that
amputations are produced by amniotic bands, pointing out that in a
case observed by him the sulci were of unequal depth and more
evident upon the dorsal than upon the plantar aspect of the foot, and
that they were associated with paralytic club-foot ; he thought they
might all be more satisfactorily explained by the action of an intra-
uterine disease of the nervous system.

These considerations suggested to some minds the idea that con-
genital amputations might be an antenatal form of ainhum, that
curious disease (at first believed to be peculiar to the negroes of
Brazil, but now known to have a much wider racial and geographi-
cal range), which is characterised by the appearance of a slowly
deepening furrow round the base of a toe (usually the little toe), the

AINHUM AND CONGENITAL AMPUTATIONS 195

swelling of the distal part, the development of a slender pedicle, and
the final separation of the pendulous digit. The microscopical
changes in ainhum consist in a thickening of the horny layer of the
epidermis of the affected toe and of the coats of the larger arteries ;
in the furrow, some of the epithelial cells are swollen and hyaline,
the sudoriparous glands are atrophied, and there is a marked small-
cell infiltration. Several articles have appeared on the subject of the
relation between ainhum and congenital amputations, including those
by G. Beauregard (Dcs difformites des doigts, Paris, 1875), by Brochin
(Gaz. d. hop., xlix. 329, 1876), by Lannelongue (1882), by Eeclus
(1883-1894), by Jeannel (1886), by Rouget (1889), by Raynaud
(1895), and by others. Rouget argued strongly against the identity
of the two morbid states ; he believed that ainhum affected only
the black race, that it attacked only adults and only the toes, and
that it had a progressive course ending in separation of the digit ;
congenital amputations, on the other hand, were met with in all
races, affected the fingers as well as the toes, were present at birth,
were associated with other malformations, and had a tendency to re-
main stationary. Reclus pointed out that some of the differences
enumerated did not hold good, and was inclined to look to disease of
the nervous system of a trophic nature for the cause ; and Lancereaux
(1894) was of the same opinion. Raynaud came to the conclusion
that while it was not proved that ainhum and congenital amputations
were the same, yet there were several points in which they strongly
resembled each other. It may be added that D. M. Moir (Indian
Med. Gaz., xxvi. p. 367, 1891) met with a case of supernumerary little
toe in a Mohammedan boy of eight years of age ; the deformity affected
both feet ; and the toes were being separated by ainhum. It does
not, however, seem to me that Moir shows reasons for believing that
ainhum was really the active process causing separation in this case.
It must, I think, be left doubtful whether congenital amputations are
the same from the pathological point of view as ainhum ; my own
opinion is that they are not, and this is supported by the differences
in microscopical characters (Broussolle, Rev. mens. d. mal. d. I'enf.,
viii. 23, 161, 1890). At any rate, it cannot be maintained that the
whole discussion on this matter tends in any degree to settle the
large question of the relation of foetal diseases to malformations and
monstrosities. It is, as I have said, only an eddy in the main current
of the problem.

Another eddy or side current arising out of the large stream of
opinion upon the nosological theory is occupied with the regeneration
of lost parts. In connection with spontaneous amputations of limbs,
J. Y. Simpson suggested that the little nodules seen sometimes on
the stump of such defective limbs were attempts at a rudimentary
reproduction of the amputated parts. This suggestion is really a
begging of the question, for all teratologists are not agreed that a
segment of the limb has actually been amputated ; but, accepting the
preliminary doubt, it may be said that a good deal of evidence has
been gathered together to show that in early embryonic life there
may be a considerable power of reproduction of destroyed structures,

196 ANTENATAL PATHOLOGY AND HYGIENE

and that in this particular the human embryo resembles the adult
form of some of the lower animals. The axolotl, for instance, can re-
produce digits which have been removed, and it is a striking fact that
the parts reproduced tend to be malformed. Some time ago I asked
Professor J. Arthur Thomson, of Aberdeen, some questions on the
zoological aspect of this matter. He kindly replied that so far as
was known there was — (1) very great power of regenerating lost ex-
ternal parts of considerable size {e.g. a limb or lens) in even adult
amphibians, such as newts ; (2) much less power of doing so in
reptiles, but, in lizards, tails and large parts of limbs might be re-
produced ; and (3) even in birds there have been some striking cases,
e.g. the notable instance of the regeneration of large pieces of bone or
horn in the upper and lower jaws of fighting male storks. I think,
therefore, there can be little doubt that similar regenerations of parts
are possible during the embryonic phase and perhaps in the early foetal
epoch of human antenatal life. To my mind, and from the theoretical
standpoint, it seems more difficult to understand how the parts can
be amputated than to admit that they may be reproduced.

To return, for a short time, to the consideration of the main
current of the nosological theory of teratogenesis, let me point out
some of the recent advances in Embryology and their possible bearing
upon this problem. It has come to be recognised, for instance, that
the early weeks of antenatal life are occupied with the formation of
the embryo and its parts, and that the later weeks and months are
characterised by the performance of festal functions and by the growth
of foetal parts. Diseases in the strict sense of the word are possible
in the later or foetal period of antenatal life ; while in the early or
formative period, malformations are the result of interference with
the normal progress of affairs. To say that a disease of the foetus is
the cause of its malformation is to invert the order of events ; until
the part is formed it cannot become the seat of the changes which
constitute a disease. As Duval (Bouchard's TraiU clc pathologic gc'ne'-
rale, p. 161, 1895) puts it, "la malformation, 1'etat monstrueux d'une
partie n'est pas la consequence d'une maladie subie par cette partie ;
cet etat monstrueux, ce developpement anormal constitue la maladie
meme." This conception of the nature and scope of embryonic as
compared with foetal pathology, of course, alters our views regarding
the nosological theory of teratogenesis, whether we look upon that
theory as depending upon the diseases of the foetus itself or upon the
maladies of its membranes ; in fact it may be said to be fatal to the
nosological theory. I have, however, already several times pointed
out that there is no hard-and-fast line between embryonic life and
foetal life ; all formation is not completed in what we term the
embryonic epoch (first six weeks or thereby), neither is the perform-
ance of functions limited entirely to the ftetal period (last thirty-two
weeks) ; some of the organs are capable of performing functions and
therefore of having diseases in the embryonic period ; and there is
still some formation going on in the foetal period, and, therefore, the
possibility then exists of some malformations arising. I hold, there-
fore, that the nosological theory has not been altogether discredited

DEVELOPMENT OF THE AMNION 197

by the new conception of antenatal life which has been arrived at ; I
maintain that diseases of formed parts of the embryo may occur and
possibly interfere with the formation of the other parts, and I think
that parts which have been developed monstrously in the embryonic
epoch will be very liable to become the seat of disease in the foetal
period. In this way diseases and malformations may coexist in the
foetus and affect each other.

But, it may be asked, how does this conception affect the theory
of amniotic disease as a factor in teratogenesis ? Two significant
facts have of late years been discovered regarding the amnion. It
has become evident that the avian method of development of the
amnion from the upgrowth of two folds of epiblast and mesoblast
round the embryo does not apply to the human embryo : it may be
that there is a sinking down of the embryonic disc into the blasto-
derm and an invagination of the membranes as suggested by Mall
(Joum. of Morphol., xii. 395, 1897) ; it may be that the amniotic sac
forms by a vacuolisation of a portion of the inner cell mass as occurs
in the bat, and is therefore a closed cavity from the very beginning
(M'Murrich, Development of the Human Body, p. 131, 1903) ; or it may
be, as Berry Hart has thought, that there is first the formation and
then the breaking down of an epiblastic plug to form the cavity of the
amnion. If we accept any of these later views on the formation of the
amnion it becomes perceptibly easier to understand the formation of
amniotic adhesions and bands and pressure ; for, if there is a stage of
breaking downof tissues in the immediate neighbourhood of the embryo,
it is not difficult to imagine an arrest in the breaking-down process re-
sulting in the formation of adhesions. There may, therefore, be no need
to invoke the occurrence of inflammation or any other disease of the
amnion ; defects in the formation of the membrane and cavity will be
sufficient to account for the acquirement of adhesions. There is, how-
ever, one other fact of recent discovery which recpuires to be taken into
account in coming to a conclusion on this matter. It has been found
that the amniotic sac is already formed in the earliest known human
blastodermic vesicle ; it is present in Peters' specimen of a few days
old. The formation of the amnion, therefore, precedes the formation of
the embryo. The conclusion I draw from this fact is that the amnion
is fully formed and capable of performing functions before the embryo
has passed through the formative or strictly embryonic stage. I
think, therefore, that, after all, it may be scientifically accurate to
speak of disease of the amnion as a possible cause of deformities of
the embryo ; amniotitis or amnionitis may be a pathological possibility
and a cause of teratological developments in the embryo. At the
same time, it appears to me that defective development of the amnion
and possibly the pressure which it thus brings to bear upon the
plastic embryo are much more likely methods for the production
of teratological conditions. Thus we have extracted from the
pressure theory and the nosological theory of teratogenesis the really
essential and valuable elements ; we hand these on to the embryolo-
gical theory to explain some conditions in it otherwise difficult of
comprehension (vide Chapter XII.).

198 ANTENATAL PATHOLOGY AND HYGIENE

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LITERATURE 199

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200 ANTENATAL PATHOLOGY AND HYGIENE

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LITERATURE 201

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Wien. Miu. Rundschau, ix. 165, 1895; Senger, Berlin. Jclin. Wchnschr., xxxi.
679, 1894 ; Sentex, Mem. et bull. Soc. de med. Bordeaux, pp, 204, 224, 1869 ;
Simonart, Arch, de la mid. beige, xviii. 112, 1845; A. R. Simpson, Trans.
Edinb. Obst. Soc, xv. 31, 1890; J. Y. Simpson, Edinb. Med. and Surg.
Journ., xlv. 265, 1836 ; J. Y. Simfson, Dublin Journ. Med. and Chem. Sc, x.
220, 1836 ; J. Y. Simpson, Month. Journ. Med. Sc, viii. 890, Edinb., 1848 ;
E. Barclay Smith, Journ. of Anat. ami Physiol., xxxi. pt. 3, 1897 (Proc.
Anat. Soc. of Grt. Brit, and Ireland, p. xxxiv) ; L. Sokolowsky, Centrlbl.
f. Gynak., xvi. 789, 1892; S. T. Sommerring, Abbildungen und Beschrei-
bungen einiger Missgeburfen, p. 23, tab. viii., Mainz, 1791 ; A. Souques and
(r. Marinesco, Corrupt, rend. Soc. de biol., 10 s., iv. 434, 1897; A. Spring,
" Monographie de la hernie du cerveau," Mem. Acad. roy. med. de belgique,
iii. 1-198, 1854; C. E. Stein, "Ein Fall von Hemicepbalie mit Yenvacb-
sung zwischen Kopf und Placenta," Diss, inaug., Marburg, 1879 ; J. Stein,
MemorabUien, xv. 113, Heilbron, 1870; P. Strassmann, Arch. f. Gynaek.,
xlvii. 454, 1894; G. Templeton, Clin. Journ,, xi. 175, 1897-98; P. H.
Tesdorpf, " Bescbreibung und Erklarungsversucb eiuer mit amniotiscben
Bandern bebafteten menschlicben Missbildung," Diss, inaug., Miincben,
1883; Therese, Bull. Soc, anat. de Paris, Ixv. 473, 1890; E. Thorner,
" Ueber eine Hemmungs-bildung des Amnion bei einem menschlicben
Foetus, verbunden mit ander weitigen Missbildungen," Abdruck aus
Reichert's und du Bois-Reymond's Archiv, Heft 2, 1869 ; C. Tournier,
Rev. d'orthop., ii. 272, 1891 ; Tronchet, Bull. Soc. d'anat. et physiol. de
Bordeaux, vi. 31, 1885; Turgard, Bull, med. du nord., xxvii. 257, 302,
Lille, 1888; Van Duyse, Hyperencephalie et perodactylie, Gand, 1894;
G. Variot and G. Chicotot, Journ, de din. et de therap. infant., vi. 961,
1898; A'eith, "Das Amnion in seinen Beziehungen zu den fotalen
Missbildungen," Diss, inaug., Miincben, 1901; E. Viehofer, "Ueber
intrauterine Amputationen," Diss, inaug., Bonn, 1887 ; R. Virchow,

202 ANTENATAL PATHOLOGY AND HYGIENE

Monatschr. f. Geburtslc. u. Frauerikr., xx. 16, 1862; W. Yrolik, Tahulm
ad illustrandam &mibryogene&in liominis et mammalium, tab. xliv., xlv.,
lxxvi., etc., Amstelodami, 1849 ; T. E. Walker, Louisville Med. Neics, vi.
Ill, 1878; F. Wiedemann, Miinchen. med. Wchnschr., xxxviii. 793, 807,
1891 ; J. William, Lancet, i. for 1886, p. 855 ; C. L. Williams, Med.
Reporter, ii. 239, Calcutta, 1893; F. von Winckel, Miinchen. med.
Wchnschr., xliii. 389, 1896; J. Wolff, "Intrauterine Spontanamputation
an den oberen Extremitaten bei einem 5 monate alten Fiitus mit voll-
standiger Erbaltung des die Amputation bedingenden Amniosfadens,"
Diss, inaug., Berlin, 1900; Arch. f. Gynael\, lx. 281, 1900; P. Zagorsky,
Mem. Acad. imp. d. sc. de St. Petersb., 6 s., iii. 3, 1834; Zini, Sitzungsb.
d. Ver. d. Aerzte in Steiermark, vii. 107, 1869-76.

CHAPTER XII

The Embryological Theory in Teratogenesis : Historical Sketch of the Theory ;
Embryology an Explanation of Teratology ; Arrested Development ; the
Amniotic Factor in Teratogenesis ; Experimental Teratogeny in the hands
of Geoft'roy Saint-Hilaire, Allen Thomson, Panum, Schrohe, Lombardini,
Dareste, Fere, and others ; the Germinal Factor : Summary.

The latest of the teratogenetic theories, which has been called
the embryological, now demands consideration. It is the latest ; it
may also be the last, for I believe that if all the facts were known
they would be found to tit this theory ; but it is dangerous to speak
of finality in these matters, and it savours of presumption, for
finalism, with its suggestion of infallibility, is obnoxious to the
scientific mind. The way is not yet closed against the advance of
new theories ; but they will require to be very satisfactory, very
conspicuously sufficient to explain the phenomena, and very con-
vincingly efficient to overcome all the difficulties, if they are to
displace the embryological theory in teratogenesis.

If the reader will at this stage re-read Chapter VII. (pp. 72 to 80),
he will gain a general idea of what is meant by the embryological
theory of the formation of monstrosities and malformations. He
will find there an Ariadne-thread to lead him through the laby-
rinthine mazes of Teratology, a threefold thread, with the three factors
of embryonic physiology, the action of the amnion, and germinal
pathology forming its constituent strands. He will be led to focus
his gaze upon Embryology ; to scrutinise all the phenomena of embryo-
genesis and of ontogenesis, so far as they are known ; to look for
his clue in the normal processes of formation and evolution of the
organism. He will discover that the most complex and perplexingly
intricate monstrosities and malformations become perceptibly less
complex and less intricate when viewed in the light which
comes from the study of the development of the parts affected.
The origin of a landscape, with its diversity of features, — mountain,
valley, river, gorge, lake, and crag, — may seem inexplicable ; but the
eye of the geologist pierces below the surface, and, conjuring up the
visual image of the prehistoric mutations of the component parts of
the earth's crust, reveals the processes and explains the methods by
which all these things came to be just what they are. So must
the teratologist look at a fully-formed malformation, not with his
gaze riveted upon what it now is, but in imagination dwelling
upon what that malformed part was in the second, in the third, or

203

204 ANTENATAL PATHOLOGY AND HYGIENE

in the fourth week of the intrauterine life of the embryo ; he will
then begin to see, like the geologist, below the surface, and to learn
from present tilings what past conditions were. Let us now study
more in detail this embryological theory ; let us see how Embryology
helps us to understand Teratology.

The embryological theory in teratogenesis dates from the time
of Harvey, who laid the foundation-stone of it in his treatise on
Generation, a work which, it is interesting to remember, he was
little inclined to publish, and only "ventured it out of his hands with
reluctance " after receiving the solicitations of Ent. To Ent, indeed,
we owe it that the Exercises on the Generation of Animals (with the
famous passage on malformations) saw the light in 1651. There, in
the sixty-ninth exercise (I quote from Willis' translation of the
Works of William Harvey, Sydenham Society, p. 487, 1847), are the
following sentences : " In the foetuses of all animals, indeed that of
man inclusive, the oral aperture without lips or cheeks is seen
stretching from ear to ear ; and this is the reason, unless I much
mistake, why so many are born with the upper lip divided as it is
in the hare and camel, whence the common name of hare-lip for the
deformity. In the development of the human foetus, the upper lip
only coalesces in the middle line at a very late period." In this
passage, Harvey, without the help of the microscope it must be
remembered, laid down the great principle of malformation by arrest
of development ; and, although he was ignorant of the exact organo-
genetic processes by which the parts about the mouth were actually
developed, yet he saw the possible results of their retardation, and
so, as has been said, laid the foundation-stone of the embryological
theory of monstrosities. It must be confessed, however, that neither
Harvey himself, nor those who immediately succeeded him in his
own country or abroad, built much upon the foundation-stone thus
laid. Not till a century had passed did any sign of building actually
appear, and then little more than indications of the walls 'of the
future edifice were to be seen above ground. In A. von Haller's
writings (Ojjcrum Anatomici Argumenti Minornm, iii. p. 135, 1768)
there is the indication of the idea that ectopia cordis may be due to
the persistence of the stage in development (seen in the chick) when
the heart lies uncovered by muscles and ribs ; some years later
(1772), C. F. Wolff (N. Comment. Acad. Scicnt. Imper. Pctropolitanv,
xvii. 540, 560, 1773) explained the origin of both ectopia cordis and
exomphalos by absence of the normal closure of the abdomino-
thoracic walls ; and J. H. T. von Autenrieth (Observation um ad
historiam cmbryonis facicntium, p. 38, 1797), towards the close of the
eighteenth century, extended the idea of arrested development till
it included not only all the monstrosities by defect of formation, but
also the double terata.

It was in the nineteenth century, however, that this theory
began, like a tree, to spread out its branches in all directions, and in
ways and with results undreamed of by the writers who helped to
plant it. J. F. Meckel (Handbuch dcr path. Anatomic, pp. 48, 51, etc.,
Leipzig, 1812), for instance, discussed in detail the various arrested

ARRESTED DEVELOPMENTS 205

developments (Hemmunr/sbildungcn), and pointed out that some of
those which occurred in the human subject represented conditions
which were to be found in the lower animals. So, out of these
observations, grew the belief that the human embryo in his ante-
natal existence is first a worm, then a fish, then an amphibian, and
then a bird ; that, in other words, he climbs up his genealogical
tree, which, as it happens, is also the zoological scale. Of this view
I have elsewhere spoken (Chapter II.) ; it is not now accepted un-
reservedly, but it had a wide vogue during the nineteenth century.
The idea that malformations were arrested developments was not,
however, inseparably bound up with the notion of the ascent of the
embryo through stages corresponding to zoological sub-kingdoms ;
its applicability to teratological problems was independent thereof,
although it must be admitted that the idea of the existence of a
parallelism between phylogeny and ontogeny was helpful in explain-
ing malformations which did not resemble any stage in the develop-
ment of the human embyro. Hare-lip, cleft palate, spina bifida, the
uteris bicornis, exomphalos, and ectopia cordis were so easily and
evidently explicable as arrested developments that they ensured for
the theory a permanent place in teratogenesis ; but there were other
anomalies which found no such easy explanation, and in their case
recourse was had to the facts of zoology.

Shortly after Meckel (op. cit.) wrote his work on Pathology, and
about the time when F. L. Fleischmann (Bildungshemmungen der
Menschcn und Thierc, p. 24 et seq., Niirnberg, 1833) was expanding
his suggestions and theories, a great discussion arose regarding the
laws of development, and. in the course of this controversy terato-
logical cases and problems were freely referred to. It would lead
us too far into general biology to follow out all the ramifications of
the theories and statements made by E. Saint-Hilaire (1822), by
Serres (1824, 1832, and 1860), by I. Saint-Hilaire (1836), by Bischoff
(1842)* by Darwin (1868, etc.), and by a legion of other scientists,
investigators, and philosophers : but certain points deserve at leasb
a passing reference.

There was, for instance, the question of the influence of one set
of organs of the body over the others, especially in relation to the
cases in which one system was the seat of malformation. Some
writers maintained that the nervous system, and especially the
spinal cord, was the centre for the regulation of the formation of all
the parts. Certain teratological facts seemed to support this view,
such as the absence of the olfactory nerves in cyclopia (in which
there is often no nose to be found), and unilateral atrophy of the
spinal cord in cases of congenital absence of one limb ; other facts,
however, seemed to tell against it, such as the advanced development
of the foetal tissues in anencephaly, the presence of many organs
and parts in the placental parasites (allantoido-angiopagous twins)
which have no brain and no spinal cord, the absence of any alter-
ation in the spinal cord in cases of absence of one limb (as in the
three-legged kitten described by me in the Interstate Med. Journ.,
vii. 367, 1900), the ability of some of the lower forms of life to

206 ANTENATAL PATHOLOGY AND HYGIENE

develop without a nervous system, the comparatively late date in
antenatal life when the nervous tissues reach functional maturity,
and the absence in some monstrosities of the nerves {e.g. vagi)
supplying certain viscera along with the presence of the viscera
themselves. It would seem, if we take a fair and comprehensive
view of the facts, that the nervous system has not the powerful
regulating action upon development and growth that was once
attributed to it ; but it would also seem that the absence of the
nerves supplying any part of the body is not altogether without
results upon the structure and functions of that part. It may
also be admitted that it is quite possible that this influence may
be either greater or less during early antenatal life than it is
after birth.

The failure, for want of evidence, of the theory of the supremacy
of the nervous system over the other systems was, doubtless, partly
the cause why some writers {e.g. Tiedemann and Serres) adopted the
view (as old as the time of Aristotle, and possibly older) that the
heart and vessels were the controlling factors in the formation of
the rest of the organism. According to this view, anencephalus,
hypospadias, hare-lip, absence of limbs, and exstrophy of the bladder
were to be explained by incomplete development of the vessels
supplying the parts which were malformed. The idea was supported
by such facts as the small size of the vertebral and internal carotid
arteries in anencephaly alongside of the large size of the external
carotids, by the small dimensions of the external carotids and the
cephalic arteries in microcephaly, and by the occurrence of mal-
formations iD the embryo when the vascular area showed defects in
its formation. These facts, however, may be perfectly true, and yet
it does not follow that the vascular defect was the cause of the
malformation ; it may, indeed, have been the consequence of it.

Another question which greatly exercised the minds' of terato-
logists and embryologists in the first half of the nineteenth century
was in relation to the direction of developmental processes : some {e.g.
Serres) believed that formation took place from the periphery towards
the centre (centripetal development), and that, therefore, the vessels
and nerves preceded the heart and cerebro-spinal axis ; while others
{e.g. Baer) maintained the theory of centrifugal development. Ap-
parent exceptions to both theories can easily be found in Teratology,
and it was necessary for Embryology to make marked advances before
these questions and their corollaries could be satisfactorily dealt with.
In the meantime these difficulties produced the effect of making the
teratologists look further back in antenatal history for the causes of
monstrosities ; so their attention came to be concentrated upon the
embryonic epoch, before the blood vessels and nerves have begun to
be functionally active.

Let us now consider to what extent a knowledge of embryonic
physiology or embryology (in its limited sense) can explain mon-
strosities and malformations ; let us take the first strand of the
Ariadne-thread that is to lead us securely through the mazes of
Teratogenesis.

ACTION OF ARRESTED DEVELOPMENT 207

We may, very suitably, begin with Harvey's hint given in con-
nection with hare-lip, in other words with the idea of arrested
development. There are many stages in ontogenesis, as the perusal
of Chapters III., IV., V., and VI. has informed the reader, and it may
be taken as possible that arrest may occur at any one of them and in
any part of the embryonic organism. There is, for example, a stage
when the limbs are but buds from the trunk ; should their formation
be then arrested an explanation is forthcoming of some of the forms
of ectromely. There is also a stage when the heart is external in
position ; it may remain so, giving rise to ectopia cordis. There is,
again, a stage in the internal formation of the heart when the ven-
tricles communicate with each other ; this state may persist and the
foetus be born with that organ possessing a single ventricular cavity.
Normally, the second branchial arch by its continued growth covers
over and then shuts in the sinus pnecervicalis ; an arrest in its
growth will leave a communication between this sinus and the
exterior. There is at one time in embryonic life the peculiar arrange-
ment of vessels known as the aortic arches ; by a series of complicated
changes some of them disappear and others remain or are fused
together to form new vessels ; arrests of different parts and at
different times will cause vascular anomalies which are easily
enough understood when the ontogeny of the parts is clearly com-
prehended.

The reader will have noted that I have already referred to two
different ways in which arrested development may show itself : it
may be evident in stoppage of the formatiom of a part, or it may be
observed in the persistence of a part which ought to disappear.
There are, however, yet other ways in which it may act : two parts
which ought to unite may, by defect of growth, fail to do so, and so
give rise to such anomalies as hare-lip and cleft palate. The cause
may possibly be a sort of unpunctuality of one part, which does not
come forward in time to meet another, and then is prevented from
ever doing so by the progression of other and intermediate parts. It
is possible, also, that in some malformations there may have been an
absolute non-formation of a part or organ, with a consequent coming
together of structures which are normally far apart ; this is an ex-
planation which may serve to account for the cyclops foetus. Again,
parts which ought to have grown out of one situation into another
may be late in doing so or may fail altogether to do so. By this
mechanism it is probable that we must account for the hernia of the
intestine into the root of the umbilical cord, sometimes found in the
new-born infant. At an early stage the intestinal loop has, so to say,
the right to lie in the umbilical aperture ; but it ought long before
birth to have been withdrawn into the peritoneal cavity ; it is not so
retracted, and thus the hernia or its subsequent development, a faecal
umbilical fistula, is produced. Yet again, some structures which are
pervious during early embryonic life close at a later stage ; arrest
may, however, seize upon them, and their patency remains, as is seen
in an umbilical urinary fistula (due to persistence of the urachus) or
in a patent ductus arteriosus. Parts which ought to atrophy or at

208 ANTENATAL PATHOLOGY AND HYGIENE

least become rudimentary in foetal life, such as the Miillerian ducts
(and their derivatives) in the male, may suffer an arrest in their pro-
cess of disappearance and remain as malformations by excess. An
example of this is found in spurious hermaphroditism of the male, in
which in addition to testicles, prostate, and the associated ducts, there
are found Fallopian tubes and a uterus.

Without multiplying instances, it becomes clear, therefore, that
simple arrest of development or of growth in the embryo serves
to account for many monstrosities and malformations. For some
anomalies, however, this explanation fails, and other modes of origin
must be adduced.

It would seem that in some instances there is a process of true
excessive development. For example, the presence in the new-born
infant of a distinct tail, due to supernumerary coccygeal vertebra,
would seem to require an explanation of this kind ; but all so-called
" tails" are not truly of this nature. Some cases of external herma-
phroditism (spurious) may also be included in this group ; thus, the
clitoris may be abnormally large and so cause a superficial re-
semblance to a penis. In a very curious group of malformations, the
heterotaxies (i.e. those in which the organs of the body are inverted,
are arranged as if seen in a mirror), it would appear as if arrest and
excess of development were combined in their teratogenesis ; thus
instead of an aortic arch on the right side, disappearing while the
corresponding one on the left side remains, the left one may disappear
and the right one remain. So-called transposition of the great vessels
is not therefore really a transposition but a combination of arrest and
persistence.

The embedding in the body-cavities of parts or derivatives of
parts which are normally external would seem at first sight to be
inexplicable by arrest of development. On further consideration,
however, some of the difficulty disappears. Is not the whole nervous
system (securely closed in within the spinal column and cranial
cavity) originally external ? Is it not ectodermic in its origin ? Is
there not, further, a stage in ontogenesis when the thoraco-abdominal
cavity is open and in a sense external ? It is not, upon reflection, so
difficult to imagine a piece of the ectoderm becoming included in one
or other of the cavities, as the cerebro-spinal nervous tube is included
within the cranio-spinal canal, if these cavities are perchance delayed
in closing. In the same way, if a branchial cleft is too long in being
shut off from the exterior, a neighbouring structure may be caught, as
it were, or snared in it. When a simple tube, such as that which
gives origin to the brain, undergoes a series of complicated flexures
and bendings. it is cpuite conceivable that if these ben dings are late
in being achieved neighbouring structures may be inserted between
them. It is perhaps in this way, and by a process which we may
describe (to borrow a French word) as " enclavement," that some
intracranial dermoid cysts may be produced.

If, as has been supposed, certain openings and canals, such as the
anus and the vagina, get their lumen by the breaking down of ecto-
dermic plugs, then it is easy to understand how an arrested breaking

THE AMNION IN TRRATOGENESIS 209

down would lead to imperforation or to septa or narrowings of these
parts.

It is evident, therefore, that the principle of arrested development
is fertile in yielding explanations of many of the varieties of mon-
strosity and malformation ; but there are cases which it is inadequate
to elucidate. For instance, there is sympodia, that curious terato-
logical state in which the lower limbs are not only fused together
into one but have evidently been rotated during the fusing ; there
are the cases of complete and partial congenital amputation and of
other forms of mutilation ; and there are all the types of true exces-
sive development and double monsters, symmetrical or asymmetrical.
Further, although arrested ontogenesis may interpret many anomalies,
it, after all, does not give any hint as to the causa causans; we have,
as yet, left unanswered the question, Why is there an arrest at all ?
To explain some of these difficulties we must pick up the second
strand of the threefold Ariadne-thread that is to guide us through
the bewildering passages of our teratological labyrinth.

A consideration of the amniotic membrane throws some light
both upon these monstrosities that are not explicable by simple
embryonic arrest, and also upon the causa causans of the malforma-
tions which are so explicable. I have already, in writing of the
nosological theory of teratogenesis, opened up the possible modes of
action of the amnion, and I need not here repeat much of what ap-
pears in the preceding chapter. It must, for the present, be left
uncertain whether the amnion acts upon the embryo by pressure due
to non-separation of the membrane from the surface of the organism
by defect of the liquor amnii, or by disease of the membrane of an
inflammatory kind leading to the production of adhesions which
compress the embryonic parts and organs ; it must also be conceded
that the exact mode of formation of the amnion in the human subject
is unknown. At the same time, it is well known that the amnion
may be found adherent to the fcetus at birth, and that the foetus,
which is thus brought into connection with it, is generally malformed
in the affected parts ; it is also clear from embryological studies that
there is a time in early embryonic life when the amnion lies in close
contact with the embryo ; and, finally, it has been shown by the
early gestation-sacs of Peters and others that the amnion is present
before the embryo. In addition, we know, from the experiments in
teratogeny of Dareste and others, that defective development of the
amnion in the chick is constantly associated with malformations ; but
I lay no great stress upon this fact, for the formation of the avian
amnion is evidently different from that of the mammalian one. I
think, therefore, that these facts lead us to the conclusions : (1) that
the action of the amnion may be teratogenic in the human subject;
(2) that certain multiple deformities, such as sulci and amputations
of the limbs and digits, and adhesion of the foetal membranes to the
cranium or abdomen or face, must be regarded as specially amniotic
in origin ; (3) that non-separation of the amnion from parts of the
embryo in the stage of architectonic activity (e.g. in the case of the
face during the growth and approximation of the various processes to
vol. n. — 14

210 ANTENATAL PATHOLOGY AND HYGIENE

each other) may be the actual means of arresting these developmental
activities; and (4) that in some monstrosities not explicable by simple
arrest {e.g. in sympodia and cyclopia), the amnion may by exercising
pressure upon contiguous parts lead to their partial or complete fusion.
The reader may now say, and there is perfect justification for the
objection, that after all, while the amniotic factor serves to explain
some monstrosities which arrest of development does not account for,
and in some measure elucidates the cause of the arrested ontogenesis
itself, still this does not bring us to the ultimate cause of these
monstrosities and malformations. In a word, we are left face to face
with the problem of the cause of the amniotic defect, disease, or
adhesion. What is it that prevents the development of the amnion
in its normal way and leads to its arrest ? The arrested development
of the embryo is, let us admit, due to an arrested development of the
amnion or to disease of it ; but what arrests the amnion or sets up
disease in it ? An attempt has been made to answer this question
along the lines of experiment ; it has not until recent years enjoyed
any measure of success, but it has thrown some light, albeit not of a
piercing kind, upon the matter. Let us then, as briefly as may be,
consider the subject of Experimental Teratogenesis.

Experimental Teratogeny.

Most of the experiments with regard to the causes of monstrosi-
ties have been made upon the chick during incubation, natural or
artificial. The reasons for this are obvious. The artificial hatching
of eggs was carried out long ago in Egypt, and the knowledge of the
methods employed and the practice of the art spread to Europe
towards the close of the sixteenth century. It was in the partial
failure of the attempts then made that the germ of the science of ex-
perimental teratogeny lay. It was often found that many of the
chickens thus artificially incubated were deformed. Such was the
experience of Olivier de Serres {TluhUre d' agriculture, v. c. ii.) in
1000 ; and in the records of the Eoyal Society of London for January
16, 1679 (T. Birch, History of the Royal Society of London, vol. iii. p. 455,
1757), the following passage occurs: "Mr. Henshaw further gave an
account of the manner, how Dr. Kuflier hatched chickens by the help
of furnaces, the process of which he had seen ; which was that the
doctor had a wire-grate placed over a balneum at a foot distance
with a cover over, pulled up by a pulley ; in which grate he set the
eggs, and so turned them every day for eighteen days together : then
he laid them on a hair-cloth in a stove near the ash-hole, where they
hatched themselves with their own bills ; in which stove he kept
them for three days, till they could feed themselves, which was when
the yolk was consumed in their bellies. He added that in Egypt
they do this with camel's dung. Sir Jonas Moore remarked that Sir
Christopher Heydon together with Drebell long since in the Minories
hatched several hundred eggs; but mentioned not the way; but that
it had this effect, that most of the chickens produced that way were
lame and defective in some part or other." Such was the statement

EXPERIMENTAL TERATOGENY 211

made by Sir Jonas Moore regarding the process of egg-hatching as
carried out in England before 1679; but he added to his statement
the following somewhat startling remark that " Drebell had an art,
by which he could produce a fly in an hour's time anywhere." We
are not told whether Drebell's flies like his chicks were usually mal-
formed. Dareste, in his work (oj). cif., p. 32), quotes from Paris's
" Memoir on the Physiology of the Egg read before the Linnean
Society of London, the 21 March 1809," the following sentence:
" During the period that I was at College, the late Sir Busick
Harwood, the ingenious Professor of Anatomy in the University of
Cambridge, frequently attempted to develop the egg, by the heat of
his hotbed; but he only raised monsters, a result which he attributed
to the unsteady application of heat." I have looked up the article
by J. A. Paris referred to above (in the Trans. Linnean Soe. Lond., x.
304, 1811), but have failed to find the sentence quoted by Dareste;
but I happened upon the following interesting statement, which has a
bearing upon the question of antenatal pathology : it is recorded that
a hen had its leg broken, and that three days subsequently it laid
several e<ws destitute of shells, and Paris thought that the calcareous
matter designed for the shell had been employed in the regeneration
of the bone.

From these quotations (and from others which could be given) it
is clear that the artificial incubation of hens' eggs was practised and
the frequent teratological results known before the beginning of the
nineteenth century. To Etienne Geoffroy Saint-Hilaire, however, the
honour apparently belongs of having conceived and carried out the
idea of turning these experiments to account in the investigation of
monstrosities and malformations. In the work of the younger Saint-
Hilaire {Histoire des anomalies, iii. 500, 1836) there are references to
the experiments made by his father and to the journals and books in
which the results of these experiments had been related. At first (in
1820 and 1822) he, Etienne Geoffroy Saint-Hilaire, employed natural
incubation ; but, on account of the small number of observations
possible by this method, he adopted artificial incubation in 1826
(Journ. comp. du diet de sc. mid., xxiv. 256, 1826). His object was
to produce anomalies in the chick at will by disturbing development
in different ways during the early days of incubation. Apparently
the time when most of the experiments were commenced was after
the third day of incubation. Various plans were used, such as
shaking the eggs, pricking them at various points, keeping them in a
vertical position, and partially covering them with wax or varnish ;
and the result was the production of a relatively considerable number
of anomalies, both simple and complex, such as triocephaly, atrophy
of the eyes, eventration, and spina bifida. No double monsters were
got. Isidore Geoffroy Saint-Hilaire states {op. cit.,\). 503) that he re-
peated the experiments of his father and made in addition others in
which he tried to produce monstrosities by disturbing incubation at
an earlier date than the third day ; the result was that he obtained
no monstrosities but got either no chicks at all or only dwarf ones.
He, therefore, came to the conclusion that monstrosities were prin-

212 ANTENATAL PATHOLOGY AND HYGIENE

cipally caused by disturbing conditions acting when certain of the
organs of the embryo were developing. These experiments were
carried out in 1831. Dareste, from a consideration of all the avail-
able information, believes that Saint-Hilaire proved, in opposition to
the views of Wolff and Meckel, that a change in the physical con-
ditions which determine development may modify the development
itself, and that, in consequence, anomalies and monstrosities do not
arise only from a virtuality placed in the germ before or at the time
of fecundation.

Other ol (servers seem soon to have repeated these experiments of
the Saint-Hilaires with or without alterations. Thus, Allen Thomson,
Professor of the Institutes of Medicine in the University of Edin-
burgh, writing in 1844 (Lond. and Edinb. Month. Journ. Med. Sc, iv.
486, 1844), made the following statement : " In 1833 and 1834, I
made several series of experiments of the same kind with these now
mentioned " (he is referring to the work of E. Geoffroy Saint-Hilaire),
" and with a similar result." Further, Thomson says that in the
course of a long series of experiments on incubation, carried on at
various intervals, he had been so fortunate as to meet with two in-
stances of very early double malformation in the bird. It does not
appear from the context whether the incubation-conditions had been
altered in any way in these two cases ; but in another part of the
same article (loc. cif., p. 579) he wrote, " Again, I may remark, I have
subjected at different periods nearly a dozen double-yolked eggs to
incubation, and have never succeeded in obtaining a double monster,
nor even two embryos at the full period from any of them." Thom-
son is most tan talisingly silent as to all the details of his experiments,
but his silence may be in part accounted for by the low value he
seems to have set upon them as means of elucidating the problems
of Teratology. While allowing that malformations might be to some
extent under the influence of mechanical causes, he thought that
there must be some original cause of an unknown nature existing in
the ovum or germ to account for the constancy with which certain
forms return, for their symmetry, and hereditary character.

1 hiring the fifty years that followed the experiments of the Saint-
Hilaires and Allen Thomson one observer stood out supreme in the
field of artificial teratogeny. I refer to Camille Dareste, who, between
the year 1855, when he published a paper on the effect which varnish-
ing the shell has on the development of the chick, and 1891, when
the second edition of his large work (Bccherches sur la 2r''(ldi(dion
artificidle des monstruosite's) appeared, carried out an enormous amount
of experimental work, and embodied the results in a long series of
monographs and articles. If Dareste was not the first to conceive
the idea of the artificial production of monstrosities as a means of
elucidating their causation, he was, beyond all question, the one to
whom the existence of experimental teratogenesis as a definite de-
partment of Biology is due. Others, such as Panum, Schrohe, Engel,
Lombardini, Lereboullet, Knoch, Szymkiewicz, Maggiorani, Gerlach
and Koch, and Warynski and Fol, were at work contemporaneously
with Dareste, and many of them added largely to our knowledge of

EXPERIMENTAL TERATOGEN Y 213

the subject ; but none of them can be said to have left his impress
upon it in the way that Dareste did, or to have carried out his ex-
periments with such whole-souled devotion. Before referring to
Dareste's work, I may briefly note the nature of the experiments of
the other authors named above.

F. P. Liharzik (Das Gcsetz dcs wenschlichen Wachsthumes, Wien,
1858) found that if he placed the eggs vertically in the stove after
the mid-term of incubation, he got chicks with the part which had
been lowest better developed than the rest ; but he got no monstrosi-
ties. P. L. Panum in his Untersuchungen fiber die Entstehung dcr
Missbildungen zundchst in den Eiern von Vogel (Berlin, 1860), and
later in two journal articles {Nord. Med. Ark., i. 1, 1869; Arch.f.
path. Anaf., lxxii. 69, 165, 289, 1878) wrote at considerable length
upon malformations in chicks and fish. He advanced a cellular
theory to explain both the normal and teratological forms assumed
by the foetus, and suggested as the cause of the cellular perturbations
in monstrosities a sort of inflammation of the embryonic structures ;
according as the cause acted before or after the formation of vessels,
the inflammation was parenchymatous or vascular. In this respect,
therefore, Panum, it is to be noted, had recourse to a nosological
explanation of the disturbances in embryogenesis. It is not clear
that there is any justification for this view ; but at the time when
Panum wrote, the suggestion was a perfectly fair deduction, for much
that we now know regarding the early stages of development was
then unknown. In his experiments upon eggs he chiefly employed
changes of temperature as the teratogenic agent, and he found that a
rise in temperature was more dangerous to the embryo than a fall
and could not be borne so long, and that a slow lowering of tempera-
ture had greater results than a rapid fall. He found that the
younger embryos were, the more likely were they to become mon-
strous and to show extensive anomalies ; it was rare that disturbing
causes produced malformations when they came into play after the
seventh or eighth day of incubation. Panum gave a very interesting
account of certain general conditions of the embryo, and named the
embryos so affected flattened, cylindrical, nodular, and atrophic ; he
also described in detail some malformations of the vascular area of a
very interesting kind. His work was most valuable, but on its ex-
perimental side it did not approach that of Dareste either in extent or
in accuracy. Joseph Engel (Wien. med. Wchnschr., xv. 17, 33, 49,
1865) made experiments somewhat resembling those of Panum. It
should be stated that Adam Schrohe (Untersuchungen uber den
Einfiuss mcchanischer Verletzungen auf die Entwickelung dcs Embryo
im Hiihnerei, G-iessen, 1862) attempted to produce monstrous chicks
artificially by a new method, namely, the wounding of the embryonic
area or embryo with a cataract knife ; he met with a measure of
success, but the method was faulty, and the same criticism applies
to the experiments of Lombardini (Forme organiche irregolari negli
uccelli c nci batraci, Pisa, 1868). Lombardini employed electricity as
his traumatic and teratogenic agency, and experimented both upon
chicks and upon the ova of frogs ; he obtained 19 deformed embryos

214 ANTENATAL PATHOLOGY AND HYGIENE

(ectopia cordis, general dropsy, amniotic adhesions to the head, etc.)
out of 95 eggs, 33 dead embryos, and 43 that had undergone no
change ; and the time when he passed the electric current through
the eggs was between the second and ninth day of incubation.

Lereboullet (Ann. des sc. not. zool., s. iv., xx. 178 ; s. v., i. 113,
257, 320, 1863 and 1864) also had carried on experiments on
fish ova (the pike was the fish chosen) ; but had, on the whole,
got negative results. The methods he employed were temperature
variations, shaking the ova during fecundation, and the like ;
and he believed that his experiments showed that only arrested
developments, deformities, and atrophies were due, and that only
occasionally, to external agencies. He thought that the cause of
monstrosities might be inherent to the constitution of the egg
itself, and thus in no way dependent upon external states. Dareste
refers to the work of Knoch, of Moscow, published in 1873 ; the
experiments were performed upon salmon embryos, but they were
(in Dareste's opinion) too incomplete to be of value in the drawing
of conclusions. In 1875, Szymkiewicz (Bcitrag zur Lchre von den
kilnstlicJicn 3fissbildungen am Hilhnereie, Wien, 1875) attempted, by
wounding the embryo chick, to produce deformities; but the embryos
either died or showed too complex deformities to be of value in
teratogen esis. S. Warynski and H. Fol (Bcv. med. dc la Suisse Bom.,
iii. 395, 1883 ; Compt. rend. Acad. d. sc., xcvi. 1674, 1883) also
tried traumatic measures for the production of monstrous chicks.
They cut a small window in the egg-shell, wounded the embryo
with a l'aquelin thermocautery or a scalpel, replaced the fragment
of shell, fixed it in position, and returned the egg to the incubator.
They obtained monstrosities such as heterotaxy, duplicity of the
heart, and spinal cyphosis. Further details of the chicks with
double hearts were given by Warynski in 1886 (Sur la production
artificielle des monstres a cceur double chez les poulets, These, Geneva,
1886). In 1883, L. Gerlach and H. Koch (Biol. Centrlbl, ii. 681)
succeeded in getting dwarf embryos by partially varnishing the
eggs; more correctly, they got embryos one part of which was
smaller than the others. In two cases they found the monstrosity
known as omphalocephaly. C. Maggiorani (Influenza del magnctismo
sulla embriogenesi, Roma, 1885) tried the effect of incubating
eggs laid between bar magnets, and found that arrested develop-
ments were four times more numerous among them than among
eggs hatched in the usual way. Magnetism was also the means
employed by Windle some ten years later, as we shall see.
A. Marcacci (Ann. di Univ. libera eli Perugia, i. 171, 1885-86 ; Bull,
d. sc. med. di Bologna, 6 s., xxii. 5, 1888; Arch. ital. dc biol., ix. 58,
1887) experimented upon eggs by shaking them either in a vertical
or in a horizontal plane by means of a somewhat ingenious
apparatus ; the results seem to have been rupture of the vitelline
membrane and death of the embryo chicks, but sometimes anomalies
in the rate of growth were obtained. Tarulli (Atti e rendic. d. Accad.
med.-chir. di Perugia, ii. 121, 1890) returned to one of the older
methods of disturbing incubation ; he employed coating the eggs

KXPERIMENTAL TERATOGENY 215

with oil ; and he regarded the death of the chick as due not only
to the interference with respiration, but also to the altered pressure
within the shell, and to the change in temperature due to the
difference in evaporation.

While these different writers and experimentalists were engaged
in their researches, Camille Dareste was steadily working at artificial
teratogeny in Paris, and publishing from time to time the results of
his researches. It is time that I gave a summary of his labours in
this held of study, which he made so peculiarly his own. Although
his death did not take place till 1899, yet his life-work was accom-
plished between the years 1855 and 1891 ; he published, it is true,
two articles, one on the action of mercurial vapours on the embryo
(Compt. rend. Soc. de biol., Paris, 9 s., v. 683, 1893) and the other
on the effect of electricity (Compt. rend. Acad. d. sc., Paris, exxi.
955, 1895), after the year 1891, but the appearance of the second
edition of his large book in that year may still be looked upon as
the close of the most active part of his scientific life. It was not
long before Dareste found that the various agents which he used to
produce disturbances in embryogenesis all led to the appearance
of the same monstrous forms ; there was no relation between the
nature of the teratogenic cause and the kind of teratological result.
Consequently he did not much employ varnishing the eggs, fixing
them in one position, and the like, but used, almost solely, variations
of temperature. Having found the normal range of temperature for
normal incubation, he raised or lowered the heat and got teratological
results. In one set of cases only was he able to predict the character
of the monstrosity to be produced : when he unequally heated the
shell he obtained elliptical deformities of the blastoderm and
vascular area. From his numerous experiments made by varying
the temperature during incubation, and from the careful examination
of the monstrous chicks thus produced, Dareste was able to draw a
great many conclusions, most of which have already been indicated
in the sketch that I have given of the embryological theory of terato-
genesis. He drew, for instance, the deduction that teratogeny was
always the consequence of a modification of embryonic development,
and instead of basing it upon theory he showed facts. He decided,
therefore, against the nosological theory. He indeed admitted
the existence of diseases in the embryo (such as dropsy, of which
he gave a very complete account) ; but he looked upon them as
producing death or disorganisation, not malformation, of the
embryo. He laid great stress upon arrest of development as the
method by which single monsters were produced ; and, with the
single exception of heterotaxy, he regarded all malformations of
the single embryo as explicable by arrest. He noted that the
earlier the anomalies were produced in embryonic life the more
grave were the resulting teratological states. He differentiated
between hemitcrata in which the arrest of development was quite
local and was due to a teratogenic cause of little intensity, and the
monstrosities proper in which it affected a whole region and was the
result of a strong teratogenic influence. He described the develop-

216 ANTENATAL PATHOLOGY AND HYGIENE

merit of blastoderms without embryos ; defect of differentiation of
the embryonic area into the embryo and the vascular area ; the
arrests of development of the blood islands and the resulting
vessels of the vascular area ; the arrests of development of the
allantois ; and, what is of the greatest interest to us in connec-
tion with the present subject, the arrested development of the
amnion, which is the primary fact of most embryonic mon-
strosities (" fait initial de la plupart des inonstruosites embryon-
naires "). He demonstrated that triocephaly and cyclopia are
due to arrest of development of the anterior cerebral vesicle, the
result of premature closure of the anterior part of the cerebro-spinal
groove ; he discovered and explained the curious monstrosity called
by him omphalocephaly ; he showed that anencephaly, pseuden-
cephaly, and exencephaly all result from the total or partial com-
pression of the cerebral vesicles ; and he described how sym podia
arose from arrest of the caudal fold of the amnion, how exomphalos
was due to defective or arrested formation of the thoracoabdominal
walls, and how ectromely was caused by an arrest in the formation
or development of the limbs. He never succeeded in producing
double monsters artificially by altering the incubation conditions ;
but he, nevertheless, threw a great deal of light upon their origin.
He believed them to be produced in most cases, if not in all, by the
union and fusion of two embryos formed on the same cicatricula or
germinal vesicle, and he, therefore, thought that the existence of
twins was determined prior to the commencement of incubation, and
probably as the result of polyspermy or the entrance of more than
one spermatozoon into the ovum. He explained, in a reasonable
way, the origin of the curious acardiac, acephalic, and aniclean
placental parasites ; and he showed that, in the absence of a twin
embryo, such very rudimentary forms ("with an absence of soli-
darity") might be initiated but failed to get beyond the earliest
stages of development ; if a second embryo with a heart were present,
they might be supported and kept in life by it, and so be carried on
to a later phase of organogenesis.

One might easily refer to many other interesting questions in
teratogenesis opened up or cleared up by Dareste, but those alluded
to seemed to the writer to have special importance. A conclusion of
great value that followed upon the whole research was the similarity
of the monstrosities met with in the chick and those found in the
mammalia and especially in the human subject. In this fact Dareste
found the germ of the hope that the causes active in the chick in
teratogenesis might yet be found to apply also to the human subject,
and that thus human teratology might likewise be elucidated.

An obvious defect or want in all Dareste's work, however, was
found in the nature of the teratogenic agents employed. One could
not imagine human or mammalian monstrosities arising on account
of variations in the temperature of the surrounding parts (save in
the rarest circumstances) ; and electricity, shaking, deficient supply
of oxygen and the like seemed out of the question. This remark
applied equally to the work of the other experimenters of Dareste's

EXPERIMENTAL TERATOGENY 217

time ; even the traumatic means adopted by Warynski and Fol and
others could hardly be imagined as having their representatives in
human teratogenesis. Experimental teratogenesis, therefore, although
it had cleared up much that was dark in t;u, mode of production of
monsters {e.g. the compressing power of the ill-developed amnion),
had left in all its native obscurity the reat problem of the causa
causans. C. Giacomini (Giorn. d. r. Acer' . trrd. ell Torino, Nos. 6 and
7, 1889), indeed, had tried experiment;! c produce monstrosities
in mammals; he had used pregnant rabl .■ and had drawn off some
of the fluid from the blastodermic vesicle, introduced a foreign body
into it, and exercised pressure upon it ; In had succeeded in arresting
development to some extent, and had i., me cases met with an
atrophic embryo : but the general resu ts were not hopeful for the
revelation of the deep-seated causes of n onstrosities. L. Blanc's ex-
periments on the effect of bright light upon the chick embryo during
incubation (Gompt. re ml. Soc.de biol., 9 s., iv. 774, 969, 1892; v. 938,
1893) did not seem to help the teratologist much in his search for the
explanation of the cause of the arrested development of the amnion;
while B. C. A. Windle's observations (Journ. Anat. <mcl Physiol., xxix.
346, 1894-95) on the influence of electricity and magnetism on chicks
and trout ova left in the mind of the observer himself the idea that
the former agency (electricity) produced an arresting effect upon
development, but that the latter (magnetism) could not be definitely
said to produce any effect at all. Experimental Teratogeny, there-
fore, had not, up to 1894, thrown light upon the cause of amniotic
arrests of development, although it had established amniotic defects
as the most probable cause of the monstrosities themselves.

About this date (1894) Charles Fere began his series of observa-
tions. He took the hen's egg as the subject of experimentation just
as earlier experimentalists had done ; but by the use of chemical
substances, microbic toxines, and venin as his teratogenic agents, he
brought the whole subject into line with general pathology and made
the facts applicable to the problems of Human Teratology. Fere's
experiments and their results were published as a long series of
articles chiefly in the Proceedings of the Society of Biology of Paris
{Gompt. rend. Soc. de biol., Paris) between 1893 and the present year
(1903); but papers also appeared in the Journal de I'anatomie et de la
■physiologic and in the Archives d'anatomie microscopique. He sum-
marised most of the results in an article contributed to the Jubilee
Volume of the Society of Biology of Paris in 1899 {Cinquantenaire de
la BoeicU de biologic, Vol. Jubllalre, p. 360, 1899), and made an in-
teresting contribution to my journal {Teratologic, ii. 245, 1895) on
the etiological relations of sterility, monstrosities, abortion, morti-
natality, delayed development, and congenital debility. His experi-
ments may be divided into three groups, with two only of which we
have now to do. The third group deals with the production of
teratomata by embryonic grafts. The first group contained those in
which the conditions external to the egg were altered : Fere, here,
repeated many of the experiments of Dareste and others, with altera-
tions of temperature, traumatism, mechanical vibrations, white and

218

ANTENATAL PATHOLOGY AND HYGIENE

coloured light, and varnishing ; but he carried out on a large scale a
series of check or control experiments, and he was careful to allow
the eggs to remain at rest in the dark before beginning any experi-
ments with them. Further, he tried the effect of gases which might
penetrate the shell ; thus he surrounded the eggs with an atmosphere
of turpentine, musk, mercury, phosphorus, ammonia, ether, chloroform,
alcohol, and tobacco before submitting them to incubation. No eggs
were used of an age greater than eight days from the time of laying.
The second group of experiments contained those in which substances
in solution were injected into the albumen of the egg ; in this way
Fere tested solutions of salt, glucose, glycerine, iodide of potassium,
bromides of potassium and strontium, peptone, creatine, xantho-
creatinine, cantharidine, nicotine, sulphate of strychnine, hydrocyanic
acid, various alcohols and essences, acetone, microbic toxines, blood,
morphine, sulphate of atropine, hydrochlorate of cocaine, and venine.
In this group, also, rigid control or check experiments were always
carried out, and large numbers of eggs were used.

The results of Fere's experimental work were very striking. He
found, for instance, that there was a direct relation between the
teratogenic and the toxic power of poisons ; this was well borne out
in connection with the alcohols, and the following table of results
shows that those which were most toxic were also most teratogenic.

FERE'S EXPERIMENTS ON THE TERATOGENIC POWER
OF THE ALCOHOLS.

First Series.

No. of

Eggs.

Nature of Injection.

Propor

tion for 100 Embryos.

r

Normal.

Non-
developed.

Monstrous.

12

Controls

66-66

25-00

8-34

45

Ethylic alcohol

68-68

13-33

17-79

22

Propylic

40-90

4-54

54*56

23

Isopropylic ,,

8-69

8-69

82-62

21

Butylic ,,

47-63

o-oo

52-37

11

Isobutylic ,,

27-27

9-09

63-64

22

Amy lie

4-54

27-27

68-19

10

Isoaniylic ,,

Secox

10-00
d Series.

10-00

80-00

24

Water ....

75-00

16-66

8*34

63

Ethylic alcoholic .

53-96

11-11

34-93

63

Methylie ,,

23-80

11-11

65-09

EXPERIMENTAL TERATOGEN Y 219

Further, the microbic toxines that are most deadly for the hen are
also the most deadly for the embryo chick, and inversely. Morphine,
for instance, which is well borne even in large doses by the fowl, may
also be injected in large doses into the egg without hurting the embryo.
The same is true of cocaine and atropine. Generally, the agents
which determine anomalies of development produce at the same time a
marked retardation of growth, so that it may be said that the anomaly
is in relation with the retardation of growth. Dwarfs were frequent in
all the conditions in which anomalies were common. Some substances
which had a dystrophic effect in large doses had a eutrophic effect
in small ones ; but in estimating such differences it was always well
to bear in mind the individuality of the germ, so strongly insisted
upon by Dareste as well as by Fere. Even in the case of prussic
acid (of enormous teratogenic power) some of the embryos escape.
This individuality is, of course, manifest in the human subject in re-
lation to various infections, etc., where it may take the form of
sporadic immunity. Further, Fere sometimes found among a lot of
eggs which had been subjected to a teratogenic influence, and most of
which had in consequence been arrested in development or mal-
formed, one egg with a development not simply normal but above the
normal. This occurrence indicated a tendency to variation, which,
according to the dose of the agent and the individual trophic equation
of the germ, might appear either in the direction of exaltation or in
that of depression. There might then be, so to say, supra-anomalies
as well as infra-anomalies. The same phenomenon was met with in
families of human beings in which men of genius existed side by side
with vicious and intellectually degraded individuals. During the
past two or three years Fere has specially experimented with sub-
stances such as caffeine which might be supposed to have a stimu-
lating effect upon embryonic development and growth, and has found
that with small doses there is some evidence of such an action. The
same was true of antipyrine.

Fere noted, also, that, while the number and gravity of the defects
of development increased with the intensity and the duration of
action of the disturbing causes, no particular monstrosity was related
to any special influence. This fact, of course, had been previously
observed by Dareste. It was also noted that a physical or chemical
agent which had a teratogenic action when the organism was in the
embryonic stage had a pathogenic action at a later date ; and as was
pointed out in the article in Teratologia (referred to above), the same
influence might, according to the time when it acted, cause sterility,
monstrosities, abortion, morti-natality, retarded growth, or congenital
debility.

Fere, in strong contrast with Dareste, paid little attention to the
monstrosities he succeeded in producing, and dwelt rather on the
causes and their nature. At the same time he was always careful to
enumerate the results of his experiments : in many cases there was
absence of development or blastoderms without embryos ; in many
others there was cyclopia, or dropsy, or heterotaxy, or doubleness of
the heart, or omphalocephaly, or atrophy of the head.

220 ANTENATAL PATHOLOGY AND HYGIENE

I may here refer very briefly to some experimental work done by
other observers. Maffucci (Centrlbl. f. allg. Path. u. path. Anat., v. 1,
1894 ; Ann. Surg., xx. 385, 1894; Policlinico, i. 33, 1894) investigated
the effect of pathogenic germs on the hen's egg and foetal rabbit,
and found that while the embryo lived, pathogenic germs did not
develop in its tissues except in rare circumstances; they might,
however, be stored up for later development in extra-ovular life.
S. Kaestner (Arch. f. Anat. u. EntweMngsgesch., 319, 1895 ; Vcrhandl.
(I. anat. Gesellsch., Jena, x. 136, 1896) made experiments by cutting
off the artificial heat for a longer or shorter time during the incuba-
tion of hens' eggs ; he thought the malformations which followed
(e.g. arrest of development of head, defect in area vasculosa) were
due to the cooling of the yellow yolk which caused the embryo to
be pressed against the vitelline membrane. P. Mitrophanow (Arch,
f. Entwicklngsmechn. d. Organ., vi. 104, 1897) tried the effect of
varnishing one side of the egg before incubation ; at ordinary tem-
peratures he got no results, but if the heat was increased the area
pellucida had an irregular shape, and there was delay in the forma-
tion of the primitive groove.

Fere has, to my mind, greatly advanced the science of experi-
mental teratogeny by breaking down the dividing wall between it
and general pathology, a dividing wall which was felt to exist in
consequence of the peculiar nature of the supposed exciting causes
of monstrosities (e.g. shaking, raising or lowering the temperature,
varnishing, and wounding). Doubtless these might be the causes of
diseases as well as of monstrosities ; but it was difficult to imagine
them in action in the case of mammals, and more particularly in the
human subject. Fere, however, at once broke down this wall when
he introduced such substances into the egg as alcohol, morphine,
strychnine, nicotine, and microbic toxines ; these were already known
to be the causes of disease, and the results of Fere's experiments
showed them to be also teratogenic. Immediately, it was seen that
experimental teratogeny fell into line with experimental pathology ;
the experimentalist felt that here he was dealing with similar things.
The results might be very different — monstrosities as a matter of
fact were very different from diseases — but it was possible to con-
sider that the differences were due, not to variations in the cause,
but to dissimilarity in the structures acted upon, namely, immature
organs as compared with mature ones. The causes then might be
similar but the effects different. Another good result followed
Fere's work : it gave to teratogeny a clinical aspect. If the causes
of disease were likewise the causes of monstrosity, then it might be
expected that in the parents, and especially in the mother of mal-
formed foetuses, some trace of such morbid causes might be found.
The question then arose whether malformed offspring were more
common in the case of alcoholic parents or of those suffering from
syphilis, tubercle, infectious fevers, and the like. That question
cannot be said to be fully answered yet, but it is in process of being
answered (as the reader will find demonstrated in the first section of
this Manual, under such headings as foetal syphilis, foetal tubercle,

RESULTS OF EXPERIMENTAL TERATOGENY 221

foetal alcoholism), and so far there has heen not a little supporting
evidence drawn out of the facts.

Let us now return from this long excursus into the realm of
experimental teratogen}7. It was made, the reader will remember,
in order to ascertain whether experiment had any answer to give to
the question of the cause of amniotic arrest or disease. It was
regarded as highly probable that defect of the amnion, acting by
pressure, was the cause of the arrested development of the embryo,
which in its turn produced the malformation or monstrosity ; but the
further question was raised, What was the cause of the amniotic
defect ? Dareste's experiments increased our knowledge of the
mode of action of amniotic defects, but did not give any utilisable
information as to the causes of them. Fere's researches, however,
showed us that probably the causes of disease are just the causes of
the malformations. But now we have to inquire whether we can
link together the results of Dareste's and Fare's work, and conclude
that the imperfect development of the amnion is the cause of tera-
tological states, and that the causes of disease are the causes of the
imperfect development of the amnion. Here a surprise awaits us,
for Fere seems, more especially in his recent writings, to be taking
up a position antagonistic to the amniotic theory- In his "Note sur
l'embryotomie teratologique " {Rev. de chir., xx. 592, 1900) he
criticises the idea of the teratogenic effect of amniotic pressure,
and points out that Dareste himself had found in some cases that
the amnion was widely separated from the embryo by a copious
supply of liquor amnii, and that the same anomalies may occur both
with and without arrest in the development of the amnion. By
varnishing a longitudinal half of the egg, Fere succeeded in obtain-
ing an embryo in which the head was separate from the trunk, and
yet the amnion formed a large ovoid sac whose walls were far removed
from the embryo. In another egg, which had been subjected to the
influence of xylol fumes during incubation, Fere discovered an
embryo with deformed cephalic and caudal parts joined by a narrow
neck of constriction ; yet the amnion formed a broad sac at the point
of constriction. The conclusion which Fere draws (and he is apparently
warranted in doing so) is that the amnion is not necessarily the
means by which ontogenesis is arrested and malformations produced.
He seems to suggest that in the place of the amniotic factor we
must put a change in nutrition. The reader may feel (and he is
justified in so feeling) that, after all, experimental teratogeny has
not done much for the understanding of the mode of origin of
monstrosities if it has weakened a belief in the inliuence of the
amnion. It may be said that the same cause may be active in pro-
ducing both the embryonic malformation and the amniotic defect,
and that that cause may be a disturbance of nutrition due to toxic
or microbic influences ; but I am not sure that this generalisation
makes up in value for the loss of the amniotic theory which it seems
to entail. Further, we still await clinical confirmation of the
hypothesis that germs and their toxines and inorganic and organic

222 ANTENATAL PATHOLOGY AND HYGIENE

poisons acting on the parents produce malformed offspring. Per-
sonally, I am loath to throw overboard the theory of amniotic
influence in teratogenesis, and to welcome in its place the hypothesis
suggested by Fere ; but it seems to me that it is not impossible to
combine the two, and to believe that, as the human amnion apparently
precedes the human embryo in the date of its development, so a
general defect of nutrition may arrest the growth of the former and
by pressure lead to arrest of the ontogeny of the latter. When the
amnion is not arrested and the embryo is nevertheless malformed, it
is permissible, I think, to imagine that the general cause (nutritional
or other) came into action after the amnion had been formed and
acted in some other (non-amniotic) way upon the embryo. The
explanation is not in all points satisfactory, but it in some measure
fits the facts as we find them. At the same time it may be repeated
that the way is not yet closed against the advance of new hypotheses
which may still better fit the facts.

Now it is quite time that I refer to the third strand in the
Ariadne-thread that is leading us through this labyrinth of hypotheses,
suggestions, and (it is feared) imaginings concerning teratogenesis.
The reader may by this time be in grave doubt as to the reliability
of the Ariadne-thread to lead him out of the labyrinth ; he may
suspect that he is only being led more deeply and hopelessly into its
perplexingly intricate and anfractuous passages. Further, he may
be reminding himself that the writer has as yet advanced no explan-
ation at all for the occurrence of double monsters and malformations
by excess, and that the hereditary transmission of special mal-
formations from parent to child has in no way been dealt with.
Obviously, neither amniotic pressure nor nutritional defects offer a
reasonable or satisfactory explanation of these problems. The writer
informs the reader that these problems are precisely the ones which
he is now to take up, and that it is for their elucidation that he
presents the third strand of his Ariadne-thread. May it be strong,
and may it lead us out of this gloomy labyrinth, and that quickly.

We have already seen that some of the morbid states found
existing in the foetal period of antenatal life had their cause and
origin in the embryonic; similarly there is good reason to believe
that some of the more peculiarly embryonic conditions, such, for
instance, as the double monsters, may originate in the pre-embryonic
or germinal epoch. In introducing the germinal factor into the
explanation of certain monstrosities, I am not trying to get out of
an impossible position by referring a difficulty, which is inexplicable
by any known mechanism in the little understood embryonic period,
back into the less understood germinal period. There are some
facts to go upon, albeit they are neither numerous nor completely
established ; perhaps they ought to be called quasi-facts. There are
certain purely physiological conditions which are apparently deter-
mined in the pre-embryonic stage of antenatal life. For instance,
when a child inherits peculiarities and characteristics from his
father, these must have been impressed on the germ prior to the

GERMINAL FACTOR 223

appearance of the embryo in the embryonic area ; they must,
indeed, have existed potentially in the spermatozoon before impreg-
nation. Again, there is good reason to believe in the determination
of sex prior to the embryonic epoch, and to accept the statement
that the ova are already male or female, although, in the case of the
higher animals, there are no discoverable structural differences which
shall tell us to which sex they belong. This, at any rate, is the
belief of M. von Lenhossek (Das Problem der geschlechtsbestimmenden
Ursachen, Jena, 1903) and several others. It must be admitted,
however, that some, e.g. A. van Lint (Qu'est-ce qui determine le sexe ?
Paris, 1902), are of opinion that the sexual future of the offspring is
not settled till impregnation, and that when a "strong" spermatozoon
meets a "weak" ovum the embryo will be female, and vice versd.
This does not, however affect the main question, for impregnation
takes place in the germinal or pre-embryonic epoch.

When we pass from physiological to pathological states, we are
almost compelled to admit that some of them, like the sex of the
embryo, and like some of the hereditarily transmitted paternal
traits of character, must be determined in the germinal period of
antenatal existence. Thus when we meet with the same mal-
formation in two or three generations of the same family, there
seems no other reasonable explanation of the occurrence than that
either in the spermatozoon or in the ovum there was a teratogenic
something which determined the abnormal development of the
embryo. There is no lack of instances of such occurrences, as,
for example, hereditary hare-lip, fistulae in the lower lip, aniridia,
coloboma of the iris, microphthalmia, fistula auris, ectromely, ectro-
dactyly, Polydactyly, polymastia, absence of the patella, hypospadias,
etc. etc. The occurrence, also, of the same malformation in several
members of the same family seems to require a similar explanation.

Then there is the question of the causation of twins, binovular
and uniovular, and of double monsters. Twinning would seem
clearly to be a state determined by the existence of several ripening
ovi-sacs in the ovary at the same time, or by anomalies in the ova
(or spermatozoa) before or during impregnation. Double terata,
likewise, may be explained by the entrance of two spermatozoa into
one ovum (polyspermy), or, perhaps, by the presence of two germinal
vesicles in one ovum. I cannot, at this point, enter into the con-
sideration of the evidence in favour of these views, — that will come
in its proper place in the chapters treating of germinal pathology, —
but it may be said that it is largely of the experimental kind. There
has been accumulated, of recent years, much evidence founded upon
experiments carried out upon the ova of various animals (especially
echinoderms and ascidians), with which the names of such well-
known experimenters as Chabry, Eoux, Driesch, Hertwig, E. B.
Wilson, and others, have been connected. These will be referred to
by and by ; in the meantime it may be taken for granted that
there is proof that altered surrounding conditions may seriously
affect the future of the ova subjected to them, and that, not in-
frequently, teratological results follow. The altered surrounding

224 ANTENATAL PATHOLOGY AND HYGIENE

conditions may be due to chemical substances or toxines, or to
mechanical agencies, and thus the causes of germinal pathology
may be brought into line with those active in embryonic, foetal, and
postnatal pathology ; the causes may be the same while the effects
are various.

The germinal factor, then, is the third causal state which is
advanced to clear up the origin of monstrosities. In it may be
found the explanation of diplogenesis (or the formation of twins),
of diploteratology (or double monsters), and of the hereditary
character of malformations in certain families. I have done little
more, at this stage, than indicate its nature ; its full consideration
must be reserved till germinal pathology is dealt with.

Summary.

To gather up all these threads of argument and proof, let me
very briefly summarise what I believe are the chief causes of
monstrosities. They are the results of disorderly embryology, of
disturbed ontogenesis and organogenesis. Many of them are arrested
developments, and represent stages which ought to have been
temporary in ontogenesis, but which have remained stationary while
other and neighbouring parts were pursuing the path of normal
development. In the case of some of them at least the amnion
would seem to act by pressure, and so delay, or altogether stop, the
progress of events in ontogenesis. The amniotic influence, in its
turn, may be the result of the action of toxines, poisons, and
mechanical states which delay the formation of the amnion; or
possibly these substances and states may act directly upon the
embryo and alter its nutrition, and consequently its development
and growth. Other monstrosities, and more particularly those by
excess and the double terata, may be due to morbid causes acting
upon the blastoderm before embryonic life begins, or upon the ova
and spermatozoa before or during impregnation. On the other
hand, there are minor monstrosities or malformations which may,
perhaps, be produced in the fcetal period by the action of intra-
uterine pressure or external traumatism ; for it must be remembered
that certain parts of the organism are still in the formative or
embryonic stage, although the organism, as a whole, has passed into
the fcetal phase. Finally, it is not impossible that the same ultimate
causes may be active in all the three periods of antenatal life,
although the results are so strikingly different.

In all this, there is much that is uncertain, obscure, dark ; but
perhaps

"A Light
Will struggle through these thronging words at last."

CHAPTER XIII

Classification of Teratology : Historical Sketch of the Systems of Empedocles,
Obsequens, Lycosthenes, St. Isidore, Weinrichius, Schenkius, Aldrovandus,
Licetus, Huber, Haller, Buffon, Malacarne, Penada, Meckel, Breschet,
Cliarvet, Saint- Hilaire, Guilt, Otto, Bischoff, Sangalli, Forster, Davaine,
Billard, Ahlfeld, Cleland, Hirst and Piersol, and Thompson Lowne ; Diffi-
culties of Teratological Taxonomy ; " Connecting Links " ; Taruffi's System
of Classification ; Teratological Nomenclature ; the Author's Classification
based upon Taruffi's ; Special Teratology of Embryonic Life, and of Germinal.

It may be stated with some assurance that many investigators have
been turned from the study of Teratology by the complexities of its
various schemes of classification and the eccentricities of its nomen-
clature. The subject has suffered and is suffering from too much
classification, and from too many names of too many syllables. The
science of monstrosities is not ready for any permanent mode of
classification ; neither, it will be remembered, was that of the
pathology of the foetus (vide Manual of Antenatal Pathology — The
Faitus, p. 174). Teratology, however, deals with matters much less
easy to distribute or arrange systematically than does Foetal
Pathology ; its classification, therefore, although much attempted,
is in a still less satisfactory state than that of Foetal Pathology.

The primary requisite of classification is that the facts or pheno-
mena should all be known, and this knowledge cannot be postulated
for Teratology. The object of such Taxonomy is to bring together
those things which most resemble each other, and to separate those
which differ ; and this object, on account of our want of knowledge of
the facts, cannot be attained in connection with Teratology. It is
necessary, however, that there be some sort of grouping, tabulating,
collocating, or disposing of the facts, such as are known, even if for
no other purpose than to serve as an aid to memory, a memoria
tcchnica. This object may be attained in several ways, but to my
mind the best, because the simplest, plan is a regional arrangement,
such as I adopted in Teratologia in 1894, and have employed ever
since. It is modelled upon Taruffi's scheme, used by him in his large
work, Storia della teratologia, and differs from it only in details.
Before, however, I give a sketch of this plan of classification, I must
refer briefly to other methods which have, from time to time, been
advocated by well-known teratologists.

In early times little or no classification of monstrosities was
attempted. In the Chaldean cuneiform tablets dealing with
vol. ii. — 15

226 ANTENATAL PATHOLOGY AND HYGIENE

monsters (Fig. 21, p. 89), the only scheme of arrangement would
seem to he into those monstrosities that were horn to ordinary
people and those forming the offspring of royal parents ; those who
are interested in such matters will find a description of the different
types in my article on the " Teratological Records of Chaldea" (Tera-
tologic!,, i. 127, 1894). Empedocles, recognising in the male semen
the great cause of generation in general, and of monstrosities in
particular, found in it six conditions which might provoke tera-
tological developments, namely, abundance of semen, defect, slowness,
aberration of movement, and division of its substance ; but it would
be difficult to work out such an etiological classification nowadays,
and we may well suppose it was no easier in the times of Empedocles
and Aristotle.

When, in the Dark Ages, the supernatural origin of monstrosities
came to be believed in, no classification in the real sense of the word
was any longer possible. Julius Obsequens in his Prodigiorum Liber
simply allocated the monstrosities known to him according to the
year after the founding of Eome in which they were born ; Conrad
Lycosthenes, in his larger Chronicon Prodigiorum atque Ostcntorum
(1557) pursued the same chronological plan, but enlarged its scope
" ab exordio mundi usque ad nostra tempora." Both these writers
included marvellous occurrences as well as human monstrosities in
their works ; so it comes about that we find the plagues of Egypt,
earthquakes, and one-eyed infants placed side by side in their
attractive and surprising pages.

The first attempt towards an arrangement of monstrosities having
any classificatioiial value seems to have been that of St. Isidore, the
archbishop of Seville, in his work on Etymologies (600 to G36 A.D.).
He placed them in ten groups, with, to distinguish them, such lead-
ing characters as large or small size, defect of parts, transformation
of parts (e.g. the minotaur) or of the whole body (as when a woman
gives birth to a calf !), transposition of parts, adhesion of them,
precocious or delayed appearance of structures (e.g. teeth and hair),
mixture of the sexes, and the presence of several different deformities
in one case. There was no place found fur the double terata. The
Isidorian system is memorable ; but it was disfigured by remnants of
superstitious beliefs, such as the possibility of animal births occurring
in the human subject. It was in advance of the systems which
prevailed about the middle of the sixteenth century, when
Lycosthenes was bringing out his Chronicon, and when Jandunius
(1543) was referring monstrosities to defect, absence, excess, or
activity of the menses or materia. There was, in fact, nothing so
good as the Isidorian arrangement till the seventeenth century, for
all sorts of fabulous monsters were believed in, and many diseases,
such as elephantiasis, were grouped with the truly teratological states.
Weinrichius (Be ortu monstrorum commentaries, 1595) used some of
Isidore's divisions; but he was, as Haller described him, a credulous
man and not an anatomist.

The Schenks, father and son, in their Obscrrationum mcdicarum,
rararum, novarum, admirabilium et monstrosarum volumen (Franco-

CLASSIFICATION 227

furti, 1G09) introduced the idea of a regional classification of
deformities ; but their work was concerned with all sorts of matters
besides monstrosities ; and the first to use this method of arrange-
ment for teratological phenomena alone was Ambrosinus in his
edition of the works of Ulysses Aldrovandus (1642). Under the
head of monstrosities Aldrovandus included the anomalies of animals
and plants, and so was the founder of the science of Comparative
Teratology, which is only now beginning to take its place with
Human Teratology in the larger subject of Anomalous Formations.
First the deformities of the head were dealt with, then those of the
arms, hands, abdomen, genitals, and feet, then those of the skin and
of the tail (in animals), then dwarfs ; and finally double monsters
were described.

There is much to be said in favour of a regional classification of
monsters in the present imperfect state of our knowledge, and I have
adopted it as the basis of the arrangement used in this work ; but
the system of Aldrovandus (witli Ambrosinus as his editor) was not
elastic enough to include all true malformations, while, on the other
hand, it provided ] daces for fabulous creatures which ought to have
been closely scrutinised before they gained admittance within the
limits of the subject. Licetus also, in his treatise De Monstr is (161G),
would have been wise had he been more sceptical regarding the
genuine character of many of the curious hybrid productions which
he pictured and placed among the multiform monsters of his classi-
fication. He divided all monstrosities into the multiform and
the uniform : among the former he placed the monsters made up of
the parts of animals of different species or of different individuals of
the same species, including the centaur, the minotaur, the infant
with a bird's wings, and diablotins ; while among the latter were
human or animal monstrosities of the mutilated type, or with
superfluous parts, or of doubtful sex, deformed, formless, and
" enormous." There are details in the scheme of Licetus which
remind us of St. Isidore's classification ; but Licetus lays no great
stress upon arrangement, and was more occupied with the enumera-
tion of causes.

No new plan of teratological classification made its appearance
during the hundred years that followed the publication of the work
of Licetus ; but in 1748 J. J. Huber (Ohscrvationes atqne cogitationes
nonnullce de monstris) distinguished nine classes of monstrosities,
among which were the well-established types of excess or defect of
development, along with such novel varieties as " union of parts
usually separate " and the " closed state of canals usually open."
Fabbri (De hicmano quodam monstro, 1767), as Taruffi states, added
another type of monstrosity to those already known, namely, that due
to a morbid process ; but it was not till the time of Haller was
reached that Teratology began to be scientifically defined, and, there-
fore, to be capable of scientific attempts at classification. Haller
himself in his work De monstris (Opera minora, iii., 1768) was not
particularly happy in his classification, for he employed different
plans, and so missed uniformity ; but he did much to rid the subject

228 ANTENATAL PATHOLOGY AND HYGIENE

of the mediasval superstitions still adhering closely to it, and so
earned the gratitude of future generations of teratologists. Button's
division of monstrosities into those by defect of development, those
by excess, and those by altered relation of parts, was not so much
a classification as a conception of the ulterior etiology of teratological
phenomena in general. It was an attempt, after the manner of
Empedocles, to introduce Teleology into Teratology, and to discuss
the final causes of things. Some twenty years after Button's Histoire
naturellc appeared, V. Malacarne {Mem. dclla soc. ital, ix. 49, 1798)
published a scheme of classification with many classes so as to offer a
place for all known monstrosities ; but although he did not succeed
altogether in this aim, he was happy in his construction of the names
of teratological types from Greek roots, and the words microsomia,
macrosomia, micromelia, macromelia, and polymelia suggested by him
have been retained by several modern writers and classifiers. Some
of Malacarne's classes, as those for men with limbs of animals and for
animals with human extremities, were ridiculous.

The nineteenth century saw a great development of the taxonomy
of Teratology, although it may be doubted whether much of the time
spent upon it might not have been better employed. In the early
years of the century J. Penada (Atti dell'Accad, ital. d. sc, I. pt. i.
284, 1810) tried to get back to the more general aspects of Teratology,
and proposed to divide monstrosities into three groups according to
the proximate causes, which he found in anomalies existing in the
germ, in external and mechanical conditions affecting the foetus, and
in diseases, such as dropsy, attacking the unborn infant. Although
there was much that was far-seeing and thoughtful in Peuada's scheme,
it was not, at the time when it was published, capable of being
utilised for the purposes of classification. J. F. Meckel, with less
originality than Penada but with more immediate advantage, adopted
Button's division of malformations into three causal sections (1,
diminished formative force or energy ; 2, increased formative force ;
3, anomalies in form and position of parts), and added thereto a fourth
containing the hermaphrodites. Meckel worked out the details of
his scheme with wonderful completeness {Handhuch der paihologischm
Anatomic, Bd. I., II., Lipsire, 1812-16), but with no little difficulty
and with many somewhat forcible disjunctions of similar things and
conjunctions of dissimilar things. His group of henna] ihrodites, also,
did not fall into line with the other three divisions, and stood rather
awkwardly by itself. The classification proposed by Breschet {Diet.
dcmkl.,\'\. 524) got over one of these difficulties by giving no separate
place to the hermaphrodites ; the third division (anomalies in form
and position of parts) was also done away with ; and, under names
derived from the Greek, four orders of teratological processes were
enumerated, namely, agenesis, hypergenesis, diplogenesis, and hetero-
genesis. The first two of these processes corresponded to the old
divisions of monstrosities by defect and excess ; the third process
was that which produced double terata ; while the fourth gave origin
to a group containing an extraordinary medley of formations, in-
cluding twins, ectopic pregnancy, albinism, and cyanopathy. Breschet

SAINT-HILAIRE'S CLASSIFICATION 229

gave to double monsters a place among four orders ; but Charvet
(Recherchcs pour servir a Vhistoire general: de la monstruosite, Paris,
1827) divided all anomalies and monstrosities into two large classes,
one of which contained the double terata, and the other the single
ones (deformed by irregularity, by defect, and by excess).

We come now, in chronological order, to the classification
elaborated by the Saint-Hilaires (Etienne and Isidore), by far the
most complete and thorough that had yet appeared. These writers,
in their works (Philosophic anatomique, ii. 1822 ; Histoirc des anomalies,
vols, i., ii., hi., 1832-37) built up nothing less than a great edifice, a
huge classiticational structure, providing accommodation fur all the
then known types of anomaly, and dwarfing all other competitive
fabrics as well by its bulk as by its intricacy. The idea adopted and
worked out by the Saint-Hilaires was similar to the natural method
of classifying plants and animals ; in fact anomalies were regarded
as constituting a "Kingdom." This kingdom was divided into four
primary divisions : there was, first, the division of the hemiterata or
simple anomalies, malformations which did not profoundly influence
the whole organism ; there were, second, the heterotaxies, the internal
anomalies which did not affect any functions, and found their typical
state in situs inversus or transposition of the viscera; there were,
third, the sex anomalies, grouped together as the hermaphrodisms ; and
there were, fourth, the monstrosities, which included internal and ex-
ternal defects of a marked kind. Thus far the classification did not differ
greatly from some others ; but the Saint-Hilaires subdivided these four
main branches or divisions in a most complicated manner into classes,
orders, tribes, families, and genera. For instance, the monstrosities
were divided into two classes — the single monsters and the composite
or double (and triple) monsters ; to take again the single monsters,
they were divided into three orders, that of the autosites, that
of the omphalosites, and that of the parasites ; then the autosites
were divided into four tribes, and these tribes were divided into
two or three families each; and, finally, each of these families, 'e.g.
that of the ectromelians, was divided into several genera. The
hemiterata were split up, although not with such complexity, into
similar groups ; while the heterotaxies and hermaphrodisms had a
much simpler arrangement. The scheme of classification of the
Saint-Hilaires by its very completeness lays itself open to criticism.
Its most serious fault, however, is found in the primary conception
that anomalous creatures form a world by themselves, a great
kingdom or sub-kingdom like that of the plants or the animals ;
there is no justification for such a comparison. Further, the idea of
genera, species, and the like, which arises out of this conception, leads
to the use of these expressions in a very misleading sense ; for while
species in Zoology are groups of living beings which transmit by
generation a complex of characters, species in Teratology are simply
individuals suffering from the same malformation. We can, however,
use the classification without accepting the ideas of species, genera,
and families which underlie ; we may simply look upon these as
larger and smaller divisions not related to each other in the intimate

230 ANTENATAL PATHOLOGY AND HYGIENE

way suggested by the Saint-Hilaires. The nomenclature is in many
respects admirable ; it is mainly formed upon Greek roots ; and, what
is greatly in its favour, it has been largely accepted not only in
France but also in other countries.

In the hands of E. F. Gurlt, Comparative Teratology was fashioned
into a science. The classification which he employed (Lchrbuch der
pathologischen Anatomic dcr Hems- Sang cthicrc, ii., 1832) was com-
paratively simple. He divided all monstrosities into two classes :
the first contained the simple or single monsters ; the second, the
double terata and the very rare triple monsters. He had, also, a
third group in which were the anomalies of individual organs of the
body. In his first class were to be found monsters by defect (monstra
per defectum), by smallness of parts (par vitas partinni), by division of
the body (fissio corporis abnormis), by union of parts (coalitio partium),
by contortion, and by excess {per execssum) ; and he placed with all
these the hermaphrodites. The difficulties of separating the single
monstrosities rigidly from the anomalies of individual parts were too
great to be overcome by any scheme, and as a matter of fact they
were not overcome by Gurlt's arrangement. Further, it is an
anomaly for cases of fcetus in fcetu to appear anywhere save among
the double terata ; Gurlt has placed them in the third group.

Otto's classification (Monstrorum Sexcentorum Dcscriptio Anatomlca,
1841) pursued the now beaten track of division into two classes,
monstra deficicntia and monstra abundantia ; but to these two a third
class was added of monstra sensic strictiore deformia, which contained
spina bifida, facial fissures, exomphalos, ectopia vesica", syndactyly,
and deforming diseases {e.g. hydrocephalus, congenital rickets, dropsy,
cysts, and tumours). Otto's collection included both human and
animal monstrosities. His third group was an awkward one, con-
taining as it did anomalies in no way resembling each other.

T. L. W. von Bischoff (in E. Wagner's Handwdrtcrbuch dcr
Physiologic, i. 901, 1842) gave a classification of much the same
kind : he had three primary divisions into monsters by defect, by
excess, and by alteration of form without either excess or defect.
He placed malformation due to atresia or to fissure in the first group,
double monsters in the second, and hermaphrodites in the third ; but
he also placed the amorphous, acormic, and acephalic twin foetuses in
the first group. Every classification founded upon the distinction of
defective and excessive development broke down in one or other of
its details; and Sangalli in 1860 (in an article quoted by Taruffi,
Storia delta tcratologia, i. 366, 1881) did not greatly diminish the
difficulty by having five primary divisions (excess, defect, anomaly of
position, abnormal division, and abnormal union).

A. Fbrster's contributions to teratological taxonomy deserve a
paragraph to themselves. At first, in his work Die Missbildungen
drs Menschen, published in 1861, he adopted the usual plan of dividing
monstrosities into those by excess, those by defect, and those " per
fabricam alienam," or monstra abundantia, deficicntia, et alienantia
s. abcrrantia ; but in a later book (Handbuch dcr pathologischen
Anatomic, ii., 1862-65) he tried to arrange teratological formations

FORSTER'S CLASSIFICATION 231

according to the time in antenatal life when they were supposed to
originate. In one group lie placed the results of the pathological
development of the germinal area, including pathological twin states
(double monsters and placental parasites), pathological development
as regards size (giants and dwarfs), and heterotaxy; in a second
group were the pathological developmental states of the extremities
(excess or defect) ; in a third were those due to abnormal develop-
ment of the spinal canal ; while in the fourth, fifth, sixth, seventh,
and eighth were those resulting from anomalies in the ontogenesis of
the face, neck, thorax, abdomen, intestines, uro-genital organs, and
vascular system. This plan was scientifically sound, and it will yet,
I believe, be accepted ; but, at the time when Forster wrote, the
details of organogenesis were not well enough known to permit of
the application of such a time-arrangement of monstrosities. Even
now, with all our additional knowledge of early antenatal affairs, it is
doubtful whether we can assign the various monstrosities to their
special dates of origin. F. v. Winckel, however, has applied the
principle very satisfactorily to uterine malformations (Uebcr die
Eintheilung, Entstchung, und Benennung dcr Bildungshemmitngcn der
wciblichcn Scxualorgane, Leipzig, 1809) ; although, of course, these
anomalies, on account of the late date when the genitals develop,
are more easily traced out and allocated to the proper weeks. For
one great generalisation we are much indebted to Forster, namely, that
Teratology is the Pathol* igy of the End iryo ; a great clarification of
teratological problems has resulted therefrom, although many things
yet remain dark, as the reader knows very well by this time.

C. Davaine in his article " Monstres " in the Dictionnaire encyclo-
pediquc des sciences medicales (2nd Series, vol. ix. p. 260, 1875) struck
out a new line in the classification which he followed. He divided
monstrosities into two divisions according as the anomalies affected
organs concerned in the life of the individual or parts devoted to the
reproduction of the species. Obviously the divisions were of very
unequal size, even although Davaine put the double terata into the
second one. As a classification little is to be said in its favour ;
indeed, its utility is less than that of the plan of C. M. Billard
{Traitt des maladies des enfans, p. 399, 1835), according to which all
congenital diseases and malformations were divided into three groups
according as they were necessarily fatal to life, or were opposed to
the development of an independent existence, or were no obstacle to
viability.

F. Ahlfeld {Die Misslildungcn des Menschen, Parts I. and il, 1880-
1882) in the two parts of his work which have appeared, deals with
double monsters, anomalies by excess of individual parts, and giant
growths in one division (" Spaltung, Doppelbildung, Vercloppelung "),
while in another (" Spaltbildung ") he considered all the malforma-
tions characterised by fissure-formation and patency of openings
which ought to close. The second group, in particular, contained a
medley of anomalies with no very strong resemblance to each other
to hold them together in taxonomic order. J. Cleland's classification
{Memoirs and Memoranda of Anatomy, \. 131, 1889), being founded,

232 ANTENATAL PATHOLOGY AND HYGIENE

as it is, upon causation, necessarily rests upon theories rather than
on established facts and individual resemblances; but it contains
much that is well worth consideration, and for that reason I repro-
duce it here in brief :

Monstrous and Anomalous Forms.

I. By Defect—

A. Without distinct external cause.

1. Dwarfing. 2. Failure of completion of form of parts.

B. Consequent on dropsies and changes connected therewith.

1. Pleuro-peritoneal. 2. Cerehro-spinal.

C. By Strangulation.

1. By thickened tissue occluding openings or preventing their

formation.

2. From obstruction to the separation and expansion of

potential parts.

3. From external causes, e.g. umbilical cord, and bands of

fibrin.
II. By Excess —

A. Hypertrophy.

1. Of whole body — Giants. 2. Of parts.

B. Multiplication of parts — Polydactylisrn.

C. Longitudinal fission of embryonic mass.

1. Complete fission.

A. Permanent (twins in one chorion).

B. Temporary (the divisions being subsequently united

by corresponding parts).

2. Incomplete fission.

A. Abcaudal.

B. Abcranial.

C. Fission simultaneously abcaudal and abcranial.

B. Cooke Hirst and G. A. Piersol (Human Monstrosities, 1891),
being dissatisfied with all the plans of classification which have been
proposed, adopted that of the Saint-Hilaires as being founded on
wide observation and possessing flexibility and familiarity. These
authors, therefore, divided anomalies into the four groups of the
hemiterata, the heterotaxies, the hermaphrodites, and the monsters
or terata (proper); but they made slight modifications in the sub-
divisions, substituting, for instance, Forster's arrangement of double
terata for that of Saint-Hilaire.

Thompson Lowne (Descriptive Catalogue of the Teratological Scries
in the Museum of the Royal College of Surgeons of England, 189,3)
adopted a regional classification as being that best suited for Museum
work. In all, eleven series were enumerated, and several of the
series were subdivided into anomalies by excess, by defect, and by
distortion. The first series, that dealing with abnormal conditions
affecting the head and trunk, was disproportionately large ; it was
divided into five classes (situs mutatus, secondary dichotomy, double
malformations, malformation with deficiency, and malformations by

DIFFICULTIES OF CLASSIFICATION 23

•>

distortion). Utility for referencing was the governing principle of
the arrangement, and in that respect it achieved its purpose.

In the preceding historical sketch of the leading systems of classi-
fication that have from time to time been in use, I have tried rather
to indicate the principles upon which each plan was founded, than
to give complicated details of the way in which it worked out. I
have passed from the chronological arrangements of the mediaeval
writers to the systems founded upon external characters, to those
based upon causes or alleged causes, to those having a regional, a
prognostic, or a medico-legal character, and t<> those in which the
course of events in antenatal life was taken into account. The pro-
gress of Embryology, the advance of Foetal Physiology, the discovery
of undescribed types of monstrosity, and further researches upon
already known types, have seldom failed to reveal the existence of
defects in these elassificational schemes, and have weakened their
utility. The observation of connecting links between different types
has been a specially fertile cause of rearrangements and reclassifi-
cation. For instance, it might appear at first sight that a line could
easily be drawn between double and single monsters, between, in
other words, united twins and monstrosities in which only one foetus
is involved. Between the Siamese Twins and an anencephalic foetus
there is all the difference possible, for the one is so obviously two
united individuals, and the other is nothing more than a single
defective foetus ; but there are double monsters which show almost
no external signs of duplicity at all. Perhaps the best instance of
such is found in the newly established type (or genus, to use Saint-
Hilaire's name) rhinodyme. P. Gilis {Jo urn. de I'anat. ct de la
physiol., xxxv. 707, 1899) has described a case of this kind in the
lamb. The body and neck were single, and at first sight the head
also appeared to be so. There were two lateral eyes ; but eacli
maxilla, especially the lower, had a notch in the median line, and on
examination of the deeper parts it was found that there were four
nasal fossae, four upper maxilla' of which the two internal ones were
much reduced in size, two mandibles whose internal rami were fused
posteriorly at the level of the angles, and two tongues fused behind
in a single body attached to a single hyoid bone. There were them
really two heads in this case, but they were not quite fused together.
A similar case in the human subject was that described by Felix
Regnault under the name of "La Femme a deux nez " (Bull, ct mem.
Soc. d'anthrop. de Paris, 5 s., ii. 333, 1901). In this woman the
eyes were wide apart, the mouth was broad, there were six upper
incisor teeth, and there were two (fused) noses of which the left had
two nares. There was an osseous plate between the two noses, which
represented two upper maxillae fused together : and there was the
rudiment of a median orbit.

Such cases as the above illustrate very well the disturbing effect
of these intermediate varieties upon classifications ; but they have at
least served the useful purpose of demonstrating that in Teratology
we have to do, not so much with a series of clearly differentiated
types, as with an almost interminable number of kinds of monstrous

234 ANTENATAL PATHOLOGY AND HYGIENE

forms related more or less intimately to each other, and shading off
into each other by almost imperceptible degrees. To attempt to
classify them by their characters is very much like trying to classify
shades of colour : we can pick out leading types and arrange all the
others according as they differ from or resemble them, but we shall
soon find that it is impossible to translate this method of grouping
into a linear series. Yet, as we have seen, this is what many tera-
tologists have tried to do. Now, the classification must never
dominate the science ; it must always be subordinate to it. It is
necessary to have some way of grouping together teratological cases,
but the cases are of far more importance than the grouping of
them.

It may be that ultimately it will be found possible to classify
anomalies and monstrosities according to their causes or according
to the time in antenatal life at which they are produced, and in this
work we are feeling our way, as it were, towards some such arrange-
ment ; but this cannot be yet achieved. The classification which I
have adopted, therefore, is founded upon the more manageable ideas
of general characters and the regional distribution of malformations.
It is, as I have said already, closely modelled upon that employed by
Tar urn" in his monumental work (Storia della tcratologia, vols, i.-viii.,
1881-94). It contains the ground principle of the division of
teratological conditions into those with excess of formation, those
with defect, and those with altered relation. By combining this way
of looking at terata with the regional arrangement of the actual
types, a fairly satisfactory working system of classification is ob-
tained. It has, of necessity, its defects ; but it has the great merit
of elasticity, and in it a place can be found for practically all forms
of malformation. If it be accepted simply as a means of convenience
in grouping together various teratological productions, and not as a
scientific setting forth of the pathological and etiological relations of
these monstrosities to each other, it will, I believe, be found to serve
its purpose well enough. It is to be remembered that it is not for a
moment intended that the larger and smaller subdivisions stand to
each other at all in the relation of orders, tribes, families, genera, and
species. It is to be thought of rather as a series of spheres of types
related in various degrees to each other, and coming into contact in
various planes, so to say.

Before I present an abbreviated sketch of the scheme of classifi-
cation which is to be employed, it may be well for me to deal very
shortly with teratological nomenclature. It is unquestionable that
teratological terms are uncouth and unfamiliar to the eye and ear
of the English-speaking medical man. It is true that some of them
are not only polysyllabic to an almost alarming degree, but are also
etymological riddles, to be read only by a good Greek scholar. It
would be easier for us to give the various monstrosities English
names ; thus, we might speak of a double-headed twin monster, or
of a brainless fcetus, or of a one-eyed monstrosity. Such designa-
tions would be more easily understood by us than such names as
dicephalus, ancncephalus, monophthalmus, or cyclocephalus ; but to

CLASSIFICATION 235

scientists of other nationalities their meaning might not be plain.
So it has come about that, for the sake of international convenience,
Greek words have been made use of to found a teratological nomen-
clature. Scientists of all nations have, or ought to have, sufficient
familiarity with Greek to be able to recognise the meaning of words
built up upon Greek roots, and thus at the expense of a little trouble
to each nation a general international advantage is gained. To the
Englishman, "two-headed" may be more intelligible than "dicephalus,"
but it is not so to the Frenchman ; while to educated Englishmen
and Frenchmen alike, " dicephalus " conveys a very distinct and
evident meaning. So it has come about that names derived from
the Greek have been given to all the known types of monstrosity.
To the beginner the nomenclature is difficult and confusing ; but,
after all, there are other departments of Science, and even of Medicine,
that have a terminology every whit as difficult as that of Teratology :
they are, however, more immediately practical or more generally
studied, and their most polysyllabic terms have in consequence
become more familiar to the eye and ear. At the same time it
will be for the good of their science if teratologists will in the
future refrain from the unnecessary coining of lengthy words to
express slight differences in structure. In this work I shall use,
as far as possible, terms derived from Greek roots.

Let me now give an outline of the plan of classification (a modifi-
cation of TarufH's) which I have adopted.

Terata (Monstrosities and Anomalies).

A. Monosomatous (a single individual involved).

I. Pantosomatous (the anomaly affects the whole or nearly the
whole body).

(a) Dwarfism (microsomia).

(b) Giantism (macrosomia).

(c) Hemi-hypertrophy and Hemi-atrophy.

II. Merosomatous (the anomaly affects only a part of the body).

(a) Partial Macrosomia and Microsomia of various Parts.

(b) Spinal Column and Contents, Anomalies of.

(c) Cranium and Contents, Anomalies of.
((f) Face and Sense Organs, Anomalies of.

(e) ISeck and Constituent Parts, Anomalies of.
(/) Thorax and Contents, Anomalies of.
(g) Abdomen and Pelvis and Contents, Anomalies of.
(//■) Limbs, Anomalies of.

(i) Annexa (Placenta, Cord, Amnion), Anomalies of.
III. Heterotaxic (the anomaly affects only the arrangement of the
parts of the body).

B. Polysomatous (two (or more) individuals involved).

I. Twins, entirely separate, but in a single chorion (monochorionic).
II. Twins, united only by the vessels of their umbilical cords
(omphalo- or allantoido-angiopagous).

(a) Paracephalic.

(b) Acephalic.

(c) Amorphous.

236 ANTENATAL PATHOLOGY AND HYGIENE

III. Twins, united more or less completely (double monsters).

(a) Symmetrically united —

1. Syncephalic.

2. I )icephalic.

3. Thoracopagous.

(b) Asymmetrically united —

1. Ceplialo-parasitic.

2. Prosnpo-parasitic.

3. Trachelo-parasitic.

4. Thoraco-parasitic.

5. Gastro-parasitic.

6. Lecano-parasitic.

7. Melomelic.

IV. Triplets, Quadruplets, Quintuplets, etc.

(a) Entirely separate.

(b) United.

1. By the vessels of the umbilical cords.

2. Directly and more or less completely.

This scheme of classification requires little explanation. In it
all teratological phenomena are divided into two great divisions;
one, that of the monosomatous terata, in which only a single in-
dividual is affected ; the other, that of the polysomatous terata, in
which two individuals (or, at least, something more than one) are
involved. Here, at once, it must be admitted that it is very
difficult sometimes to allocate exactly every specimen to its proper
place in one or other of these two divisions. The difficulty does
not arise in respect to well-marked types, such as anencephaly or
cyclopia and the Siamese Twins, but with regard to the inter-
mediate types or connecting links, such as Polydactyly or the
diphallic monstrosity. An individual possessing no other signs of
excessive formation than an extra digit or two penes instead of
one does not at first sight show much resemblance to such a pheno-
menon as the Siamese Twins ; but the reason is that they are on
opposite sides of the sphere of types which has " excessive formation "
as its one character. In the same fashion low types of animal life
show little resemblance to the mammalia, and are at first sight more
like plants; but they may be animals all the same. Of course,
there are spurious signs of excessive development. A good instance
of this is got in the so-called " double " uterus, which is, as every student
of Embryology knows, simply an ununited or imperfectly united organ.
These cases fall not under the polysomatous but under the monoso-
matous terata. The great majority, however, of cases showing excess
of formation are grouped with the polysomatous terata, even although
they exhibit somewhat scanty indications of their double nature.

The division of the monosomatous terata corresponds roughly with
the two groups of "defects of development " and "altered relation-
ship of parts." It includes the arrested developments and nearly all
the conditions which have been called hemiterata or anomalies in
the narrow sense of the word. The types are very numerous and
have been subdivided according as the whole body is affected

MEROSOMATOUS TERATA 237

(pantosoniatous) or only a part of it (merosomatous) ; and I have
made a third division to hold the heterotaxies, the states in which
the viscera are more or less accurately transposed. The only
difficulty met with in this group arises with regard to the giants
and hemihypertrophies. These are indeed " excessive," but the
excess is apparently one of growth and not of development ; and so
they fall among the single monstrosities and not among the double.
The subdivision of the merosomatous terata is the largest of all,
and has had to be split up according to the region affected. A slight
difficulty arises with the cases in . which two or more parts of the
body are simultaneously malformed, " connecting links " again play-
ing havoc with the best laid plans of the taxonomist ; but the
difficulty may be surmounted by classifying the case according to
its major malformation, and calling the minor ones complications,
e.g. anencephaly, with its complications spina bifida and defects of
the sympathetic nervous system. In turn, the spinal column,
cranium, face, neck, thorax, abdomen, pelvis, and limbs are dealt
with according as the anomaly affects one or other of them ; and
I have made a special group to contain the malformations of the
foetal annexa, including the placenta and funis, for they are as truly
foetal organs as are the liver and spleen, and their malformations
have received little attention and deserve more. I have made no
special subdivision for the hermaphrodites, but have placed them
along with other malformations of the genital organs under the
region of the pelvis. It is true that they have a peculiar importance
on account of social difficulties to which they may give rise, and
of the medico-legal questions which may emerge from their ano-
malous position ; but to the antenatal pathologist they are simply
cases of malformation of the genital organs. Most of them belong-
to the monosomatous terata, but it is possible that a few ought to
be grouped with the polysomatous.

The polysomatous terata are subdivided into four groups. The
first contains the monochorionic or uniovular twins. It is possible
that here also ought to be placed the dichorionic or binovular twins,
for twinning is an anomaly in the human subject : but the point is
not pressed. It is on account of their twin-ship that monochorionic
twins are included in this group ; they need not necessarily exhibit
any individual malformations. It is, however, a remarkable fact that
twins often are malformed, one or both ; and it is still more significant
that they may exhibit identical malformations. In the second group
of the polysomatous terata are the allantoido-angiopagous twins or
placental parasites, those curious cases in which a very rudimentary
foetus is found existing in utero in a parasitic fashion, being attached
to his co-twin by the vessels of his umbilical cord vid the placental
circulation. They are placed among the polysomatous terata because
they are monochorionic twins in the first place, and on account of
their malformed state in the second place ; for it must be remembered
that one of the twins may show no malformations at all, although
there is reason to believe that from the standpoint of antenatal
physiology he is seldom quite normal.

238 ANTENATAL PATHOLOGY AND HYGIENE

The third group of the polysomatous terata contains the double
monsters or united twins commonly so-called. In them the union is
between the two foetuses and not by means of their annexa
(membranes, cords, and funic vessels) ; and it contains such well-
known types as the Siamese Twins, the Hungarian Sisters, the
Scottish Brothers, and the like. It also includes the cases in which
a rudimentary foetus is inserted on to the other foetus in a parasitic
manner, as in the famous Laloo and in the Chinaman, Ake.

The fourth group of the polysomatous terata contains the plural
pregnancies, in which more than twins are present in the uterus.
With regard to triplets, quadruplets, quintuplets, and sextuplets
(genuine cases of which in the human subject are very rare), there
can be no doubt that their place is among teratological formations.
Whether they show individual malformations or not, the very
presence of three foetuses in the human uterus at the same time is
teratological. As a matter of fact, however, triplets, etc., are very
often malformed. They may consist of two fairly normal foetuses,
and of a parasitic one attached to the placenta of one of the normal
ones ; in this case they are similar to the allantoido-angiopagous
twins. In other, but exceedingly rare, cases three foetuses may all
be fused together leading to the production of a triple monster or
united triplets.

Such is the scheme of classification which is used in this work.
Its most serious defect is its overlapping, but this is unavoidable. It
must also be remembered that on account of our defective knowledge
of all the facts of our science, no perfect scheme of arrangement is at
present possible ; this classification, then, is not a scientific setting
forth of the exact pathological and etiological relations of mon-
strosities to each other, but simply a convenient method of grouping
them. We must not expect to introduce into our subject a mechani-
cal " precision which has no counterpart in nature."

There is an interesting speculation connected with the classifying
of monstrosities to which I have not yet referred ; I mean the ques-
tion whether we can separate the teratological conditions which arise
in the embryonic period of antenatal life from those which are
initiated in the germinal epoch. The reader who has perused the
chapters dealing with Teratogenesis will be prepared for the state-
ment that I think we can so separate teratological productions. I
am of opinion that most of the monosomatous terata arise during the
embryonic period, while the double terata are probably due to causes
acting upon the organism in the germinal epoch before the first
rudiments of the embryo can be recognised in the embryonic area.
For this reason I shall describe the types of polysomatous terata
under the Pathology of the Germ, along with deformities of the
blastoderm and hydatid moles. I have found great difficulty in
arriving at any conclusion regarding the position of congenital
tumours : some of them, such as cysts of the neck and of the sacral
and perineal regions, doubtless are formed during the embryonic
epoch, and possibly also to some extent in the foetal ; but others, I
think, must be regarded as germinal in origin. I shall, therefore,

TERATOLOGICAL TYPES 239

describe some of them with the types of embryonic pathology and
others with those of germinal pathology. Teratology, then, includes
more than the Pathology of the Embryo ; it contains part also of the
Pathology of the Germ. The Pathology of the Embryo and the
Germ, on the other hand, includes more than Teratology; it contains
morbid states which are not usually named teratological. I freely
admit that there is much that is uncertain about this arrangement;
but, as I have already said, we are only as yet feeling after some
such grouping of anomalies according to the periods of antenatal life
in which they originate.

I have now reached the end of what may be called the General
Principles of the Pathology of the Embryo, and can, therefore,
proceed to describe some of the leading types of the results of the
action of morbid causes upon the new organism in the embryonic
epoch. There is to be a long procession of such types, and it will
be my purpose and my endeavour to give to the reader a clear,
concise, and intelligible descriptive account of them as they pass by.

CHAPTER XIV

Teratological Types : The Types and their Bibliographies : Microsomia or
Dwarfism ; Growth Processes ; Embryonic, Fcetal, and Postnatal Micro-
somia ; Generalisations ; Literature : Microsomia or Giantism ; Embryonic,
Fcetal, and Postnatal ; Literature : Unilateral Microsomia or Hemi-
hypertrophy : Unilateral Microsomia or Hemi-atrophy.

The teratological types form a long series, a very long series, as the
reader will immediately perceive, if he have not already done so.
We are now in a position to begin, with some profit, our study of
these types. It may be that the reader has, long ere this, begun
somewhat impatiently to look for some type-descriptions, and to
marvel at their non-appearance. " So much time has been spent
over general principles," he has been saying to himself with the
suggestion of a grievance in his tones, " that there is little left for
the actual description of the malformations and monstrosities them-
selves." And this is quite true if the present work is a catalogue of
monstrosities ; but, then, the writer ventures to hope that in some
measure it is more than a teratological descriptive list. It is indeed
put forth as an attempt to discover how there come to be monstrous
formations at all, what the causes of teratological phenomena are, and
how the prevention of such things is to be brought about. To arrive
at conclusions on those matters, general principles, if we can get at
them, are more likely to help us than are descriptions of individual
types. No one surely wishes to retain the teratological types as a
permanent possession of scientific medicine ; there might well be
rejoicing if there were any prospect of their being seen no more on
this planet ; like smallpox, they might happily be forgotten, were
there but a similarly effective means of getting rid of them. We are
not, of course, within measurable distance of any such consumma-
tion ; but it is well to remember that the wished-for goal of this, as
of all departments of the healing art, is prevention. We can at any
rate look towards it. Meanwhile let us to the description of the
teratological types.

It is obvious that there are types : the hasty survey of any collec-
tion of monstrosities suffices to make this plain. Further, some
types are relatively commoner than others ; and some are more
clearly marked off from the rest than are others. Of the commoner
and more clearly marked types I trust I may be able to paint fairly
complete word-pictures ; the rarer and less sharply delimited varieties
of monstrosity will be indicated only by a few strokes, as it were, in

240

TYPES 241

outline, in black upon white. The former we may regard as full-
length portraits, the latter as little more than vignettes.

The word " type " is used here in its simplest and most obvious
sense ; by it is meant only a " sort " or " kind " of monstrosity.
Thus employed, it has neither ulterior significance nor specific mean-
ing. It is no more than a conveniently short term to express an
outstanding and fairly well delimited variety of monstrosity. The
types, further, are taken up in the order of the system of classifica-
tion sketched in the preceding chapter ; but it is not suggested that
they stand in any peculiar or vital relationship to the other members
of the series in which they are placed. In other words, I do not give
to the term " type " its common biological signification, for, as has
been already explained, I do not regard Teratology as a " kingdom "
in any sense resembling that of the plants or the animals.

Around each teratological type has grown up a bibliography,
sometimes of considerable dimensions, and occasionally of enormous
size. What to do with these lists of literature has been the subject
of much thought. I have, in the past, put a high value upon such
things, and there can be no doubt that they constitute the framework
of the subject with which one is dealing; but it is possible to be
overburdened with bibliographies, and for the general reader they are
of minor importance so long as the facts have been extracted out of
them and are presented to him in attractive form and with reasonable
fulness. I shall, therefore, generally place them at the end of the
descriptions; the individual references will be arranged neither
alphabetically nor chronologically, but simply in the order in which
they are alluded to in the text. The serious student, who may wish
to investigate fully any special type, will find useful literature lists
in Taruffi's compendious work (titoria delta teratologic*,) and in the
Index Catalogue of the Washington Library.

The types selected for description in this chapter are not, so to
say, typical types ; they have been chosen because they are not
typical. Let it be borne in mind that the pathology of the embryo
deals with malformations, with disturbances of ontogenesis and
organogenesis ; in this respect it differs from the pathology of the
fuetus, which has to do with diseases, with disturbances in the per-
formance of the functions of formed organs. It follows, therefore,
that the types of embryonic pathology are monstrosities, while those
of foetal pathology are antenatal diseases. Now, the types which
are to be described in the following pages are neither malformations
nor diseases, although they are doubtless related to both : they are
disturbances of growth, and growth is a phenomenon common to
embryonic and to foetal life. Indeed, growth is common to the early
part of postnatal existence as well as to the whole of antenatal life.
Disturbances of growth, therefore, are not typical of embryonic
pathology ; they are not even typical of antenatal as contrasted with
postnatal pathology. Dwarfism, giantism, and hemi-hypertrophy are,
in consequence, quite non-typical of embryonic pathology; but they
are of great value on this account, for they serve as connecting links
between the pathology of the foetus and that of the embryo, and even
VOL. II. — 16

242 ANTENATAL PATHOLOGY AND HYGIENE

between the pathology of the child and that of the unborn infant.
The}' are placed here because they bridge over the gap between
Section I. of this Manual and the present part ; they give the
reader a general idea of the resemblances which exist between the
pathological processes of the antenatal and postnatal parts of life.
He will very soon be dealing with the dissimilarities, and it will be
necessary for him to remember that there are some similarities after
all, although they may be few in number. So, because they are not
typical of embryonic pathology, the anomalies of growth are taken
here and made to serve as an introduction to the types which are in
every sense typical of embryonic pathology.

Microsomia (Dwarfism).

Microsomia is a better name than dwarfism for the teratological
type to be described. The word " dwarf " is very old, and in its
progress down the centuries it has gradually taken on various mean-
ings, and in none of these is it exactly synonymous with microsomia.
To most people, " dwarf " means a diminutive adult human being,
bringing with it memories of General Tom Thumb, the Midgets,
and one of Sir Walter Scott's characters. To a few persons, well
read in Spenser's Faerie Qaecnc and mediaeval romances, it signifies
an attendant upon a knight or a lady ; and to some others, versed in
mythological lore, it suggests the race of metal-working and (too
often also) mischief-working elves. Even in its restricted meaning
of a diminutive adult, it includes cases of stunted stature due to
postnatal diseases of the spine and of the lower limbs ; and it has
been applied to the results of antenatal maladies, such as achondro-
plasia. Microsomia is at once something less and something more
than all these. It is more nearly equivalent to what Sir George
Humphry (1) has called " true dwarfism," although it differs slightly
even from that : a true dwarf is a person of unusually small
stature, not as the result of any particular disease or deformity, but
merely from growth having fallen much short of the usual standard.
Microsomia I define as a " monstrous smallness " of all the parts of
the individual, whether that individual be an embryo, a foetus, a child,
or an adult; for the age reached, the size is much below the normal,
growth having been arrested. This definition introduces the possi-
bility of the smallness being corrected or undone in a later period of
existence, a modification which I regard as of some importance in
relation to antenatal microsomia.

The following facts about growth in antenatal and in postnatal
life are worth remembering in connection with microsomia. Growth
is going on during the whole of antenatal life, with great rapidity in
the early part of it, less quickly towards the close ; but it does not
cease at birth as everyone knows, for the infant continues to increase
in size until he is a child, and the child goes on growing till the
eighteenth or nineteenth year. It may be concluded, therefore, that
growth may be arrested either before or after birth, at any age be-
tween minus nine months and plus nineteen years. Consequently,

MICROSOMIA 243

microsomia may be antenatal or postnatal. It follows, further, that
it may be arrested either in embryonic or in foetal antenatal life, and
that, therefore, there may be embryonic and fcetal microsomia.
Facts in support of these statements will be brought forward imme-
diately ; in tbe meantime, let them be taken on trust, in order that I
may lead the reader on to some other conclusions. Growth is not
the only process going on at these different epochs of life. In em-
bryonic existence growth is accompanied by, nay, is overshadowed by
development or ontogenesis ; increase in size is made almost insignifi-
cant by increase in complexity ; accrescence is hidden by organo-
genesis. Growth asserts itself in foetal life, for the processes of
ontogenesis have slackened ; but there is still some development going
on, and so the two vital phenomena run on side by side. But further,
in foetal life the formed organs have begun to perform functions, and
so a third manifestation of life is added to the other two. There is
growth and development and the performance of organic functions ;
possibly the order of arrangement represents the order of their
importance at this period of antenatal life. After birth, both growth
and development slacken very perceptibly, indeed ontogenesis almost
disappears and is represented only by some changes in the brain and
genital organs, and by the process of dentition. On the other hand,
the physiological activities of the various organs greatly increase, and
gradually a fourth series of vital processes, the mental, are super-
added. Now the question arises — and it is an important one — when
microsomia comes on at any of the periods above mentioned, will it
be accompanied by an arrest of the other vital processes (onto-
genesis, physiological activities of body and mind) which are peculiar
to that period, or will it not ? There is reason to believe that the
arrest sometimes affects growth alone, and sometimes includes also
the other activities of the individual. In the former case it must be
assumed that the cause of the arrest, whatever it may be, has a
discriminating power which enables it to pick out and stop the
phenomena of growth alone ; in the latter case it may be supposed
that its action is a more general one, such as produces instances of
dwarfism combined with malformations or with diseases or with
mental obtuseness. There is also some evidence that one form of
vital activity may be arrested while another is accelerated, although
this would appear to be rare. Let us now turn to the recorded facts,
and see how they accord with the statements which have just been
made. Let us consider, in order, microsomia as it affects the embryo,
the foetus, and the child.

Embryonic Microsomia.

Sometimes when we are engaged in examining an abortion sac
we find to our surprise that it contains an embryo of a size much
smaller than the size of the sac had led us to expect. For instance, an
abortion sac the size of a hen's egg may disclose an embryo only
2 or 4 mm. in length. This is what I understand by embry-
onic microsomia, and the embryo I call a " dwarf embryo." I have

244

ANTENATAL PATHOLOGY AND HYGIENE

examined several specimens of this kind. One (2) was from the
ninth pregnancy of a woman, thirty-six years of age, who had on two
previous occasions miscarried. Three months after the cessation of

Fig. 38. — Abortion Sac, 13 cms. in circumference, containing dwarf embryo (E),
2 mm. in length. Specimen No. 42.

menstruation she expelled an intact abortion sac measuring 7 cms.
in length and 13 cms. in its minor circumference. It had the shape
and size of a large hen's egg, and there was a small haemorrhage in
the decidual membranes near the narrow end of the ovoid. I ex-

EMBRYONIC MICROSOMIA 245

pected to find in it an embryo of about the length of 6 cms. ; but the
contents consisted of nothing more than a tablespoonful of somewhat
muddy liquor amnii, and an embryo measuring little more than
2 mm. in length lying close to the sac-wall (Fig. 38). In another
specimen (3), the sac measured 7 cms. by 5 cms., and contained 6 drms.
of clear liquor amnii, and an embryo 4 mm. in length, with an
umbilical vesicle (45 nun. in length) attached to it. In this case
also there was a haemorrhage in the decidual membranes, but at the
broad end of the ovoid. The embryo showed no trace of limbs,
while in the first case they were present but deformed. In yet
another specimen which I have had an opportunity of examining
(unpublished), the disproportion between the size of the embryo and
the containing sac was not so great, but was still quite recognisable.
In the second and third cases there had been two months amenorrhoea.
Specimens not unlike the above have been described by other ob-
servers, as by Isbrand de Diemerbroeck (4), L. Ciniselli (5), and G.
Colombo (6); but the most complete reports have come from His (7),
C. Giacomini (8), and F. P. Mall (9).

I look upon these cases as instances of dwarfism of the embryo ;
but I need hardly add that they are not so regarded by all patho-
logists. According to some writers they are instances of early
hydramnios : thus Keppie Paterson (10), not long ago, described a case
of " hydramnios of the ovum," in which the amniotic sac was as large
as at the third month of pregnancy, while the embryo was of the size
of the third week. I had an opportunity of examining this specimen,
and noted that the embryo had a development considerably in advance
of its size ; I, therefore, was inclined to regard it rather as a case of
dwarfism of the embryo than of hydramnios. Doubtless there was
an excess of liquor amnii, but the more important phenomenon was
the embryonic microsomia. Other writers, again, have explained
these abortion sacs as examples of early embryonic death with con-
tinued growth of the chorionic vesicle. I admit that it is quite
possible that the chorion and decidual membranes may go on growing
after embryonic death ; for they have a large amount of individual
vitality, so much that they have even been regarded as constituting a
separate larval stage in human ontogenesis (J. Beard, 11). I do not,
however, think that there is clear evidence of the death of the embryo
in these cases; certainly in my specimens, which I examined by
means of serial sections, there was no signs of anything more than
recent death ; the changes seen in them truly furnished me with a
riddle in morbid ontogenesis which I have not yet succeeded in
reading, but they did not appear to be of a post-mortem nature. At
the same time, I freely admit that we know little about post-mortem
changes in the embryo ; thus, it is not, in my opinion, proven that the
dead embryo can entirely disappear by dissolution in the liquor
amnii. But, further, there is evidence of a positive kind in support
of the view that these small embryos are instances of microsomia.
Fere (12), in the course of his experimental work, almost constantly
found dwarf chicks among the deformed ones ; and Panum (op. cit.,
1860) and Dareste (op. cit., 1891) also noted that teratogenic agencies

246 ANTENATAL PATHOLOGY AND HYGIENE

produced an imperfect and unequal development of the area vasculosa
with "atrophic" and dropsical embryos. Gerlach and Koch (13), by
partially varnishing eggs in incubation, succeeded in getting partly
dwarfed chicks, a result ascribed by them to diminished penetration
of the shell by oxygen. Henry de Varigny's experiments (14) on the
artificial production of dwarfism in fresh water snails (Lymnceus
stagnalis et auricularis) have been quoted by some writers in con-
nection with human microsomia, but the conditions of life are too
dissimilar to permit of the drawing of any deductions therefrom ; the
same objection applies to the experiments of Driesch, Wilson, Zoja,
Morgan, 0. Schultze, and 0. Hertwig (15), who worked with the eggs
of sea urchins, medusae, ctenophores, frogs, and of amphioxus, and
who were able by destroying half the ovum (e.g. one blastomere in
the two-blastomere stage) to get not half an embryo (as some other
observers got) but an embryo half as large as usual.

There is, it seems to me, sufficient evidence, both clinical and
experimental, to warrant a belief in the occurrence of microsomia or
dwarfism of the embryo ; but it does not, of course, follow that the
causes of the arrested growth which have been demonstrated or
suspected in the case of the chick will be found in action in the human
subject. I am inclined to attach great importance to haemorrhage
into the decidual membranes as a determining cause of the dwarfism
in the specimens seen by me ; but other causes, predisposing and
determining, may have a value. There are several other questions
concerning embryonic microsomia to which no answer can as yet be
given : thus, there is no information to be had as to the fate of a
dwarf embryo when the pregnancy does not result in abortion. Does
it become a dwarf foetus? Is, on the other hand, the embryonic
dwarfism replaced by accelerated growth in the fu?tal period ? We
can say nothing with any definiteness, but analogy, as we shall see,
suggests that the second hypothesis is the more likely one. There
is another question which may be asked, and to which a sort of half
answer may perhaps be given — Is embryonic microsomia commonly
associated with embryonic malformations and monstrosities ? As
has been explained already, growth in embryonic life is concurrent
with development (ontogenesis), and it is possible, therefore, that the
cause that arrests growth and produces microsomia may also arrest
ontogenesis and produce malformations. There is evidence that this
sometimes happens : in one of my specimens the embryo was de-
formed as well as dwarfed, and one of Mall's cases showed club-hands
and feet and a defective ear. There is also evidence that growth
alone may be interfered with, the constructive processes going on
normally. We do not know why it is that the arresting cause in
some cases expends its force upon growth, and in other instances acts
upon the constructive or architectonic part of embryonic existence ;
but apparently it is so. We shall see, further, that this character
is not peculiar to embryonic microsomia. There is another question
still that may be asked — May an accelerated rate of development in
the embryo lead to a slackened rate of growth I The only hint of
an answer that we can get to this question comes from Dareste's

FCETAL MICROSOMIA 247

experimental work (16) ; lie found that by over-heating eggs in in-
cubation he was able to expedite development and to retard growth,
and so get dwarf chicks.

In this, as in so many other parts of Antenatal Pathology, the
gucesita arc many and the data sadly few. The careful observer who
wishes to make available new data will do well to examine carefully
all abortion sacs.

Fcetal Microsomia.

Since foetal life lasts for seven months it is theoretically possible
that at any time in this period growth may be arrested and micro-
somia result ; but, as a matter of fact, little is known of the earlier
arrests, and we can only speak with certainty of the dwarfism which
is recognised at birth at the full term. Obviously, a new-born infant
may be small and yet not a dwarf ; it may be prematurely born and
small on that account. Of course it may be both dwarfed and pre-
maturely born, but it will always be difficult to do more than suspect
such a combination. The case easiest of recognition is that in which
an infant is born at the full term of gestation (carefully calculated)
with all the signs of maturity save size and weight ; such an infant
may be regarded as an instance of fcetal microsomia, it is a dwarf
foetus. If the dwarfism is accompanied by malformations, as there is
reason to believe it often is, it becomes next to impossible to be
certain regarding it, and the case will in all probability be classed by
most observers as one of monstrosity and not as one of microsomia.
To give some degree of definiteness to a somewhat indefinite subject,
let us say that a foetus born at the full term of pregnancy, weighing
less than 3 lb. and measuring less than 12 inches in length,
but possessing the other signs of maturity (finger-nails, absence
of lanugo, etc.), is a case of foetal microsomia.

Possible instances of microsomia occurring early in foetal life are
recorded by Montgomery (17), who described a foetus of the size of two
months in association with a placenta (diseased) of the size of three
months, and by Cruveilhier (18), who discovered a foetus of the size of
two and a half months in a plural pregnancy of six months. In
relation to Cruveilhier's case, it may be remarked that the presence
of a co-twin in utero often has a dwarfing effect upon the other foetus
(in the human subject). Illustrative instances of full-time or nearly
full-time foetuses were these reported by Sir Everard Home (19),
T. E. Baker (20), G. A. Mursick (21), and S. J. Scott (22). Of one
of these we are fortunate in possessing the after-history ; it deserves
a paragraph to itself.

It is the case reported by Sir Everard Home some eighty years
ago, and recently brought to light again by Hastings Gilford (23).
The dwarf foetus was the offspring of an Italian woman, who travelled
with the Duke of Wellington's army, and who was so terrified by a
monkey when three months pregnant that she was thrown into con-
vulsions ; she did not miscarry but went to the full time, and gave
birth to a female child weighing 1 lb. and measuring 7 inches. This

248 ANTENATAL PATHOLOGY AND HYGIENE

child grew up (to speak somewhat paradoxically), and was known
as Caroline Crachami, " the Sicilian dwarf " ; she was exhibited in
London, and died at the age of nine years. She measured nearly
20 inches in height, and her ossification, dentition, and mental powers
were those of a child of two rather than of nine years. In this case,
therefore, there was both arrested growth and arrested development ;
and to the syndrome thus produced it has been proposed (23) to give
the name " ateleiosis " (Gr. anXr,?, unripe, imperfect), but it is doubtful
whether any benefit flows from the introduction of the new term. Foetal
microsomia may or may not be accompanied by arrested development.

Several other well-known dwarfs seem to have been, in their
origin, instances of foetal microsomia. Nicholas Ferry, better known
as Bebe (24), was born in 1741, when he weighed less than a pound, and
had a length of 8 inches ; at five years he weighed 9 lb. 7 oz.,and was
about 22 inches high; and he died when twenty-two years old
(height then being 33 inches). He lived in the times when dwarfs
formed part of the fashionable equipment of European Courts, and
Bebe was devoted in a somewhat doglike way to his master Stanislaus,
ex -King of Poland. He was apparently almost microcephalic, if we
may judge from the details which Saint-Hilaire (24) gave about him.
Another congenital dwarf was Antonio Toselli, who was described by
L. Trevisani (25) ; he was born in 1808, and his mother, whose eighth
child he was and was forty-six years of age at the time of his birth,
had had an accident in the early months of her pregnancy. Antonio
was very small at birth (full-time), and when twenty years old was
only 40 inches in height. He had rather a large head. The " Princess
Paulina" (26) was only 12 inches in length when born; at her death,
aged nineteen, she measured 24 inches. The " Marquis Wolga " (27),
born in Hungary in 1857, measured 9 inches at birth and weighed
not quite 2 lb. ; he married the liliputian " Marquise Louise." " Mary,"
known as the " Living Doll," was born in Hull in 1895, when she
weighed not quite 1 lb. and measured 7 inches in length ; at the age
of four and a half years she was 17 inches high and weighed about
5 lb. (28). Other dwarfs who appear to have been dwarfs at birth were
described by Dupuytren (29), Virey (30), P. Dubois (31), and by E.
Magitot (32). Dupuytren's dwarf had the measurements of a five
months' foetus, although he was born at the full term ; Virey 's case,
Babet Schreier or the " German Liliputian," was also the product of
a full term pregnancy, although she only weighed 1^ lb. at birth and
measured 6 inches in length ; Dubois' dwarfess was the daughter of
a dwarf father and a normal-sized mother, she had two dwarf sisters,
and she herself gave birth to a premature foetus weighing 3 lb. 12 oz. ;
and Magitot's dwarf, born in 1867, was very small when born, although
his mother had arrived at the full term of pregnancy.

The causes of foetal microsomia are as little known as those of
embryonic dwarfism, and the reader will cease to wonder thereat if
he will peruse what is written on p. 168 of the first section of this
Manual on the subject of the variations in the normal weight of the
foetus. He will also be prepared, from his studies of teratogenesis
(Chapters VIII. to XII.), to find the maternal impression alleged as

POSTNATAL, MICROSOMIA 249

a cause and maternal traumatism in pregnancy hinted at as a factor
in the production of dwarfism. Doubtless the problem in its ulti-
mate aspect is to be regarded as one of nutrition ; doubtless, also, the
state of the placenta has something to do with bringing nutritional
defects into action ; but beyond these general statements it is rash
to go. The occasional occurrence of several dwarfs in one family,
and the still more occasional occurrence of direct heredity in micro-
somia, make it evident that the arrestment of growth has been
determined in the germinal period of life. Some further notes on
etiology will be added after the description of postnatal microsomia.

Postnatal Microsomia.

All dwarfs are by no means born so ; the retardation in growth
may begin at one, two, three, or four years of age ; indeed it may not
commence until early adolescence. With these cases of postnatal
dwarfism, antenatal pathology, properly speaking, has no concern,
except in so far as they may be predisposed to by antenatal influences
(heredity, postponed effect of foetal and embryonic states) ; but a few
paragraphs may be given to them, chiefly to show that the same laws
apply to them as to embryonic and foetal microsomia.

There is no evidence that Jeffery Hudson (33) was a congenital
dwarf. He was born at Oakham, in Eutland, in 1619, and was
scarcely 18 inches high at the age of nine years. His parents were
of average height. He had some chequered experiences in a
peculiarly adventurous life: he was presented "in a pie" to King
Charles I. ; he fought a successful duel, for " although a dwarf Jeffery
was no dastard " ; and he was sold as a slave in Barbary. This last
and most trying adventure has a special interest for us, for it caused,
or at least preceded, the recommencement of growth at the age of
thirty years.

Joseph Boruwlaski (34) was born in Polish Galicia in 1739, and
died in Durham in 1837 at the great age of ninety-eight. Shortness
of stature, let it be noted, is not incompatible with length of days.
He was never more than 39 inches in height. He had two brothers
older than himself : one measured 41 inches and the other 6 feet
4 inches ! Dwarfism may run in families, as we have said ; true, but
it sometimes runs alongside of giantism, as we now note. Boruwlaski
also had a sister who measured only 2 feet 4 inches at her death,
which took place when she was twenty years old. He was a great
traveller, a good linguist, and wrote his own memoirs in an interest-
ing fashion. He had several children of ordinary size.

Postnatal microsomia, then, is not always preceded by foetal
microsomia ; nay, it sometimes develops in an individual who has
been unusually large at birth. The famous " General Tom Thumb,"
for instance, was above the average weight of the new-born ; the
dwarf exhibited in London in 1848 (35) weighed 9 lb. at birth;
John Thomson's dwarfed girl (36), whose thymus was found to be
hypertrophied at the time of her death (age then four years and
eight months), was a " big baby " ; and F. Bohrer's case (37) was of

250 ANTENATAL PATHOLOGY AND HYGIENE

at least normal dimensions when born. Further, postnatal micro-
somia may in some cases be temporary and give way to growth ; thus,
as has been already noted, the " strenuous " Jeffery Hudson began
suddenly to increase in height at the age of thirty years, and, in
more recent times, the dwarfess Magri (described by Taruffi, 38)
grew after her nineteenth year. In this clinical fact lies the germ of
the treatment of dwarfism, if it could be discovered what it is that
retards growth.

Many causes have been alleged for postnatal dwarfism : among
these may be mentioned poor or bad food, the infectious fevers,
injuries (especially those affecting the head), chronic hydrocephalus,
foetal rickets (45), and idiocy. Of course several of these alleged
causes might with equal probability be regarded as concomitant
results of an as yet undiscovered etiological factor. Alcoholism of
the parents has also been blamed : Langdon Down (39) found that
three children begotten by a man during a period of excessive in-
dulgence in alcohol were dwarfed, while those borne by his wife
before and after the alcoholic period were normal. If we admit an
antenatal cause in these cases of microsomia which do not develop
till childhood, we must suppose that it remains latent or is held in
check during the early months of life. The occasional occurrence of
family prevalence in dwarfism would seem to make it necessary to
admit not only an antenatal but even a germinal causal factor. This
phenomenon of family prevalence was noted, it will lie remembered,
in the case of Boruwlaski ; it was present also in the observati< >n
made by Clauder (40), in which each alternate child in a family of
eight was a dwarf; less striking instances were those reported by
C. Taruffi (41), Schaaffhausen (42), and G. 0. Jacobsen (43). There
was also direct heredity in P. Dubois' case (loc. cit.). Consanguinity and
advanced age of the parents, if they could be substantiated as causes,
would likewise favour the idea of a germinal etiological inllueiice.

With postnatal, as with embryonic and foetal microsomia, anom-
alies in development (malformations) may be found associated, but
they are likely to be of a less grave type and they affect the structures
developed in postnatal life alone. Thus, dentition may be incomplete
or retarded ; the general ossification of the skeleton may be tardy ; the
skin may be hairless and the fingers almost nailless (44) ; the repro-
ductive organs may be defective, as shown by sterility and want of
sexual instinct in both sexes, by cryptorchidy in the male, and by
absence of pubic hair and delayed menstruation or oligonienorrhoea
in the female; and the mental powers may be of a low order,
pointing to incomplete development of the higher nerve centres. Of
course, exceptions to all these statements can be discovered: some
dwarfs, for instance, have been perfectly formed ; some have been
able to have children, generally of normal size; some have lived to a
good age; and some have had fair intellectual powers. At the same
time, I think it may be said that a general impression is left upon
the mind that the dwarf is in many ways imperfectly fitted to fight
the battle of life. Dwarfism and infantilism and idiocy have more
than an occasional and accidental association (46).

MICROSOMIA 251

Generalisations.

The reader will now realise that I was right when 1 described
microsomia as a somewhat non-typical type, so to say, of embryonic
pathology. Microsomia, as has been fully proved, may attack the
organism in the i'cetal and postnatal as well as in the embryonic
period of life; it is not, consequently, peculiar to anyone of these
epochs. In each of them it may be accompanied by arrests of the
other vital activities of the period, and these arrests show themselves
in malformations and in the defective performance of such functions
as those of reproduction and cerebration. It may also, apparently,
be unaccompanied by these anomalies, growth alone being affected.
There is some reason for thinking that the microsomia of one of these
periods may be prolonged into the next ; for instance, an embryonic
dwarf may grow into a dwarf foetus, and it, in its turn, may be
continued into postnatal microsomia. There is also, however, some
evidence to support the idea that sometimes the dwarfism of one
period of life may be corrected in the next, and the dwarf fcetus, for
instance, not become a dwarf child. There are also some fragments
of proof that growth may recommence at an unusually advanced
period of life.

Growth, by cell division, is the earliest vital phenomenon of the
impregnated ovum ; and it continues during the whole progress of
antenatal life and stretches on into postnatal existence. There is
growth, therefore, before there is an embryo and before there are any
organs ; growth also continues after all the organs and tissues have
appeared. These facts are to be kept in mind when we try to arrive
at any conclusions regarding the causes of the arrest of growth and
of the dwarfism or microsomia that follows it. In the early stages of
antenatal life the arrest obviously cannot be due to inaction or over-
action of any separate organ, for separate organs do not then exist ;
at a later stage it may be so caused, but, if so, it becomes necessary to
admit different causes for different periods of life. It is evident that
there must either be a common cause of arrest which is in action
during the whole of life, antenatal and postnatal ; or there must be
special causes for the special periods. I am inclined to think that
the predisposing cause of microsomia is often in the impregnated
ovum, and that in each of the succeeding periods of life certain
circumstances may serve to prevent or to accelerate its action as the
case may be. Syphilis, which is adduced as an important factor by
Founder (48), can hardly be the common cause for which we are
looking.

Literature. — (1) Sir G. Humphry, Brit. Med. Joum., ii. for 1891, p.
1187. (2) J. W. Ballantyne, Trans. Edinb. Obst. Soc, xvii. 242, 1892.
(3) J. W. Ballantyne, Trans. Edinb. Obst. Soc, xix. 51, 1894. (4) Is-
brand de Diemerbroeck, Anatome corporis humani, Lib. i. cap. 28, 1672.
(5) L. Ciniselli, SoiJra il gabinetto anatomo-patologico . . . di Cremona,
p. 70, 1869. (6) G. Colombo, Gazz. med. ital. lomb., 7 s., iv. 221, 1877.
(7) His, Anatomie menschlicher Embryonen, Fasc. ii., 1882. (8) C.

252 ANTENATAL PATHOLOGY AND HYGIENE

Giacomini, Su alcune anomalie di sviluppo delV embrione umano, Torino,
1888, 1889, 1892, 1894; Anat. Hefte, Abt. ii., iv. 617, 1895. (9) F. P.
Mall, Johns Hopkins Hosp. Rep., ix. 6, 1890. (10) G. K. Paterson,
Trans. Edinb. Obst. Soc, xxvi. 97, 1901. (11) J. Beard, Anat. An::., viii.
22, 1892-93. (12) C. Fi£re, Cinquantenaire de Ja Society de biologie, Vol.
Jubilaire, p. 365, Paris, 1899. (13) Gerlach u. Koch, Biol. Centrlbl., ii.
681, 1883. (14) II. de Yarigny, Joum. de Vanat. et physiol., xxx. 147,
1894. (15) Driesch, ''Embryology," Encyclopaedia Britannica, xxviii.
(16) C. Dareste, Production des monstruosites, p. 126, 1891. (17) Mont-
gomery, Todd's Cyclopaedia of Anatomy, ii. 318, 1836-39. (18) Cruveil-
hier, Anatomie patholo'gigue, Livr. vi. pi. vi. 1829-35. (19) Sir E. Home,
Philos. Trans., cxv. 76, 1825. (20) T. E. Baker, Trans. Med. and Phys.
Soc. Calcutta, i. 364, 1825. (21) G. A. Mursick, Amer. Joum. Med. Sc,
n.s. lxviii. 575, 1874. (22) S. J. Scott, Brit. Med. Joum., ii. for 1885,
pp. 646, 913. (23) H. Gilford, Practitioner, lxx. 797, 1903. (24) I. G.
Saint-Hilaire, Histoire des anomalies, i. 148, 1832. (25) L. Trevisani,
Rendic. Accad. med.-chir. di Ferrara, p. 60, Bologna, 1843. (26) J. D.
Nagel, Pediatrics, ii. 369, 1896. (27) Saltarino, Abnormitdten, p. 30,
1900. (28) Ibid., p. 74. (29) Dupuytren, Bull. Fac. de med. de Paris,
i. 146, 1806. (30) Yirey, Diet, d, sc. med., xxxv. 145, 1819. (31) P.
Dubois, Arch. gen. de med., 3 s., vii. 513, 1840. (32) E. Magitot, Gaz.
hebd, de med., 2 s., xviii. 692, 1881. (33) Diet. Nat. Biogr., xxviii. 149,
1891. (34) Ibid., v. 410, 1886. (35) Lancet, ii. for 1848, p. 490. (36)
J. Thomson, Scott. Med. and Stirg. Joum., vi. 209, 1900. (37) F. Rohrer,
Arch. f. path. Anat., ci. 197, 1885. (38) C. Taruffi, Riv. din. di Boloqna,
s. 2, viii. 33, 65, 1878. (39) L. Down, Trans. Path. Soc. Bond., xx. 419,
1869. (40) Clauder, Miscell. Acad. nab. curios., Dec. ii., Ann. viii. 543,
1689. (41) C. Taruffi, Storia delta teratologia, v. 446, 452, 1889. (42)
Schaaffhausen, Verhandl. d. natur. Ver. d. preuss. Rheinl. u. Westplial.,
xxv. 26, 1868. (43) G. O. Jacobsen, Lancet, i. for 1891, p. 1040. (44)
J. Hutchinson, Arch,. Surg., vi. 140, 1895. (45) S. Garnier et A.
Santenoise, Arch, de neurol., 2 s., xvi. 31, 1903. (46) Bourneville, Arch,
de neurol., 2 s., xvi. 38, 1903. (47) Oui, Gaz. hebd, d. sc, med. de Bordeaux;
xv. 245, 1894. (48) E. Fournier, Stigmates dystroqjhiques de I'herSdo-
syphilis, p. 147, 1898.

Macrosomia (Giantism).

Macrosomia is the converse of microsomia, and may be denned as
a " monstrous largeness " of all the parts of the individual. It may,
like microsomia, have its origin before or after birth, but the more
characteristic cases are postnatal. It is, therefore, comparatively
unimportant from the standpoint of antenatal pathology ; and I shall
give no lengthy description of it, but simply some condensed notes,
some facts extracted from a scattered literature and compressed
within limits.

Concerning embryonic macrosomia or giantism of the embryo,
little or nothing is known. Theoretically, it may be expected to be
met with, as much as embryonic microsomia ; but there is next to no
proof tbereof. Mall's case (1) of an amniotic sac too small for its
contained embryo may perhaps be regarded as one of an embryo too
large for its containing amniotic sac. Experimental teratogeny,

MICROSOMIA 25

Q

however, throws a little light here : Fere (2) found that substances
which produce dwarfing or deformity of the chick embryo will some-
times cause excessive growth with or without excessive development
(ontogenesis); and exaltation or depression of growth or development
apparently depends upon the dose of the teratogenic agent and the
" individual trophic equation of the germ." Of course, this " trophic,
equation of the germ," being, so to say, a personal equation, is not
easily to he calculated. If it could be determined, many other
problems than those of microsomia and macrosomia would be in a
fair way to settlement. Embryonic macrosomia lies meanwhile in
a neglected corner of Antenatal Pathology, which the systematic
examination of early abortion sacs may yet serve to redeem.

Fatal macrosomia or giantism of the foetus, on the other hand, is
well known. Of course, a new-born infant with a weight above the
normal is not necessarily an instance of foetal macrosomia; it may be
simply a case of postmaturity, the child having by his longer sojourn
in the uterus acquired an increased size and weight. Macrosomia is
an undue largeness of the foetus for the time in antenatal life arrived
at. It must also be kept in mind, however, that a postmature
infant may be at the same time the subject of macrosomia, as in
Rachel and Neumer's case (.">), and in Fuchs' two cases (49). The
great criterion of macrosomia is weight, whereas postmaturity is
characterised by advanced development (ontogenesis) as well. In
the " problem of the postmature infant " (4), I have discussed this
and allied questions, and I refer the reader, who is interested in the
matter, to it. The postmature infant is larger and further developed
than the ordinary new-born child, simply because he has had a month
or six weeks longer life in the uterus; the subject of macrosomia is
larger than he ought to be for the age at which he has arrived, and
he may be developed in proportion to his weight or only in pro-
portion to his age. The two conditions may, as I have already
indicated, be combined ; but in the paper to which I have referred (4),
a still more remarkable association was pointed out. One of the post-
mature infants therein described was also the subject of anencephaly
(i.e. arrested development of the cranial vault), and at the same time
exhibited in other parts of the skeleton a degree of ossification not
equal to but actually in excess of that which the postmature condition
warranted : this remarkable case, therefore, was at one and the same
time an instance of macrosomia, of postmaturity, and of arrested
development. Let us, however, direct our attention to the uncom-
plicated cases at present.

Some instances of foetal macrosomia may be cited : S. S. Smith's
case, 22 lb. (5); G. Olano's, 22 lb. (6); C. W. Chubb's, 21 lb. (7);
A. E. Rice's, 20 lb. 2 oz. (8); W. P. Johnston's, 20 lb. (9); R. L.
Dickinson's, 20 lb. (10); J. Perrins', 19f lb. (11) ; T. P. Satterwhite's,
19 lb. (12); the cases of O. 0. Burgess (13), E. Gordon (14), and A.
Meadows (15), 18 lb. each; T. Wilmot's, 17| lb. (16); the cases of
Kilham (17) and R. P. Myers (18). 17i lb. each ; H. Ludwig's case,
17 lb. (19); the cases of H. A. Cooper (20) and G. Read (21), 16^ lb.
each; F. S. Jaquett's, 16 lb. (22); C. Waller's, 15 lb. 15 oz. (23); H.

254 ANTENATAL PATHOLOGY AND HYGIENE

W

Oppenheimer's, 1U lb. (24) ; E. R. Lewis' case, 15 lb. 3 oz. (25) ; 1
E. Kirby's, 14 lb. 15 oz. (26) ; the cases of D. J. F. Bennett (27),
Brothers (28), and Cameron (29), 14 lb. each; Maygrier's, 13| lb.
(30) ; the cases of A. H. Cuartero (31), G. G. Detharcling (32), and B.
Wolff (33), 12 lb. each; W. Woodward's, 11 lb. 10 oz. (34); and E.
A. Tucker's, 11 lb. G oz. (35). A greater weight than any of the
above was attained by the infant reported by A. P. Beach (36), but
the father of it was 7 feet 7 inches in height and the mother 7 feet
9 inches ; it was the second child, and weighed 23f lb. and had a
length of 30 inches; the first child born to these same parents
weighed 18 lb. and was 24 inches in length.

Many of these giant foetuses died at birth, and it is unfortunate
that as a rule no information is forthcoming regarding their morbid
anatomy ; others survived, but rarely have we any record of their
after-history. A few details I have gathered together and present to
the reader herewith.

The case recorded by Cameron (29) was that of a female child
weighing 14 lb. at birth ; she weighed 69 lb. at twelve months, and had
a height of 35£ inches ; at seventeen months the weight was 98 lb.; she
was weaned at two years, when also she began to walk. Great size was
here maintained up to two years (we have no further information) ;
but it was not accompanied by any physiological precocity, on the
contrary there was delay in walking and possibly in other functions
as well. But there is some evidence that large size at birth occa-
sionally precedes a precocious puberty. The large size may not be
maintained, but there is a premature development of hair on various
parts of the body, and an early awakening of sexual functions
(Kussmaul, 37). Stacker's case (38) was a most remarkable one:
the giant infant was one of twins, both female ; and their mother
had altogether eleven children, including triplets once and twins
twice ; the large twin showed some signs of a vaginal discharge at
birth, and regularly menstruated at the age of three years; when
eight years old, she had all the appearance of a girl of twelve,
measuring 139 cms. in height and having well-developed genitals and
mammae. Moreau's giant boy (39) weighed 16 lb. at birth ; he had a
beard at seven years ; at ten he measured 4 feet 5 inches ; his testicles
were large ; and he was very powerful, but of a timid disposition.
Apparently, the big boy of Willingham — "Prodigium Willing-
hamense," as Dawkes styled him (40) — was of large size from birth.
He was born at Willingham, near Cambridge, in 1741 ; before he was
three years old he was 3, feet 8 inches in height, and had the marks of
puberty. I do not know whether the case of early puberty described
by Molitor (41) was also one of foetal macrosomia, as I have not been
able to refer to the original article : it was that of a girl who had
hair on the pubes at birth ; puberty came on at four years ; when
eight years old she measured 133 cms. (53 inches) in height; soon
thereafter she became pregnant, had intractable vomiting and jaundice,
and expelled a hydatid mole containing a small embryo (35 mm. in
length) ; she had large breasts and long hair. By a curious
coincidence, the earliest pregnancy that I have met with also

MACROSOMIA 255

terminated in the expulsion of a hydatid mole : it was the case of a
Polish Jewess, aged fifteen and a half years, who was said to have
been impregnated by her cousin, a boy of thirteen years ; she was
large for her age.

From these case-records it will be clear that foetal macrosomia
may come into relation with several postnatally developed morbid
states : it may, for instance, run on into infantile macrosomia
(" pedomacrosomia," Taruffi) ; it may be followed by a precocious
puberty, by early menstruation, and by an early pregnancy ; and
there is some evidence to show that it may be associated with the
growth of tumours of the ovaries and testicles. J. Halliday Croom
(•42), for example, reported a case of ovarian sarcoma in a child of
seven years who had many signs of precocious puberty, and about
whom it was stated that her mammary glands were large even at
birth. E. Sacchi (43), also, put on record an instance of infantile
macrosomia in a boy, who, in addition to having an early puberty,
was the subject of a tumour of the left testicle of an epithelial
nature and containing coccidia ; it is a noteworthy fact that the
removal of the testicular tumour was followed not only by an arrest-
ment of growth, but by the disappearance of some of the signs of
sexual maturity. These various morbid states, however, lie outside
the province of Antenatal Pathology ; so also does true giantism
(" neanio-macrosomia," Gr. nuvtag, young man, Taruffi) and the con-
ditions which are related to it (acromegaly, leontiasis ossea, etc.), in
which the excessive growth of the individual begins at or after
the fourteenth year. They may, of course, be antenatal in origin
although postnatal in development, and be the postponed effects of
causes acting before birth ; but so may many other morbid conditions,
and it is necessary somewhere to draw the line bounding this ever
growing province of Medicine.

The causes of macrosomia are even less understood than are those
of microsomia. Speculation, however, has travelled along very much
the same lines, but it would be of little profit to follow it. A few
conclusions seem warranted, but even these must be accepted with
caution, and will probably ere long call for revision. Great size at
birth (foetal macrosomia) and giantism developed later would seem to
be commoner in the races in which the average height is great ; great
height is sometimes hereditarily transmitted, but in many cases there
is no such transmission ; macrosomia and sexual precocity are some-
times associated, but on the other hand the giantism of adolescence
sometimes appears to follow as a consequence of premature cessation
of sexual development (45) ; and, from what is known of the morbid
anatomy of acromegaly and of its relations with giantism, it may be
that increased growth is due to disease of some organ which normally
checks growth, or to increased activity of some other organ which
normally stimulates it. In relation to the last statement, it is
interesting to remember that the large foetus described by B. Wolff
(33) had a disproportionately large heart and disproportionately
small thymus and thyroid glands; interesting, but not, in all
probability, throwing much light on etiology. If it turn out to be

256 ANTENATAL PATHOLOGY AND HYGIENE

true that macrosomia is due to sexual infantilism and to retarded
ossification of the epiphyses (46, 47), then, after all, this anomaly will
have to be reckoned as an arrest.

In all speculations about the etiology of anomalies of growth, it
has constantly to be borne in mind that a theory to be satisfactory
must account for the facts, and for all the facts. Now, since both
dwarfism and giantism may be antenatal as well as postnatal in
origin, a theory of causation to be acceptable must be capable of
application to the conditions which prevail both before and after
birth ; it must also take into account and explain the commonly
associated conditions, such as infantilism in microsomia and pre-
cocious puberty in macrosomia (or vice versd) and malformations in
both ; and it must also give a reason why these commonly associated
states are not constantly associated. I know of no theory which
fulfils these requirements ; certainly, the idea of variations in the
"trophic equation of the germ" brings us no nearer the truth than
to say that a person with a high temperature is suffering from a
thermometric perturbation. Syphilis, likewise, does not explain all
the circumstances.

Tkerapia nulla. — It may yet be found that one of the organic
extracts may check excessive growth, and so an empiric mode of treat-
ment be rendered possible ; but of that there is no indication now.

Literature. — (1) F. P. Mall, Johns Hopkins Hosp. Rep., ix. 6, 1900.
(2) C. F£re, Compt. rend. Soc. de biol., 10 s., iii. 790, 1896. (3) Eachel
and Neumer, Med. Monatschr., New York, Hft. 9, p. 422, 1890. (4)
.1. W. Ballantyne, Journ. Obst. and Gyncec. Brit. Emp., ii. 521, 1902.
(5) S. S. Smith, Med. and Surg. Rep., xxxviii. 512, 1878. (6) G. Olano,
El Monitor Medico, xi. 260, 1896. (7) C. W. Chubb, Brit. Med. Journ.,
i. for 1879, p. 143. (8) A. E. Bice, Boston Med. and Surg. Journ., xcv.
110, 1876. (9) W. P. Johnston, Amer. Journ. Med. Sc, n.s., xxi. 340,
1851. (10) B. L. Dickinson, Brooklyn Med. Journ., viii. 225, 1894.
(11) J. Perrins, Eclect. Med, Journ., xxxviii. 501, 1878. (12) T. P.
Satterwhite, Arch. Pediat., xiv. 914, 1897. (13) O. O. Burgess, Pacific
Med. and Surg. Journ,, ix. 179, 1875-76. (14) E. Gordon, Brit. Med.
Journ,, i. for 1884, p. 664. (15) A. Meadows, Med. Times and Gaz., ii.
for 1860, p. 105. (16) T. Wilmot, Brit. Med. Journ,, i. fur 1901, p. 146.
(17) Kilham, Lancet, i. for 1895, p. 620. (18) B. P. Myers, Boston Med.
and Sun/. Journ,, c. 138, 1879. (19) H. Ludwig, Centrlbl, f. Gynak., xx.
64, 1896. (20) H. A. Cooper, Kansas City Med. Pec, vi. 179, 1889.
(21) G. Bead, Australas. Med. Ga::., xii. 184, 1893. (22) F. S. Jaquett,
Med. and Surg. Rep., xi. 571, 1858. (23) C. Waller, Trans. Obst, Soc.
Bond., i. 309, 1860. (24) H. Ofpenheimer, Lancet, i. for 1896, p. 262.
(25) E. B. Lewis, Boston Med. and Sun/. Journ., cxix. 543, 1888. (26)
W. E. Kirby, Brit. Med. Journ., i. for 1901, p. 16. (27) D. J. F. Bennett,
Lancet, i. for 1896, p. 99. (28) A. Brothers, Amer. Journ. 014,, xxxiii.
528, 1896. (29) Cameron, Trans. Obst. Soc. Lond., xviii. 115, 1877.
(30) Maygrikr, Rev. obstet. et gynec, vii. 136, 1891. (31) A. H. Cuartero,
El Siglo Med., xlii. 134, 1895. (32) G. G. Detharding, Arch. f. d.
Geburtsh., iii. 625, 1791. (33) B. AVolff, Ztschr. f. Geburtsh. u. Gynak.,
xlv. 381, 1901. (34) AV. AVoodward, Lancet, ii. for 1895, p. 1497. (35)
E. A. Tucker, Med. Rec, xlviii. 334, 1895. (36) A. P. Beach, Med. Rec,

HEMI-HYPERTROPHY 257

xv. 271, 1879. (37) A. Kussmaul, Wiirzb. vied. Ztschr., iii. 321, 1862.
(38) 0. Stockeh, Corr.-Bl.f. schweiz. Aerzte, ix. 261, 1879. (39) Moreau,
Journ. d. med., chir., pharm., xii. 274, 1806. (40) T. Dawkes, Prodigium
Willinghamense, London, 1747. (41) Molitor, Bull. Acad. roy. de med,
de Belg., 3 s., xii. 77, 1878. (42) J. H. Croom, Trans. Edinb. Obst. Soc,
xviii. 58, 1893. (43) E. Sacchi, Arch, di Ortoped., xii. 305, 1895. (44)
A. Brunauer, Wien. med. Wchnschr., xlii. 4, 1892. (45) L. Capita^
Compt. rend. Soc. de biol., lv. 63, 1903. (46) P. E. Launois et P. Roy,
Rev. intemat. de med. et de chir., xiii. 397, 1902; None, iconogr. de la
Salpetriere, xv. 540, 1902. (47) A. Poncet, Corrupt rend. Soc. de bio/.,
lv. 65, 1903. (48) F. Ortega, Nouv. arch, d'obst. et de gynec, vi. 481,
1891. (49) H. Fuchs, Miinchen. wed. Wchnschr., 1. 1411, 1461, 1903.
For the Literature of Early Menstruation, Pregnancy, and Puberty, vide
lists in Index Catalogue, ix. 162, 1888; xi. 599, 761, 1890. The literature
of Macrosomia in Childhood will be found in Ta rum's Storia delta terato-
logia, v. 344-395, 1889; viii. 84-147, 512, 1894.

Unilateral Macrosomia (Hemi-hypertrophy).

Unilateral Macrosomia, more commonly but less correctly termed
Hemi-hypertrophy, may be considered here, although, strictly speak-
ing, it is not a malformation of the whole body but only of one side
of it. It is a one-sided enlargement of the individual, a " laterality "
or asymmetry of the body. It has been supposed by J. B. Milne (1)
and others that while the one side-half of the body is enlarged the
other side-half is not normal but diminished or " atrophied " ; if this
be so, which is doubtful, then the malformation after all would be
one affecting the whole individual.

A well-marked case of unilateral macrosomia draws attention
by its strange and peculiar appearance. Proudly conscious, perhaps,
of the strict symmetry of the two side-halves of his own body,
the observer cannot but remark upon the grotesqueness of this
anomaly or inequality of growth. But let him pause and inquire
whether, after all, he himself is the possessor of perfect bilateral
symmetry. Most probably he will find that he is not — his boots,
gloves, and photographs bearing witness against him ; more than
this, he will most likely discover that he is unequally asymmetrical,
while the subject of unilateral macrosomia may be, so to say, equally
asymmetrical. It is the irregularity of his asymmetry that makes
him normal, while it is the regularity of the asymmetry of the
individual with hemi-hypertrophy that makes him abnormal. A
strange paradox surely !

It would indeed appear to be true that the two side-halves of the
body have a symmetry which is never entirely perfect. " Invarial >ly
Nature varies " (Paget, 2) ; and the two halves of the head and face,
the two eyes and ears, the two arms and legs, and even the two
breasts are slightly unlike in everyone. The right arm is usually
longer and thicker than the left (Tuckerman, 3) ; the right leg may
also have dimensions which exceed those of the left, but some
statistics {e.g. those of CI. Guldberg, 4) have shown the left leg
larger than the right. Crossed asymmetry has been noted — right
vol. ii. — 17

258

ANTENATAL PATHOLOGY AND HYGIENE

arm larger than left, but left leg larger than right — in 40 per cent,
according to Guldberg; along with this morphological inequality it
has been discovered that there is often a functional asymmetry (one
side stronger than the other), and in this association has been found
a possible explanation of why it is that a person lost in a fog tends
to wander in a circle.

Unilateral macrosomia, however, differs from this " normal " or
" physiological " inequality. In it the laterality is complete, or
nearly so : all the parts on one side of the body are enlarged ;
they are usually enlarged in all their constituent parts ; and in
general they are proportionately enlarged. These, at any rate,
were the characters of the case of unilateral macrosomia which I had

Fie 39. — Case of Hemi-Hypertrophy or Unilateral Macrosomia. No. 224.

an opportunity of examining with Dr. J. S. Fowler in March 1899.
The details of this example of unilateral macrosomia were afterwards
published by Fowler and Johnston (5), and are accessible to the
reader ; for this reason I shall be brief in my references thereto.

The patient, a little girl, was ten months old when I saw her ; was
the first child of healthy parents (ages twenty-two and nineteen) with
no hereditary history of malformations ; had been artificially fed, but
had had no serious illnesses. The right foot had been observed to be
larger than the left at the time of birth, when, also, numerous capillary
naevi had been noticed on various parts of the body. Only on the fore-
head were the nsevi unilateral in their arrangement. With regard
to size, the patient showed an almost complete laterality : the right
side-half was larger than the left. The enlargement, it is true, was

HEMI-HYPERTROPHY 259

slightly more obvious in some parts than in others ; thus, it was
more noticeable in the forearm than in the upper arm, in the foot
than in the leg, in the leg than in the thigh, and was hardly dis-
cernible in the nose and mouth, although quite perceptible in the
ear and in the external genitals. The sensory functions were
unimpaired, and the reflexes were not abnormal ; but no reliable
observations on the temperature of the two halves of the body
could be made. The appearances are well displayed in Fig. 39.

Some additional facts regarding this striking malformation may
be brought together here from various sources in order to give com-
pleteness to the clinical picture. As the individual grows in size
the disproportion between the two sides of the body becomes more
apparent, but there is no evidence that the larger side gains much,
if at all, upon the smaller side ; it would appear as if the one side-
half had got, as it were, the start of the other, but does not further
increase its lead. During dentition, it may be noticed that the teeth
are cut sooner on the enlarged side than on the other (1); walking
may be interfered with by the laterality of the body which causes
some obliquity of the pelvis. The hair may grow more quickly on
the enlarged side, may curl more, and be coarser in texture ; there
may be increased desquamation of epidermis, the nails may be
thicker and curved, and there may be unilateral hyperhidrosis. The
tongue and tonsils may also show laterality (Finlayson, 6) ; or there
may be macroglossia of the whole tongue (Kopal, 7), due to the
enlargement of its muscle. One testicle may be larger than the
other (Redard, 8). The internal organs have been found to show
unilateral hypertrophy as well as the external parts ; thus, at the
autopsy in Arnheim's case, even the valves of the right side of the
heart were larger than those of the left (9). Other malformations
may be present, but rarely ; for example, there was Polydactyly
in the cases of Bull (10) and Keuscher (11), and syndactyly in that of
Wittelshbfer (12). Foetid macrosoniia may coexist (Hymanson, 13).

The general health is usually uninterfered with, but occasionally
the mental powers may be weak (J. Thomson, 14). The enlarged
parts would appear to be more liable to ulceration, to erysipelatous
infection, and to lymphangitis. The association of nam and telan-
giectases with lateral macrosoniia has been noted in several cases,
e.g. S. S. Adams' (15) and B. Duzea's (16) ; but it is most noteworthy
that these vascular disturbances have seldom been strictly unilateral
in their distribution, a fact which militates against the theory ad-
vanced by Trelat and Monod (17) and Duzea (16) that they are the
cause of the macrosomia.

Knowledge regarding the etiology and teratogenesis of unilateral
macrosomia is sadly lacking ; theories there are in plenty and of the
greatest variety, but no uniformity of belief. In Demme's case of
congenital hemi-hypertrophy of the muscles in a female infant (30),
it was thought (after the autopsy) that there was an increased number
of ganglion cells in the anterior horns on the left side in the cord
and in the nucleus of the hypoglossal (the tongue showed remarkable
hemi-hypertrophy) ; and Demme thought the malady showed analogies

260 ANTENATAL PATHOLOGY AND HYGIENE

with Thomsen's disease on the one hand and with pseudo-hypertrophic
paralysis on the other. It is difficult, however, to formulate a theory
of origin from these data and resemblances. Syphilis, maternal
impressions, heredity, and injuries have been adduced, and have been
supposed to act through disturbances of the vessels, lymphatics, or
nerves of the affected parts ; further, all sorts of ingenious explana-
tions have been brought forward to account for the morbid anatomy
of the malformation, but no satisfactory reason has yet been alleged
for its one-sided character. It is no explanation to point out that
some other antenatal morbid states, such as nsevus neuroticus unius
lateris, congenital white streaks (28), and tumours (29), have this
unilateral character ; this brings us no nearer the cause. It would
be a real help if it could be shown whether the macrosomia develops
in the foetal period or is already present in the embryonic. There is,
as the reader will remember, a degree of spiral ity about the embryo
at the third week, but I know not whether along with it there may
be also a predominance of one side of the body over the other. In
some measurements made upon embryos and foetuses by L. W. Fox
(27) it was found that in the region of the face the right side was
larger than the left ; but the observations were too few (seven) to
warrant their use in deciding the question of embryonic laterality. If
unilateral macrosomia can be shown to be present in embryonic life,
then Leblanc's theory of the action of the metameres (18) may be
accepted as giving the most probable explanation of its mode, of
origin ; but it must be kept in mind that metamerism l itself is as
yet but a theory, and not an established fact in ontogenesis. It may
be asked whether the latericumbent position of the embryo in the
embryonic area has any causal effect ; but no answer can be given.
The statement that congenital hemi-hypertrophy is due to " an in-
herent tendency of the tissues to appropriate an excess of nutri-
ment " is more ingenuous than ingenious. We may frankly admit
that we do not know the causes of unilateral macrosomia or their
mode of action ; indeed we do not know those of general macrosomia,
and we can hardly, therefore, expect to be more successful in our
attempts to arrive at these of the partial variety of giant growth.
It may be noted, before the subject is left, that the enlargement, as
a rule, affects all the layers of tissue of which the part is made up
(skin, subcutaneous tissue, muscle, bone) ; it is not, therefore, to be
confounded with congenital elephantiasis (vide Manual, i. 300).

Literature. — (1) J. B. Milne, Quart. Med. Journ., iii. 243, 1894-95;
Lancet, i. for 1895, p. 752. (2) Sir Jas. Paget, Internat. Journ. Med. Sc,
n.s., xci. 41, 1886. (3) F. Tuckerman, Journ. Auat. and Physiol., xix.
307, 1S85. (4) G. Guldberg, Norsk. Mag. f. Laegevidensk., 4 R., xii.
180, 1897. (5) .1. S. Fowler and K. J. Johnston, Edirib. Hosp. Rep., vi.
113, 1900. (6) J. Fixlayson, Glasgow Med. Journ., 4 s., xxii. 327, 1884.
(7) Kopal, Prag. med. Wchnschr., xx. 161, 341, 1895. (8) P. Kedard,
Arch. gr/t. de vied., i. for 1890, p. 31. (9) G. Arnheim, BerJ. /din.

1 A metamere is a pair of mesodermic somites ; it is supposed that the body is made
up of a series of metameres, but it is difficult to trace this metameric arrangement in
all regions of the embryo.

UNILATERAL MICROSOMIA 261

Wchnschr., xxxiii. 46, 1896. (10) G. J. Bull, Boston Med. and Surg.
Journ., xciii. 293, 1875. (11) C. Reuscher, "Bericht iiber einen Fall
von totaler halbseitiger Kbrpernypertrophie," Diss, inaug., Giessen, 1897.
(12) Wittelshofer, Arch, f. Jclin. Chir., xxiv. 57, 1879. (13) A. Hy-
manson, Arch. Pediat., xx. 428, 1903. (14) J. Thomson, Edinb. Med.
Journ., xxxvii. 249, 1892. (15) S. S. Adams, Arch. Pediat., xi. 901,
1894. (16) R. Duzea, " Sur quelques troubles de developpement tlu
squelette dus a des angiom.es superficiels," These, Lyon, 1886. (17) U.
Trelat et A. Monod, Arch. gen. de vied., i. 536, 676, 1869. (18) E.
Leblanc, These, Paris, 1897. (19) A. Massonnaud, These, Paris, 1874.
(20) H. True et Masme.iean, Montpelier mid., 2 s., x. 257, 1888. (21) S.
Hornstein, Arch. f. path. Anat., exxxiii. 440, 1893. (22) Duplay, Gaz.
held. d. mkl., n.s., 'ii. 529, 1897. (23) C. Turbil, Giorn. d. R. Accad. d.
med. di Torino, lix. 9, 1896. (24) R. Jasinski, Przeglad lek., i. 83, 1893-94.
(25) V. 1'. Zhukovskt, Arch, psichiat., xxvi. i. 1895. (26) P. Wagner,
Deutsche Ztschr. f. Chir., xxvi. 281, 1887. (27) L. W. Fox, Mitth. a. d.
embryol. Instit. d. h:-l: Univ. in Wien, i. 280, 1880. (28) J. Hutchinson,
Smaller Atlas of Clinical Surgery, Plates i. and n., 1895. (29) Ibid.,
Plate xliii. (30) Demme, Wien. med. JBlatt., xiv. 17, 1891.

Unilateral Microsomia.

In a certain sense every case of unilateral macrosomia might be
regarded as one of unilateral microsomia ; instead of looking upon
the one side as hypertrophied or overgrown, we might look upon the
other side as atrophied or undersized. The question of deciding which
side is abnormal has usually been answered by finding which of the
two sides departs further from the normal measurements of the
body for the particular age arrived at. In this matter, especially in
the case of the foetus, it is of course quite possible to be in error,
and it may be that among the cases of unilateral macrosomia have
been included instances of unilateral microsomia. Further, it is
also possible that just as the one side-half of the body exceeds the
normal size, so the other side-half may not come up to it ; the
macrosomia of one side may be compensated, as it were, by a micro-
somia of the other side. In all these cases much depends upon
the fixing of the normal size and measurements for the age of ante-
natal or postnatal life reached, and, unfortunately, little certainty has
been attained to in this part of anthropometry.

The cases to which the name congenital unilateral microsomia
may be given must not be those in which there is one-sided atrophy
of the brain and cranium with atrophy of the opposite side of the
body and limbs ; such cases are to be accounted for in another way
and come under another category (result of foetal brain disease).
Several of the reported instances of unilateral microsomia must be
excluded on this account ; and very few true cases are left. I have
met with a malformed foetus (exomphalos, facial fissures, tail-like
projection, etc.) in which the right arm wTas manifestly stunted
(Fig. .°>0) ; but it would be incorrect to call it an instance of uni-
lateral microsomia, even if the right leg had also been small, so long
as there was not right-sided smallness of the head. Scheiber's

2G2 ANTENATAL PATHOLOGY AND HYGIENE

case (1), if not a genuine instance of unilateral atrophy or micro-
somia, is very nearly so : it was that of a boy with complete left-sided
hemi-atrophy, who had had an attack of apoplexy at the age of seven
years. The writer was uncertain as to the origin of the atrophy, and
did not decide between a developmental fault and cerebral haemor-
rhage ; but he tentatively suggested that there might have been a
developmental fault on one side of the brain and a haemorrhage on
the other side. N. Obolonsky's case (2), also, is not a clear instance
of unilateral microsomia : it was a new-born male infant, with
unilateral smallness of the left leg and left side of the body and of
the middle fossa of the cranium on the left side ; the left arm, how-
ever, showed no marked difference in size ; there was also hydro-
cephalus, and there were pre-auricular appendages on both sides,
malformations of the left hand (syndactyly and perodactyly), talipes
varus of the left foot, a tail-like appendage, enlargement of the
penis, and hypertrichosis in the lumbo-sacral region and on the left
thigh ; the two cerebral hemispheres were symmetrical. C. Hennig's
case of hypertrichosis and hemi-atrophy (3) was one in which only
the right arm showed evident smallness ; Forster Brockman's
instance of hemi-atrophy (4) was a dissecting-room body, about
which no history was obtainable ; and in Caizergues' patient (5)
there had been fever with convulsions on one side at the age of two
years. All these cases must therefore be excluded ; but possibly
that reported by Wallis Ord (6) may be looked upon as true uni-
lateral microsomia : it was that of a girl of twelve years, whose
whole right side below the level of the eyebrows was smaller than
the left, who had no hemiplegia, and whose mother had had a severe
fright during the time she was pregnant. Sumner Pixley's case of
" congenital unilateral atrophy " (7) may likewise be a genuine
instance.

It is quite evident, from what has been noted above, that very
little is known about unilateral microsomia, that very few cases have
been reported, and that no satisfactory explanation of their origin is
forthcoming. It may be that some instances of unilateral macro-
somia ought rightly to have been included here ; it may also be
suggested that both these morbid states (macrosomia and micro-
somia) take origin in the embryonic period by reason of some cause
{e.g. pressure) which acts upon one side of the organism and not on
the other ; but facts are so scanty that hypotheses become more than
usually dangerous. One thing, however, seems fairly certain : there
is a form of congenital unilateral smallness of the body which is not
satisfactorily to be accounted for by disease of the brain arising
during foetal life, and for which, therefore, it is necessary to look for
an explanation in the embryonic or germinal period.

Literature. — (1) S. H. Scheiber, Wien. Tclin. Wchnschr., v. 369, 1892.
(2) N. Obolonsky, Ztschr.f. Heilk., x. 246, 1890. (3) C. IIennig, Jahrb.
f. Kinderhlk., xl. 107, 1895. (4) F. Brockman, Journ. Anat. and Physiol.,
"x. 786, 1876. (5) Caizergues, Montpel med., xliii. 151, 1879. (6) W.
Ord, Brit. Med. Journ., i. for 1895, p. 587. (7) S. Pixley, Med. News,
xli. 146, 1883.

CHAPTER XV

Teratologieal Types : Merosoinatous Terata : Partial Macrosomia affecting (1) the
head and face, (2) the extremities, and (3) one extremity or part of an
extremity; Pathogenesis and Treatment : Partial Microsomia.

In the preceding chapter the pantosomatous terata, to use Tarufn's
name for them, were dealt with. They were the malformations, it
will be remembered, in which not a part but the whole Of the organism
was affected ; the whole body, trunk as well as limbs, was enlarged
(macrosomia) or diminished in size (microsomia). They were anomalies
of growth rather than of organogenesis or construction, and on that
account were not clearly typical of embryonic pathology ; but it was
noted that they were not uncommonly accompanied by errors of
organogenesis, showing that the two kinds of anomaly may coexist,
indeed tend to coexist. Unilateral macrosomia and microsomia
were also considered, although in one sense they were not panto-
somatous terata; but they were evidently closely allied to the
generalised forms of giantism and dwarfism. Now, in the present
chapter, I proceed to the description of the clearly localised mon-
strosities (merosomatous terata of Taruffi), selecting partial macrosomia
as the first type. In this way the anomalies of growth, already
considered, are seen to be linked on to the anomalies of construc-
tion, which are about to be discussed, by these intermediate types,
unilateral and partial macrosomia.

Partial Macrosomia.

By partial or localised macrosomia is meant a local as contrasted
with a general overgrowth of the body ; one part, such as one-half of
the face, a leg, an arm, or a toe, considerably exceeds the other parts
in size ; and the overgrowth, as a rule, involves all the constituent
structures of the affected part. From the standpoint of Antenatal
Pathology we have only to do with the congenital cases ; and among
them there are some which may be truly embryonic in origin while
others are apparently foetal. It is often exceedingly difficult to
separate the giant growths which are really due to an original
anomaly of growth from those which arise in .the foetal period of ante-
natal life and are caused by such diseases as congenital elephantiasis
and angiomata ; on this matter the reader will find some remarks in
the first section of this Manual (pp. 300-305). Bearing these facts

263

264 ANTENATAL PATHOLOGY AND HYGIENE

well in mind, we may proceed to consider macrosomia as it affects
(1) one side of the face, (2) the arms or legs, or (3) one limb.

Partial Macrosomia- of the Head and Face. — Lateral enlargement of
the head and face would appear to be rather rare ; but within recent
years I). M. ( Jreig (1) has succeeded in gathering together thirty-five
illustrative cases, a great advance upon Taruffi's fourteen instances (2).
In ( Ireig's list, cases of congenital elephantiasis, of sarcoma, and of
leontiasis ossea have been carefully weeded out. The bones and soft
parts may both participate in the unilateral enlargement, or the
soft parts alone, or the bones alone may be affected. When the
latter alone are enlarged, the morbid state is commonly described as
originating after birth, and this is quite in keeping with the general
principles of the subject according to which early interference with
the processes of growth have more widespread results. Statistics
do not show marked predominance of one sex or of one side of the
face in these cases, although some writers have thought that males
suffer more than females, and that the right side is more often the
site of the enlargement than the left (Barwell, 3).

The laterality of the head and face is usually unmistakable. The
one cheek is larger than the other ; the angle of the mouth of that
side is lower ; the alveolar portion of the superior maxilla, half the
tongue, the ear, and the tonsil show hypertrophy (Ziehl, 4) ; so do
the lips, the eyelids, half the nose (Schieck, 5), and even the teeth
when they appear (Passauer, 6). There may be a more marked flow
of saliva on the side which is enlarged. Sometimes, but not often,
the lower jaw participates in the laterality (Schneider, 7). The
subcutaneous tissue generally shows thickening, and there may be
recognisable lipomata (Schneider, 7). The mental conditions may,
as the infant grows, be discovered to be defective.

The causes which are usually alleged in connection with all
teratological cases are also advanced to explain unilateral macrosomia
of the head and face ; and the theory that in some way the over-
growth is due to perverted action of the nervous system (foetal
meningitis) would appear to be the most probable. On the other
hand, there is much to suggest that the macrosomia originates before
the foetal period of life, and therefore before foetal diseases can be
invoked as causative agencies. Our knowledge of the teratogenesis
of this, as of all forms of macrosomia and microsomia, is, after all,
close neighbour to nothing.

Literature.— (1) D. M. Greig, Edinb. Hosp. Rep., v. 212, 1898. (2) C.
Taruffi, Storia delta teratologia, v. 410, 1889. (3) E. P.arwell, Med.
Times and Gaz., i. for 1881, p. 361 ; Trans. Path. Soc. Lond., xxxii. 282,
1880-81. (4) F. Zieiil, Arch. f. path. Anat., xci. 92, 1883. (5) Schieck,'
Berlin, klin. Wchnschr., xx. 700, 1883. (6)0. Passauer, Arch. /.path.
Anat., xxxvii. 410, 1866. (7) A. Schneider, Diss, inaug., Strasburg, 1885.
(8) N. Friedreich, Arch. f. path. Anat., xxviii. 474, 1863. (9) E. Kiwull,
Fortsrhr. d. Med., viii. 121, 1890.

Macrosomia of the Extremities. — Sometimes all the four extremities
exhibit a certain degree of enlargement ; when this occurs in associa-

PARTIAL MACROSOMIA 265

tion with hypertrophy of the head the clinical picture formed is that
of acromegaly. This malady occurs, as a rule, after adolescence, and
so does not at all fall within the province of the antenatal pathologist.
" Congenital " cases have been described {e.g. those of Cenas, 1, and
Jacobson, 2) ; but it is doubtful whether the affection was true
acromegaly. The tendency of the malady to be associated with
giantism has been already noted ; and it may itself be regarded as
giantism occurring in adult life, and limited, therefore, to the parts
of the body where growth is still possible — the terminal parts ; it has
been named, on this account, peripheral macrosomia (Taruffi). Its
pathogenesis, like that of all the anomalies of growth, is quite un-
certain ; it may be due to the neoplasm of the pituitary body, so often
found on post-mortem examination, but the evidence is by no means
conclusive. It has also been regarded as a reversion to the type of
the anthropoid ape, a theory which, if true (the writer very much
doubts it), would bring the malady well within the province of the
antenatal pathologist after all. Here we leave the matter, adding
this one sentence that the cases in which the long bones participate
in the overgrowth are probably to be regarded not as true acromegaly
but as delayed giantism {macrosomia scrotina, Taruffi).

A curious form of enlargement of the extremities has been
described by H. Friedberg (3) and A. 1*. Gould (4) ; in it there is a
sort of crossed macrosomia, the right leg and the left arm being
hypertrophied (3), or the left leg, the left side of the head, and the
right arm being the overgrown parts (4). In Friedberg's case there
were also telangiectases and lipomata. Associated with this type of
macrosomia is that in which both arms or both legs are affected
{bilateral macrosomia, Taruffi). Gherini's patient (5) had both lower
limbs enlarged, the left showing the macrosomatous state more than
the right ; there were also nsevi on the skin ; while in Boechat's case
(6) the enlargement affected the hands and had existed for four
generations. E. Kapin (8) has reported a case of congenital oedema
of the two upper limbs and of the right side of the face. Some
years ago I received from Dr. P. H. Nightingale the photograph of a
Siamese woman, which is reproduced here (Fig. 40) ; the whole of the
left upper limb and the right hand are much enlarged, and the case
appears to be one of bilateral macrosomia of a very marked type. I
could get no details of her history beyond the facts that she lived, in
1895, at Klong Bang Luang, that she was forty-three years of age,
and a widow. Both her hands measured 13 inches long and 7 inches
broad ; the forefinger was about 4 inches in diameter. She could use
the ring and little ringers of the right hand for eating her rice, but
the rest were useless. Andre Thomas (7) has reported a curious case
of unilateral enlargement of the left arm and leg and of the right
side of the face and tongue (" hemihypertrophie alterne").

Literature.— {1) Cenas, Loire mkl., ix. 313, 1890. (2) D. E. Jacobson,
Arch. f. path . Anat., exxxix. 104, 1895. (3) H. Friedberg, Arch. f. path.
AnaL, xl. 358, 1867. (4) A. P. Gould, Trans. Path, Soc. Lond., xxxiii.
222, 1881-82. (5) A. Gherini, Contribwdoni alia chirurgia sui bambini,

2G6

ANTENATAL PATHOLOGY AND HYGIENE

3-6, 63-66, 1876. (6) Bo^chat, Congr. period, intermit. d. sc. vied.,
Genrve, v. 691, 1878. (7) A. Thomas, Nouv. iconogr. de la Salpitriere, xiv.
508, 1901. (8) E. Rapin, Nouv. iconogr. de la Salpitriere, xiv. 493, 1901.

Macrosomia. of one Extremity. — A considerable number of cases
may be grouped together here, including those in which the whole of
one limb or a part of it (an arm or leg, a foot or hand, or a digit) is
the subject of congenital hypertrophy. According to Taruffi's

Fig. 40. — Siamese Woman with Bilateral Macrosomia
affecting Upper Extremities.

statistics the upper extremity is more often affected than the lower,
and the left side than the right. Such names as cheiromegaly,
megalodactyly, pes gigas, macropodia, macrodactyly, and sciapody,
have been given to these localised giant growths.

In the cases in which the whole upper limb participated, it was
occasionally found that there was some enlargement of the corre-
sponding part of the chest (Wagner, 1). In several of the recorded
instances, while the whole arm was larger than normal, only two or

MACRODACTYLY

267

three of the fingers showed marked hypertrophy (Beck, 2 ; Horing,
3 ; MacGillivray, 4). Sometimes the macrosomia was limited to
one hand ; and the classical scholar will remember that the Persian
king, Artaxerxes I., was named Longimanns or Macrochir because he
was the subject of this localised form of giant growth. Less ancient
instances have been reported by Guersant (5), F. Wulff (G), Ewald
(7), D. Halderman (8), and Richardiere (9). The commonest form of
localised enlargement in the case of the upper limb is that which
only involves one or two fingers (megalodactyly or macrodactyly).
Fig. 41 represents a skiagram of this type of macrosomia ; I am

Fig. 41. — Case of Macrodactyly.

indebted for it to Dr G. Owen C. Mackness of Broughty Ferry. The
patient in whom the deformity occurred was a boy, two years of age,
and otherwise well formed ; it was present at birth. In this instance
the affected digits were the index and the thumb ; but in the
majority of cases the middle finger is the hypertrophied one (Annan-
dale, 10; Curling, 11; and others). It is not uncommon for the
enlarged digit to be dislocated or partially dislocated, and to lie more
or less crosswise as regards the others. All the tissues of the part
may be involved, and the metacarpal bone may likewise show
enlargement (Piothschild et Brunier, 12). The fingers may be
webbed (syndactyly) as well as enlarged (R. Abbe, 10 ; Begouin et

26 8 ANTENATAL PATHOLOGY AND HYGIENE

Sabrazes, 39), and there may also be a record of syndactyly in some
relative of the patient (Morton, 13). In Cayla's case (14) there was
asymmetry of the face and a certain degree of microcephaly; and
Wahl's patient (15) was insane. The finger which is hypertrophied
at birth may simply grow with the rest of the body ; but it would
seem that more often it increases at a disproportionately rapid rate,
necessitating amputation (Lejars, 17). The examination of the
amputated digit may show interphalangeal osteophytes and anchy-
loses (Planchu, 18) ; and as the head of the corresponding metacarpal
may be similarly affected it is wise to remove it. Hypertrophy of a
supernumerary digit (thumb) has been noted by Kirmisson (18a).

Sometimes it is the lower extremity that exhibits this partial
form of macrosomia. The whole limb may be enlarged (Tesnieres,
19); the enlargement may be associated with vascular naevi on the
affected part (Adams, 20), or, somewhat curiously, on the limb of the
opposite side (Chassaignac, 21); and there may be a great deposit of
adipose tissue, which accounts in some measure for the great size of
the extremity (Little, 22). The foot, again, may alone show hyper-
trophy, as in the cases reported by Busch (23), Albert (24), D'Urso
(25), Piatt (26), Blackader (27), and Allingham (28). In the instance
described by Shoemaker (29), a brother of the patient had a super-
numerary thumb ; but most often, as in Galvani's case (30), there was
no family history of deformities. Finally, to bring to a close this
long list of partial macrosomias, the hypertrophy may affect only one
or two of the toes. The great toe alone may be enlarged (Fuller, 31),
or the second (Boehm, 32 ; Folier, 33), or the second and third
(Pughe, 34), or the first and second (Taulier, 35), or the first and
third (Fischer, 36), or the first, fourth, and fifth toes (ibid). Not
uncommonly, when two toes have been hypertrophied, these digits
have been fused (syndactyly) as well ; and in Carvallo's case (37) the
syndactyly was present at birth but the hypertrophy only appeared
at a later date. Hofmokl's observation (38) was peculiar : in it there
was microdactyly at birth and afterwards hypertrophy of the second
and third toes (macrodactyly) developed. In several other cases the
enlarged digits continued to grow rapidly after birth, necessitating
amputation. When the whole limb or a large part of it has been
affected with macrosomia, ligature of the chief artery has occasionally
been tried to check the growth, but with indecisive results.

Literature. — (1) Wagner, Med. Jahrb. d. Jr. Jc. usterr. Staates, n.R,
xix. 378, 18.39. (2) Peck, Med. Ann., Heidelberg, ii. 89, 1836. (3)
Horing, Med. Corr.-Bl. d. ttriirttemb. arztl. Ver., xiv. 39, 1844. (4)
P. H. MacGielivray, Austral Med. Journ., xvii. 9, 1872. (5) Guersant,
Bull. Soc. de chir. de Paris, viii. 101, 1857-58. (6) F. Wulff, St. Petersb.'
med. Ztschr., i. 281, 1861. (7) A. Ewald, Arch. f. path. Anat., lvi. 421,
1872. (8) D. Halderman, Med. Rec, xxiii. 320, 1883. (9) Richardiere,
Bull. Soc. franc, de dermat. et syjth., ii. 128, 1891. (10) T. Annandale,
Malformations . . . of the Fingers and Toes, pp. 5, 6, 1866. (11)
Curling, Med. Chir. Trans., London, xxviii. 337, 1845. (12) H. de
Rothschild et L. Urunier, Per. d'hyg. et de mid. infant., ii. 232, 1903.
(13) T. K. Morton, Med. News, lxiv. 294, 1894. (14) Cayla, Novo.

PARTIAL MACROSOMIA 269

iconogr. de la Salpetriere, xvi. 41, 1903. (15) L. Wahl, Compt. rend. Soc.
de biol., lv. 595, 1903. (16) R. Abbe, Trans. Neio York Acad. Med., x.
639, 1894. (17) F. Lejars, Nouv. iconogr. de la Salpetriere, xvi. 37, 1903.
(18) Planchu, Lyon med., lxxxiv. 372, 1897. (18a) E. Kiemisson, Traite
de* maladies chirurgicales d'origine congenitale, p. 742, 1898. (19)
Tesnieres, Rec. de. mem. de med. . . . mil., xxix. 385, 1830. (20) J.
Adams, Lancet, ii. for 1858, p. 140. (21) Chassaignac, Bull. Soc. de chir.
de Paris, viii. 452, 1857-58. (22) Little, Trans. Path. Soc. Lond., xvii.
434, 1865-66. (23) W. Busch, Arch. f. Idin. Chir., vii. 174, 1866. (24)
E. Albert, Wien. med. Presxe, xiii. 10, 71, 1872. (25) G. D'Urso,
Pol idin., ii. 353, 1895. (26) Platt, Bull. John* Hopkins Hosp., vi. 49,
1895. (27) A. D. Blackader, Arch. Pediat., i. 626, 1884. (28) H. W.
Allingham, Proc. Med. Soc. Lond., x. 106, 1886-87. (29) G. E. Shoe-
maker, Med. Neirs, lxiv. 296, 1894. (30) Galvani, Per. d'orthop., ix.
421, 1898. (31) K. P. Fuller, South African Med. Journ., vi. 13, 1898.
(32) T. Poeiim, Diss, inaug., Giessen, 1856. (33) C. Folier, Bull. </. sc.
med. di Bologna, 6 s., ix. 19, 1882. (34) R. M. Vvghe,i Illust. Med. News,
v. 78, 1889. (35) Taulier, Mem. et Compt. rend. Soc. d. sc. med. de Li/on,
(1867), vii. 75, 1868. (36) H. Fischer, Deutsche Ztschr. f. Chir., xm 57,
1879. (37) D. Carvallo, EI Progreso med., Chile, vi. 296, 1896. (38)
Hofmokl, Ber. d. k. k. Krankenanst. Rudolph-Stiftung in Wien, p. 406,
1890. (39) P. Begouix et J. Sabuazes, Now:, iconogr. de la Salpetriere,
xiv. 305, 1901.

All these forms of partial macrosomia have a pathogenesis which
is ([iiite obscure. Doubtless they are due not to one but to several
causes. Some of them are to be regarded as the result of foetal
diseases and are to be explained by lymphatic obstruction (Busey, 1)
and lymphangiectasis, or by a diffuse fatty infiltration of the sub-
cutaneous and muscular layers of the part (Duplouy, 2). I have
seen a case of unilateral hypertrophy of the tongue in a young man
shown by Mr. Wallace at the November Meeting of the Edinburgh
Medico-Chirurgical Society (1903) in which lymphatic obstruction
may possibly have been the cause of the enlargement. In other
instances there is a general enlargement of all the structures in the
part, and two theories have been invoked to explain them : the
vascular theory of Trelat and Monod (3) and others which ascribes to
slowing of the capillary circulation an increased nutritive activity of
the tissues ; and the nervous theory which looks to a trophic action
of the special nerves of the part, and in order to explain the dis-
tribution of the hypertrophy has to have recourse to the idea of the
hypothetical metameric arrangement of the body in embryonic life
(Leblanc, 4). It seems to me that the cases of macrosomia in which
all the tissues of the part are involved must be referred to causes
acting before the foetal period ; but while accepting an embryonic
cause, I do not think it necessary to regard it as nervous in its
nature. I look upon the cause rather as a sort of tendency of the
tissues to grow out of due proportion, a tendency present before the
trophic action of the nervous system comes into play. I need hardly
add, for the reader will already have it in his mind, that some of the
cases of partial macrosomia can scarcely be separated from the
neoplasms.

270 ANTENATAL PATHOLOGY AND HYGIENE

Three chief methods of treatment have been tried. First, elastic
compression of the hypertrophied parts maintained for some time ;
second, ligature of the chief artery supplying the enlarged area ; and,
third, amputation of the digit, hand, foot, or entire limb. The first
and second plans deserve a wider trial than they have yet received ;
the third, on the other hand, ought not to be so readily recommended
and so hastily put into practice as has been done in the past, for it is
sometimes followed by the excessive growth of neighbouring structures
(eg. other digits), and a hypertrophied part is sometimes more useful
than none at all, while the aesthetic advantage is dubious. We may
sum up in the words of Kirmisson (5) : " le traitement . . . est loin
jusqu'ici d'avoir donne des resultats brillants."

Literature. — (1) S. C. Busey, Amer. Journ, Obst., x. 1, 223, 420, 571,
1877. (2) Duflouy, Bu71. et mem. Soc. de chir. de Paris, n.s., xii. 343, 1886.
(3) Trelat et Monod, Arch. gen. de med., i. 536, 676, 1869. (4) Leblanc,
Thhe, Paris, 1897. (5) E. Kirmisson, Traite des maladies chirurgicales,
p. 749, 1898.

Partial Microsomia {Mcro-microsomia, Tarutti).

By partial microsomia is meant the disproportionate smallness of
one part of the body as compared with the rest ; it may be one side
of the face or a limb or a digit. It is the converse, therefore, of
partial macrosomia. It is as yet an ill-defined morbid entity ; and
time will be required before it is differentiated from localised atrophies
coming on in infancy due to disease or defective development of the
nervous system {e.g. poliomyelitis anterior) on the one hand, and from
arrested growth of the limbs due to absence of one or more of the
component bones (e.g. femur, radius, tibia) on the other. If these
two groups of cases could be excluded we should then be left with
the true instances of partial microsomia of antenatal origin. It has
been thought that some of them may be caused by poliomyelitis
anterior occurring in foetal life, followed by paralytic club-foot and
wasting of the limb, as in Wharton Sinkler's case (Keating's Cyclo-
paedia of Diseases of Children, iv. 684, 1890), where there was also a
maternal impression in pregnancy. Others, I think, are to be looked
upon as intranatal in origin, and take their place in the group of
obstetrical or birth palsies. But paralysis is by no means a constant
accompaniment of microsomia. Yet others are due, there can be
little doubt, to the pressure of amniotic bands or of the umbilical
cord wound round a limb, as was described in Chapters X. and XL ;
in such cases there may be no paralysis at all.

Further space, however, need not be given to the consideration of
partial microsomia. As a matter of fact dwarfing in size of any part
of the foetus or embryo is usually accompanied by arrest in develop-
ment ; and since the latter is the more striking anomaly it absorbs
attention, and the atrophic state which exists alongside of it passes
almost unnoticed ; the monstrosity or error in organogenesis over-
shadows the dwarfing, which is only an error in growth. A common
instance of this combination of malformation with microsomia is

PARTIAL MICROSOMIA 271

found in syndactyly ; here the fused or webbed ringers or toes are
usually dwarfed in size. The reader will remember that syndactyly
was also seen to be associated with macrosomia ; but this is only
another instance of the way in which both overgrowth and defective
growth may be found along with the same type of malformation.

"With reluctance we must leave this teratological type (partial
microsomia) in its ill-defined state. Some earnest worker will one
day, after much labour and great searching in the literature of the
subject, be able to sort out from among a large number of cases of
structural anomaly those in which true dwarfism of a part of the
body exists independently of defective development of that part ; but
that day is, as yet, far off, and the type must perforce remain dim in
our sight and obscure. Some of the difficulties, however, are even
now capable of being removed and ought to be removed : for instance,
a careful scrutiny of the cases ought to enable us to separate out
those due to birth-injuries from those caused by foetal disease of the
nervous system, and those again from the cases in which morbid
agencies have been at work in the embryonic epoch. Instances of
microsomia of two or three toes and of a whole hand have been
referred to by E. Fournier (Stigmates dystrophiques de VMrtdo-syphilis,
p. 146, 1898), and might be used as the nucleus of a collection of
cases of arrested growth apart from arrested development. Fournier
ascribes them to the effect of the syphilitic poison, but does not in
his description clearly distinguish between arrested growth and
arrested development, a distinction which, I cannot help thinking, is
of considerable importance.

CHAPTER XVI

Teratological Types : Merosomatous Terata (cord.) : Monstrosities affecting the
Spine and Head : Inienceplialy ; Definition ; Illustrative Instances ;
Morbid Anatomy and Clinical History ; Associated Malformations ; Tera-
togenesis ; Literature.

The teratological types which now fall to he considered are those
in which the spine is the region most involved. The reader will
remember that it was decided (in Chapter XIII.) that it would be
most convenient to adopt a regional classification of the monoso-
matous terata ; and, in accordance with that decision, anomalies of
the spinal column and its contents naturally take their place among
the first types. After these have been discussed, I shall describe
the monstrosities in which the head is the part most affected. There
is, however, a very interesting type, known as iniencephaly, in which
both the spine and the head are very constantly associated in mal-
formation ; it is convenient to deal with it first.

Iniencephaly.

In the monstrosity known as iniencephaly (Gr. hiov, nape of the
neck, and lyxsipaXog, the brain), the centre of interest is the nape of
the neck or inion. Through the extreme backward bending of the
head upon the dorsal region of the trunk the nape of the neck loses
its external position and is enclosed within the somewhat globular
mass which is formed by the head and the trunk (Plates XV., XVI.,
XVIL, and Figs. 42-49). In well-marked specimens the nape of
the neck may be described as the centre of this rounded head-body,
from which the limbs stand out as little projections. The mon-
strosity well deserves the epithet of tres curieux given to it by
Isidore G. Saint-Hilaire (1), who wras also the first to describe and
to name it.

I have met with seven specimens of this type (Nos. 25, 44, 162,
225, 251, 252, and 277), a very exceptional experience if the great
rarity spoken of by Saint-Hilaire be a correct representation of the
facts (which I doubt). All these specimens showed very clearly
the three cardinal characteristics of iniencephaly, to wit, imperfect
formation of the occiput in the neighbourhood of the foramen mag-
num, spina bifida of considerable extent, and retroflexion or back-
ward bending of the spine. The reader will best understand the

272

INIENCEPHALY 273

monstrosity if I describe first a specimen showing a minor degree
of it.

The fcetus whose external appearances are represented in Fig. 42
was given me for dissection by Dr. W. 1ST. Elder in 1893, and. was
shown by me at a meeting of the Edinburgh Obstetrical Society in
the same year (2). The noteworthy facts in the clinical history of
the case were that the mother in her first pregnancy had given birth

/

-„

A

\

,V r

"\4

■

\

J /

\ 1 1

v - ' > \

■

- /

■> - ■ - -

Fig. 42. — External Appearances of Female Iniencephalic Fcetns.
Specimen No. 44.

to a female child with a spina bifida, her second and third preg-
nancies had ended in the birth of normally formed male, and her
fourth in that of the female iniencephalic fcetus to be described
immediately. The labour was complicated by hydranmios. The
mother was the younger of twins, and her mother had on two
occasions given birth to twins ; the father's mother had once had
twins.

The foetus measured 30 cms. in length, and the mass formed by

VOL. II. — 18

274

ANTENATAL PATHOLOGY AND HYGIENE

the head and trunk 19 cms. The face was directed upwards, and
the whole head was sharply Hexed backwards, so that the occiput
came into close relation with the open spinal canal in the lumbar
region. The skin of the head, however, was not continuous with
that over the sacrum, and, in this respect, the specimen differed from
the most marked type of iniencephaly. There was no indication of
a neck-groove anteriorly, the skin passing directly from the chin

— ;*>->

Fig. \'i. — Internal Appearances of Iniencephalic F<etus as seen in vertical mesial
section. The parts are named in the Figure. Specimen No. 44.

to the thorax. The abdomen and thorax were prominent, but
showed no defect. The vulva was normal, the anus was patent, and
the limbs, although slender, were well formed.

The arrangement of parts in the interior of the foetus was made
plain by a vertical mesial section (Plate XV., Fig. 43) ; on account
of slight lateral distortion of the trunk the middle line was not found
in the whole of the section, the saw having passed a little to the

Plate

Diffluent
brain.

4 \-Cervical spine.

Tongue.

Basis crani

Thymus.

Umbilicus.

276

ANTENATAL PATHOLOGY AND HYGIENE

The foetal retroflexion is more nearly complete, the vertex of the
head coining into close connection with the lower end of the spinal
column ; and the skin passes directly from the head to the gluteal
region. The extraordinary distortion of the vertebral column is very
evident ; the cervical part lies nearly in the centre of the foetal
mass (fused head and trunk) ; from this point the column passes
downwards and forwards at a short distance, and then turns back-
wards with a downward inclination to traverse the body and end
near the skin surface immediately above the anus. There is no
vertebral canal properly so called, for the spine is open in its whole
extent ; but, on account of the retroflexion, an artificial cavity, which

Fig. 44. — External Appearances of Female Iniencephalic
Foetus. Specimen No. 25,

may be called cephalo-rachidian, is formed. The cephalic and spinal
parts of this cavity communicate freely through the defect in the
squamous part of the occiput. The basis cranii is directed back-
wards, the oral aperture looks directly upwards, and there is no sign
of a neck : these anomalies in construction are evidently associated
with the sharp retroflexion of the whole foetus. Further, through a
defect in the postero-mesial part of the diaphragm, the spleen and
intestines have been dislocated upwards into the thoracic cavity,
where they lie behind the heart. Dissection revealed two small
lungs, two kidneys and supra-renal capsules, and a normal uterus
and annexa.

INIENCEPHALY

277

In a small foetus, which I received in April 1902 from Grimsby,
the external appearances of iniencephaly are again very clearly
shown (Fig. 4G) ; unfortunately no clinical details came with the
specimen. Another small iniencephalic foetus (Fig. 47), a female,
was sent to me by Dr. G. H. Pirie of Dundee in 1895 (4). It was the
product of the tenth pregnancy of a woman, forty-two years of age,

as -

J

Fig. 45. — Internal Appearances of Iniencephalic Foetus, as seen in a sagittal mesial
section. B., brain ; C, umbilical cord ; C.V., cervical vertebra ; h., heart ; %.,
intestine ; La., larynx ; Li., liver ; L.K., left kidney ; L.S.C., left supra-renal
capsule; in., mouth; n., nose; O.S., occipito-spinal space; R., rectum;
Sp. , spleen ; St. , sternum ; t. , tongue ; Thyr. , thyroid gland ; Thym. , thymus
gland; V. , vagina.

who had had eight full-time confinements and one previous mis-
carriage. The age of the pregnancy was between the sixth and
seventh month, when the whole contents of the uterus came away
intact. On opening the amniotic sac it was found that there was
about a pint of thick liquor amnii of a yellow colour, and in it was

278

ANTENATAL PATHOLOGY AND HYGIENE

the small iniencephalic foetus attached by a cord 18 inches long.
The footus was macerated, and had apparently been dead for over a
month. When lying upon a plate, the monstrosity had sufficient
resemblance to a frog to justify the comparison (Fig. 47). Yet
another iniencephalic fostus came into my hands in May 1900 ; it

k

Fig. 46. — External Appearances of small Iniencephalic Foetus.
Specimen No. 277.

occurred in the practice of Dr. Murray Cairns of Liverpool, and was
the cause of a prolonged labour.

Two specimens, showing in an even more marked fashion the
chief characters of iniencephaly, were examined by me by the
sectional method in 1890 and 1900. One of these (Specimen No. 251)

Plate

Anterior^
fontanclle.

Cranial,
defect.

f I Thymus.

Intestines

Tongue.

Hyoid.

Cervical
vertebra.

Thyroid.

Uterus

Vagina.

280

ANTENATAL PATHOLOGY AND HYGIENE

i

1

Fig. 48. — External Appearances of Iniencephalic Foetus. Specimen No. 251.

Plate

Tongue.

Thyroid.

Thymus.

Heart. 1

Liver.

Umbilical vein.

Umbilical cord.

A nterior
fontamlle.

Defect in
cranium.

Spinal cord.

Rectum.
Bladder.

INIENCEPHALY

281

This specimen, therefore, had an unusual interest, for it exhibited
iniencephaly unassociated with an extensive spina bifida, and pre-
sented the extraordinary picture of two separate portions of the
brain lying posterior to the spinal cord in the cervical and upper
dorsal region. The external appearances of this foetus are repre-

sented in Fig. 49.

Fig. 49. — External Appeai-ances of Iniencephalic Fcetus with Placenta.

Specimen No. 225.

The reader will have gained some ideas of the morbid anatomy
of iniencephaly from the descriptions of cases which I have given
above. Let me note some additional points gathered from other
records. The neck-groove is generally absent both anteriorly and

282 ANTENATAL PATHOLOGY AND HYGIENE

posteriorly, but, sometimes, as in Lop and Pujol's specimen (5), it is
indicated in front. In this case, also, there was not simply retro-
flexion of the head on the trunk, but the occipital bone was actually
fused with the vertebral column at the level of the seventh dorsal
vertebra ; most of the cervical vertebra' were absent, and, through
the bifurcation of the upper part of the dorsal portion of the spine
and its fusion with the occiput, the first six pairs of ribs seemed
to arise from the basis cranii. H. F. Lewis (6) collected together
twenty-two cases of iniencephalus, including three described by
himself. He divided them into two groups. In the first group were
the cases in which the cephalo-rachidian cavity formed by the bend-
ing back of the head was complete, and contained the whole brain ;
this type Lewis termed iniencephalus clausus. To the second group
he gave the name of iniencephalus apertus, and included in it all the
specimens in which there was a small or large encephalocele, some of
the cranial contents lying outside the cephalo-rachidian space. In
this group we see the curious spectacle of part of the brain lying
not only outside the proper cranial cavity but actually outside the
new cavity formed by the cranium and the open spinal canal. One
of Lewis's specimens was a male twin ; nearly all the others seem to
have been females and the result of single pregnancies. In a majority
of the cases the mother was a primipara ; but this is probably an
accidental result, which more extended statistics might reverse.

The defect in the occiput varied considerably in individual in-
stances. In Leith Napier's specimen (7), the basi-occiput as well as
the squamous part was absent, and the basi-sphenoid bounded the
much enlarged foramen magnum anteriorly ; in Eeinfry's case (8),
however, the basi-occiput and the ex-occipitals were developed, while
the squamous part was represented by two crescentic curved plates
of bone with a large opening between them. In Ptemfry's case, also,
the curious anomaly was observed of the origin of part of the
gluteus maximus (of the left side) from the left upper part of the
defective occiput. A good diagrammatic sketch of the relation of
parts in a typical iniencephalus is given by W. Hoffmann (9) ; in
this instance the two rudimentary bones representing the occiput
were separated by a distance of 4 cms. The spinal defect varied
considerably in extent, being limited in some instances (as in my
specimen No. 225, Plate XVI I.) to the cervical region, and in others
affecting the whole column. In most of the cases some vertebrae are
absent. The altogether peculiar character of the spinal defect in
iniencephaly is that the open canal produced by the want of develop-
ment of the lamina' and spinous processes is closed in again by
the retroflexed head and the skin covering it and extending to the
sacrum.

Other malformations may be associated with iniencephaly : dia-
phragmatic hernia would seem to be one of the commonest of these ;
exomphalos, umbilical hernia, hydrocephalus, cyclops, horse-shoe
kidney, and club-foot have also been noted.

Attempts to explain the origin of iniencephaly have, as usual,
followed the lines indicated in the chapters on Teratogenesis. Eefer-

TERATOGENESIS OF INIENCEPHALY 283

ence has been made to maternal impressions by some writers, to
pressure, uterine or intra-uterine, by others, and to the amnion by-
yet others. Amniotic pressure is probably the active cause ; but in
order to understand the modus operandi, it is necessary to take into
account the normal mode of development of the head and spine of
the embryo. If the reader will refer back to Chapter III. and look
at Fig. 12 (p. 34), lie will see that there is a stage of ontogenesis,
corresponding to the third week, in which there is a normal retro-
flexion of the embryo corresponding to that found in iniencephaly.
Under ordinary circumstances, that embryonic retroflexion is quickly
replaced by the attitude of the fourth week (Fig. 13) ; but it is com-
paratively easy to understand how, if the bending of the spine be
fixed, the iniencephalic state is produced. Let us suppose, for
instance, that uterine pressure or more probably amniotic pressure
(due to non-development of the amnion) is brought to bear upon
the retroflexed embryo : the spinal curves will be retained and
exaggerated ; as the head develops, the occipital part will find its
expansion hindered by the adjacent spine and the spine will have its
closure posteriorly checked by the occipital part of the head, and in
neither of these parts will ontogenesis be able to pass on to com-
pletion. It is quite possible that retroflexion may be produced at
other periods in embryonic and ftjetal life, and it may then be due to
a short umbilical cord or to the entire absence of the cord (Duncan
and Hurry, 10); but the anomaly so caused will not be iniencephaly.
If the view that this monstrosity is due to the retention and
exaggeration of a state of matters which is normal at the third week
be true, then we ought to find in early abortion-sacs some evidence
of it. This evidence, I think, we do obtain in such a specimen as
that of C. B. Lock wood (11), which was regarded as about fifty days
old and which showed retroflexion of the body and defective develop-
ment of the cartilaginous spinal column.

It must, of course, be remembered that retroflexion of the foetus
and iniencephaly are not one and the same monstrosity : there may
be retroflexion of the spine with no other anomaly, the foetus being
born with the head bent back and the limbs also lying behind the
trunk, but with no other malformation (12) ; or there may be
anencephaly with retroflexion (Fig. 28, p. 173), and this may or may
not be conjoined with exomphalos ; or, again, there may be exom-
phalos, without anencephaly, and with retroflexion (13). It is the
defective state of the occiput and the condition of spina bifida,
which, together with the retroflexion of the whole embryo, produces
iniencephaly. Which of these anomalous states is primary does not,
I think, matter very much : there is a stage in development when
they are all normally present, and this stage, which ought to be very
transitory, is permanently fixed and maintained in iniencephaly.
Further ontogeny is hindered and cramped, and so the associated
malformations {e.g. diaphragmatic hernia) arise.

Literature. — (1) I. G. Saint-Hilaire, Histoire des anomalies, ii. 310,
1836. (2) J. W, Ballanttne, Trans. Edinb. Obst. Soc, xviii. 226, 1893;

284 ANTENATAL PATHOLOGY AND HYGIENE

Teratologia, ii. 88, 1895. (3) J. W. Ballantyne, Teratologic, ii. 287,
p ^ 1895. (4) J. W. Ballantyne, Trans. Edinb. Oust. Soc, xxi. 55, 1896.

(5) Lop et G. Pujol, Rev. obstet. internal., ii. 145, 1896. (6) H. F. Lewis,
Amer. Journ. Obstet., xxxv. 11, 1897. (7) L. Napier, Trans. Obst. Soc
Lond., xxxvi. 116, 302, 1895. (8) Remfry, Tran*. Obst. Soc. Land., xxxvi.
227, 1895. (9) W. Hoffmann, "Zwei Falle angeborner Difformitat,"
Inaufj.-Diss., Erlangen, 1883. (10) J. j\I. Duncan and J. L\ Hurry, Trans.
Obst Soc. Lond., xxvi. 2-06, 1885. (11) C. B. Lockwood, Trans. Obst. Soc.
Lond., xxix. 234, 1888. (12) R. M. Murray, Trans. Edinb. Obst. Soc,
vii. 59, 1882. (13) A. R. SmrsoN, Trans. Edinb. Obst. Soc, vii. 80, 1882.

CHAPTER XVII

Merosomatous Terata (cont.) : Malformations affecting the Spine ; Spina Bifida
or Rhachisehisis ; Synonyms ; Varieties; Holo-Rhachischisis ; Mero-Rhachi-
scliisis ; Spina Bifida Occulta ; Localised Rhachisehisis without a Sac ;
Spina Bifida with Rachicele ; Morbid Anatomy ; Teratogenesis ; Treatment;
Bibliography.

Spina Bifida (Rhachisehisis).

As the outstanding feature in the ontogenesis of the spine is the
gradual closure of the medullary canal to form the central canal of
the spinal cord and the gradual growth round the closed cord of
mesenchymatous masses (derived from the sclerotomes of the meso-
dermic somites) to build up the vertebral column ; so the commonest
and most typical teratological state of the spine is an open condition
of the vertebral canal, and sometimes also of the spinal cord, the
result of the imperfect performance of this piece of organogenesis.
The details of the process of chondrification of the sclerotomes to
form the cartilaginous framework of the spine are not well known in
the human .subject; this is doubtless the reason why there are some
parts of the teratogenesis of spina bifida which are obscure. Speak-
ing generally, however, it may be said that the backward and inward
growth of the laminae (of one or of several vertebraj) is arrested and
a wide or a narrow space is left unclosed in the posterior wall of the
spinal canal. Through this opening, as a rule, a larger or smaller
portion of the spinal contents (membranes and cord, in a more or
less altered state) protrude, to form a tumour in the dorsal region.
Such is, stated very generally, the idea that is to be formed of spina
bifida and its mode of origin.

Synonyms.

In the Obscrvationes Mcdiccv of Tulpius (1) there is the record of a
case of " Spina dorsi bifidi," which from the accompanying description
and illustration was evidently an example of lumbo-sacral rhachisehisis ;
the name " spina bifida " thus given to the anomaly some two hundred
and sixty years ago is still that by which the malformation is best
known at the present time. The term, in its etymological sense,
emphasises the state of imperfect closure of the vertebral canal ; but in
common use it has come to be applied also to the tumour or swelling
(consisting of the spinal membranes with or without the cord) which

285

286 ANTENATAL PATHOLOGY AND HYGIENE

projects through the opening. These two anatomical characters, the
divided spine and the protruding swelling, are found in all the
synonyms which have been proposed. " llhachischisis " (2), for
instance, emphasises the former character ; while " spinal hernia "
and " rachicele " direct attention specially to the latter. The term
" hydrorrhachis " refers to the fluid contents of the sac ; and the
names meningocele, myelo-meningocele, and myelo-cystocele indicate
the component parts of the sac. The defect in the spine and the
protrusion of the spinal contents through it are commonly but not
constantly associated : there may thus be spina bifida or rhachischisis
in the strict sense of the words without rachicele or spinal hernia,
and to this kind of case the name spina bifida occulta has been
given ; on the other hand, a hernial protrusion which at one time has
been in communication with the interior of the spinal canal may
afterwards be cut off therefrom, and so give rise to a tumour which
is often classed simply as a " sacral cyst."

Varieties.

In naming the synonyms of " spina bifida," I have incidentally
referred to some of the varieties of the malformation and to some of
the structural peculiarities of these varieties ; but it is necessary now
to consider these matters more in detail. The following classification
of the varieties may be adopted : —

I. Holo-Rhachischisis or Total Spina Bifida : the whole canal is widely

open and there is no sac.
II. Hemi-Rhachischisis, Mero-Rliachischisis, or Partial Spina Bifida.

A. Spina Bifida Occulta or Crypto-Mero-Rhachischisis.

B. Spina Bifida without Spinal Hernia : the canal is Avidely

open at one or more places, but there is no sac.

C. Spina Bifida with Rachicele (common variety).

1. Meningo-myelocele (common sub-variety).

2. Meningocele.

3. Myelo-cystocele, Syringomyelocele, or Myelocele

(Taruffi).

Not a little difficulty has been introduced into this subject by
want of care in the naming of the varieties ; hasty identification of a
given case with one described by another writer in another language
is perhaps in large measure responsible for this. Bland-Sutton
(3), for instance, applies the term " myelocele " to a condition differ-
ing (as it seems to me) from that described by Taruffi (4) under that
name ; Taruffi, again, blames the English writers for giving the name -
" syringo-myelia " to cases of " myelocele," while the word they really
used was " syringo-myelocele." Some of the confusion thus intro-
duced is, I fear, almost irremovable ; I can only endeavour not to
add more to it. I shall describe each of the varieties of spina bifida
named above, but I shall group most of the information round that
known as meningo-inyelocele, for that is, as it were, the central
type.

SPINA BIFIDA 287

Holo-Rhachischisis.

Under the name " Holo-Rhachischisis " may be conveniently
grouped all these cases in which the whole spinal canal from end
to end is open, in which all the vertebral arches are imperfectly
developed. According to Taruffi, the first case of this kind to be
described was that reported by P. Zacchia in 1661 (5), and since then
many specimens have been noted. As a general rule, this widespread
defect of the vertebral arches is conjoined with anencephaly, the
cerebro-spinal canal being then open from end to end (vide
Plate XXL, Fig. 50). There are a few exceptions to this rule, in
which, although the spinal part of the canal is open, yet the cranial
part is closed (save only in the occipital region) ; and these ex-
ceptional cases have been described in the previous chapter (vide
F'ig. 45) under the name of " iniencephaly." They are really
instances of spina bifida occulta in its most extreme form. But the

•"■ ■■ '■" ','^'

Fig. 50. — External Appearances of Foetus with complete Spina Bifida and Anencephaly.

Specimen No. 6.

common type of holo-rhachischisis is that associated with anen-
cephaly. In it, as may be seen in Plate XXI. and also in Fug. 50,
the open spinal canal is continuous with the broad gutter or furrow
on the basis cranii. The total number of vertebral bodies is generally
diminished, and those in the cervical region show marked lordosis.
The vertebral arches not only fail to unite posteriorly, they are
turned outwards and so produce the wide groove or gutter which
traverses the middle line of the back. This gutter is covered, it is
stated, with the ventral portion of the dura mater, and possibly also
with the corresponding part of the pia mater ; in it may be found
some strands of nerve tissue representing the spinal cord or a bifid
cord in an atrophic state. Traces of the ganglia and spinal nerves
may be found. Sometimes the bifid condition of the spine may
extend to the bodies as well as to the arches, as in Lallemand's case
(6) in which the first thirteen vertebra3 were thus divided, the
oesophagus finding lodgment between them. This was also the state
of matters in the case reported by Cleland (7). It was one of holo-

288 ANTENATAL PATHOLOGY AND HYGIENE

rhaehischisis and anencephalus in an embryo measuring § inch in
length ; there were ragged and torn walls round the open neural canal,
which Cleland interpreted as the burst walls of a dropsical sac and re-
garded as confirmation of the view that anencephaly and spina bifida
are due to dropsical distension of the cerebro-spinal tube. They are
capable of this interpretation; but they may also be explained as
due to adhesions to the amnion or chorionic sac, for the early embryo
described by Knoop (8) was attached to the chorion by its back.
These questions, however, are more properly considered later, as are
also the appearances, naked-eye and microscopic, of the spinal cord
in such cases.

Mero-Rhachischisis.

Under the names " Mero-rhachischisis," " Hemi-rhachischisis,"
" incomplete spina bifida," and " localised spina bifida " are grouped
all the cases in which the defective development of the vertebra?
affects only a part and not the whole of the spinal column. These
malformations are of more general interest, for they are not incom-
patible with postnatal existence, and some of them indeed may
only be accidentally discovered in adults ; holo-rhachischisis, on the
other hand, prevents any but the most fleeting extrauterine life.
It is also a comparatively common anomaly, occurring about once
in a thousand births. If club-foot be reckoned as the most frequent
of all the malformations, then spina bifida may perhaps be placed
second in frequency.

In mero-rhachischisis the defect in the spinal canal may be un-
accompanied by any protrusion of its contents, or it may be asso-
ciated with a more or less marked swelling, consisting of the
membranes of the cord or of the cord itself, or of both. In the
cases in which the anomaly is unaccompanied by a swelling, the
aperture in the spinal canal may be small and the skin and sub-
cutaneous tissues may bridge it over and leave no external sign of
the defect ; or, on the other hand, the canal may be completely open,
presenting simply a groove or gutter. To cases belonging to the
former category the name of spina bifida occulta has been given ;
while those of the latter group may be termed localised rhaehischisis
without hernial protrusion. A short description of these two types
of mero-rhachischisis must suffice.

Spina Bifida Occulta.

Masked spina bifida and crypto-mero-rhachischisis (Taruffi) are
names which have been given to the cases in which there is no
obvious external evidence of the defective state of the vertebral
canal : there is no solution in the continuity of the skin covering
the imperfectly ossified area, and there may be no swelling. This
peculiar anomaly seems to have been first definitely recognised by
Virchow (9) in 1875, although there were records of cases of it
anterior to this year (10, 11, 12). Many instances have been re-

SPINA BIFIDA OCCULTA 289

ported in recent years. In some of the cases it was found that the
skin covering the defect in the spinal canal carried a tuft of hairs
often of considerable length (14, 15, 1G, 17, 18, and 10); but although
this association is common it is not constant, for in A. H. Dodd's
case (20) there was no spina bifida and yet there was marked lumbar
hypertrichosis, and in L. Mayet's patient (21) there was no sign of
any defect of the vertebrae. On the other hand, there may be spina
bifida occulta without hypertrichosis, as in the instances noted by
Kirmisson (22), Bohnstedt (2.'}), and others. At the same time, the
discovery of lumbar or sacral hypertrichosis ought always to lead to
a careful examination of the underlying part of the spinal column.
In other forms of spina bifida, there is also a tendency to the develop-
ment of hairs, and then they are usually arranged round the sac.

Spina bifida occulta commonly affects the lumbar part of the
spine, but it may also be met with in the sacral region or in both
lumbar and sacral regions. In quite exceptional cases it is situated
in the dorsal region (as in one of Bland-Sutton's specimens, 24).
The skin surface over the spina bifida may show either a depression
or dimple or a slight elevation ; and it may, as has been stated, be
covered with hairs, often of considerable length and sometimes
extending to the neighbouring parts (buttocks, loins). The opening
in the spine may be quite palpable, and may involve the spinal
processes and laminae of three or four vertebrae ; or it may be little
more than a small fissure in the arch of one vertebra, when it may
easily be passed over unobserved. In a case reported by Taruffi (25)
there was no defect in the bone, but the ligamentous structures were
absent. When there is an aperture in the arches, it is commonly
closed by fibrous membrane, but the closure may be incomplete,
allowing the subcutaneous tissue and fat to enter the canal. Further,
a pad of fat may lie over the opening and conceal it ; and in Lind-
say's case (26) the diagnosis between spina bifida occulta and con-
genital lipoma remained doubtful. The spinal cord may be length-
ened, reaching a level in the spine which it usually attains only in
embryonic life (the tip of the coccyx in Bland-Sutton's case, 24) ;
and opposite the defective arches and spines the central canal of the
cord may be dilated, but there may be a degree of meningocele as
well as of myelo-cystocele.

Some interesting anomalies in the vertebrae adjoining the defective
ones may be met with. Half vertebra?, for instance, have been
found ; and curious false joints in the laminae behind the position of
the future articular processes have been discovered (24). The whole
spine, also, may show marked curvature, lordosis, or, more rarely,
cyphosis; and the spinal canal may be wide (23). The coccyx may
be shortened or absent (22). Club-foot is a not uncommon compli-
cation ; and the association of this anomaly with lumbo-sacral spina
bifida occulta and a tuft of hair in the position of a tail has sug-
gested to Bland-Sutton (3) that perhaps the idea of fauns and goat-
footed satyrs had origin in the observation of such malformations.
Other concomitant teratological states may be exomphalos (11),
atresia ani, pharyngeal imperforation, absence of one kidney, fibrous
VOL. ii. — 19

290 ANTENATAL PATHOLOGY AND HYGIENE

changes in the appendix vermiformis (24), congenital dislocation of
the hip (18), and dermoid tumours in the neighbourhood of the
spinal defect or a fibro-myo-lipoma in the canal itself (Reckling-
hausen).

Unlike other varieties of spina bifida, the occult type does not
prevent postnatal existence ; but some paralytic phenomena and some
atrophy of the lower limbs may result, and in adult life perforating
ulcers of the feet or buttocks (66) may develop and lead to anxiety.
Thus, in a case reported by Mr. Jones (27), the patient showed no
abnormal signs till the age of seventeen years; then he developed
paralytic symptoms and perforating ulcers of both feet ; the spine
was trephined in the region of the second sacral vertebra to relieve
the pressure on the cauda equina due to a fibrous band, and the
result was satisfactory. It is seldom, however, that operative inter-
ference is required in spina bifida occulta (28).

The theory of the causation of the masked variety of spina bifida
can hardly be considered apart from that of rhachischisis in general
(q.v. p. 302) ; but it may be stated here that there is some evidence
that spina bifida occulta is really an ordinary spinal meningocele or
myelo-cystocele which has undergone Nature's cure during intra-
uterine life. It is thought that the meningeal protrusion has gradu-
ally re-entered the spinal canal, and cicatricial tissue partly or wholly
closed the aperture, and this is not out of keeping with the state of
the spinal membranes (absence of dura mater) and the presence of a
cutaneous cicatrix in some instances. At the same time it must be
remembered that great obscurity surrounds all the processes of intra-
uterine repair, and especially with regard to the power of the parts
to form structures at a late date which ought to have been developed
early. Much has been written and speculated regarding the cause
of the frequent association of hypertrichosis with this form of spina
bifida, bat without shedding any appreciable light; and the same
must be said of the hypotheses advanced to explain the heterotopic
presence of various tissues (muscle, fat, etc.) within the spinal canal
(13, 23). Both these matters, however, seem to me to point to
marked vitality of the surrounding structures, although the vitality
has anomalous effects ; herein may lie a hint as to the nature of the
reparative processes.

Localised Rhachischisis (without Hernial Sac).

In Holo-Ehachischisis the spinal canal was open from end to end,
and showed very rudimentary traces of either the cord or its mem-
branes ; the type at present being described resembles it in the com-,
plete openness of the canal and in the state of its contents, but the
anomaly is localised in extent, affecting only one region of the back.
Both types are generally associated with grave cranial defects, such
as anencephalus or mero-acrania. The spinal defect may be in the
cervical region, and then there is produced the variety of anencephaly
known as derencephaly ; the spines and laminae of the cervical
vertebra? are absent, the bodies are exposed, and the cord begins in

LOCALISED RHACHISCHISIS

291

the dorsal region. Again, the canal may be closed in the dorsal
region and open in the lumbar or lumbo-sacral (Fig. 51). It may,

Fig. 51. — External Appearances of Foetus with Lumbar Spina Bifida, Mero-acrania,
Caudal Projection, and Anchylosed Joints. Specimen No. 113.

however, sometimes be difficult to determine whether in these cases
a hernial protrusion may not have existed and then disappeared,

292 ANTENATAL PATHOLOGY AND HYGIENE

being ruptured in labour. At any rate, the malformation is not
compatible with postnatal life. I have met with several instances of
it in association with anencephaly (vide Plate XXIL), generally in
the cervical region but sometimes in the lumbar ; in one case
(Fig. 51) I found it coexisting with defective ossification of the
anterior and upper part of the cranium.

Spina Bifida with Rachicele.

Spina Bifida with Eachicele (hernial protrusion of the contents of
the spinal canal) now falls to be described. It is the form which is
usually meant when the name Spina Bifida is used without qualifica-
tion. It is also compatible with a certain length of life after birth,
and it is often made the subject of surgical interference. It has,
therefore, a high importance and calls for a detailed description.
There are three varieties of it, according to the composition of the
rachicele or hernial protrusion : these are myelo-meningocele, men-
ingocele, and myelo-cystocele. There are also varieties according to
the region of the spine affected (cervical, dorsal, lumbar, or sacral),
and according to the part of the circumference of the spinal canal left
open (posterior, lateral, anterior). The whole subject is a large one,
and I must economise time and space ; I shall, therefore, try to con-
centrate the attention of the reader upon its antenatal aspects,
leaving the description of clinical postnatal phenomena and com-
petitive plans of operation to the Surgery Text-books. For more
elaborate details of the pathological characters of the malformation
reference may profitably be made to the classic articles of W.
Koch (29), F. von Becklinghausen (13), and to the Eeport of the
Committee of the Clinical Society of London (30).

Morbid Anatomy.

In presenting the morbid anatomy of Spina Bifida I shall first
deal with the structural peculiarities of the vertebral column, then
with those of the contents and of the hernial protrusion, and finally
with the commonly associated malformations in the cerebrum and
elsewhere.

1. The Spine. — The region most often affected with spina bifida,
as Buy sch (31) pointed out long ago, is the lumbar or lumbo-sacral,
and next in order of frequency is the sacral; in about 77 per cent,
of all cases the defect is either in the lumbar or sacral region
or in both. Far less frequently is cervical spina bifida met with
(it is even doubtful whether some of the cases of so-called cervical
spina bifida were not, after all, instances of occipital meningocele) ;
and dorsal, dorso-lumbar, and cervico-dorsal specimens are the
rarities of Museums. The reason why the lumbo-sacral region is the
commonest site of spinal fissure is probably the ontogenetic one,
that there the medullary groove is latest in closing to form the
neural canal. The 8 mm. human embryo described by Tourneux
and Martin (32), in their important article, had a neural canal still

SPINA BIFIDA 293

open in the lumbo-sacral region, and was regarded by Dareste (33),
as an early instance of spina bifida without a rachicele ; Tourneux
and Martin, however, thought that if the embryo had lived on, the
hernial sac would have formed ; but the value of the specimen
resided in the presence of the open canal in that position in so young
an embryo.

In the great majority of cases the defective closure of the spinal
canal is found in the middle line posteriorly ; in two or more verte-
brae the spinous processes are absent, and the halves of the laminae
do not meet, or are actually directed outwards, and therefore away
from each other. " In extreme cases they are so everted as to lie in
a transverse vertical plane " (30). Sometimes, however, the osseous
defect is situated laterally ; 1\ Hewett (34), Taruffi (35), and others
have recorded instances of this, but it is rare. In Hewett's case it
was associated with hydrocephalus and in Taruffi's with exomphalos ;
and in 1896 Mr. Cotterill (36) brought under my notice a boy, nine
years of age, suffering from a spina bifida which protruded laterally
through the sacrum through an opening opposite the right sacro-
iliac synchondrosis, and showing in addition a number of minor
malformations (congenital hydrocele and inguinal hernia, non-descent
of testicle, etc.). Sometimes, again, the hernial protrusion may take
place between the arches of two successive vertebrae, e.g. the fifth
lumbar and the first sacral (37), or the seventh cervical and the first
dorsal (38); this variety has been termed interstitial spina bifida.
Yet again, the spinal defect may be situated anteriorly, affecting the
bodies of the vertebra' ; it may then be conjoined with posterior
spinal deficiency, as in the case of Tulpius (1), or may exist alone,
as in Kroner and Marchand's specimen (39). Anterior spina bifida
existing alone may easily lead the surgeon into diagnostic and
therapeutic error, as in the case (39) just referred to, where an intra -
pelvic tumour (a sacral meningocele) in a young woman was re-
garded as a cyst of the broad ligament, and was twice tapped, with
a fatal issue. In Emmet's case the sac resembled an ovarian
cyst (40).

The vertebral column may show structural peculiarities other
than those observed in the defective spinous processes, laminae, and
bodies. Certain vertebrae may be altogether wanting (Hauner, 41) ;
or there may be half vertebrae, causing lateral distortion of the spine
(Specimens 25 and 26 reported on by the London Committee, 30) ;
and, perhaps in consequence of these anomalies, there is shortness
of the whole vertebral column. A very striking and suggestive
anomaly that has occasionally been found in spina bifida is the pre-
sence of osseous or cartilaginous processes from the posterior surface
of the bodies of the vertebrae, which project into the spinal canal,
and completely, or more often incompletely, divide it into two com-
partments. This condition may be accompanied by a more or less
complete division of the spinal cord into two halves, each with a
central canal (diastematomyelia). Instances have been recorded by
Cruveilhier (42), Boullard (43), Houel (44), Humphry (45), the
London Committee (30), Eecklinghausen (13), Cleland (46), Taruffi.

294

ANTENATAL PATHOLOGY AND HYGIENE

(47), and others. The anomaly may be associated with the presence

of two centres in the body of
one vertebra (30). One of these
interspinal osseous projections is seen
in Fig. 52, which represents the
vertebral column of a case of spina
bifida and commencing hydrocephalus
examined by me in 1899. The exact
significance of such growths is at
present uncertain, but I have called
them "suggestive," because of their
possible bearing upon the causation
of spina bifida (q.v.).

2. The Hernial Protrusion {Rachi-
ccle). — In each case of spina bifida
there is commonly one hernial sac
alone, and that sac has only one
cavity ; to the latter rule there are,
however, exceptions, and multilocular
sacs have been noted by the London
Committee (30) and others ; in them
the loculi communicated by small aper-
tures. The swelling is usually rounded
in form resembling a tomato (Fig. 53),
but it may also be oval with the long
axis running vertically ; sometimes it
is slightly flattened, and when it is
multilocular the internal subdivisions
may be indicated externally. A slight
depression or umbilicus on the out-
side may point to the insertion of the
spinal cord on the inside. In size the
sac may be that of a pea or a bean,
or that of a child's head or even larger
{e.g. 25 inches in circumference) ; all
intermediate dimensions may be met
with. It may be sessile or pedun-
culated, and its pedicle may be broad
or, less often, narrow. It may be
covered by skin, but this is rather
rare ; the coverings usually consist of
the more or less altered meninges,
the skin stopping at the base of the
swelling. When the skin is present,
it may be thick and vascular and
provided with a considerable adipose
layer ; and it may exhibit a ring of
hairs or be surmounted by a tuft of
them. Occasionally the skin is thick-
ened and leathery (34), and sometimes the subcutaneous tissue is of a

Fig. 52. — Spinal Column and Cord
in a case of Spina Bifida (Speci-
men No. 233), showing new
growth from third lumbar
vertebra into spinal canal,
which consisted of bone and
cartilage.

RACHICELE

295

case (48), and in one
Generally, the skin,

myxomatous type, as in Matthews Duncan's
examined by Kirmisson and Berger (22).
when it is present, is thin-
ned out ; and Beringier and
Chambard (49) have given
details of its histology, among
which the disappearance of
the subcutaneous fat and of
the hair follicles and sweat
glands is a conspicuous fea-
ture. When the cutaneous
covering gets so altered it
becomes difficult to distin-
guish it from the dura mater ;
and when the sac -wall is
purely meningeal it may
have an almost transparent
appearance. This leads me
to touch upon the most diffi-
cult point in the morbid
anatomy of Spina Bifida, the
composition of the sac-walls
and sac, a matter deserving
a new paragraph.

The sac may consist sim-
ply of the spinal membranes
(spinal meningocele). This
form I describe first, not
because it is the commonest
but because it is the sim-
plest ; as a matter of fact it
is a rare variety, representing
about 8 per cent, of all spinal
hernias (30). It is a local
hernia of the membranes ;
the spinal cord itself is, as a
rule, normal in position, but
if the osseous defect be large
there may be some prolapse
of the cord or of its termina-
tion (cauda equina) into the
sac. It probably more often
occurs in the sacral than in
the cervical region, for some
of the recorded cases classi-
fied as cervical in position
were no doubt occipital and
not spinal meningoceles. It is exceedingly rare in the dorsal, and
is only occasionally met with in the lumbo-sacral region. I have
met with two instances of it, both in the sacral region (Figs. 29

Fig. 53. — External Appearances of Foetus with
Lumbar Spina Bifida and Club Feet.
Specimen No. 233.

296

ANTENATAL PATHOLOGY AND HYGIENE

and 54) ; when it is so situated the sac may protrude from the sacral
hiatus and the coccyx be split into halves. The sac may be of all
sizes, and it is not uncommonly very large when sacral in position.

Fig. 54. — External Appearances, posterior view, of Foetus with Sacral Spina Bifida,
and Exoiuphalos and Ectopia Vesicae. Specimen No. 200.

Its wall has been regarded as composed of the dura mater and the
arachnoid ; but Recklinghausen (13) and Muscatello (50) both be-
lieve it to consist of the arachnoid alone, often somewhat thickened.

MYELO-MENINGOCELE 297

The whole sac may be subcutaneous, or there may be a skinless
area on its convexity ; the membranes (or membrane) may be
adherent to the cutaneous covering. The inner aspect of the sac-
wall has the appearance of a serous membrane.

In the majority of cases of spina bifida, however, the hernial pro-
trusion contains in its wall the flattened-out spinal cord itself as well
as the membrane {myelo-meningocele). The fluid lies not between the
membranes as in the former variety, but between the cord and its
envelopes. The cord is commonly found flattened out in the
posterior wall (Fig. 52) ; and that the cavity is not due to dilatation
of the central canal of the cord is proved by the discovery of that
central canal in an undilated state in the substance of the posterior
wall of the sac (30). The spinal nerves pass forwards across the
interior of the sac to the intervertebral foramina. Tourneux and
Martin (51) have described the spinal cord passing obliquely from
above downwards and from before backwards to penetrate the wall of
the sac and to find lodgment between the membranes (arachnoid and
dura) and the more or less altered skin; but Recklinghausen (13) has
investigated very thoroughly a more common arrangement of parts.
This latter observer has not found either the membranes (arachnoid
and pia) or skin at the apex of the spina bifida sac ; there he discovered
only exposed spinal cord, or rather its remnants continuous with the
cord above and with the filum terminale below. Myelo-meningo-
celes are common in the luinbo-sacral region, and according to Reck-
linghausen they exhibit at the apex a reddish velvet-like area (vasculo-
niedullary) made up of numerous blood vessels with nerve fibres and
cells supported by the ventral pia mater ; at the upper pole of this
area is a small oblique funnel-shaped depression (infundibulum)
marking the point of insertion of the cylindrical spinal cord within
the sac; round the vasculo-medullary area is a thin smooth /one
(epithelio-serous) formed by the pia mater and not covered by
remains of nerves but by an epithelial stratum with a thin epidermic
layer ; and round this zone, again, is the skin of the back. The three
zones are seen in Fig. 53, and a good coloured plate showing these
areas is given by Bockenheimer (78). All myelo-meningoceles do
not, however, show these characters. In some instances there is no
velvet-like medullo-vascular area but only a smooth surface, like a
congested serous membrane, constituted by the exposed pia mater
with no nerve remnants, and to be accounted for perhaps by the
action of the liquor amnii (13) or by the great degree of distension
of the sac, especially if it correspond to a cyphotic curve of the
spine (52). In other instances, the myelo-meningocele is covered
entirely with skin ; and in yet others with an epidermic layer, which
possibly represents skin in an early stage of development. The
cavity of the sac in myelo-meningoceles represents, therefore, the
ventral part of the expanded subarachnoid space.

In a third group of cases the sac is constituted by an expansion
of the central canal of the spinal cord (myelo-cystoccle, syringo-
myelocele). This is a rare variety of spina bifida, and is apt to be
associated with marked spinal curvature (scoliosis, lordosis) and with

298 ANTENATAL PATHOLOGY AND HYGIENE

such monstrous states as exoniphalos and ectopia vesicae (Fig. 54).
Recklinghausen (13) and Muscatello (50) have especially investigated
this type. In it the osseous defect is more often situated laterally
than in the other two varieties. The sac is covered by the skin,
subcutaneous fat, and aponeuroses ; and the wall consists of the thin
spinal membranes (without any trace of the dura mater), lined
internally by cylindrical epithelium. The spinal nerve roots pass
forwards at the sides of the sac, and are not found inside it ; they lie
between the inner lining membrane and the other layers of the wall.
A meningocele may coexist with such a myelo-cystocele as has been
described (54) and give rise to the compound myelo-cysto-men-
ingocele ; in it there is fluid not only in the dilated central canal of
the cord but also in the subarachnoid space, dorsal or ventral. This
third variety of spina bifida can only be distinguished from the first
by dissection. The central canal of the cord may show dilatation
above the level of the sac, but this in itself does not prove the sac to
be a myelo-cystocele, for such dilatations may occur in other varieties
of spina bifida and also apart from it altogether.

To summarise briefly the three varieties of sac, it may be stated
that the spinal meningocele is simply a local herniation of the
membranes, the cord being practically normal ; the niyelo-men-
ingocele has a sac-wall composed of the membranes and the cord,
which latter structure appears as if it has been flattened out and
pressed against one wall of the sac and united with it ; and the
myelo-cystocele has a sac-wall made up of the cord and the
membranes, the fluid accumulation being in the central canal of
cord. In the two former varieties the fluid is outside the cord, in
the third type it is inside the cord.

The fluid contents of the spina bifida sac have been analysed
several times. W. D. Halliburton made three analyses for the
Committee of the London Clinical Society (30), one of which is given
here. The fluid was drawn from a lumbo-sacral sac in the case of a
female infant eleven days old, and it was the first tapping.

Water ..... 989-877

Total solids ..... 10-123

Proteids (containing globulin) . . . L602

Soluble salts . .... 7 -544

Insoluble salts ..... '346

Extractives . . . . . '631

The fluid was clear and faintly yellow in colour, had a slightly
alkaline reaction, and showed a slight trace of sugar. The specific
gravity is low (usually about 1007), and boiling produces an opales-
cence but no coagulation.

3. The Spinal Corel and its Membranes. — Not only does the spinal
cord show anomalies of structure when it forms part of the spina
bifida sac (as in my^lo-meningocele and myelo-cystocele) but it may
also do so in the simple meningocele ; further, it may exhibit
malformations in other parts of its extent than in the immediate
neighbourhood of the spinal defect. I have already referred to the

SPINA BIFIDA 299

condition of diastematomyelia, in which, especially in spinal men-
ingocele, the cord divides into two strands, each with a central canal,
and unites again into one band at a level below the sac ; this state
may or may not be associated with the presence of an osseous or
cartilaginous projection into the spinal canal. Sulzer (55) describes
fully a case of division of the cord in which such an exostosis existed,
and refers at length to other cases of diastematomyelia ; and War-
rington and Monsarrat (5G) give full details of another instance in
which there was also arrested development of the cerebellum and
hydrocephalus. In the latter case (56) the division of the cord and
the exostosis were both situated in the dorsal region above the level
of the spina bifida (which affected the fourth and fifth lumbar
vertebne) ; this would seem to show that the exostosis has not the
teratogenic effect on the spina bifida which some writers have
ascribed to it. Further, Theodor's observation (97) of a cord double
from the third dorsal vertebra backward would seem to show that
the duplicity was due to a wedge of grey and white matter pushing its
way into the cord posteriorly and so separating the posterior columns :
the central canal was also double, and these gave off several branches.

Apart from division of the spinal cord, other malformations of it
may be present in cases of spina bifida. Dilatation of the central
canal, for instance, has been noted ; it is commonly situated above
the level of the spina bifida. Hydromyelia, however, may occur
quite apart from spina bifida, and it is not proven that it often has
a causal relation to it ; but sometimes the canal in the cord would
seem to be continuous with the sac of the spina bifida (30). The
cord itself may be normal in size, but often it is attenuated ; it may
end abruptly or be too long for the canal.

I have already referred to the difficult question of the arrange-
ment of the membranes in cases of spina bifida. The difficulty seems
to be largely due to the fact that in the sac-wall the membranes may
be found in an undifferentiated state. Thus, in the posterior wall
(in cases of myelo-meningocele) we may find the structures which
normally precede and give rise to the spinal membranes : these
structures, mesoblastic in origin, ought to be developed between the
epiblast of the cord and that of the surface ; but they either remain
undifferentiated, or only advance to the stage of myxomatous tissue,
or are absent altogether. In this way the peculiar relations of the
cord to the surface may arise. At the neck of the spina bifida sac,
however, it is generally possible to trace the membranes : the
arachnoid, at any rate, can lie seen there, and may be followed for a
greater or less distance in the sac-wall. The dura mater, often in a
congested state, may be recognisable anteriorly in connection with
the bodies of the vertebra? ; but, as a rule, it cannot be traced in the
wall of the sac posteriorly. Both the pia mater and the arachnoid
are commonly found thickened. Bockenheimer (78) gives useful
although somewhat schematic representations of the supposed
arrangement of the membranes in the different types.

4. Complications. — It would, of course, be out of place here to
enter in detail into the morbid anatomy of the complications of spina

Q

00 ANTENATAL PATHOLOGY AND HYGIENE

bifida; that would be to anticipate the description of many of the
succeeding teratological types. At the same time, the commonly asso-
ciated malformations of the brain deserve a few lines of description.

(a) In the Brain. — Hydrocephalus, as is well known, is a frequent
concomitant of spina bifida. It may only become apparent after
some operation upon the sac has been attempted ; but, as Plate XVIII.
shows, it may be present without there being any external sign of it
at all, and before any active treatment has been initiated. This fact
must be taken into account in deciding whether an operation should
be attempted and at what time. The association of spina bifida and
hydrocephalus is well shown in Specimen No. 246, represented in
Plate XX.; in it the section has passed to the side of the middle line
in the cephalic region, and, therefore, the distension of the lateral
ventricle is well shown.

Anencephalus as a complication of spina bifida has been already
referred to ; but there are other changes in the brain besides those of
anencephalus and hydrocephalus. In a painstaking and beautifully
illustrated article, Nicolas Solovtzoff (57) has described five cases of
spina bifida. From the examination of these specimens he comes to
the conclusion that spina bifida is sometimes accompanied by dropsical
enlargement of the fourth ventricle, leading to its elongation in a
downward direction ; this may be associated with downward dis-
placement of the whole medulla oblongata, and in some instances the
posterior part of the medulla is so displaced as to lie upon the spinal
cord or to be united directly with it. The result of these changes
was that the fourth ventricle, which normally can only be seen when
the cerebellum is raised, was visible below the level of the latter
structure ; it was covered only by the valve of Tarin and a mem-
branous expansion of the cerebellum.

The development of the cerebellum itself was defective in most of
Solovtzoffs specimens (absence of the vermis, etc.). In my specimen
(No. 233) shown in Plate XVIIL, a somewhat similar displacement
of parts has evidently taken place ; the cerebellum and medulla
oblongata are both at a lower level than is normal and the basis
cranii looks backward in a marked degree. Gudden (96), also, has
described a case of dorsal spina bifida in a human embryo, in which
there was a remarkable swelling of the inferior vermiform process of
the cerebellum, which projected into the fourth ventricle ; the spinal
cord at its origin from the medulla was bent upon itself, and near
the spina bifida it divided into two parts.

(b) Outside the Nervous System. — Another complication which
occurs not uncommonly in cases of spina bifida is the presence of
congenital neoplasms, some of them of the nature of teratomata, otlrers
teratoid in character, and yet others more simple in structure ; most
of these tumours are attached to the sacrum and coccyx in the
neighbourhood of the spinal defect. I have described a case
(Specimen 113) in which a lipoma was situated in the gluteal
region just below an open spina bifida (Fig. 51). Sometimes the
growth has been found to communicate with the interior of the
canal. I shall speak again of these sacral neoplasms.

Plate xviii.

Tongue.

Fluid in
Ventricle.

Cerebellum.

Stomach
(cardiac end),

Liver.

Spina bifida.

Intestines

Bladder

./

COMPLICATIONS OF SPINA BIFIDA 301

Another complication is club-foot, double or single ; this was the
case in my specimen, No. 233, represented in Plate XVIII. and
Fig. 53, as well as in No. 113 (Fig. 51). In the latter case there was
also congenital dislocation of the hip joints ; this complication has
been observed by Kirmisson (22) and others. I have referred in the
first section of this Manual (p. 384) to the curiously frequent
association of congenital prolapsus uteri and lumbo-sacral spina
bifida ; prolapse of the rectal mucous membrane has also been noted,
and, in other cases, atresia ani (58, 59). An imperforate state of
other parts of the intestine has occurred as a complication of spina
bifida (e.g. of the duodenum, as in Jackson's specimen, 60). Various
degrees of incomplete closure of the anterior abdominal wall (exom-
phalos) and of the bladder (vesical exstrophy) and genital organs
(epispadias, etc.) have also been met with (61-65) ; and some writers
(13, 24) have seen in this association a teratogenetic relationship
between the abdominal and the spinal malformation, more especially
when the latter is of the variety known as myelo-cystocele. More
rarely other anomalies occur in foetuses with spina bifida, such as
sympodia (Cosentino, 67 : Arnold, 68), hernia funiculi umbilicalis
(Bergh, 69), defect of chest-wall and hernia of lung (Sabrazes, 70),
cleft palate (Charrier, 71), and pseudo-sclerema (Pieynolds Wilson,
72).

For some reason, unknown to us, spina bifida would appear to be
rare in the Mammalia, with the exception of man ; a few cases have
been noted in calves and lambs. Avian rhachischisis, however, is
more common, and has been noted more particularly in artificially
incubated chicks (Dareste, '■'>■'<).

5. In order to complete this sketch of the morbid anatomy of
spina bifida, I must add that changes may take place in the sac both
before, during, and after birth. Doubtless rupture of the sac from
pressure not uncommonly happens during the birth of the infant ;
and sometimes it is tapped because of the delay which it causes in
labour (73). In other cases a sort of natural cure would appear to
take place before birth. This, at any rate, is what is supposed to
have happened in cases of spina bifida occulta and in congenital
fistula?, especially in the sacral region (Betz, 74 ; Beboul, 75).
Sometimes the sac ruptures spontaneously after birth, and then the
rupture may be followed by shrinkage and cure, but more often it is
the prelude to septic infection and meningitis and myelitis. Doubt-
less herein lies the great difference between antenatal and postnatal
rupture : the former is not followed by inflammation, for it takes
place in an aseptic cavity, and so the tissues are able by their
growth partly at any rate to repair the defect and close the spinal
aperture; the latter, on the other hand, is succeeded by sepsis,
inflammation, and death. In both instances the morbid anatomy
will be modified : in the former case by the repair-processes of ante-
natal life of which we know so little, and in the latter by inflam-
matory exudations, adhesions, and the like, about which we unfortu-
nately know far too much. Changes in structure may also occur
after operative interference : sometimes these are of the nature of

302 ANTENATAL PATHOLOGY AND HYGIENE

cicatrisation with a shrinkage of the sac and more or less complete
cure, especially in meningoceles ; at other times they consist in
inflammatory processes which affect the cord and brain. The reader
can, therefore, understand how difficult it is to recognise the indi-
vidual spinal membranes and parts of the spinal cord in the spina
bifida sac which has passed through the vicissitudes enumerated
above.

Teratogenesis.

To attempt to describe the different explanations of the origin of
spina bifida that have been advanced by various writers would simply
be to tell over again the greater part of the story of the terato-
genetic theories of the past and present already dealt with in an
earlier part of this volume (pp. 107-224). I shall be brief.

There was, first, the theory of maternal impressions. It was
never difficult to discover that at some time or other in her preg-
nancy the mother had seen a case of spinal disease, or had received
a blow on the small of the back, or in some way had got a fright. In
a case seen by myself in the Maternity Hospital in December 1903,
the mother had had a fall from a car between the fourth and fifth
month of pregnancy, and she was inclined to ascribe the spina bifida
in her infant to that cause ; but she had also had a severe illness
(catarrh of the stomach and inflammation of the lungs) at the sixth
month, and thought it might also have played a part. I believe the
spina bifida was in existence long before either the fall or the illness
occurred.

Then came, in their turn, the theories of traumatism and pressure.
A blow on the mother's abdomen during her pregnancy might
injure the foetal back and so cause the spinal aperture, through
which the contents would then protrude. A sharp curve in the
vertebral column (and such curves are, as we have seen, associated
with spina bifida) might by its mechanical action reopen the
medullary canal ; a cyphotic curve would specially tend to open the
posterior part of the canal (76), and the cyphosis itself might be due
to traction on the umbilical cord, from excess of the liquor amnii or
disorderly foetal movements, etc. (77). Although Lebedeff (76) and
Marchand (77) both supported this view, Recklinghausen (13) has
adduced arguments against it, among which is the fact that spina
bifida may be found associated with lordosis as well as with cyphosis.
At the same time there may be an element of truth in the theory,
for the curves which are found in the spine of the foetus when born
may be different from those which were present when the spina
bifida was being induced. The pressure of the amnion has also been
invoked to explain the various forms of spinal defect and herniation :
but the discussion of this possible factor is more correctly taken later.

Many pathologists have looked to disease of the cerebro-spinal
nervous system as the cause of spina bifida. At first sight it is
reasonable enough to believe that there is dropsical distension of the
spinal cord or of its membranes, and that the consequent enlarge-

TERATOGENESIS OF SPINA BIFIDA 303

ment prevents the closure of the spinal canal ; but there are diffi-
culties in accepting the view, as we shall see. The association of
hydrocephalus with spina bifida was early noted, and it was thought
that the latter was due to the former; it was not clear what the
sequence of events was, but it was conjectured that the cerebral
ventricles (e.g. the fourth) might rupture, and so the contained fluid
might make a way for itself down into the vertebral canal. Some
cases seemed to favour this view, but in others no such communica-
tion could be found, and in them it was thought that the fluid had
descended under the membranes, following what might be called
natural routes. Morgagni (79) found it necessary to admit a spinal
origin of the fluid in some cases, for otherwise it was impossible to
account for the cases in which there was spina bifida without hydro-
cephalus ; in other instances the cephalic origin might be allowed to
exist, for in them rupture of the spinal sac caused shrinkage of the
hydrocephalic enlargement. Morgagni's views held sway for some
time, and they were rather strengthened by the inclusion of the idea
of a dropsical distension of the central canal of the spinal cord
(Portal, 80). But the variety of spina bifida in which the sac is due
to distension of the central canal of the cord is, as we have seen, a
rare one. Further, (Jruveilhier (81) noted the independent occur-
rence of hydrocephalus and spina bifida, and had a difficulty in
explaining the adhesion of the spinal cord to the meningeal sac' (in
meningo-myeloceles) by Morgagni's theory. Some modern authors,
however, have not found such adhesions incapable of explanation.
Tourneux and Martin (32), for instance, have ascribed them to the
pressure of the fluid within the subarachnoid space ; and Hofmokl's
experiments (82) with a model of a spinal canal and cord seemed to
support this hypothesis, although W. Koch (29) pointed out possible
fallacies in them. Of late, however, the general trend of terato-
logical opinion has been away from the theory that dropsy of the
cerebro-spinal nervous system was the cause of spina bifida. Even
Solovtzoff (57), whose observations demonstrated the presence of
dropsical enlargement of the fourth ventricle along with a series of
dislocations of the cerebellum, medulla oblongata, and cord, does not
claim that there is a constant association of the spina bifida and the
ventricular changes. At the present time the idea of diseased con-
ditions of the brain and cord as causes of spina bifida has largely
given place to that of arrested development ; at best, it takes a
subordinate place in the latter theory. To this view I must now
direct the reader's attention ; the theory of the action of the amnion
is almost constantly combined with it.

What has been called the embryological theory in teratogenesis
would seem at first sight to offer a real solution of the difficulty
met with in the explanation of the origin of spina bifida. There is
delayed closure of the medullary groove due to imperfect develop-
ment of the medullary folds (due in its turn, perhaps, to amniotic
pressure from maldevelopment), and so an open condition of the
spinal canal is produced. This apparently simple explanation, how-
ever, does not account for all the facts. By the above-described

304 ANTENATAL PATHOLOGY AND HYGIENE

mechanism we are able to explain the cases of holo-rhachischisis in
which there is a wide vertebral groove or gutter with a layer of
rudimentary nerve tissue covering it; in such instances there is
evidently a want of closure of the medullary groove, and of all
the later formed structures which lie posterior to it, including the
laminae and spines of the vertebra-. In other cases, however, such
as those in which the spinal cord is present and contains a central
canal, it is evident that the medullary groove has closed ; in them
the cause of the spina bifida would seem to lie in the imperfect
differentiation of the tissues which are found between the spinal cord
and the dorsal epiblast, including the membrana reuniens superior,
which is the anlage at the corium, spinal laminae, and membranes
(Ranke, 83). For a time the spinal cord and the dorsal epiblast are
in apposition, and then they are gradually shut off from each other by
the mesodermic prolongations ; it is easy to imagine how a defect in the
ingrowing mesodermic plates would allow spinal structures to remain
attached to epidermic ones, and so produce some of the varieties of
spina bifida. Secondary factors may come into play as well as this
primary fault in the mesoderm ; individual vertebrae will be ill-
developed, and thus bendings of the spine may be produced, and the
canal become too short for its contained cord (Recklinghausen, 13).

Attempts have been made to arrange in series the varieties of
spina bifida according to the stage in development when the arrest in
ontogenesis occurred. Thus Bland-Sutton (3) has five types. In the
first the medullary folds unite imperfectly, and this gives rise to
what Bland-Sutton terms a myelocele, but is perhaps better dis-
tinguished as a simple rhachischisis ; in the second the medullary
folds unite throughout, but fail to separate from the surface epiblast,
and the central canal subsequently dilates, myelo-cystocele or syringo-
myelocele ; in the third, the cord, it is supposed, is normally closed,
but before it separates from the surface epiblast it becomes com-
pressed by a collection of fluid within the meningeal spaces, myelo-
meningocele ; in the fourth, the cord is normal, but there is a local
hernia of the membranes, meningocele ; and in the fifth, the cord and
membranes are normally formed, but the arches of one or more
vertebrae are defective, although no protrusion of spinal contents
takes place, spina bifida occulta.

Taruffi (84) has thought it necessary to conjoin with the theory
of arrested development that of disease of the parts. In this he has
followed Recklinghausen and others. Accordingly he ascribes the
state of the cord and its membranes to the destructive effects of a
sort of angiomatous process similar to that which he thinks has led
to the changes in the brain in pseudencephalus (q.v.). The factors,
are a pathological development of the vessels along with exudations
and haemorrhages ; and these, it is supposed, lead to destruction of
certain parts of the cord and its membranes. It seems to me, how-
ever, that it is not necessary to bring forward this factor, for it is
possible that the changes found may not be due to destruction of
previously existing parts but to non-formation of them, or rather to
imperfect differentiation of each one of them from the rest. Further,

TREATMENT OF SPINA BIFIDA 305

the vascular theory of Taruffi is only of use in explaining myelo-
meningoceles; it fails in the case of myelo-cystocele and of men-
ingocele.

Cleland (7, 85), as has been stated already, favours the view of a
reopening of the medullary canal from excessive collection of fluid
within it in order to explain some varieties of spina bifida. He has
also introduced (7) a new element into the teratogenesis of spina
bifida and allied conditions by suggesting the factor of over-stimula-
tion. He thus accounts for the osseous and cartilaginous projections
which have been noted within the spinal canal ; and the double state
of the cord in some instances he ascribes to partial fission of the
early embryo. The fission has not extended far, having resulted
simply in the production of these " vestiges of duplicity " on the
dorsum ; but possibly (Cleland thinks) specimens of spina bifida may
yet be discovered containing a trace of appended limbs.

1 am of opinion that Cleland is right in introducing the idea of
excess of development into the teratogenesis of spina bifida. I believe
that the chief factor is arrested ontogenesis, and that the most of the
structural peculiarities are its results, modified probably by the con-
tinued and unopposed growth and development of neighbouring parts
and possibly also by pathological processes ; but it seems to me that
there coexists with this arrest of development a degree of excessive
development, leading to the growth of cartilaginous and osseous pro-
jections from the vertebral bodies, to the presence of teratomata or
teratoid neoplasms, and to the duplicity of the cord and its central
canal (diastematomyelia). It is not uncommon to find this combina-
tion of defect and excess of ontogenesis in teratological specimens ;
and it is due, I believe, to what I have named the germinal factor
in teratogenesis (p. 222). The tendency to excessive development is,
I think, impressed upon the organism in the germinal or pre-em-
bryonic epoch, and manifests its influence later. Perhaps 0. Hertwig's
experiments (Arch. f. mikr. Anat., xxxix., 1892) on the production in
frogs' eggs of malformations resembling spina bifida by means of
polyspermy may be regarded as supporting this opinion. That there
is sometimes a germinal factor in the production of spina bifida is a
view which is also supported by the occurrence of several instances
of the malformation in the same family (J. Burgh, 86 ; 0. Zimmer-
mann, 87 ; E. M. Pendleton, 88 ; P. Winge, 98 ; Mearns, 99 ; A. C.
Butler-Smythe, 89), and in twins (Windle, 90). In considering this
possibility, however, we must remember that we are forcing our way
very far into the deepest mysteries of Nature's arcana, concerning
which we must confess that our knowledge is but slight.

Treatment.

In the absence of any accurate knowledge as to the manner in
which Nature attempts and sometimes successfully carries through
the antenatal cure of a spina bifida, we have to think solely of
postnatal reparative procedures. One thing, however, we may learn
from Nature's works in utero — the necessity for asepsis. Whatever

VOL. II. — 20

306 ANTENATAL PATHOLOGY AND HYGIENE

may be the process by which a defect in the spinal column is repaired
before birth, we may be quite sure it is carried out aseptically. Now,
since this book deals with Antenatal Hygiene it is not essential that
I describe postnatal operative methods ; so I shall simply refer to
some of the plans that have been used and to them only in so far as
a knowledge of the antenatal may throw light upon the postnatal.

It is doubtful whether tapping, compression, ligature, electrolysis,
and simple incision of the spina bifida sac should be any longer
countenanced by the surgeon or described in the text-books. None
of these methods seems to me to be warranted by what is known of
the morbid anatomy of the malformation. It is true that in a few
cases cysts have been found in the neighbourhood of the spine and
unconnected with its canal (Trowbridge, 91), and it has been thought
that they represented spina bifida sacs shut off from the vertebra in
antenatal life by a natural process of compression or ligature ; but
these cysts are capable of being accounted for in other ways, and,
even if they are the results of Nature's cure, we may be quite sure
her methods had very little in common with such a crude method as
ligature of the base of the sac. Even if a separation of the sac from
the canal by a growth of the surrounding parts at its neck does occur,
it must be supposed to be a very gradual process in no way resem-
bling the sudden process of ligature ; and, as has been already pointed
out, it occurs in the aseptic medium of the uterine cavity. J. Morton
(92), it is true, has obtained wonderfully good results (79'3 per cent,
recoveries) from the combined method of tapping the sac (drawing off
a portion of the fluid contents) and injecting the iodo-glycerine
solution (10 grs. of iodine, half a drachm of iodide of potassium, and
1 oz. of glycerine) into the half-emptied cavity ; but the operation
most in favour at the present time would appear to be excision.
Excision (described by Newbigging (95) in 1834) is not limited, as
was at one time thought, to the meningoceles, but may be usefully
employed in myelo-cystoceles and in many myelo-meningoceles. Of
course in the case of myelo-meningoceles, the spinal cord and nerve
roots must be preserved ; if they are adherent to the sac they must
be separated and returned to the canal, and if they are not detach-
able the cord must be put back with the part of the arachnoid to
which it adheres. The attempt to bridge across the defect in the
spinal canal by chiselling through and turning inwards the laminae of
the vertebra' or by bone grafting is unnecessary and, as H. J. Stiles
(9."») says, "only adds to the gravity of an already serious operation."
In spina bifida occulta we find the opening in the canal closed by
fibrous tissue, and there is often a pad of fat and connective tissue
over it ; in operative closure we ought to aim at the same result and
bridge across the aperture with the edges of the sac and perhaps with
periosteum, covering over the weak spot with two flaps taken from
the erector spinal muscles and sutured together. In this way the
opening can be closed and protected after closure by a sort of natural
truss.

The coexistence of hydrocephalus is a contraindication to opera-
tion, as is also the presence of complete paralysis of the lower part of

BIBLIOGRAPHY OF SPINA BIFIDA 307

the body and the association of the spina bifida with grave malforma-
tions in other regions. Large size is not necessarily a contraindica-
tion, for it is remarkable to what an extent skin can be drawn from
surrounding parts to supply the deficiency. The most serious
impediment to success is the unsuspected presence of hydrocephalus ;
for I have shown how this may be found after the death of the infant
although it could not be diagnosed during life {vide Plate XVIII. ).
The operation need not be postponed much after the first month of
life is past; it may, if necessary, be performed earlier (93). Spina
bifida, therefore, is in some of its forms (e.g. the occult) not necessarily
fatal to postnatal life, while in other forms it may be operated upon
with a fair hope of ultimate success if hydrocephalus be not associated
with it. The results of Stiles (93), Nicoll (9-4), and a great many
other surgeons, both at home and abroad, are decidedly encouraging,
especially when it is remembered that it is not so long ago since all
operative interference was regarded as well-nigh hopeless. Reparative
Surgery has done much and will do more for the correction of ante-
natal malformations.

BIBLIOGRAPHY.

(1) N. TuLrius, Observations Mediae. Lib. iii. c. xxix., xxx. pp. 240,
242, Amstelodami, 1652. (2) A. Forster, Die Missbildungen des Menschen,
p. 78, Jena, 1865. (3) J. Bland-Sutton, Tumour*, Innocent and Malignant,
p. 522, 1903. (4) C. Taruffi, Storia della teratologia, vi. 214, 1891.
(5) P. Zacchia, Quest ionum Medico-legalium, Lib. vii. quest. 9, p. 500,
Lugduni, 1661. (6) C. F. Lallbmand, Observatioiis pathologiques, p. 25,
Paris, 1818. (7) J. Cleland, Memoirs and Memoranda in Anatomy,
p. 161, 1889. (8) H. Knoop, Beitr. z. Geburtsh. u. Gyncek., vii. 284,
1903. (9) R. Virchow, Ztschr. f. Ethnol., vii. 279, 1875. (10) Friedinger,
Wchnbl. d. k, k. Gesellsch. d. Aerzte in Wien, i. 489, 515, 1855. (11) L.
Calori, Mem. d. Accad. d. sc. di Bologna, s. 2, ii. 244, 1862.
(12) Ornstein, Ztschr. f. Ethnol., vii. 91, 2*79, 1875. (13) F. v. Reck-
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494, 1887; cxxix. 246, 1892. (16) G. Joachimsthal, Berl.klin. Wchnschr.,
xxviii. 536, 1891 ; Arch. f. path. Anat., cxxxi. 488, 1893. (17) E. Saal-
feld, Arch. /. path. Anat., cxxxvii. 384, 1894. (18) J. Schou, Bed. klin.
Wchnschr., xxxi. 113, 1894. (19) Furst, Deutsche med. Wchnschr., xxi.,
Ver.-Beil, 103, 1895. (20) A. H. Dodd, Lancet, p. 1063, ii. for 1887.
(21) L. Mayet, Nouv. iconogr. de la Salpetriere, xiv. 266, 1901. (22) E.
Kirmisson, TraitS d. vial. chir. d'origine congenitale, pp. 7-36, 1898.
(23) G. Bohnstedt, Arch. f. path. Anat., cxl. 47, 1895. (24) J. Bland-
Sutton, Trans. Bath. Soc. Bond., xxxix. 437, 1888. (25) C. Taruffi,
Storia della teratologia, vi. 245, 1891. (26) Lindsay, Edinb. Med. Journ.,
xl. 664, 1895. (27) Jones, Brit. Med. Journ., p. 173, i. for 1891.
(28) Maass, Centrlbl. f. Chir., xxv. 22, 1898. (29) W. Koch, Beitrdge
zur Lehre von der Spina bifida^ Kassel, 1881. (30) Report of the Com-
mittee appointed to inquire into the Anatomy of Spina Bifida, etc., Trans.
Chir. Soc. Lond., xviii. 339, 1885. (31) F. Ruysch, Ohservationum
anatomico-chirurgicaruni centuria, obs. xxxiv. p. 45, 1691. (32) F.
Tourneux et E. Martin, Journ. de Vanat. et de la phgsiol., xvii. 1, 383,

308 ANTENATAL PATHOLOGY AND HYGIENE

1881. (33) C. Dareste, Production artificielle des monstruosites, Paris,
1891. (34) P. IIewett, Loud. Med. Gaz., n.s., xxxiv. 459, 1844. (35) C.
Taruffi, Storia della teratologia, vi. 173, 1891. (3G) J. M. Cotterill,
Tram. Med.-Chir. Soc. Edinb., xv. 49, 1896. (37) B. Trompei, Ann. nniv.
di med., xv. 27, 1820. (38) Dubourg, Gaz. d. med.-prat., Paris, i. 84,
1839. (39) T. Kroner and F. Marchand, Arch. f. Gyncek., xvii. 444,
1881. (40) T. A. Emmet, Amer. Journ, Obst, iii. 623, 1870-71.
(41) Hauner, Illust. wed. Ztg., i. 230, 1852. (42) Cruveilhier, Bull. Soc.
anat. de Paris, xxviii. 347, 1853. (43) Boullard, Bull. Soc. de med. et de
pharm. de la Haute- Vienne, p. 118, Limoges, 1855. (44) Houel, Bull, et
mem. Soc. de chir. de Par., n.s., iii. 325, 1877. (45) Humphry, Journ.
Anat. and Physiol., xx. 585, 1885-86. (46) J. Cleland, Memoirs and
Memoranda in Anatomy, p. 161, 18N9. (47) C. Taruffi, Storia della
teratologia, vi. 197, 1891. (48) J. M. Duncan, Edinb. Med. Journ., xxi.
343, 1875-76. (49) Beringier, Bull. Soc. anat. de Pari*, liv. 796, 1879.
(50) Muscatello, Areh.f. klin. Chir., xlvii. 214, 257, 1894. (51) F.
Tourneux et E. Martin, Journ. de Vanat. et de la physiol., xvii. 8, 1881.
(52) C. Taruffi, Storia delta teratologia, vi. 210, 1891. (53) A. \Yiede-
mann, Diss, inaug., Breslau, 1872. (54) H. H. Glutton, Trans. Clin. Soc.
Lond., xix. 99, 1886. (55) P. Sulzer, " Ein Fall von Spina bifida mit
Verdoppelnng und Zweitbeilung des Riickenmarks," Diss, inaug., Jena,

1893. (56) W. B. Warrington and Keith Monsarrat, Brain, xxv. 444,
1902. (57) N. Solovtzoff, Nouv. iconogr. de la Salpetriere, xiv. 118, 251,

1901. (58) J. Guerin, Bull. Acad, de med., 2 s., viii. 1022, 1879.

(59) W. Taylor, New Orl. Med. and Surg. Journ., ix. 314, 1852-53.

(60) J. B. S. Jackson, Boston Med. and Surg. Journ., lix. 355, 1858-59.

(61) V. Colomiatti, Frammenti di embriologia patologica, p. 16, 1880.

(62) G. Friedlander, Monatschr. f. Geburtsk. u. Frauenkr., vii. 243,

1856. (63) A. Wasseige, Bull. Acad. roy. de med. de Belg., xii. 701,
1852-53. (64) O. Grundies, Diss, inaug., Erlangen, 18S3. (65) B.
Gaedkens, Diss, inaug., Berlin, 1880. (66) M. Katzenstein, Areh.f. Idin.
Chir., lxiv. 607, 1901. (67) G. Cosentino, Arch, di ostet. e ginec, i. 41,

1894. (68) J. Arnold, Beitr. z. path. Anat. u. allg. Path., xvi. 1, 1894.
(69) C. A. Bergh, Hygeia, Ivii. 560, 1895. (70) Sabrazes, Rev. de med.,
xiv. 1010, 1894. (71) Charrier, Bull. Soc. anat. de Paris, xxxii. 249,

1857. (72) W. Reynolds Wilson, Amer. Journ. Obst., xxxiii. 9, 1896.
(73) M. Gunsburg, Trans. Edinb. Obst. Soc, xxi. 252, 1896. (74) F.
Betz, Memorabilien, xvi. 171, 1871. (75) J. Reboul, Bull. Soc. anat. de
Paris; lxii. 605, 1887. (76) A. Lebedeff, Arch. f path. Anat., lxxxvi.
263, 1881. (77) Marchand, Real-Encyclopddie d. gesammten Heilkunde,
xviii. 437, 1889. (78) P. Bockenheimer, Arch. f. Bin. Chir., lxv. 697,

1902. (79) G. B. Morgagni, Seats and Causes of Diseases (Alexander's
translation), i. Letter xii. art. ix. p. 255, 1769. (80) A. Portal, Mem. de
TAcad. d. Sc, Paris, Annee mdcclxx., Hist., p. 40 ; Mem., p. 238 ;
Cours d'anatomie medicate, i. 303, 1803. (81) J. Cruveilhier, Atlas
d'anatomie pathologique, i. Livr. xvi., PI. iv., 1829-35 ; Traite d'anatomie
patholog. generate, i. 201, 598, 1849. (82) Hofmokl, Med. Jahrb., Wien,
viii. 443, 1878. (83) H. Ranke, Jahrb. f. Kinderh., n. F., xii. 116,
1877-78; Centr.-Ztg. f. Kinderh., i. 195, 1877-78. (84) C. Taruffi,
Storia delta teratologia, vi. 265, 1891. (85) J. Cleland, Journ. Anat. and
Physiol., xvii. 257, 1883. (86) J. Burgh, Miscell. nat. curios., Dec. ii.
Ann. vi. obs. lviii. p. 138, 1688. (87) O. Zimmermann, Med. Ztg., Berlin,
xv. 45, 1846. (88) E. M. Pendleton, South. Med. and Surg. Journ.,

BIBLIOGRAPHY OF SPINA BIFIDA 309

Augusta, n.s., xiii. 215, 1857. (89) A. C. Butler-Smythe, Lancet, i. for
1889, p. 272. (90) B. C. A. Winule, Journ. Anat. and Physiol., xxvi.
295, 1891-92. (91) A. Trowbridge, Boston Med. and Surg. Journ., i.
pt. 2, 753, 1828-29. (92) James Morton, The Treatment of Spina Bifida,
1st edit., 1877; 2nd edit., 1887. (93) H. J. Stiles, Brit. Med. Journ,, ii.
for 1902, p. 673. (94) J. H. Nicoll, Brit, Med. Journ., i. for 1902,
p. 1532. (95) P. S. K. Newbigging, Probationary Essay on Spina Bifida,
Edinburgh, 1834. (96) H. Gudden, Arch, f. Psychiat,, xxx. 866, 1898.
(97) F. Theodor, Arch, f. Kinderh., xxiv. 344, 1898. (98) P. Winge,
Norsk May. f. Laegevidensk,, 4 R., i. 271, 1886. (99) Mearns, Rep. Proc.
Nortliumb. and Durham Med. Soc, p. 271, 1894.

CHAPTER XVIII

Merosomatous Terata (runt.): Malformations of the Spine (cont.): Minor Types
of Spinal Deformity; Spinal Curvature; Scoliosis; Cyphosis; Lordosis;
Spinal Retroflexion ; Spondylolisthesis ; Numerical Anomalies in Vertebrae ;
Fusion of Neighbouring Vertebrae ; Assimilation; Foetus Anurus ; "Tails" ;
Tailed Races; Sporadic Cases of Homo Caudatus ; Spurious Tails; True
Tails, Varieties, Complications, Teratogenesis ; Sacral Dimples and Infundi-
liula; Miscellaneous Malformations of the Spine; Malformations of the
Spinal Cord (Double Central Canal ; Heterotopia of White and Grey
Matter ; Hydromyelia and Syringomyelia).

Spina bifida may be regarded as the central or major type of all the
malformations that affect the spine and its contents. For this reason
I described it in some detail, giving, as it were, a f ull-length portrait
of it ; but there are minor types of spinal deformity, and they require
only to be sketched in outline. Some of them, however, are interest-
ing both from the theoretical and the practical standpoint, and each
one of them suggests attractive problems in disorderly ontogenesis.
Many of them may be met with in association with spina bifida;
they may also, however, occur alone.

Spinal Curvature.

As has been shown in the two preceding chapters, spinal curvature
of a marked kind is present in iniencephaly and spina bifida ; it will
be met with again when the consideration of anencephaly and ex-
omphalos and sympodia is undertaken. In these monstrosities the
spine may show cyphosis, lordosis, or scoliosis; the last-named de-
formity has rendered itself particularly prominent in my eyes when
I have been engaged in the making of frozen sections of teratological
specimens, for, on account of it, the middle line of the body was lost
at one point or another in the section. But spinal curvature may
exist in the fcetus apart from other malformations, and it is with that
form that I have here to do. There can be no doubt that some of
these cases are to be regarded as foetal diseases ; in other words, they
arise in the foetal period of antenatal life from such causes as mus--
cular retraction or paralysis and a faulty or restrained attitude of the
unborn infant (Perdu, La Scoliose,Yavis,\). 18, 1902). On this subject
Guerin's work (BechercJies sur les difformitvs coiujenitalcs, Paris, 1880)
may be consulted with profit. But there are also cases in which the
spinal distortion would appear to be due to causes arising in the
embryonic period. To this group belong the instances of scoliosis
arising from defects in the vertebral bodies.

310

CONGENITAL SCOLIOSIS 311

Congenital Scoliosis.

A. Mouchet (Bull. Soc. anat. de Paris, 6 s., i. 972, 1890) has
reported three cases of congenital scoliosis due to vertebral anomalies ;
two of these were dead-born foetuses showing numerous malforma-
tions (exencephaly, congenital amputations, facial anomalies), and
do not at present concern us ; the third was a living female
child who showed no other deviations from the normal. In this
last-named instance the spinal anomaly was discovered by the
radiograph, and consisted in the presence of a supplementary half-
vertebra between the first and second lumbar vertebral bodies on the
left side. The half-vertebra was wedge-shaped, the base being to the
left and the apex to the right. A similar case was reported by G.
Sangiorgi (Arch, di ortopcd., xviii. 65, 1901). There is a specimen of
congenital malformation of the spine in the Museum of the Royal
College of Surgeons of England (Catalogue of the Tcratological Series,
p. 95, 1893), in which there is a sharp lateral curve in the dorsal
region, apparently due to absence of the right half of the third dorsal
vertebra ; there are other malformations, such as the presence of
only seven ribs on each side and osseous union of the left scapula to
the spine. Noble Smith (Clin. Sketches, ii. 72, 1895) has referred to
similar cases. Half-vertebra' have been observed by other observers
and without the association of scoliosis ; in such cases there may be
a compensatory atrophy of the bodies of adjacent vertebra?, or there
may be a second half-vertebra on the opposite side of the spine
(Salaghi, Arch, di ortopcd., xvi. 172, 1899). Salaghi (Joe. cit., p. 86)
has also recorded a case of congenital dorso-lumbar scoliosis on the
right side, in which the whole of the left half of the body and head
of the infant was unduly small and in which there was left-sided
torticollis ; the cause, in this instance, may have been amniotic com-
pression. Perhaps the amniotic pressure may, in some cases, be the
agent which produces the half -vertebra : but even if this be so,
it seems necessary to suppose some local defect in the affected
vertebra to explain why it in particular and not the whole spine
should be influenced by the compression. Whatever may be its
cause, congenital scoliosis is a rare affection, occurring, according to
Coville (Rev. d'orthop., vii. 301, 1896) only once in one thousand new-
born infants. A recent case is that reported by H. Maass (Ztschr. f.
orthop. Chir., xi. 411, 1902-03). It would seem to occur also in
the lower animals, for there are specimens of it in fish, in birds, and
in a calf in the Museum of the Royal College of Surgeons of England
(op. cit., p. 94), and Gurlt (Lchrhuch d. path. Anat. d. Haus-Sdugc-
thicre, ii. 173, 1832) has described cases in the calf and the lamb
under the name Campylorrhachis scoliosa.

Congenital Cyphosis and Cypho-Scoliosis.

Cyphosis of one or other region of the spine may be met with in
association with spina bifida, anencephaly, exomphalos, and other
anomalies ; but it may also occur, in rare cases, practically alone, as

312 ANTENATAL PATHOLOGY AND HYGIENE

in L. Bernhardt observation {Arch. f. Kinderli., xxx. 31, 1900). In
this case there was abnormally advanced ossification of some parts
of the skeleton, and it was noted that attempts had been made to
bring the pregnancy to a premature end. The cyphosis was in
the upper dorsal region, and there was no compensatory lordosis.
Cyphosis is usually conjoined with lordosis, the one curvature com-
pensating the other ; but the compensation is often incomplete.
Instances of cyphotic scoliosis of antenatal origin in the lower
animals have been recorded by Gurlt, Goubaux, Billiet, and Gotti
(Taruffi, Storia dclla tcratologia, vii. 135, 1894).

Lordosis, Spinal Retroflexion, Spondylolisthesis.

Antenatal lordosis and spinal retroflexion are generally coml lined
with such grave types of monstrosity as exomphalos, iniencephaly,
and anencephaly (q.v.) ; but Matthews Duncan (Trans. Ohstct. Soc.
Zond., xxvi. 217, 1885) has described an interesting case in which
none of these teratological states was present. It was that of a full-
time male foetus with a large (slightly hydrocephalic) head ; the
trunk was apparently normal, but the head and all the limbs were
doubled backwards towards the spine, and fixed in their abnormal
position by " amniotic " bands consisting of skin. Matthews Duncan
believed the specimen to be unique, and I have met with no similar
one in teratological literature ; but a case resembling it in some
points has been reported by C. B. Lockwood (Trans. Obstet. Soc.
Lond., xxix. 234, 1887). Lockwood's specimen was a fifty days old
human embryo, showing a remarkable degree of retroflexion, but
with no amniotic bands to account for it such as were found in
Duncan's fcetus ; it had no spinal cord, and the vertebral column was
very short and without neural arches or spines ; there was a noto-
chord which was single in the upper cervical and dorsal regions, and
double in the lower cervical. Lockwood thought that the cause of
the retroflexion was to be found in the fact that the spinal column
had failed to develop, while the viscera at its ventral surface had
both developed and grown and forced the pelvis and lower spine
dorsally. Whether Lockwood's explanation be correct or not, the
observation is a most interesting one on account of the early period in
antenatal life to which it is necessary to refer the production of the
lordosis ; it is evident that one is warranted in regarding some at least
of the cases of congenital spinal curvature as embryonic in origin.

The Comparative Teratology of lordosis suggests some interesting
reflections. It has been found that retroflexion of the spine is out of
all proportion more frequent in the calf than in any other animal ;'
in fact, with the exception of one case in a foal (Joly, Mem. de
I' Acad. d. sc. Toulouse, i. 259, 1845), it would seem to be only met
with in the calf. This is one of the most interesting lines along
which research may yet travel in the solution of the problems of
Teratology ; for there must be some reason why one monstrosity
specially affects one animal type rather than others. In the case of
the calf the retroflexion of the spine is almost constantly associated

RETROFLEXION OF THE SPINE 313

with an open condition of the anterior (or inferior) thoracic and
abdominal wall ; more than this, the ribs are curiously turned back-
wards (or upwards) to form a sort of spurious thoracoabdominal
cavity above the dorsum. This new supradorsal cavity has, therefore,
walls lined on the outside with a serous membrane and on the
inside by skin ; to the lordotic spine are attached the thoracic and
abdominal viscera which hang downwards in two packets, as it were,
with traces of the diaphragm separating them. On account of the
open state of the thoracoabdominal cavity, Gurlt {Lehrbuch d. path.
Anat., etc., ii. 137, 1832 ; Ueber thierische Missy eburten, p. 25, 1877)
gave to this type the name schistosomas refiexus ; while Joly {Ann. cl.
sc. nat., 3 s., iii. 374, 1845), looking to the general turtle-like appear-
ance of the monstrosity, called it chdonisonia. Dareste {op. cit., pp,
418, 549, 1891) has given a good description of two specimens.
Attached to the ends of the ribs which are turned backwards over
the dorsum are parts of the divided sternum, and inside the new
costal cavity are the scapuhu Further, the head may be turned
back and lost from view in the cavity; in other cases it is the tail
and posterior extremities that thus pass from sight ; the former
arrangement would seem to be the rarer one. There may also be
incomplete degrees of both displacements associated with rotation of
the spine and resulting in the production of very curious and
puzzling forms of monstrosity; and, finally, both the anterior and
posterior limbs, the tail, and the head may be enclosed in the dorsal
cavity, giving rise to an ovoid mass to which are attached the
thoracic and abdominal viscera.

Spondylolisthesis may be considered along with lordosis, although
it differs therefrom in certain particulars. It consists in a disloca-
tion forwards and downwards (into the pelvic brim) of the body
of the fifth lumbar vertebra, which, therefore, comes to lie more
or less in front of the first sacral vertebra ; it is, in consequence,
a malformation which takes on great importance from the point of
view of the obstetrician. F. Neugebauer {Arch. f. Gyna:h., xix. 441 ;
xx. 133, 1882; xxi. 196, 1883; xxii. 347, 1884; xxv. 182, 1885;
Ann. dc gyne'e, xxi. 100, 1884) and many other writers have inves-
tigated the form of pelvis obtccta which is produced by the over-
hanging and displaced lumbar vertebra. The displacement probably
occurs only in postnatal life ; but in some cases at least it is made
possible by an antenatal defect. Lambl {Beitr. z. Geburtsh. u. Gynmk.,
iii. 1, 1858) regarded the defect as of the nature of a localised form
of spina bifida, but Neugebauer thought it much more probable that
it was due to an imperfect ossification and incomplete union of
the mterarticular parts of the vertebral arch of the fifth lumbar
vertebra (" spondyloschizis interarticularis congenita arcus verte-
bralis"). Lambl {Ccntr.f. Gyndk., ix. 356, 1885) made a strongly worded
reply to Neugebauer's criticisms, and, on the whole, it seems to me to
be quite possible that something of the nature of spina bifida might be
the cause of the separation of the body of the vertebra from its arch.
It may be mentioned here, as a curious addendum to the matter
under discussion, that steatopyga is apparently due to or associated

314 ANTENATAL PATHOLOGY AND HYGIENE

with a separation between the body of the last lumbar vertebra and
the base of the sacrum (D. Lambl, Ccntr.f. Gyndk.,\. 256, 281, 1881),
producing a backward tilting of the whole sacrum. Steatopyga, it
will be remembered, is the name given to the accumulation of fat on
the buttocks which is found in several African races, and especially
in the women of the Hottentots or Bushmen. It is not clear
whether the spondylolysis associated with steatopyga is a racial
antenatal peculiarity or not ; but investigations of an interesting
nature seem to be possible in this direction. It is perhaps somewhat
significant that a so-called racial birth-mark over the back in the
region of the sacrum has been described (Matignon, Arch. din. dc
Bordeaux, v. 416, 1896). It is said (Ashmead, Sei-i-Kwai Med.
Journ., xiv. No. 1, 1896) to be present in all Japanese babies as
a dark blue spot which vanishes during the first two years of life ;
and it is believed to be present as early as the fifth month of foetal
life. The pigment is situated in the corium. This so-called racial
birth-mark has been referred to by many recent authors. J. Deniker
{Bull, et me'm. Soc. d'anthrop. dc Paris, 5 s., ii. 274, 1901), for instance,
says it is not peculiar to the yellow races of mankind ; it is found
in Polynesia and among the Esquimaux. Although neither steato-
pyga nor this lumbo-sacral naevus can be regarded as peculiar to or
constant in any one race, they nevertheless suggest two reflections :
one of these is the tendency which some malformations have to affect
one race rather than another ; and the other is the frequency with
which the lumbo-sacral region is the seat of anomalies of various
kinds. From such racial and regional peculiarities it ought to be
possible to learn something of the ultimate causes of monstrosities.

Numerical Anomalies in the Vertebrae.

An increase or a decrease either in the total number of vertebrae
(33) or in the number of them in each region may occasionally
occur. Numerical anomalies, like the malformations above referred
to, seem to be commoner near the posterior (caudal) end of the
spine ; they are also commoner in the terminal members of the
various series than in the middle ones. It is rare, therefore, to find
either an increase or a decrease in the number of cervical vertebrae.
There are, however, some cases of eight cervical vertebrae (Eegalia,
Arch, per I'antrop., Firenze, x. 305, 1880 ; Dubreuil, Gaz. vied, dc
Par., 3 s., iv. 871, 1849, etc.), although some doubt may exist
whether the additional vertebra was a cervical one (with or without
ribs) or a dorsal. Six cervical elements have also been noted (H. Ooote,
Med. Times and Gaz., n.s., viii. 56, 1854), but diminution in the-
number would seem to be nearly always associated with such grave
states as anencephaly and spina bifida. An additional dorsal verte-
bra; (making thirteen in all) may be found at the caudal end of the
series ; in such cases there may be twenty-six instead of twenty-four
ribs. On the other hand, the twelfth dorsal vertebra, with its
ribs, may be wanting (Tenchini, Ateneo med, parmensc, i. 97, 1887 ;
P. Topinard, Rev. d'anthrop., vi. 577, 1877). Sometimes these

SPINAL ASSIMILATION 315

numerical defects or excesses in one region would appear to be com-
pensated by excesses or defects in another region of the vertebral
column ; but this is not constant. Variations in the number of the
lumbar, sacral, and coccygeal vertebrae are comparatively common ;
they will be referred to later (p. 317). These numerical variations
in the vertebra1 must have their origin early in embryonic life, if we
accept the theory that they are due to an original excess or deficiency
in the number of the mesodermic somites and of the sclerotomes
derived from them. Such anomalies may be expected to throw some
light upon the metameric theory of the constitution of the body.

Fusion of Neighbouring Vertebrae, Assimilation, etc.

Several cases are on record of fusion of contiguous vertebra'.
This process is normal in the sacral and coccygeal regions, and may
on this account be termed sacralisation of the spine. It has been
noted in the case of the atlas vertebra and the occiput by K. Bock-
shammer (Diss, inaug., Tubingen, 1861), T. C. F. Serger (Diss,
inawj., Halle a. S., 1888), D. D. Slade (Boston Med. and Surg. Joum.,
exxxiii. 57, 1895), Mouchotte (Bull. Soc. anat. de Paris, s. 6, i. 873,
928, 1899),Eegnault (ibid., ii. 691, 1049, 1900), and Apert (ibid., in.
58, 1901); in the case of the odontoid process and the atlas by G.
Romiti (Boll. d. Soc. tra i. cult. d. sc. med. in Siena, iv. 99, 1886) ; in
the case of the axis and third cervical by Bancroft (Proc. Acad. Nat.
Sc. Phila., p. 419, 1891); and in that of the sixth and seventh
cervical vertebrae by S. Bianchi (Arch, per I'antrop., Firenze, xix. 93,
1889). Far more interesting, however, are the anomalous relations
which may exist between the last lumbar and the first sacral verte-
bra or between the last sacral and the first coccygeal. Under normal
circumstances the sacrum is formed by the union of the 25th, 26th,
27th, 28th, and 29th segments of the spinal column. Occasionally,
however, it is made up of six segments, and the additional vertebra
may be either the fifth lumbar (the 24th) or the first sacral (the
30th). Now there exists at various levels in the spine a tendency
for the terminal members of one region to take on some of the
characters of the members of the immediately adjacent region ;
these vertebra* may be described as showing assimilation. A good
example is found in the presence of a foramen at the root of the
transverse process of the first dorsal vertebra (W. Gruber, Arch. f.
path. Anat., lxvii. 341, 1876 ; W. Turner, Joum, Anat. and Physiol.,
xvii. 255, 1882-83 ; xviii. 223, 1883-84) ; but a better is to be had in
connection with the sacrum. Not only may the fifth lumbar (24th
segment) resemble the first sacral in its characters, but it may also
imitate it in its relation to the vertebra immediately following it, in
other words it may fuse with it. This assimilation and fusion may
be bilateral and complete or unilateral and imperfect, and in this
way are produced two varieties of abnormal pelvis, that deformed
symmetrically by assimilation of the fifth lumbar vertebra, and that
deformed asymmetrically. In other cases it is the first coccygeal
vertebra which takes on sacral characters and is fused with the fifth

Q

16 ANTENATAL PATHOLOGY AND HYGIENE

sacral ; in this way another type of the pelvis deformed by assimila-
tion is produced. The last-named arrangement differs from the
simple anchylosis of the coccyx to the sacrum in the sacral characters
assumed by the first coccygeal vertebra. E. Tridondani {Ann, di
ostet. e ginec, xxiv. 1-44, 1902) has written a very interesting article
on these " pelves by assimilation " (" baeini da assimilazione "), in which
he shows, among other things, that such deformed pelves often show
other anomalies besides the fusion of the fifth lumbar and first
sacral vertebrae : spina bifida, for instance, may coexist, as may also
incomplete fusion of the sacral segments themselves, and traces of im-
perfect union of the pubic and ischial bones. Tridondani concludes
that the sacral anomaly is not due to rickets but is an arrested de-
velopment, and, from the fact that in the anthropoid apes the vertebra
which forms the sacral promontory is variable, he thinks that atavism
may possibly be invoked to explain the arrest in development.

Other abnormal fusions may take place in connection with the
sacral vertebrae. The obliquely-contracted pelvis of Nmgcle is well
known to obstetricians, and is generally regarded as due to a
unilateral anchylosis of the sacrum and the ilium ; according to
some there is a premature ossification in the cartilages of the sacro-
iliac articulation leading to the synostosis, and this may be due to a
fcetal osteoarthritis ; according to others there is an antenatal defect
in the development of the lateral mass of the sacrum and of the
adjacent ilium ; and, according to yet others, the starting-point is
unusual intrauterine pressure coining to bear upon the pelvis and
flattening it, and the pressure may be due to absence of liquor amnii
or to the existence of a malformation of the mother's uterus (uterus
unicornis, etc.). In the pelvis of Robert both the sacral wings are
absent, and there is bilateral sacro-iliac synostosis ; it may be due to
the same antenatal causes as produce the Ntegele pelvis; at any
rate, an instance of it has been met with in the foetus at birth (0. Graf,
Ein Fall von angcborcnem querverengtem Becken, Zurich, 1864). In
connection with the antenatal origin of the Eobert pelvis it is inter-
esting to note that F. Billitteri {Arch, di ostet, c ginec, i. 421, 498,
1894) has recorded a case in which this pelvic deformity was found
in two sisters (family prevalence).

In the two malformations just described (the Na-gele and Robert
pelvis) the sacral defect is in the ala3 and lateral masses, but there
are cases in which the sacral bodies themselves may be absent.
When this is the case the coccyx also is wanting. As we shall see,
when we come to study sympodia, sacral and coccygeal defects are
so common as almost to constitute an integral part of the monstro-
sity ; but they may also occur, although rarely, in cases which show no '
signs of fusion of the lower limbs. A. F. Hohl {Zur Pathologic des
Bechcns, p. 61, Leipzig, 1852), for instance, has described the case of
a new-born infant in which the sacrum was represented only by two
thin pieces of cartilage corresponding to the first two segments of the
bone: and H. Litzmann {Arch. f. Ghyncek., xxv. 31, 1884-85) has put
on record the case of an adult woman who died from peritonitis after
her third confinement, and in whom the fifth lumbar vertebra was

FCETUS ANURUS 317

rudimentary, the sacrum was practically absent, and the coccyx was
represented by a small nodule of cartilage. Litzmann's patient was a
twin, and he thought the pressure of the co-twin in utero might have
caused the defect at the posterior end of the spine. C. C. Werthheim
(Monatschr. f. Geburtsk. u. Frauenlcr., ix. 127, 1857) has met with
absence of both sacrum and coccyx in a new-born infant ; and Bland-
Sutton (Introduction to General Pathology, p. 85, 188G) tells of a
prematurely born infant in whom all the vertebra' below the first
lumbar were absent, and adds that the mother had fallen in the fifth
month of gestation and probably fractured the back of the foetus.
Yet another specimen exists in the Anatomical Museum of Brussels;
it was shown to Neugebauer by Professor Albrecht (Arch. f. Gyncelc.,
xxv. 22.">, 1885); and in it only the first and second segments of the
sacrum were represented. Albrecht called it a pseudo-pithecoid
pelvis, from its resemblance to what is found in the apes. 0. Piering
(Ztschr. f. He ill:., x. 215, 1890) has recorded a case of short sacrum
and apparent absence of the coccyx in an adult woman ; there was an
unusually long vulva with atresia ani vaginalis, the right scapula was
situated at a higher level than usual, and some of the ribs were absent.
To the pelvis without a sacrum, Taruffi (Storia clella tcratologia,
vii. 544, 1894) gave the name acocono-lecanus ; he described instances
of it in the calf and the pig; but he does not seem to have been
acquainted with any instances in the human subject. In Compara-
tive Teratology the term perosomus elumbis has been assigned by
Gurlt (Lchrb/ieh, ii. 88, 1832). The description of these specimens
of excess or defect of the sacral vertebra naturally leads me to the
consideration of the tailed and tailless foetuses ; but to them special
paragraphs must be given.

Foetus Anurus.

Since the human fcetus is normally tailless in the ordinary sense
of the word it is hardly correct to speak of an anourous type of
malformation in connection with it. At the same time the human
embryo in an early stage of its existence is distinctly " tailed," and
the remnant of that tail is found in the coccygeal vertebra ; so that
it is not inappropriate to regard absence of the coccyx as equivalent
to absence of the tail. The human coccyx is generally looked upon
as a part in process of suppression ; it is disappearing from the race.
It is common to find numerical variations in such parts, and in this
respect the coccyx forms no exception to the rule. The normal
number of segments is four; but Calori (Mem. d. r. Accad, d. sc. di
Bologna, s. 4, viii. 181, 1887) found five segments in 16 per cent, of the
cases examined by him, and Staderini (Monitore Zool. ital., v. 56, 95,
1894) put the percentage as high as 23. The latter writer met with
three coccygeal vertebra in 17 per cent, of the spines examined by
him. Within normal limits, therefore, the number of the coccygeal
vertebra:' may be said to range from five to three. In the early
embryo the number is probably always greater. When, therefore,
the coccyx in the human subject is absent, either alone or in conjunc-

318 ANTENATAL PATHOLOGY AND HYGIENE

tiou with some of the sacral vertebra', it is not incorrect to speak of
the foetus as anourous or tailless. I. Newton Snively (Med. News,
lxv. 545, 1894), for instance, has reported the case of a new-Lorn
male infant with several deformities, including absence of the coccyx
and backward displacement of the lower end of the sacrum. Prob-
ably, if the foetus anurus were more carefully looked for it would
be more often noted.

As might be expected, absence of the tail is more commonly
observed in the lower animals. The caudal defect may be either a
shortening or an entire absence. It has been observed in a number
of mammals, including the calf, sheep, pig, horse, cat, dog (Taruffi,
Storia della teratologia, vii. 563, 1894), mouse (Catalogue of the Tera-
tologkal Series, etc., p. 80, London, 1893), and rabbit (ibid., p. 120) ;
but it may also occur in other types, e.g. in the sole among fish
(ibid., p. 120). In some of the recorded cases the coexisting mal-
formations (e.g. anencephaly, existence of a cloaca, etc.) were so
severe as to dwarf the importance of the anourous state. Gurlt
(Zehrbuch, ii. 94, 1832) gave to the uncomplicated form the name
perocormus ecccudatus; when it was associated with absence of the
anus and cloacal formation, he called it atretoeormus aprochis; and
when it was combined with anencephaly and rhachischisis, Joly
(Gaz. d. hup., Ann. xxviii. 191, 1855) described it as anencephalus
anurus. The tailless state may occur sporadically : the anourous
animal is the offspring of those that possess tails, and some curious
family histories have been recorded. Mazzarelli (quoted by Taruffi,
wp. cit., vii. 5G5, 1894), for instance, writes of a tailed male cat which
impregnated two females which had both previously had normal
kittens ; on this occasion the kittens of both mothers had much
shortened tails along with other abnormalities. Apparently, how-
ever, absence of the tail may become the character of a race, as in
the famous cats of the Isle of Man and of Japan ; the name anurus
endemicus has been given to this racial type. In these animals the
tail is not, as a rule, entirely absent, but it is reduced to five or six
instead of twenty-two or twenty-three caudal vertebrae, and these
are often somewhat twisted or fused together. Some interesting
observations have been made on the results obtained when such
tailless cats have progeny by the ordinary tailed variety. E. Anthony
(Bull. Soc. d'anthrop. cle Paris, 4 s., x. 303, 1899), for instance, has
analysed the litters of kittens which a female Manx cat had by ordinary
cats ; among the twenty -four kittens (in six litters) there were ten
that had long tails (like the father) and fourteen that had short
ones like the mother, or none at all, or short ones although not so
short as the mother's. The proportions in the various litters differed ;
thus, in one litter, there were five kittens resembling the mother
and one resembling the father, whilst in another the paternal infiu-
ence was seen in three and the maternal in one. Anthony concludes
that the anourous state of the Manx cats is an anatomical character
acquired long ago and so well fixed as to constitute a race.
Whether, however, the originator of this tailless race was a sporadic
case or the result of an accidental mutilation is not, of course,

"TAILS' 319

known. Here the individual case widens out suddenly into the
large question of the heredity of acquired conditions. Into the fierce
and prolonged discussion that has taken place round this disputed
matter I cannot here enter, for it is rather a question of germinal
than of embryonic pathology ; but it may be said that there is, to
my mind, some evidence, although it is neither large nor conclusive,
that animals which have had their tails removed artificially do
occasionally have short-tailed individuals in their progeny (11. Bonnet,
Beitr. z. 'path. Anat. u. ally. Path., iv. 67, 1888-89). Whether, how-
ever, they have them more frequently than can be accounted for
by the occurrence of the usual sporadic cases is not clearly estab-
lished. (A summary of recent views on this subject will be found
in Delage's L'HMdiU, 2nd ed., pp. 222-226, 288, 297, 849, 1903.)
With regard to the mode of origin of the sporadic cases, several
theories have been advanced, but they are not altogether satisfactory :
perhaps in some cases the mother animal bites off the tails of her
offspring at birth, either intentionally or by accident (mistaking the
tail for the umbilical cord) ; perhaps in other cases there is a paternal
influence acting in some mysterious manner, as Mazzarelli's case
suggests ; and there are other hypotheses.

" Tails."

Not Teratology only but Anthropology and Ethnology are all
concerned in the discussions which have taken place regarding the
occurrence of "tailed infants"; homo caudatus or uro-anthropos
(Taruffi) has always excited curiosity and aroused controversy. In
Mythology, also, the records of tailed individuals and races are not
wanting.

A great deal has been written and more has been rumoured about
the existence of a tailed race of human beings. From the earliest
times of Herodotus and Pliny and Ctesias, up to the most recent
reports of modern African explorers, the credulous public has
been asked to believe in the presence somewhere on the earth's
surface of men with tails. Eumour has been busy with reports as to
the geographical location of this tailed race : now, on an old map,
we find it in Tierra del Fuego, the abrupt and laconic legend being
" homines caudati hie " ; again, it is in Polynesia (in New Britain),
and the infants that are born without the caudal appendage are, so
we are told, summarily killed ; again, it is near the sources of the
Amazon (" cependant je laisse a chacun ajouter la foi qu'il voudra a
ces faits," adds a critical reporter), or in Trebizond in Asiatic Turkey
among the descendants of Constantine the Great (" d'ailleurs la
puissance de Dieu est infinie ; il fait tout ce qu'il lui plait, et cree,
quand il le veut, des objets incoimus aux hommes "), or in Turkestan
(" stinkendes Ungeziefer mit Schwanzen "), or in India in Eajputana
(" the long-tailed Eanas of Saurashtra "), or in China, or in Japan,
or in Formosa, or in the Malay peninsula, and the islands of Borneo,
Sumatra, and the Philippines ; but in Africa more perhaps than in
any other part of the world have there been reiterated " travellers'

320 ANTENATAL PATHOLOGY AND HYGIENE

tales about tails " (in Marocco, among the Niam-Niams of Central
Africa, in Loango, in Bomu, etc.). Even in Europe, more especially
in the west and south, there are legends, usually of considerable
antiquity, of the occurrence of tailed peoples. Scotland and England
are not without their records ; and the people of Eochester (" ilia
gens incredula ") seem to have been most unfortunate, for by
offending first St. Augustine and later Thomas a Becket, their
children were born with tails, as a mark, so the chroniclers say, of
God's displeasure. The reader who may desire to know more con-
cerning these quaint beliefs of the past will find what he wants in
Bartels' classical papers {Arch. f. Anthrop., xiii. 1, 1881 ; xv. 45,
1884) and in the many references which they contain. Some articles
which have appeared in recent years and which may also be con-
sulted are by Tirant (Bull. Soc. d' anthrop. dc Lyon, i. 158, 1881-82;
ii. 157, 1883), J. Wilson (Ztschr.f Mhnol.,xii. 74, 1880), T. H. Parke
(My Personal Experiences in Tropical Africa, -p. 397, 1891), P. D'Enjoy
(Anthropologic, vii. 531, 1896), and Zaborowski (Bull. Soc. d'anthrop.
dc Paris, 4 s., viii. 28, 1897).

Some parts of the evidence regarding the tailed races have been
hearsay, and, therefore, outside the province of scientific inquiry ;
and other parts of it have been founded upon observations upon
tailed individuals made at such a distance as to leave the possibility
that the caudal appendages were of the nature of attached ornaments
and were not structurally united to the body. The tailed peoples
have always shown a tendency to recede as the curious traveller or
anthropologist advanced towards them, and when he reached his
furthest point it was to find that the "homines caudati" were still
three or four days' journey further on. There is no really reliable
evidence of a tailed race of human beings, although, as we shall see
immediately, there are not a few records of individuals with tails or
with caudal appendages resembling tails. " Homines caudati hie "
cannot yet be written over any part of the world's surface ; but it is
more than likely that in every country a tailed baby now and again
makes its appearance, to cause loud wonder among the vulgar and
suppressed scientific excitement among the learned.

Let us look now at the individual or sporadic cases of caudal
appendage which have been put on record at various times and in
various places. Some of the older records contain in them a sug-
gestion of the mythical, although the illustrations that accompany
them are apparently most convincing. As a type of such ancient
and dubious cases I may cite that reported by Krahe (Phil. Trans.,
xiv. 599, Oxford, 1684). It is entitled " The Description of a Mon-
strous Child, born Friday, the 29th of February 1684, at a village
called Heisagger, distant about four English miles from Hattersleben,
a Town in South Jutland, under the King of Denmark's Dominion,
communicated by Mr. Christopher Krahe, a member of the Ecclesi-
astical Consistory and Provost of all the Churches belonging to the
said Diocess." The record, then, is most circumstantial, and the
reader who doubts may find Hattersleben (or Hadersleben) on the
map, in South Jutland, just as is affirmed. The mother of the child

"FALSE TAILS" 321

was wife of a soldier ; the infant's legs looked as if they had been
hacked, and there were swellings on them like bullets ; and a maternal
impression is therefore indicated. " The face did look pretty old " ;
and at the forehead there were excrescences, " as if it were artificial
laces." " With the left eye it did look fiercely, keeping that other
close." There were also malformations of the back of the head, of
the left arm, and of the toes. The description of the tail (which is
shown almost reaching the ground in the figure) is disappointingly
short : " the Tail, which was strangely grown out of the back-part,
was a quarter of a Sealandish Ell long." The mother had two other
sons, alive and well ; " but this Monster, after it had cryed out 2
or 3 times, died presently." The tail, long and thin, is represented
as springing from between the buttocks just above the anus. It is
difficult to know what to make of this record : but the most probable
explanation is that an infant deformed in a way resembling the de-
scription given was actually born and died where and when it is stated ;
and that the artist considerably lengthened the tail, and tried to
give some touches of artistic verisimilitude to the lower limbs in
accordance with the father's military experiences. A suspicion of
the same aesthetic amplification attaches to the illustration which
accompanies J. S. Elshott's description of a " Puella monstrosa," who
had an umbilical hernia, a left arm ending in a point, deformed digits,
and two rat-tail-like appendages, one attached to the right thigh and
the other over the tip of the coccyx. By a curious coincidence, the
father of this infant was also a soldier (Miscel. Acad. not. curios.,
iv.-v., Append, p. 80, 1676). I need cite no others of these seven-
teenth century cases, for the two already mentioned will serve as
types. It is to be regretted that the shadow of doubt rests upon
them, for if they were free from it they would stand out prominently
as the best instances that we possess of truly tail-like tails in the
human subject. The shadow, however, does rest upon them.

Before I proceed to describe some modern examples of tailed
infants, it may be well worth while to narrow down the whole sub-
ject by excluding the "false tails." In the first place, it is necessary
to set aside sacro-coccygeal neoplasms of all kinds. I recently saw
(with Mr. Stiles) a female infant with a large tumour attached to the
post-anal region which, when examined by the Bontgen rays, was
found to contain a spinal column and ribs ; obviously this was not a
tail but an attached twin or parasitic foetus. Teratomata, teratoid
growths, and dermoid cysts may also grow in this region ; Thirk's
case (Oesterr. med. Wchnschr., N. 36, 1121, 1847) and Jacob's (Dublin
Hosj). Rep., iv. 571, 1827) probably both belong to this group.
Lipomata, also, of the sacral region are not tails, although when they
become pendulous, as in one of Bartels' cases (Deutsche Ztschr. f.
Chir., xx. 100, 1884) and in Fieux' case (Rev. mens, de gynic. d'obst.
et paed. de Bordeaux, iv. 456, 1902), they may closely simulate them.
All caudal appendages composed of fat are not, however, of necessity
lipomata, for Virchow found in one of them (Greve's specimen) some
traces of the notochord (Arch. f. path. Anat., lxxii. 129, 1878 ;
lxxix. 176, 1880). Again, the sac of a spina bifida of the sacral

VOL. II. 21

322 ANTENATAL PATHOLOGY AND HYGIENE

region must not be mistaken for a tail (Fig. 54) ; at the same time a
caudal appendage and a spina bifida sac may be associated, as we shall
see immediately Neither can we regard the hairs which sometimes
arise over a spina bifida occulta, and grow to some length, as a true
tail. These various structures may all become caudiform, but they
are to be separated off from human tails.

I have personally been able to examine several cases of " tails "
and "pseudo-tails." In one of these (Fig. 51) there was an open
lumbar spina bifida, and below it a thick caudiform growth with a
little button-like projection at its lowest point ; it seemed to be no
more than a lipoma. In another case (Fig. 29) there was a sacral
spina bifida sac ; and in another (Fig. 31) there was a conical pro-
jection which seemed to contain the lower end of the spinal column,
and from the tip of which an amniotic band took origin. The latter
of these two cases has some claim to be regarded as a tail. In another
case, which I saw in January 1902, there was a curious combination
of spina bifida and tail : the child, a male, was fifteen months old when
brought under my notice by Dr. T. J. Thyne ; it was born six months
after the death (from typhoid fever) of the father, and the mother
had one other child, a female, nine years of age, and normally formed ;
the infant had a sacral spina bifida sac, covered with skin, and
measuring G inches in circumference, and from the apex of this
there projected a tail-like appendage. The " tail " was 1 inch in
length, and was covered with normal skin ; it was quite soft ; it
consisted of two parts, a proximal, resembling the first phalanx of
the little finger, and a much thinner distal part, more tail-like. Un-
fortunately, a photograph of this caudal appendage was not obtained.
It had resemblances to that described some time ago by Edmund
Owen {Trans. Path. Soc. Loud., xxxix. 425, 1888), who compared it
to a " fat little finger." In yet another case which came under my
notice there was a " tail." This was the foetus, appearing in Figs. 48
and 49 in vol. i. of this Manual (p. 341); it suffered from an exu-
berant overgrowth of cartilage in various parts of the skeleton
(chondrodystrophia fcetalis liypcrplastica), and the coccyx, participat-
ing in this change, grew into a marked " tail."

What may be called " true tails " may be subdivided into those
which contain bone or cartilage and those which do not ( soft tails),
or into those which are attached or adherent (" die angewachsenen
Schwiinze," Bartels) and those which are free (" die freien Schwanze,"
Bartels). Of tails containing bone or cartilage or rudiments of
vertebrae there are very few specimens known. T. Bartholin (Hist,
anat. ct vied, rar., cent, vi., hist. 44, p. 268, 1681) and M. F. Lochner
(Miscel. Acad. nat. cur., Dec. ii., Ann. vii. p. 230, 1689) have recorded
cases in which it is stated that more than five coccygeal vertebrae
were present in the tail ; but it is a pity that the details are not more
convincingly stated, and that Lochner writes "in a merry vein," as
Jacob (loc. cit.) puts it. No modern instance has been reported of a
human tail containing more than the usual number of coccygeal
vertebra', namely, three to five, and even of them there are very few
(Voltaire, Diet, philos., xi. 211, 1832 ; Ornstein, Ztschr. f. Ethnol.,

"TAILS" 323

xi. 303, 1879 ; C. Hennig and A. Eauber, Arch. f. path. Anat., cv.
8)5, 1886). Of specimens containing cartilage or rudiments of the
notochord I may refer to those of J. Wilson (Ztsclir. f. Ethnol., xii.
74, 1880), of S. Blancard {Collectanea medico-physica, Amsterdam,
1080-88), of Fleischmann (1841) and Gerlach (Morphol. Jahrb.,
vi. 106, 1880), and of J. H. F. Kohlbrugge (Natuurk. Tijdschr. v.
Nederl. Indie, lvii. 163, 1898). As an example of this group of tails,
1 may cite the case described by Hennig and Eauber (loc. cit., supra).

The mother of the tailed infant seen by Hennig was a I -para ;
she was a dairymaid, and had got a fright at the parturition of a cow.
Her child, a female, presented by the breech, and there was very little
liquor amnii ; it was only 29 cms. in length, the lower limbs being
short. The upper part of the body was well formed, and the
number of vertebrae above the sacrum was normal. The lower limbs
showed several malformations (peromely), and there was atresia ani
vestibularis and absence of the labia majora. There was a post-anal
dimple (fovcola coccygca) 1 inch above the base of the tail ; it corre-
sponded to the apex of the sacrum, and it was surrounded by a ring
of hairs. The sacral vertebra? were only four in number. There was
a groove from the post-anal dimple to the root of the tail. The tail
itself was a little over an inch in length (2*7 cms.), was somewhat
triangular in form, was covered with normal skin, had some lanugo
hairs at the tip, and possessed a hard core. The core consisted of
cartilage, made up of a proximal and a distal part, apparently
representing five vertebras. The tail was somewhat flattened at the
tip, and contained, in addition to the cartilage, some muscular fibres
(levator caudae or extensor coccygis, etc.). On more minute examina-
tion the two segments of the tail were found each to consist of a small
cylinder of bone (diaphysis), with two extremities (epiphyses) of
hyaline cartilage ; they may be regarded either as vertebras arrested
in development, or as a parasitic appendage, but the authors regard
the former as the more probable conclusion. They were attached by
a fibrous band to the sacrum.

Of soft tails several specimens have been reported. They may
be attached or free: of the former I may mention Labourdette's
case (Journ, gin. de mkl., chir., et pharm., xxxii. 375, 1808), and
those of Bartels (Arch. f. Anthrop., xiii. 1, 411, 1881) and of H. W.
Freund {Arch. f. path. Anat, civ. 531, 1886) ; and of the latter, those
of Oskar Sclueffer (Arch. f. A7ithrop.,xx. 189, 1891-92), of Pyatnitski
(Diss, inaug., St. Petersb., 1892), of K. K Vinogradoff (Vrach, xv.
901, 1894),Cof W. Scheboldaeff (Zemsk. Vrach, vi. 2, 28, 1893), of E.
G. Harrison (Johns Hopkins Hosp. Bull., xii. 96, 1901), and of E.
Hagenbach (Arch. f. Bin. Chir., lxvi. 426, 1902). The attached tail
usually consists of a triangular elevation of the skin covering the
lower end of the sacrum ; it passes to one side of the anus and may
become continuous with the raphe in front of it. Harrison's
example of a free soft tail is a typical one, and calls for a brief
description.

In the case exhibited in 1900 by Watson (Johns Hopkins Hosp.
Bull, xi. 114, 1900) and examined by Harrison (ibid., xii. 96, 1901),

324

ANTENATAL PATHOLOGY AND HYGIENE

the tail was attached to a male infant, who showed no other
anomaly save shortening of the toes of one foot (two phalanges in
each). The tail (Fig. 55), which was If inch long at three weeks and
2]- inches at three months, arose about | inch below the coccyx.
Above its root was a groove leading to a well-marked post-anal dimple
(foveola coccygea). It was covered with normal skin, had a firm but
not a hard consistence, and it had three recognisable segments. The
basal segment was short and was evident only on the ventral side ;
the middle part was an inch in length, and was clearly separated by
a constriction from the end segment ; the last-named portion was
curved to the right and ventrally, and ended in a rounded blunt
extremity. There were numerous hairs, especially near the tip. It
is very noteworthy that the tail was slightly movable : when at rest

Fig. 55. — Harrison's Patient with Soft Tail.

it lay extended in the middle line, but, when the child was irritated,
it was contracted and the terminal segment was drawn in and flexed
to the left side. After the tail had been amputated it was carefully
examined. The skin was normal save on the ventral aspect, where
there was some thickening of the epidermis. The sebaceous and
sudoriparous glands were normal, and the hairs were obliquely in-
serted pointing towards the tip. The bulk of the tail was made up-
of areolar tissue containing much fat. There was no trace of noto-
chord or spinal cord ; but there were some bundles of voluntary
muscular fibres which took origin from the subcutaneous tissue near
the proximal end of the middle segment, and were inserted into the
skin of the terminal segment mostly on its left side. No muscles
passed from the trunk to the tail. There were several arteries and
some small veins, also a number of small nerve trunks.

« TAILS "

325

Before I refer to the mode of production of tails I may take note
of the fact that such structures are usually found in foetuses which
show other malformations.
Schseffer (loc. cit.) has made a
careful analysis of the associated
malformations : among them
were atresia ani, exomphalos,
exstrophy of the bladder, asym-
metrical development of the
whole body, defective limbs,
anomalies of the genital organs
(e.g. hypospadias), amniotic
bands, anencephalus, hypertric-
hosis, polymastia, and club-feet.
A tail has also been found in
double terata (Hohlfeld, Diss.
inaufj.,WuYzhuY\r, 1861 ; Becker,
Diss, inaug., Gottingen, 1889)
and in several instances of sym-
podia. Its association with fusion
of the lower limbs (sympodia)
is particularly interesting : when
it occurs it would seem that in
some instances (e.g. Euge's case,
Arch. f. path. Anat., cxxix. 381,
1892) the "tail" is due to a
dislocation of the lower end of
the spine backwards and to one
side (as in one of my cases, Fig.
56) ; while in others it is simply
a soft caudiform structure which
is met with (" penis-like " in W.
Maclaren's specimen, Edirib. Med.
Journ., xviii. 658, 1873 ; xix.
590, 1874; "soft, vascular,
spongy, and obtuse " in J.
Hofer's, Acta Hclvct., iii. 066,
1758 ; like a pig's tail in D.
Superville's, Phil. Trans.,x\i. 302,
London, 1744 ; and with its tip
of livid colour and divided into
two lobules in B. W. Huesker's
case, Diss, inaug., Gryphias, 1841).

It is necessary, now, to con-
sider very briefly what explan-
ation Teratology has to offer
for the occurrence of such true
" tails " as have been described.

I

Fig. 56. — External Appearances, posterior
view, of Uromelic (Sympodial) Fcetus with
Caudal Projection. Specimen No. 250.

Embryology supplies part of the

answer, and the general principles of Teratogenesis when applied to
this part of ontogenesis complete the solution of the problem. There

326 ANTENATAL PATHOLOGY AND HYGIENE

is a stage in the development of the human embryo when it possesses
a real tail ; it is best marked in embryos of about 14 mms. in length
(fifth week); and it may be well seen in Figs. 12, 13, and 14, and
especially well in Fig. 17 (p. 54). Harrison's description of the tail in
the human embryo (founded on the examination of Mall's specimens)
is good, and I quote from it here. There are seven coccygeal vertebra-
in embryos 14 mms. long, and from the third of these onwards is the
'• tail," i.e. the part which projects free from the trunk. Half of the
length of the projection is occupied by the vertebra1, while the distal
part contains no vertebrae ; the former part may be called the coccy-
geal projection (Stcisshdckcr), and the latter the "caudal filament."
The caudal filament is bent towards the dorsum and ends in a slight
knob ; the medullary cord (spinal cord) runs to the tip of it and
ends in a vesicular swelling ; the notochord and the terminal branches
of the aorta (middle sacral) and inferior vena cava also pass into
it, but not to the same distance as the medullary cord ; and there
is a mesenchymatous network in it with a thickened area, perhaps
representing the post-anal gut of earlier embryonic life. At the
level of the thirty-second vertebra (just above the base of the tail)
the medullary cord suddenly becomes constricted to form the filum
terminale ; there are few or no neuroblasts beyond this point ; and
the walls are composed of columnar epithelial cells. As embryonic
life goes on the caudal filament atrophies ; the part containing
vertebrae (coccygeal projection or StcisshocJcer) remains for a time as
a prominence, but gradually disappears by the growth and alterations
in surrounding parts and by the reduction (by fusion) of the seven
or six coccygeal vertebra? to five, four, or three. It begins, now, to
be clear that these human " tails " are the result of arrested develop-
ment. The soft, boneless tails are due to persistence of the caudal
filament (His), and the rarer cartilaginous or osseous tails are due to
the arrest of development in the later stage, when only the Steiss-
hocker is left. We are not in a position to explain completely how
the arrest is brought about, or how the development of neighbouring-
parts affects it or is affected by it ; but we may surmise, with
Schaefier (loc. cit.) and others, that either amniotic compression or
amniotic adhesions or both have something to do with it. It is
interesting to note that several of the associated malformations
{e.g. sympodia) are ascribed to amniotic narrowness, and it is quite
possible that the caudal fold or part of the amnion may remain
attached to the caudal filament or Steisshocker and prevent its
normal atrophy or its embedment in the surrounding structures.
Human " tails," therefore, would seem to be due to the persist-
ence of a state which is transitorily present in the human embryo
itself ; no evidence can be extracted from them to support the
view that they are atavistic revivals of the animal tail of a far-
back ancestor. Whatever may be its origin, the human "tail "had
best be removed by the surgeon's knife to relieve the patient from
its " discomfort and reproach."

Literature. — In addition to the references on " Tails " already given, I

COCCYGEAL FOYEOL.E 327

may add some others which have appeared, most of them subsequent to
Bartels' articles in 1881 and 1884. 0. T. J. Mohnike, Uber geschwdnzte
Mensehen, Minister, 1878; Albrecht, Ber. ii. d. Verhandl. d. deutsch.
Geselhch./. Okir., p. 58, 1885 ; A. Ecker, Arch. f. Anthrop., xii. 129, 1880 ;
xiii. 483, 1881 ; Lissnek, Arch./, path. Anat., xcix. 191, 1885; E. Sonnen-
bubg, Berlin. Id in. Wchnschr., xxi. 790, 1884 ; H. W. Eaton, Science, iii.
673, 1884; A V. Elissejeff, Vrach, vii. 295, 1886; H. E. Steinbach,
Diss, inaug., Berlin, 1889; Ch. Eer£, Nouv. iconogr. de la Salpet., iii. 45,
1890; F. Keibel, Anat. Am., vi. 170, 1891 ; Arch. f. Anat. u. Entwcklng-
sgesch., 356, 1891 ; K. A. Belinovski, Chir. Laitop., ii. 57, 1892; Dickin-
son, Brooklyn Med. Journ., viii. 568, 1894; J. Berry, Memphis Med.
Month., xiv. 105, 1894; V. N. Kniazeff, Vrach, xv. 903, 1894; C.
Garcia, Med. contemp., Lisbon, xv. 93, 1897; Unger u. Brugsch, Ztschr.
f. Ethnot., xxxv. 469, 1903; B. Chatterton, Indian Med. Gaz., xxxviii.
300, 1903; Hennig, Deutsche med. Wchnschr., xxix., Ver.-Beil, 101, 1903.

Coccygeal Foveolae, Infundibula, and Fistulae.

What Max Bartels did for the study of " tails," Ecker may be
said to have accomplished for the curious minor malformation known
as the post-anal dimple or foveola coccygea (Arch. f. Anthrop., xi.
281, 1879 ; xii. 129, 1880). The little depression or fossa situated
behind the anus (which occasionally draws to itself the surgeon's
attention by becoming a sinus, a fistula, or a cyst) had indeed been
recognised before Ecker's time ; but after his articles had made their
appearance it was realised that a new teratological type had been
added to the already long list, and that it had come to us along
with an adequate embryological explanation of its mode of origin
and meaning. Of contributions prior to the appearance of Ecker's
papers I select two for brief note. One of these was by Lawson
Tait (Nature, xviii. 481, 1878; Rep. Brit. Ass. Adv. Sc, xlviii. 606,
1879): he found a well-marked sacral dimple or cutaneous de-
pression over the lower end of the sacrum in 23 per cent, of the
patients in the Birmingham Hospital for Women ; he thought it
indicated a cicatrix ; and, from an observation on a tailless kitten, he
suggested, somewhat fancifully, that it might be the hereditary
cicatrix of the spina bifida by which the human tail had been lost,
In the same year, Fere (Bull. Soc. anat. de Paris, 4 s., iii. 309, 532,
1878) described an infundibuliform circular depression at the level
of the lower part of the sacrum in a child with a double uterus and
vagina ; he also met with a case in which the posterior surface of the
sacrum-coccyx was vertical, while the point of the coccyx was turned
backwards and had the infundibulum or dimple just over its tip,
the skin being adherent to it by fibrous bands. He saw several other
instances, and pointed out how cysts might form in them by reten-
tion of products from the glands of the skin. He regarded them as
due to imperfect closure of the " posterior umbilicus," a somewhat
hypothetical aperture.

Ecker's contributions to the subject (loc. cit.) contain also an
investigation into the anatomy of the posterior end of the trunk in
embryonic and in early foetal life. He found in the case of the

328 ANTENATAL PATHOLOGY AND HYGIENE

young foetus and new-born infant that the lanugo hairs converge to
form a vertex or vortex over the coccyx ; this he callecT the vertex
coccygeus (Steisshaarwirbel). Above the vertex was a small hairless
area, the glabella coccygea ; and in its neighbourhood there was some-
times a distinct depression or hollow, the foramen caecum retro-anale
(Hyrtl) or foveola retro-analis (Luschka). Ecker preferred to call
the depression the foveola coccygea (Steissbeingrubchen). He found
it in the foetus, in the new-born infant, and even in adults, and
sometimes it occurred in combination with a " tail." It usually was
single and occupied the exact middle line, very nearly over the tip of
the coccyx and at least 1 cm. behind the anal aperture ; but it might
be double and asymmetrical. It was not restricted to the foetuses of
European races, but occurred also among the Chinese and Mulattos.

In 1882, Terrillon (Rev. de chir. ii. 269, 1882) pointed out the
association of the foveola with fistula^, sinuses, and abscesses over
the sacrum in the adult, and recorded three instances of inflammatory
swellings in the intergluteal fold having this congenital origin.
Eeclus (ibid., p. 342) described an instance of fistula arising from
such a foveola, and Lannelongue (ibid.) spoke of a case in which a
clinical thermometer was by mistake placed in such a depression
instead of in the anus. The latter writer found foveola' in 95 cases
out of 130, while Heurtaux (ibid., p. 343) met with 42 in 960, or 1
case in 23. A. Dunlop (Lancet, i. for 1882, p. 729) described several
interesting instances of sacral dimple ; he showed how in some cases
it occurred in association with a coccyx which had a vertical direc-
tion or was turned back at the tip, while in others the coccyx
was quite normal. Peyramaure-Duverdier (These, Paris, 1882), J.
Couraud (Thtec, Paris, 1883), H. Wendelstadt (Diss, inaug., Bonn,
1885), and 0. Madelung (Centrlbl. f. Chir., xii. 761, 1885) also made
contributions to our knowledge of this foveola or para-coccygeal in-
fundibulum. F. Tourneux and G. Herrmann (Journ. de I'anat. et
physiol, xxiii. 498, 1887) tried to find a more satisfactory explana-
tion of its origin : they showed that at first the medullary canal
reaches the tip of the coccyx, where it becomes adherent to the
deeper layers of the derma ; then through the quicker growth of the
spinal column the medullary canal (spinal cord) ascends ; but coccy-
geal remnants (" vestiges coccygiens ") remain uniting the coccyx to
the skin and constituting the " caudal ligament " ; about the sixth
month of intrauterine life these remnants atrophy, the surrounding
soft parts grow, the coccygeal projection is effaced by the straighten-
ing of the spine, and so the skin is drawn in as a dimple, foveola, or
infundibulum. W. Zoege von Manteuffel (Deutsche mcd. Wclmschr.,
xvi. 473, 1890) described two cases in which fistuke resulted from'
inflammation in the foveoke, and T. Wette (Arch./. Tclin. Chir., xlvii.
343, 1894) recorded seven instances of abscess and fistula1, pointing
out also the possible relation of the foveola to dermoid sacro-coccygeal
cysts.

Fere (Nouv. iconogr. de la ftedpet., x. 195, 1897) has in recent
years drawn an interesting analogy between the hair vortex over the
sacrum and that which exists over the cranium. He thinks they

MALFORMATIONS OF SPINAL CORD 329

represent the posterior and anterior ends of the dorsal groove of
embryonic life ; each is, therefore, of the nature of an umbilicus, the
posterior dorsal and anterior dorsal umbilicus. Anomalies may
occur in connection with both : there may be two vortices, for
instance, or the single one may be displaced to one side {Famillc
neuropathigue, p. 259, 1898). P. Godin (Compt. rend. Soc. dc biol., 10
s., iv. 656, 1897) has noted hereditary transmission in relation to this
malformation, and has described fistulse in a father, his son, and two
grandchildren ; Lawson Tait (loc. cit.) had already referred to cases
of sacral dimple in a mother and all her children. M. Salaghi {Arch.
di ortoped., xvi. 10, 1899) has pointed out that cysts developed in
these foveokc might be mistaken for spina bifida.

I have met with quite a number of sacral dimples in the series of
over three hundred deformities which I have examined. They may,
for instance, occur in association with spina bifida and with anen-
cephaly. They are cutaneous invaginations ; and they probably
account not only for the sinuses and fistuke which arise over the
sacrum and coccyx, but also for the secpuestration-dermoids of this
region. Bland-Sutton (Tumours, p. 337, 1903) has compared these
sinuses to the interdigital pouch of the land). The teratogenesis of
sacral dimples has been already referred to ; but it may be added
that Cleland regards them as due to arrest of growth of the not<>-
chord (Journ. Anat. and Physiol., xvii. 289, 1883; Memoranda in
Anatomy, i. 185, 1889).

Miscellaneous Malformations of the Spine.

In addition to the anomalies already described, I may name some
others ; space forbids that I do more. There is the presence of a
foramen at the root of the transverse process of the first dorsal
vertebra (W. Gruber, Arch. f. path, Anat,, lxvii. 341, 1876; W.
Turner, Journ. Anat. and Physiol., xvii. 255, 1882-83; xviii, 223,
1883-84). There may lie anomalies in the form and connections of
the atlas and axis vertebrae (G. Sangalli, P. 1st. Zomb. di sc, e lett.,
2 s., hi. 515, 1870 ; W. W. Keen, Amer. Journ. Med, Sc, n.s., lxvii. 412,
1874 ; G. Romiti, Poll, d. Soc, tret i cult, d. sc. med, in Siena, iv. 99,
1886; W.Turner, Journ. Anat. and Physiol, xxiv. 358, 1889-90;
H. Waterhouse, Proc. Anat, Soc. Gr. Brit, and Ireland, p. xxxiii., 1893).
Persistence of the notochord in the lumbar region of the spine in an
adult has been described by J. Musgrove (Journ. Anat. and Physiol.,
xxv. 386, 1891). Half-vertebra3 have been already referred to.

Malformations of the Spinal Cord.

Of the malformations of the spinal cord apart from spina bifida
comparatively little is known. Doubtless ere many years go by there
will be much work done in this direction. Meanwhile I may note
that a double canal or multiple canals have been met with both in the
human subject (J. Wagner, Arch. f. Anat., Physiol., u. wissenseh,
Med,, 735, 1861) and in the chick (G. Cutore, Atti d. Accad. Giocnia

330 ANTENATAL PATHOLOGY AND HYGIENE

di sc. nat. in Catania, 4 s., xii. and xiii., 1898-1900); these have
been found not to open into each other nor into the true central canal,
although they may be lined by the same kind of epithelium. Dupli-
cation of the spinal cord has also been met with apart from spina
bifida (F. Cecco, Morgagni, i. 307, 1857 ; P. Foa, Arch, per Ic sc. med., xi.
423, 1887); and absence of some of the nerve roots would appear to
be not uncommon (A. Adamkiewicz, Arch. f. path. Anat., lxxxviii.
388, 1882).

I have recently seen sections of the spinal cord of a seven months'
foetus, which Drs. Matthew and Waterston were examining (Bel).
Neurol, and Psychiat., i. 233, 1903) ; in the cervical region it showed
marked asymmetry from almost total absence of the pyramidal fibres
on one side, and there was a fissure in the position where the fibres
should have been found ; in the medulla an explanation of the ano-
maly was found in the fact that at the level of the decussation of
the pyramids the crossing of fibres took place from one side only.
This, then, was an instance of heterotopia of the white columns of
the cord.

Several cases of heterotopia of the grey matter in the spinal cord
are on record. The grey matter may be found in separate masses or
in parts of the cord which normally do not contain it {e.g. between
the white columns). P. Kronthal {Neurol. Ccntrlbl., vii. 97, 1888)
described a case of this kind, and drew together out of literature a
number of other instances. H. T. Tooth (Trans. Path. Soc. Lond.,
xlii. 14, 1891) and C. M. Campbell and W. Aldren Turner {ibid.,
p. 20) also described cases ; in one of these there was general
paralysis and in the other myelitis. It does not appear that the
cord anomaly gives rise to symptoms by itself, but it would seem to
predispose to diseases such as myelitis. There may also be marked
asymmetry between the grey substance in the two halves of the
cord, as was seen by P. Schiefferdecker {Arch. f. mikr. Anat., xii. 87,
1875). Myelitis in foetal life has been regarded as a cause of the
heterotopia of the grey substance; by it the neuroblasts may be
hindered from reaching their proper places (K. Fusari, 1896). Much
more information, however, is needed before the vexed question of
teratogenesis can be decided.

Distended cavities in the spinal cord may be met with, and these
would appear to be due to localised dilatation of the central canal or
hydromyelia. Hydromyelia occupies an ill-defined position in relation
to two other morbid states, spina bifida and syringomyelia. Ap-
parently it may sometimes exist alone without giving rise to any
symptoms ; at other times, as has been shown in Chapter XVII., it
may either coexist with or be in causal connection with spina bifida ;
and at yet other times it may, according to some observers, be in
intimate relation with syringomyelia. So strongly has the last-named
relationship been believed in, that hydromyelia and syringomyelia
have been used as synonymous terms (E. Leyden, Arch. f. path. Anat.,
lxviii. 1, 1876). Other authorities think that syringomyelia is an
acquired condition, due sometimes to injury to the cord, followed by
hemorrhages and the development of gliomata or myxoglioinata and

HYDROMYELIA 331

their subsequent degeneration (G. Hinsdale's article, Internat. Med.
Mag., v. 617, 681, 745, 1896-97). The two views need not be regarded
as irreconcilable, for congenital hydromyelia and syringomyelia,
although not identical, may yet be intimately related ; and it may be
supposed that gliomatous tissue will be prone to grow in cavities due
to developmental defects. The reader who may wish to follow out
these arguments must be referred to the special literature (now very
considerable) of syringomyelia. Hydromyelia in relation to spina
bifida and anencephaly has been specially studied by E. B. Block
(Contributions to the Science of Medicine, Vy the piqyils of W. II
Welch, p. 805, 1900).

The Antenatal Pathology of the Spine and its Contents is yet, as
has been gathered, far from completely understood. Many problems
still surround spina bifida, and, as has just been stated, the malfor-
mations of the spinal cord are full of difficulties of interpretation
and causation. Observers and investigators will do well to
remember that, on account of the late development of the nervous
system, embryonic malformations and foetal diseases are doubtless
more intimately connected and more directly interdependent in the
cord than elsewhere in the body.

CHAPTER XIX

Merosomatous Terata (rout.) : Malformations of the Cranium : Anencephaly or
Acrania ; Definition ; Nomenclature ; Historical Notes ; Clinical Details ;
Morbid Anatomy, Cranial Vault, Varieties of Defect, Face, Basis Cranii,
Cerebral Structures, Eyes, Spinal Cord, Supra- renal Capsules, Sectional
Appearances, Musculus Sternalis ; Associated Malformations ; Teratogenesis ;
Literature.

In describing the malformations of the cranium and its contents I
shall pursue the same plan as that adopted for anomalies of the spine,
namely, I shall give fairly full details of one type of malformation, and
little more than short notes of the other varieties. I have had little
difficulty in deciding which type ought to be described at length, for
the teratological state known as anencephaly or acrania has sovereign
claims to be regarded as the master monstrosity of this part of the
body. For one reason, it is, speaking relatively, a common mon-
strosity : few obstetricians who have attended more than two or
three thousand labours have failed to meet with at least one instance
of it ; and in 325 cases of monstrosity and fuetal disease examined by
myself there have been forty-six anencephalics, or 14 per cent.
Again, it is a very striking monstrosity ; and, yet again, it is an
anomaly which suggests many interesting problems affecting the
development and functions of the nervous system and its relation to
the nourishment and growth of the body. Obviously, if any one
monstrous state of the cranium is to be singled out for special
description, anencephaly it must be.

Anencephaly (Acrania).

Anencephaly may be defined as that teratological state of the head
in which the osseous vault of the cranium is more or less defective,
in which the scalp is absent in whole or in part, and in which the
brain is more or less gravely altered and may even be entirely
wanting.

The nomenclature is somewhat complicated, for many more or less
synonymous terms have been used to describe this monstrosity. In
the early days anencephalic ftrtuses were simply termed " cat-like "
or " frog-like," or were more accurately described as " puellus " (or
" puella ") " sine cerebro natus " (" nata "). During the eighteenth
century such deformities began to be called " acephalous " (e.g. by Gf.
van Doeveren, specimen dbscrv. acad. ad monstrorum historiam, p. 46,

332

HISTORY OF ANENCEPHALY 333

Groninga?, 17G5); but the name is erroneous, for they are not
" headless " in the sense that the grossly defective twin foetuses are
" headless," and the latter alone ought to be termed " acephalic " or
"acephalous."1 Nevertheless the name thus unfortunately intro-
duced has never been entirely abandoned ; it is in use at the present
day ; and the expressions " spurious acephalus," " semiacephalus,"
and " hemicephalus " are, all of them, attempts (by Prochaska, Rossi,
and Meckel respectively) to bring the terminology into correct rela-
tions with the morbid anatomy. Better names, however, were to be
invented, and V. Malacarne {Mem. Soc. ital, xii. pt. 2, p. 173, Modena,
1805) was fortunate enough to suggest the two expressions which
have most successfully met the requirements of the case. These
were the names " acrania " and " aencephalus " ; and the latter in
its more euphonious form, " anencephalus," is probably the most
commonly employed at the present time. Neither of them is a
perfect expression, for " acrania " concentrates attention upon the
defective state of the bones of the head, and " anencephalus " refers
only to the morbid condition of the cranial contents ; but if the
reader will keep them both in mind he will have a fair notion of the
nature of the malformation for which they stand. At the same time
neither of the names is to be taken in its literal signification : in
" acrania " it is mainly the bones of the cranial vault that are absent,
not those of the base ; and in " anencephaly " the brain may not be
entirely absent, but may be represented by a smaller or a larger
portion in a less or more altered state. There are also the inter-
mediate types or connecting links so troublesome to the taxonomist :
in some cases, for instance, it is only the anterior part of the cranial
vault that is absent, and in others the supra-occiput alone is at fault ;
and, again, there is no clear line of division between acrania and the
different varieties of cerebral hernia (encephalocele, meningocele, etc.),
although we may try to make the absence of the scalp over a greater
or a smaller extent of surface the differentiating character.

The nomenclature, therefore, is not perfect. At the same time, I
prefer the words " acrania " and " anencephaly " to such others as
" cranioschisis," Forster's comprehensive term, and "crania bifida,"
which is little more than an attempt to establish a somewhat
artificial bond of union between the present malformation and spina
bifida. Some confusion exists ; the fact is patent ; my endeavour is
not to increase it. I, therefore, employ " acrania " and " anencephaly "
as synonymous expressions, intending to designate by them the group
of malformations as a whole ; other names to mark off sub-types will
be introduced later.

It is an interesting fact in the history of this monstrosity that a
specimen exists which is much older than the earliest reference to
the anomaly to be found in literature. The mummy anencephalic
fcetus found at Hermopolis in Egypt, to which I have already referred
(p. 101), has obviously a much greater antiquity than the earliest
record we possess, for it dates only from the sixteenth century. It is

1 The converse mistake of calling an acephalus " anencephalus" was made by J. B.
Kiombholz (Anatomisches BeschreiMmg eines . . . Anencephalus, Prag, 1830).

334 ANTENATAL PATHOLOGY AND HYGIENE

a curious fact that nothing which can be identified with anencephaly
is to be found in the teratological lists of ancient Chaldea, although
it seems very improbable that this monstrosity did not exist in
Mesopotamia in these early times. The earliest recognisable account
of an anencephalic foetus would appear to be that given by Lycos-
thenes (Prodigiorum . . . Clironicon, p> 585, 1557). The author is
enumerating the prodigies of the year 1544, and after referring to the
birth of joined twins at Heidelberg, he proceeds as follows : " Quinto
Calend. Septembris, Argentina^ nobilis Alsatire metropolis, infans
foeminei sexus, horrendo, mostroso, atque in superiori parte, aperto
plane capite, lato ore, bovinis oculis, naribus aquilinis natus est."
The description and the illustration both point to this as a genuine
instance of anencephaly, although we may be inclined, from what we
know of present-day specimens, to doubt the presence of the " aquiline
nose." In the same year, the chronicler goes on to tell us, " Henricus
Anglie rex Gallos bello vexavit," a not unusual state of matters in
these troublous times. Lycosthenes' case of anencephaly, " naribus
aquilinis," has been followed by a long series of observations, so that
Taruffi (op. cit., vi. 95, 1891) had no difficulty in collecting together
more than two hundred of them from medical literature, and one
might with little trouble greatly increase the length of the list. It
was not, however, till near the close of the eighteenth century that
good descriptions of the anatomy of anencephaly began to be pub-
lished, and the nineteenth century was well advanced before satis-
factory theories of causation began to be advanced to explain the
origin of the monstrosity. All the best known teratologists, such as
Meckel, the Saint-Hilaires, FOrster, Ahlfeld, Dareste, and Taruffi,
have made important contributions to our knowledge of the subject ;
and the number of monographs and articles in journals has been
very large and is ever increasing. In the present work I have
already referred many times to anencephaly; indeed the consider-
ation of the theories of teratogenesis in several instances resolved
itself into the consideration of the theories of the production of
anencephaly. Further, the monstrosity has already passed before
the reader's eye in Figs. 25, 26, 27, 28, 32, and 33, and is represented
also in Plates XXI. and XXII. It is partly by reason of its
frequency that anencephaly has been described so often and so fully,
for it has been said to occur once in 1460 labours, and, as has been
stated already, I have had forty -six specimens of it put for examina-
tion into my hands during the past sixteen years ; it is also a very
arresting and wonder-provoking monstrosity.

Clinical Details.

The pregnancy that ends in the birth of an anencephalic foetus
may be quite uneventful; but there is reason to believe that more
often it is abnormal in one way or another. One cause which leads
to the frequent association of some maternal distress with such a
gestation is the very frequent* presence of hydramnios as a com-
plication. Indeed the symptoms that are noted in connection with

ANENCEPHALY 335

it are generally those of hydramnios (J. Laulaigne, These, Paris, 1883),
over-distension of the uterus, interference with respiration and circu-
lation, vomiting, pain in one side of the abdomen, etc. It is difficult
to hear the foetal heart, and when it can lie heard it is irregular, slow,
and weak, a character upon which G. Giglio {Ann. di ostet., xix. 328,
1897) is inclined to rely for diagnostic purposes. On account of the
excess of liquor amnii it is also difficult to palpate the foetal parts
and so to be sure of the cranial malformation. Hydramnios, however,
is not constant, and I have met with at least two cases in which the
opposite condition (oligohydramnion) was present; in such instances
auscultation and palpation may be expected to be more helpful in
diagnosis.

There is sometimes a history of abdominal traumatism during
pregnancy, a fact which led the Saint-Hilaires to conclude that it had
a causal connection with the malformation, and to which reference
has already been made (p. 139). Further, maternal impressions are
often alleged, an allegation which the resemblance of the anencephalic
foetus to a frog or a cat renders easy.

Family prevalence has been observed in connection with anence-
phalv. Two at least out of my forty-six cases were instances of this ;
in both of them the mother had previously given birth to an anence-
phalic foetus, and in one the sex of the monstrosity was different
(Trans. Edinb. Obst. Soc, xxiv. 16, 1899). G. Lammert (Arch. f. path.
Anat., xxii. 230, 1861) has also noted this, and Carli (Bull. d. sc. med.
di Bologna, 4 s., xix. 30, 1863) has reported the birth of three suc-
cessive anencephalic foetuses. J. Martin (Med. Exam., Philadelphia,
n.s., v. 23, 1840) put on record the extraordinary occurrence of six
anencephalic births in the reproductive history of one woman ; she
had two normal infants and one other monstrosity. The monstrous
infants do not always follow each other in successive pregnancies, for
Bruneel's patient (Ann. soc. mkl.-chir. de Bruges, 2 s., ii. 73, 1854)
had fourteen labours, of which the fourth, twelfth, and fourteenth
resulted in the birth of an anencephalic foetus. Occasionally an
anencephalic foetus is born with a healthy co-twin, as in A. Paterson's
case (Trans. Edinb. Obst. Soc., iv. 376, 1878) and in Blandin's (Joum.
hebd. de med., i. 107, 1828), or with a hydatid mole (P. Pagello, Giorn.
veneto di se. med., 2 s., i. 636, 1853), or with a foetus papyraceus
(Curatulo, Polieiinico, i. 379, 1894 ; Ann. di ostet., xvii. 287, 1895).
One of triplets has also been found to be anencephalic (namely, case
reported by Plancus and cited by Taruffi, Storia, vi. 148). Further,
both twins may be anencephalic, as in Otto's remarkable case
(Monstrorum Sexcentorum Descriptio, obs. xlix. 32, 1841), in which
they were both males and were born at the seventh month to a
young primipara. In fused or conjoined twins, also, this monstrosity
may be met with : instances are found in F. Noll's specimen, a
hemicephalic male foetus with epignathus (Diss, inaug., Marburg,
1882), in F. G. Parsons' case, a male anencephalous foetus with three
arms and three lower limbs (Joum. Anat. and Physiol, xxx. 238,
1896), and in S. T. Sommerring's four examples of dicephalic mon-
strosities (Abbildungen und Beschrcibungen einiger Misgeburten, Plates

33 G ANTENATAL PATHOLOGY AND HYGIENE

iii., v., vi., vii., Mainz, 1791). It may be noted as a fact of some
possible importance that in the reproductive history of a woman who
has had an anencephalic monster there may be the record of the
earlier or a later birth with hydrocephalus or spina bifida ; I have
met with two or three instances of this. An anencephalic foetus has
been carried in a bilocular uterus (P. L. Gardini, Ann. di ostct. e ginec,
xxi. 705, 1899); it has also been found in a tubal gestation sac
(Centrlbl. f. Gyndk., xxv. 385, 1901). Finally, the manner in which
anencephalic foetuses occur in groups in general obstetric practice
may be referred to simply as a matter of curiosity. P. Del-Vesco
(Gazz. med. ital. prov. venete, Padova, ii. 149, 1859) had the following-
extraordinary experience : for nine years in practice he never saw a
single monstrosity, then within twelve months he assisted at the
birth of five anencephalic fcetuses ; there followed a period of three
years without teratological manifestations, then within one month he
met with three anencephalics ; eight years then elapsed during which
he saw no further cases. I have had no experience like this, but I
have received two very similar specimens of pseudencephaly (Figs. 25
and 26) from different parts of the country within twenty-four hours,
and I have on several occasions had two anencephalics handed to me
within a few days ; but these are commonplace occurrences compared
with Del-Vesco's.

It would seem that the anencephalic fcetus is usually of the
female sex. At any rate there are thirty females and only ten males
in my collection of forty-six anencephalics ; the remaining six cases
may have been either males or females, the fact was not noted or else-
only the upper part of the body came into my hands. Meckel
(Handbuch der path. Anat., i. 234, 1812) found a somewhat less
marked excess of females over males (44 : 36). Forster (Die
Missbildungen des Menschen, p. 81, 1865) doubts its greater frequency
in either sex ; but it seems to be a well-established fact.

The birth of an anencephalic foetus is not necessarily rendered
difficult by the malformation ; but the malformed state of the head
makes a malpresentation more probable and so may introduce delay,
and the frequent coexistence of hydranmios may cause uterine inertia
and predispose to haemorrhage. Further, when the malformed head
presents it does not fit accurately into the pelvic brim, and on this
account the cord or the hands may prolapse ; again, it acts as an
unsatisfactory dilator, and so delay is caused ; and again, even when
the head is born there may be retardation in the birth of shoulders,
requiring the division of one or both clavicles (cleidotomy). It
would seem, also, that the labour is apt to be complicated in other
ways, e.g. by placenta previa, as in a case recently reported by'
myself (Jour/i. Obstet. Gyncec. Brit. Empire, v. 148, 1904).

Several interesting questions group themselves round the pheno-
mena of life manifested by the anencephalic foetus. That they live
in utero, of course, is evident ; further, they move freely inside the
uterus even when the spinal cord as well as the brain seems to be
entirely absent, as in one of the cases shown by myself in 1898
(Trans. Edinb. Obst. Soc, xxiii. 83, 1898). More than this, their life

LIFE OF ANENCEPHALICS 337

in utero may be unduly prolonged, for although most anencephalics
have been born prematurely some have come postmature into the
world. I think it is possible that the postmature anencephalics may
owe their longer sojourn in the uterus to absence of excess of liquor
amnii ; at any rate in the three cases which I have met with in which
anencephaly and postmaturity were associated there was no evidence
of hydramnios (Jourri. Obstet. Gyncec. Brit. Empire, ii. 530, 1902).

When an anencephalic foetus is born alive it usually quickly
succumbs ; but some remarkable instances have been reported of
continued postnatal life. This is more likely to occur when the
labour is at the full term, when the whole brain is not absent, and
when part at least of the cranial vault is intact ; but even in cases
of complete absence of vault and cranial contents signs of vitality
may continue for some minutes or even some hours. Instances of
the postnatal life of anencephalics have been recorded by Burrows
for six days (Med.-Chir. Trans., 3rd ed., Lond., ii. 52, 1817), by
Polaillon for twenty-four hours (Bull. Soc. de cliir. de Paris, 3 s., iii.
4:! 5, 1874), by S. S. Purple for two and a half days (New York Journ.
Med., n.s., v. 40, 1850), by W. Lawrence for four days (Med.-Chir.
Trans., Lond., v. 165, 1814), by C. Lucia for thirty -four hours (Sigh
mid., Madrid, iii. 187, 1856), by A. A. Spessa for eleven hours (Ann.
univ. di med., lxiv. 189, 1832 ; Gaz. med. de Paris, 2 s., i. 46, 183-">),
and by Stapfer for at least an hour and a half (Ann. de gynec., xxiii.
127, 1885). W. Ross's case (Trans. Obst. Soc. Lond., ix. 31, 1868) would
seem to have lived for more than sixteen days.

The vital phenomena which have been shown by anencephalic
ftetuses after birth have excited considerable interest ; and they
proved puzzling in the extreme to the physiologists of the past, who
did not realise the capabilities of the spinal cord. Purple (loc. cit.)
watched his case carefully, and noted that it breathed as soon as
it was born although inspiration was spasmodic in character, that
the skin of the face and chest and the eyes became congested for a
time, that deglutition was at first somewhat difficult, that it sucked
with considerable force, that its pulse was irregular and slow (68 to
76 per minute), that it passed urine and fa-ces freely, and that they
were natural, that it made frequent and partly successful efforts to
cry, and that it made convulsive movements when its skin was
pinched or the deformed cerebral mass touched. The temperature
was normal.

In a well-marked case of anencephaly seen by Saviard (Obser ra-
tions chirurgicales, p. 257, Paris, 1702) the infant lived for four entire
days ; it opened and closed its eyes, it cried, it took the breast, and
it swallowed the milk. Another infant (op. cit., p. 249) lived for six
hours, often opened its eyes, especially the left, and swallowed a little
sugar and wine. John Hey sham's female anencephalic child (Duncan's
Medical Commentaries, Dec. 2, iii. 429, 1789) lived for five days
and twenty-one hours ; she swallowed nourishment well, moved her
limbs " with apparent agility," cried, and had slight convulsions for
some time before her death. The excrescence upon the basis cranii,
after the most accurate post-mortem examination, showed not the

VOL. II. — 22

338 ANTENATAL PATHOLOGY AND HYGIENE

■smallest trace of a brain. " This," says Heysham, " may justly be
considered as a very curious, and perhaps an important circumstance
in physiology." So, in effect, say also N. Yaschide and C. Vurpas,
who in recent years have turned to the study of the biological
phenomena of the anencephalic feet us, bringing with them all that
modern physiology can give to help in the solution of the problem
(Compt. rend. Acad. d. sc, exxxii. G41, 1901 ; Now: iconogr. de la
SalpSt., xiv. 388, 1901). A male infant, with no trace of cerebral
hemispheres or cerebellum, with an incomplete medulla and pons,
with some rudiments of the corpora quadrigemina, and with an
apparently normal spinal cord, was born at the tenth month, was
resuscitated, and lived for thirty-nine hours. During his short life he
had a low rectal temperature (95° F.) ; his respirations were nine per
minute, and of a marked Cheyne-Stokes type ; the pulse was 138,
with some intermissions during inspiration ; his skin was cyanosed ;
his patellar reflex was difficult to elicit owing to the contracted state
of the legs, but the reflexes of the forearms were exaggerated ; idio-
muscular reflexes were also present ; the eyes were prominent ; and
there was very pronounced external strabismus and dilatation of the
pupils. There was, however, immobility of the pupils and absence of
reaction even to bright light. He sucked and swallowed, and drew
the body away when the skin was pinched or pricked or heated. He
made a few cries. There seemed to be no sense of taste (e.g. for
quinine), and camphor did not seem to affect the nasal mucous mem-
brane, but the vapour of ammonia made him draw back his head.
Loud sounds and bright lights had no apparent effect. Twenty hours
after birth he began to have convulsions; these began in the left
upper limb and were soon general, being followed as a rule by
micturition. The case presented a number of very puzzling problems,
such as the origin of the spontaneous and associated movements, the
normal character of the muscles (absence of atrophy, etc.) in the
presence of such a defective cerebro-spinal nervous system, the cause
of the signs of intense inflammatory change in the nerve tissues,
membranes, and vessels. Vaschide and Vurpas evidently hesitated
to draw conclusions from these data which would be contradictory
to established beliefs in the action of the nervous system, and dreaded
advancing " adventurous hypotheses." At the same time it is clear
that physiologists must find explanations for these facts ; for it is
no longer possible to say, as Duncan did (in 1789) about Heysham's
observation, that " a case so singular can hardly be supposed to
warrant any general conclusion whatever."

Morbid Anatomy.

In anencephaly the cranium is the part most affected, so I shall
consider that region first. The cranial vault bones and the scalp
covering them may be absent in part or in whole : it is upon this
character that Taruffi (op. cit., vi. 117, 1891) founds his primary
division of all specimens of the monstrosity into mero-acrania (partial
absence of vault, with protrusion of cranial contents) and holo-

CRANIAL VAULT IN ANENCEPHALY 339

acrania (complete absence of vault, with loss of cerebral tissues).
The former group corresponds with the exencephalic and partly with
the pseudencephalic families of Isidore Saint-Hilaire, and the latter
with that writer's anencephalics proper and partly with his pseuden-
cephalics (ffistoire des anomalies, ii. 293-374, 1836). Mero-acrania
also corresponds witli A. Spring's group of " ectopia of the cerebrum "
(Man. Acad. roy. de mid. dc Bclgique, iii. 179, 1854).

Cranial Vault.

According to the position and extent of the cranial defect Tarufri
obtains secondary divisions of mero-acrania. When it involves the
frontal plates alone he calls it (1) anterior mero-acrania : in this type,
which is rather rare, the margins of the parietals are usually slightly
affected, the orbital plates of the frontals are commonly present, the
rest of the vault is flattened, and the supra-occiput below the pro-
tuberance is perpendicular. It corresponds with proencephalus of
Saint-Hilaire. In (2) median mero-acrania (podeneephalus, Saint-
Hilaire) the upper part (vertex) of the cranium is defective, and the
greater part of the contents lies outside ; the bones chiefly affected
are the parietals, but the contiguous margins of the frontal plates
and of the supra-occiput may likewise be defective, and the temporals
are approximated anteriorly. Thus a diamond-shaped opening in
the vault is produced, the shortest diameter of which is the antero-
posterior. This type is, like the former, comparatively rare, and
Taruffi enumerates only thirteen specimens. Among these is the
case reported by W. Boss (Trans. Obst. Soc. Lond., ix. -31, 18G8), a
negro infant, in whom there was considerable arrest of development
of the parietal and temporal bones ; the frontal bones and the lower
jaw were well formed, but the superior maxilla was deformed, as were
also the fingers and toes. The monster lived for more than sixteen
days. A coloured plate accompanies the record. Spina bifida does
not appear to be associated with this form of acrania.

A third variety of mero-acrania is called antero-median by Tarufri
(op. cit., vi. 121, 1891). In it both the parietals and the frontals are
largely absent ; it is thus similar to the former type save in the
larger extent of the osseous defect. The name nosencephalus
(" morbid brain ") was given to it by Saint-Hilaire, but it is some-
what inexact. About ten cases have been reported, including one by
F. P. L. Cerutti (Rarioris monstri . . . descriptio, Lipsise, 1827), in
which, in addition to the cranial deformity, there were facial fissures,
ectopia cordis, and other malformations, the whole picture drawing
from the author (not without reason) the exclamation, " En mirabile
visu monstrum ! " In another instance, that reported by P. Pezerat
(Journ. compl. du diet. d. sc. mid., xxix. 252, 1827), the right arm was
absent, the parietals were entirely absent, and the frontal plates were
represented only by two osseous fragments. Under the somewhat
cumbersome and ambiguous name, " antero-tubercular mero-acrania,"
Taruffi has placed together seventeen specimens in a fourth group ;
in them the whole cranial vault was absent save that part of the

340 ANTENATAL PATHOLOGY AND HYGIENE

occiput lying below the level of the occipital protuberance. They
correspond roughly to Saint-Hilaire's division of the hyperencephalics,
and they are more numerous than any of the previously named types.
A good example was well described by L. Heydenreich (Arch f. path.
Anat., c. 241, 1885) : the exposed brain consisted of three lobes
covered with the membranes, there was also a median fissure of the
face, and amniotic bands were attached to the stunted lingers ; the
author is inclined to ascribe all the deformities to the amniotic
adhesions. Taruffi has constructed a fifth and final group of mero-
acranial monsters with the name postero-spinal : its leading character
is absence of the supra-occiput, extension of the foramen magnum,
an open condition of the cervical (and sometimes of the dorsal) spine,
and flattening of that part of the cranium roofed in by the frontals
and parietals ; and it includes Saint-Hilaire's genera of iniencephalus,
exencephalus, and notcnccphalits. It has seemed to me more con-
venient and correct to consider this as a teratological state by itself
and quite separate from anencephaly ; I have, therefore, described it
in Chapter XVI., to which I now refer the reader.

Leaving out of account the cases of iniencephaly (postero-spinal
mero-acrania), it may be said that mero-acrania is rarely associated
with spina bifida, although there may be anomalies of other kinds
affecting the vertebras. In holo-acrania, on the other hand, spina
bifida is practically constant. In complete acrania (" holo-acrania,"
as Taruffi prefers to call it) the whole vault of the cranium is absent :
the parietals, the squamous parts of the temporals, the frontal plates,
and the supra-occiput including the foramen magnum are all wanting,
although sometimes remnants of these bones are turned backwards
and downwards at the sides of the basis cranii, where they project
like wings or horns. When the basis cranii is uncovered by either
nervous or vascular tissue and simply shows a more or less thickened
membrane (dura mater) Saint-Hilaire called the condition ancnce-
phalus, although he also used the same name for the larger group ;
when the base is covered by a spongy, vascular mass he named it
pseud enccphahcs ; when the vascular mass is present along with a
very minor degree of cervical spina bifida it was thlipsencephctfus ;
and when there is spina bifida and absence of the spinal cord only in
the cervical region it was derencephalus. Obviously, these varieties
are refinements of classification and unnecessary for ordinary purposes.
A few words, however, may be said regarding the kind and degree
of spina bifida or rhachischisis which accompanies holo - acrania.
There may, for instance, be an open spinal canal all the way down
the back, constituting a gutter with everted edges and shallow every-
where save sometimes in the cervical region where a lordotic curve
of the vertebral bodies may exist. The gutter may be covered
simply with a layer of dura mater, or it may contain a tube of spinal
membranes, or it may show some nervous filaments or a duplicated
spinal cord. Again, the spina bifida may affect only the dorsal and
cervical regions, and in these regions the cord is commonly absent,
although it may be present lower down. Yet again, the open state
of the spine may exist only in the cervical region ; indeed, only the

FACE IN ANENCEPHALY 341

first two cervical vertebra (even the atlas alone) may be imperfect.
Sometimes the division of the spine may affect the vertebral bodies
(anterior spina bifida) as well as the arches, and this gives rise to
what has been called " anencephalus perforatus." I have seen one
specimen of this, and in it there was also exomphalos and absence
of the symphysis pubis, with the result that a foetus was produced
whose two lateral halves were united almost solely by the basis
cranii (Specimen No. 28).

Face and Basis Cranii.

I now pass to the description of the other parts of the head in
anencephaly (the basis cranii and the face). The absence of the
cranial vault gives to the face an appearance of breadth, but, as a rule,
the whole head is rather small, all the diameters of the base being
diminished. In a female anencephalic foetus (Fig. 57) measuring
38 cms. in length I found that the " occipitofrontal " diameter was
5*5 cms., the " occipito-mental " 6-8 cms., and the transverse both in
front of and behind the ears was 6 cms. ; the transverse measurement
of the face between the malar bones was 5"5 cms., and the vertical
or " fronto-mental " was 6 cms. In this instance the transverse
diameter of the shoulders was only 4 cms., but it is common for it
greatly to exceed the corresponding head diameter ; in fact, the broad
shoulders usually form an obstacle in the delivery of the anencephalus.
The face is prominent, partly on account of the thick layer of fat
which covers the cheeks, and partly by reason of the projection of
the lower jaw (its hyper-prognathism). For the relation of the
lower to the upper jaw it will be well to contrast Plate XIX. with
Plates XXL and XXII.

The eyes are large and seem to bulge out of the orbits (which
are shallow); and in the typical anencephalic they look almost
directly upwards, hence the somewhat fanciful name " uranoscopic "
(star-gazing) given sometimes to this type of monstrosity. The eye-
lids are often thick and oedematous in appearance. The nose is
almost always flattened, and the mouth is generally open, with the
tongue protruding (Plate XXI.). The ears have a twisted and
deformed appearance and rest on the shoulders ; the auditory meatus
passes downwards and forwards. There is no forehead, the eyes and
root of the nose forming the upper limit of the face.

The head is deeply set on the shoulders and the skin of the face
commonly passes directly on to the chest, there being no indication
of a neck groove. This is not constant, for now and again we meet
with a specimen in which there is a slight sulcus and sometimes
with one in which there is a true neck, but they are rare. The
anatomical cause of the neckless appearance will be referred to
immediately.

The basis cranii as studied by sectional and dissectional methods
shows various anomalies. It may look directly upwards (as in Fig.
57, and in one of Bauer's cases, Diss, inaug., Marburg, 1863), or
partly upwards and partly backwards (as in Plate XXII.), or almost

342

ANTENATAL PATHOLOGY AND HYGIENE

directly backwards (as in Plate XXI. and Fig. 58). The last-named
arrangement is the most typical, and probably represents the least
degree of commencing retroflexion of the spine (which is so often
combined with anencephaly, vide Fig. 28). There is an advanced
degree of ossification observable in the bones of the base : the pre-

sphenoid and basi-sphenoid are com-
monly fused together into one bone,
and this may likewise be merged with
the osseous basi-occiput (Fig. 57),
but not constantly (Fig. 58, and Plate
XXL). The petrous parts of the tem-
porals are short and thick, and may
be fused with the sphenoid to form
a large keystone in the centre of
the base of the cranium. The squam-
ous temporals and also the tympanic
rings are commonly entirely absent.
The stapes may not reach the fenestra
ovalis (T. Biancini, Di una anen-
crfalia, Pisa, 1829), and may be de-
formed (A. Wallmann, Arch./, path.
Anat., xi. 503, 1857 l) ; and various
other anomalies of the internal ear
(of the tympanic cavity, of the cochlea,
vestibule, and semi-circular canals)
have been described (E. Voltolini,
Afonatschr. f. Ohrenhlk.,iv. Ill, 1870).
In one of my specimens (Journ. Anat.
and Physiol., xxvi. 516, 1892) there
was no condyle on the petrous part
of the temporal for articulation with
the lower jaw, and in its place was
a small spine which projected be-
tween the coronoid process of the
jaw and a projection representing
the neck of that bone below the
condyle, for the condyle itself was
absent. The malar bones, in this
case, were large, and each articulated
with the external angular process of
the frontal and with the great wing
of the sphenoid, but not directly
with the zygoma of the temporal.
The external angular process of the
frontal articulated with the zygomatic process of the temporal.
Doubtless these anomalies are not present in all anencephalics.

To return to the description of the sphenoid, it may be found that
the great wings are rather stunted, and that the small wings are

1 The Index Catalogue gives the year as 1856, one of the very few inaccuracies I have
deteeted in this fine work.

Fig.

57. — Vertical Mesial Section of
Head and Trunk of Anencephalic
Foetus (left face shown and reduced
one-half). A, hones fused together
to form basis cranii ; 15, spina bifida ;
C, 7th cervical vertebra ; D, hyoid
bone ; E, cricoid ; F, thyroid gland ;
G, thymus ; H, heart ; I, liver ;
K, lobus Spigelii ; L, pylorus ; M,
transverse colon ; N, third part of
duodenum ; 0, second lumbar ver-
tebra ; P, cesophagus ; Q, aorta ;
K, right pulmonary artery ; S,
trachea.

Plate

Basis crani

Tongue.

Small
intestine.

Colon.

Umbilicus.
Uterus.

Bladder.

Symphysis
pubis.

BASIS (RANI I IN ANENCEPHALY

343

either absent or fused with surrounding parts (perhaps with the
orbital plate of the frontal, as in one of my specimens in which the
frontal bone was represented by the nasal spine and posterior part
of the orbital plates). The pterygoid processes, if present, may be
deformed. The fusion of the pre-sphenoid and basi-sphenoid has
been referred to already. The single bone thus produced may lie in
the same plane with the basi-occiput (Fig. 57) ; but more often it
forms a sharp angle with it (Plates XXL, XXII. ; Fig. 58), so that
while the upper surface of the sphenoid may look upwards, that of
the basi-occiput is directed backwards. The depression of the sella
turcica is thus usually entirely effaced. Some very ingenious theories
regarding the sphenoidal angle and its mode of production in
anencephaly have been advanced by F. G. J. Bauer (Diss, inaug.,
Marburg, 1863), by C.
T. Ackermann (Die
Schadclil ifiorm itdt lei dcr
En ceph a locele congen Ha,
Halle a. S., 1882), by
W. Quicken (Diss, inn /"/.,
Halle a. S., 1885), and
1 >y C. Taruffi (Storia della
tcratologia, vi. 1 1 1,189 1 ).

It is unnecessary to con-

sider these theories in
detail ; but it would ap-
pear that the sharpness
of the angle between the
sphenoid and the basi-
occiput depends greatly
upon whether there is
liolo -acrania or mero-
acrania. In the latter
case, and especially
when the mero-acrania
is anterior or median,
the angle may be much
less marked. Sometimes the basi-occiput, instead of being bent
downwards from the sphenoid, may be flexed upwards upon it. The
change in the direction of the basis cranii is intimately connected
with the presence of lordosis of the cervical part of the spine. The
degree of the lordosis varies greatly, but there is nearly always
lordosis and very rarely cyphosis (Fig. 58). The cervical lordosis
accounts, also, in large measure, for the neckless state of the
anencephalic foetus ; but cyphosis has the same effect. The only
specimen of anencephaly which I have met with which possessed
a distinct neck had a spinal canal closed right up to the occiput,
so that the presence of spina bifida would appear to have much to do
with this neckless state. The cervical vertebrte are often reduced in
number, fused together, or otherwise abnormal, circumstances which
also tend to shorten the neck.

U"7'f/ 'li'fflj it) ' yjifm

Fit;. 58. — Vertical Mesial Section of Head of Anen-
cephalic Foetus (right face shown, natural size).
A, sphenoid ; 15, basi-occiput ; C, body of axis
vertebra ; D, hyoid bone ; E, thyroid cartilage ;
F, septum of nose; (!, Eustachian tube; H,
tongue.

344 ANTENATAL PATHOLOGY AND HYGIENE

Cerebral Structures.

Turning now to the structures found lying on the basis cranii or
contained within the rudimentary cranial cavity, I may state that
great differences exist. As a rule, the more complete is this absence
of the cranial vault the less likely is it that any nervous tissue will be
found on the base ; in such cases of holo-acrania not even a mem-
branous sac may be found, and all that is present is a layer of tissue
(dura mater ?) with some vascular portions (pia mater ?) adhering to
it and covering the bones. Earely in holo-acrania and commonly in
mero-acrania a spongy mass of tissue is seen occupying the position of
the brain (pseudencephaly) : this consists of a sac made up of the more
or less altered cerebral membranes and containing more or less altered
cerebral substance. The sac, according to Bauer (op. cit.), has an
external, smooth surface consisting of connective tissue adhering to
the dura mater, which contains no sinuses ; occasionally the smooth
surface is lost and in its place are membranous fragments or fila-
ments which had attached it to the amnion. On the inner surface of
the sac-wall it is difficult to trace a distinct arachnoid membrane.
The contents may consist of greatly softened cerebral substance,
infiltrated with blood, but in which, occasionally, definite parts of the
brain can be made out. In other cases they are made up entirely of
a red spongy " fungous " substance, consisting of a network of con-
nective tissue in which are numerous vessels distended with blood.
These vessels may show aneurismal swellings, and doubtless they are
derived from the pia mater and really represent the cerebral arteries
and veins. In yet other instances the interstices of the network are
filled with serum or the tissue itself is converted into one or more
cysts with clear contents. In F. G. Gade's anencephalic fetus, the
third born to the same mother, there were some traces of the pitui-
tary body (Norsk. Mag. f. Lcegevidensk., 4 R, ix. 715, 1894) ; but these
are rarely seen. In Vaschide's specimen (loc. cit.) the corpora quadri-
gemina were recognisable, as was also the medulla oblongata, but in
the latter organ the restiform bodies, the inferior olive, and the arci-
form fibres were wanting. It is quite the exception for distinct
traces of any parts of the nervous system above the level of the
medulla to be met with in anencephaly. It is, on the other hand, not
uncommon to recognise some of the cranial nerves lying on the basis
cranii (H. Mattersdorf, Diss, inaug., Berlin, 1 836). They either lie
with free blunt or tapering ends on the bones or are inserted into the
meml >ranous sac ; and they may or may not contain nerve fibres. The
cerebral vessels usually show marked anomalies. The circle of Willis
is not complete ; the carotid arteries are small or follow an unusual
course ; and the vertebral arteries may be obliterated. It has to be
noted, however, that exceptions occur, for the carotids and vertebrals
may have their usual arrangement and be of normal calibre (Bauer).

The Eyes in Anencephaly.

Several observations have been made upon the eyes of anen-
cephalic foetuses, for the fact could not pass unobserved that these

SPINAL CORD IN ANENCEPHALY 345

organs often had a perfectly normal appearance, although, of course,
they had no connection with a brain. Eduardus de Wahl (Diss, inaug.,
Dorpati Livonorum, 1859) examined the microscopic characters of the
retina in a case of anencephaly and amyelia ; W. Manz (Arch. f. path.
AnaL, li. 313, 1870), C. Hitter (Arch. f. Augenh., xi. 215, 1881-82),
Joseph Hegler (Diss, inaug., Wiirzburg, 1893), F. G. Gade (loc. cit.), and
Vaschide and Vurpas (Cqmpt. rend. Acad. d. sc, exxxiii. 304, 1901)
made similar investigations. Gade found, as might be expected from
the close relations existing between the brain and the eye, that the
lens, the cornea, the ciliary body with the iris, and the ganglionic layer
of the retina were absent, and that, in one eye, there was coloboma
of the retina and choroid. The specimen of Vaschide and Vurpas,
however, gave very different results : to the naked eye the organ was
normal, and under the microscope the retina showed wonderfully
little change ; even the layer of rods and cones existed (although not
so distinct as usual). It is evident that another puzzling problem in
Embryology and Physiology arises in connection with the eyes of the
anencephalic foetus.

Spinal Cord in Anencephaly.

The condition of the spinal cord, like that of the brain, varies
greatly in different specimens of anencephaly; indeed, much that has
been said regarding the cord in spina bifida might be repeated here.
There may be practically no trace of a cord, or there may be all the
gradations between entire absence (amyelia) and a nearly perfect
structure. Within recent years three careful histological examina-
tions have been made : those of Bulloch (Journ. Anat. and Physiol.,
xxix. 276, 1895), of Vaschide and Vurpas (Now. iconogr. de la
SalpSt., xiv. 388, 1901), and of D. Waterston and E. Matthew (Rev.
Neurol, and PsychioL, i. 465, 1903). In Bulloch's case (in which the
upper part of the cord was wanting) the white matter in the cord was
small in proportion to the grey, the anterior horn contained normal
multipolar ganglion cells, Clarke's column contained very few cells,
and the lateral horn of the dorsal region was quite rudimentary ; with
regard to the white matter, the pyramidal, direct cerebellar tracts,
and the tract of Lissauer were absent. In Vaschide's specimen there
was a degenerated condition of the nerve cells in the anterior horn ;
the white matter was small in amount (the lateral cords being
especially reduced) ; there was an increased number of neuroglia cells
and signs of intense inflammatory action (dilated blood spaces) ; just
behind the anterior fissure fibres could be seen passing from one
anterior horn to the other ; and the nerve roots were practically
normal ; in this instance there were some traces of the mid-brain.
In the specimen of Waterston and Matthew there was no nervous
tissue above the medulla oblongata ; the cord showed normal cervical
and lumbar enlargements ; in the sacral and lumbar parts the lateral
columns of white matter were small, but the motor cells of the
anterior horns were large and well developed, and the central canal
was fairly well formed ; in the dorsal region there were numerous

346 ANTENATAL PATHOLOGY AND HYGIENE

1 dood vessels of large size and masses of red blood cells resembling
haemorrhages in both the white and grey matter, the lateral columns
were much diminished and their surface fissured ; in the cervical
region there were two (and at one part, three) distinct central canals,
the direct and crossed pyramidal tracts were absent, and there was no
Assuring of the surface over the lateral columns, perhaps on account
of the presence of many commissural fibres. It is evident, therefore,
that the histology of the cord in anencephaly differs considerably ; it
also contains problems for the neurologist of a somewhat complex
kind, as Vaschide and Vurpas {Compt. rend. Acad. d. sc, cxxxiii. 116,
1901) clearly point out.

The supra-renal capsules in anencephaly are often found to be
wanting (seven times in seventeen specimens, according to 11. Lomer,
Arch. f. path. Anat., xcviii. 366, 1884), and in other instances they
are diminished in size (Bender et Leri, Compt. rend. Soc. dc biol.,
lv. 1137, 1903). I have noted these facts in some of my specimens,
and in one case {Journ. Anat. and Physiol., xxvi. 516, 1892) the left
adrenal was absent while the right was present. These organs, how-
ever, are not constantly affected in anencephaly ; they may be
normal. In this connection, observations have also been made upon
the sympathetic system in anencephaly. Thus, in a case in which the
supra-renal capsules were incompletely developed, C. Weigert {Arch,
f. path. Anat., c. 176, 1885) found that the superior cervical
ganglion of the sympathetic was absent ; but the association of the
two defects is not constant, as Weigert himself showed {Arch. f.
path. Anat., ciii. 204, 1886). Karl Biesing {Diss, inaug., Bonn, 1886)
also investigated the supra-renal capsules and sympathetic system
in anencephaly ; he examined nineteen ftetuses (ten females and six
males, three unknown), and found a greater or less degree of defec-
tive development of the adrenals in them all, but the degree of defect
was not in proportion to that of the skull ; the sympathetic cord was
unaltered in all the cases. It may be concluded, therefore, that
while there exists some relation between absence of the brain and
defective development of the supra-renal capsules, the state of the
sympathetic system is not necessarily influenced by that of the
cerebro-spinal. Herein is one other problem for embryologists and
physiologists to give their attention to.

Sectional Appearances in Anencephaly.

Eighteen out of my forty-six specimens of anencephaly were
studied by means of frozen sections, and some of the sections have
been reproduced for this work (Plates XXL, XXII. ; Figs. 28, 57, 58)
and in journal articles {Physician and Surgeon, i. 891, 1900; Journ.
Obstct. Gi/nwc. Brit. Empire, ii. 521, 1902). It is unnecessary that I
discuss in detail the various parts of the regional anatomy of
anencephaly ; but I may refer to one or two matters of special
interest. In sixteen of the cases in which the sectional method was
followed it was possible to note the curvature of the spine in its

Plate xxn

Blood-clot

Basis cranii.

Cervical
spina bifida.

Nasal septum.

Upper jaw
with tooth.

Mouth.

Tongue.
Lower jaw
with tooth.

first Dorsal

Vertebra-
Spinal cord.

Thymus.
Sternum.

Heart.
Diaphragm.

First Lumbar
Vertebra.

Pylorus.

Colon.

Umbilical
cord.

Uterus.

Rectum,

Bladder.

Vagina..
Symphysis pubis.

ANENCEPHALY 347

whole extent. In fifteen of these there was cervical lordosis : it was
marked in six cases, extraordinarily marked in one (Fig. 28), and
slight in one, while in the remaining seven it was quite recognisable ;
in the sixteenth case the whole spine, including the cervical region,
was practically straight. In no instance was there cervical cyphosis,
although this has been noted by other observers {e.g. Taruffi, Storia
della teratologic/,, vi. 101, 1891). In eight of the sixteen cases there
was dorsal cyphosis, and in five of these the cyphosis was slight ; in
seven the dorsal region was straight or nearly so*; and in one only
was there lordosis in the upper and cyphosis in the lower dorsal
region. In eight instances there was lumbar lordosis ; in three there
was cyphosis, and in five the lumbar region was straight or nearly so.

Another fact brought out by the sectional anatomy was that the
structures in the neck all lay at a lower level in relation to the
cervical vertebra; than is usual in the fcetus (although not in adult).
The organs in the upper part of the thorax also lay at a lower level
qua the spine ; in the abdomen there were often alterations in the
relation of the organs to each other, traceable in some instances to
associated malformations, such as absence of the supra-renal capsule,
presence of a diaphragmatic hernia, and the like. The main fact
made clear by the sectional anatomy was the existence of cervical
lordosis and the consequent dislocation of the relation of structures
in the neck and thorax.

Before I pass from the morbid anatomy of the anencephalic
foetus I may refer to F. J. Shepherd's article (Journ. Anat. and
Physiol., xix. 311, 1885) on the occurrence of the musculus sternalis
in this type of monstrosity. It was found in six anencephalics
examined, and in three of them it was bilateral. Its association with
the teratological state suggested to Shepherd that it might be a
rudimentary structure or reversion. The association of anencephaly
with the musculus sternalis had been previously pointed out by 1'. S.
Abraham (Trans. Acad. Med. Ireland, i. 301, 1883). It is not a con-
stant association.

Associated Malformations.

Anencephaly, being a comparatively common monstrosity, is not
infrequently met with in association with other teratological states.
I can do little more than name these complications here.

Reference has already been made (p. 335) to the fact that anence-
phaly may occur in the different forms of double monster. The
malformations now to be noticed are in the single foetus. There may,
for instance, be facial fissures of greater or less extent, including
hare-lip and cleft palate ; three at least of my specimens showed this
combination, and in two of these there was adhesion of the amnion
(covering the placenta) to the head. There may be cyclopia in some
cases (von Lenhossek, N. Jahrb. d. teutsch. Med. u. Vhir., iii. 1, 1821 ;
Planehon, Bull. Soc. anat. de Paris, -xliii. 541, 1868), exomphalos in
others (as in three of my forty-six cases), and diaphragmatic hernia
with displacement of the abdominal contents into the thoracic cavity

348 ANTENATAL PATHOLOGY AND HYGIENE

in others (as in one of my specimens). Other associated malforma-
tions are digital defects (Ballantyne, Edinb. Med. Journ,, xl. 1020,
1895), anal imperforation (Perkins, Med. Bee, xlviii. 492, 1895),
vascular anomalies, solitary or horse-shoe kidney (Gade, loc, cit,), and
genital defects such as a rudimentary state of the testicles or ovaries
or of the penis (Appert, Arch, de tocol., xxi. 874, 1894). Bifid tongue
has been noted (Joly, France mid., xiii. 343, 1866). Club-feet are
common, and spina bifida, as has been pointed out, is so common as
almost to constitute a part of the malformation. Club-hand is rarer
(A. G. Luecke, Diss, inaug., Halis Saxonum, 1854), so is the uterus
duplex (J. Klatt, Diss, inaug., Leipzig, 1892), and the cystic liver
and kidneys (0. Witzel, Gentrlbl.f. Gynal:, iv. 561, 1880).

Teratogenesis.

It is unnecessary to consider at length the various theories of the
causation and mode of production of anencephaly. In discussing
teratogenesis I constantly had to refer to this monstrosity, for all the
theories were made by their proposers to apply to it. Thus we find
it explained by means of a maternal impression (p. 112), by injury to
the mother (pp. 137, 139, 140), by a foetal disease such as hydro-
cephalus (p. 159 ct seq.), by amniotic (or placental) adhesions (p. 168
et scq.), and by arrested development (pp. 206, 216). A few words
are all that is necessary on the three last suggestions.

The idea that a foetal disease such as hydrocephalus is the cause
of anencephalus has been popular since the time of Morgagni (E.
Mayer, Amcr. Journ. Med. Sc, n.s., lxxxiii. 118, 1882; and many
others). With the progress of time and the acquirement of further
knowledge its terms have been altered ; but the notion of a distended
condition of the cerebral ventricles preventing the formation of the
cranial vault and resulting ultimately in rupture, is in all respects
the legitimate modern representative of the old theory. Two great
difficulties face the supporters of this view : one is the state of the
eyes in anencephalics, for, unless we are to change our ideas of the
development of these organs, it is difficult to explain their occasion-
ally perfect formation in association with widespread destruction of
the cerebral vesicles, including, of course, the anterior one ; the other
is the convex curve of the cranial base, for if there lias indeed been
great intracranial pressure, we should have expected a concavity.
If we are to accept this theory we must believe that the destruction
of the anterior cerebral vesicle takes place after the outgrowth of the
optic evaginations from the telencephalon, and that pressure on the
basis craiiii is in some way or another abolished; there are manifest
difficulties in connection with both. Taruffi {Storia, vi. 158, 1891)
was inclined to adhere to a pathological theory of origin, but a
different one from that referred to above. He saw, in the excessive
ossification of the bones of the basis cranii and in the spongy vascular
mass occupying the position of the brain, signs of a process analogous
to inflammation which had resulted in the production of an erectile
tissue (an angiomatous formation arising from the cerebral blood

TERATOGENESIS OF ANENCEPHALY 349

vessels) and in the destruction of the nerve tissues. The difference
between the results in anencephaly and in hydrocephalus is due to
the effusion being interstitial in the former and interventricular in
the latter. I think, however, that it must be admitted that there are
grave difficulties in accepting Taruffi's theory of vascular origin for
any form other than pseudencephaly. Further, all the theories of
f(etal disease as a cause of embryonic monstrosity are to my mind
difficult of acceptance, for I believe that the monstrosities arise at a
time anterior to the stage of development when diseases, as such,
are possible.

It must at the same time be admitted that the strictly embryo-
logical theories of anencephaly also contain numerous difficulties.
That amniotic attachments and consequent traction upon parts of the
head account in part at least for many of the cases of mero-acrania
cannot, 1 think, be doubted. The cases of adhesion of the placental
amnion to the deformed cranium are sufficiently numerous to permit
us to draw this deduction. Further, the evidence of Comparative
Teratology is in favour of this, for all known specimens (there are not
many) of anencephaly in the mammalia are instances of mero-acrania,
and in some at least of these (p. 188) amniotic bands were present.
But this view does not satisfactorily account for the cases of complete
anencephaly (holo-acrania) which are the more numerous in the
human subject, and which are wholly absent in the other mammals.
This negative fact must have a significance : the absence of anence-
phaly in mammals other than man must have a bearing upon the
mode of origin of the monstrosity. I believe its significance has to
do with the mode of development of the amnion in the human
subject, which, there is reason to maintain, differs from that in the
lower animals.

Taking into account all the facts, I am inclined to think that the
cases of complete absence of the cranium (holo-acrania) at any rate are
arrested developments due to amniotic pressure, while some at least
of the cases of partial anencephaly (mero-acrania) are caused by
amniotic adhesions (or non-separations), which, while they do not
altogether prevent the formation of parts, seriously disturb their
normal organogenesis. The arrest in development obviously affects
the medullary groove primarily and most markedly, and we may
reasonably suppose it to be due to defective formation or separation
of the amnion over the cephalic end of the embryo. Some of the
differences in the appearances found in different cases may be clue to
the angle at which the head-end lies at the time when the anomaly is
produced (Lebedeff, Arch./, path. Anat., lxxxvi. 263, 1881) ; we know
that the embryonic axis in its upper part changes its direction during
ontogenesis, and in this way different parts of it may fall under the
action of amniotic pressure. Further, different effects may be pro-
duced by the different dates at which the pressure may cease to act,
for it is not maintained that the compressing force continues to act
till the end of antenatal life ; a certain amount of subsequent
development and even of repair may, therefore, follow the terato-
genetic epoch and mask the original appearances. The investigation

350 ANTENATAL PATHOLOGY AND HYGIENE

of the early stages of the development of both the human embryo and
the human amnion must be awaited before this whole matter can be
further elucidated. It is, as I have said, noteworthy that anence-
phaly is so common in the human subject and so rare in the
mammalia generally ; it is also very uncommon in birds, and, as
Dareste points out, it has very seldom been met with among the
results of teratogenetic experiments on the hen's egg {Production
artificicllc dcs monstruosite's, p. 384, Paris, 1891).

Another factor in the teratogenesis of anencephaly which we
cannot afford to neglect is the germinal. Direct heredity, of course,
is impossible ; but the frequency with which family prevalence
occurs is most striking ; in fact when a woman has once given birth
to an anencephalus there is almost a presumption that she will give
birth to a second, or at any rate to some other form of monstrosity
(Ballantyne. " Recurrent Monstriparity," Amer. Journ, Obsf.,.xM. 577,
1900). This would seem to show that anencephaly may be deter-
mined prior to the appearance of the embryo and the amnion ; even
if this be so, however, it is not to be concluded that the actual
formation of the monstrosity is not worked out by amniotic pressure
due to amniotic defective development. It may be the amniotic
defect which shows the family prevalence.

LITERATURE.

In addition to the references already mentioned in the text (about
seventy in number), the following may be named as of special
interest ; many others are in the Index Catalogue and elsewhere : —

J. Arnold, Beitr. z. path. Anat. u, allg. Path., xi. 407, 1892 ; L. Ball,
Diss, inaug., P>onn, 1896; C. N. Ballard, Amer. Gyrwec. and Ohst.
Journ., ix. 744, 1896; A. Betancourt, Progreso mSd., Habana, viii. 81,
1896; A. Brothers, Arch. Pediat., xiii. 614, 1896; J. M. Carafi, Butt.
Soc. anat. de Paris, lvi. 733, 1881 ; Cleland, Journ. Anat. and Physiol.,
xvii. 257, 1883; W. L. Conklin, Buffalo Med. Journ., n.s., xxxv. 638,
1896; H. Coote, Med. Times and Gaz., n.s., iv. 488, 1852; E. Crosnier,
Thhe, Paris, 1875 ; C. L. Dana, Journ. Nerv. Ment. Dis., xiii. 21, 1888 ;
J. M. Duncan, Trans. Edinb. Obst. Soc, iii. 72, 1875; G. Eustache,
Arch, de tocol., vi. 84, 1879; Gadaud, Bidl. Soc. d'anthrop. de Paris, 2 s.,
iii. 153, 1868; J. Guthrie, Edinb. Med. and Surg. Journ., xxvi. 28, 1826;
H. Habgood, Brit. Med. Journ., i. for 1876, p. 505; A. Haller, De fetu
. . . sine cerebro, Gottingse, 1745; A. Hannover, Den menneskelige
Hjerneskals Bygning red Anencephalia, Kjobenhavn, 1882; Dublin Journ.
Med. Sc, lxxv. 396, 1883; C. A. Hensche, Diss, inaug., Halis Sax., 1854;
Herbet, Bull. Soc. anat. de Par., Ixxii. 703, 1897 ; C. Herrick, Journ.
Comp. Neurol., iv. 1, 1894; B. Jungbluth, Diss, inaug., Berolini, 1843;
J. L. Kerr, Lancet, ii. for 1896, p. 380; C. E. H. Knackstedt,
Anatomi*che Bexchreibung einer Missgeburt., St. Petersburg, 1791 ; N. I.
Knapp, Med. Pec, New York, li. 530, 1897; A. Lagahde, La Semana
mi'dica, Buenos Aires, ii. 377, 1895 ; O. Y. Leonowa, Arch. f. Anat. u.
entwcMngsgesch., p. 403, 1890; P. Macedonio, Archivio di ostet., v. 73,
1898 ; R. Magnus, Diss, inaug., Konigsberg, 1889 ; Osmont, Centrlbl. f.
Gynak., xx. 203, 1896; G. B. Pellizzi, Ann. di freniat., xiii. 51, 1903;

LITERATURE OF ANENCEPHALY 351

K. Petren und G. Petren, Arch. f. path. Anat, cli. 346, 438, 1898; J.
Platon, Marseille med., xxxiv. 785, 1897; E. Pruneau, These, Paris,
1837 ; H. Ribbert, Arch.f. path. Anat., xciii. 396, 1883 ; A. Routii, Trans.
Obst Sqc. Land., xxxviii. 92, 1897; A. Sachsalber, Ztschr.f. Augenh., ix.
354, 1903; E. Sandifort, Anatome infantis cerehro destituti, 1784;
Septours, Union med., 3 s., xxi. 209, 1876; Souligoux et Paquy, Arch.
de tocol., xxi. 474, 1895 ; F. A. Stahl, Amer. Journ. Old., xxxiii. 501,
1896; M. Sternberg, Deutsche Ztschr. f. Nervenh., xxiv. 209, 1903 ; T.
G. Stevens, Trans. Obst. Soc. Lond., xxxviii. 346, 1897 ; E. W. Taylor,
Journ. Boston Soc. Med. aS'c, ii. 31, 1897-98; E. L. Twombly, Ann. Gynwc.
<in<l Pa'diat., xi. 63, 1896-97; C. Vurpas et Leri, Bull, med., xvri. 698,
1903; Compt. rend. Acad. d. sc, cxxxvii. 213, 1903; A. C. Wood, Univ.
Med. Mae/., Philadelphia, viii. 948, 1895-96 ; W. A. Wood, Intercolon. Med
Joum. Anstratas., i. 342, 1896 ; R. Zander, Beitr. z. 'path. Anat. n. allg.
Path., vii. 441, 1890.

CHAPTER XX

Merosomatous Terata (cont.)-. Malformations of the Cranium. Cephalocele :
Definition, Synonyms, Historical Note, Clinical History, Morbid
Anatomy, Position, Sac, Foramen, Contents (Hydro-men ingocele, Encepha-
locele, fiydro-enceimalocele, Hydro-meningo-encephalocele), Basis Cranii,
Associated Malformations, Comparative Teratology, Teratogenesis. Hydro-
cephalus : Varieties, Definition, Clinical History, Morbid Anatomy,
Associated Malformations, Surgical Treatment. Microcephalics : Definition,
Historical Note, Clinical History, Morbid Anatomy (Cranium, Brain,
Varieties, Other Parts, Associated Malformations), Teratogenesis, Surgical
Treatment. Miscellaneous Malformations (Cranial Perforations, Poren-
cephaly, etc.). Omphalocephaly (Dareste).

Having in the preceding chapter described in some detail the
important monstrosity known as anencephaly, I now proceed to deal
with several other malformations of the cranium. The limitations of
the available space in a work of this size lead me greatly to curtail
the descriptions of them. That the reader may realise this, I may
mention that Taruffi in his Storia della teratologic/, gives eighty-
eight pages in his sixth volume and sixty-four pages in his eighth
to the study of cephaloceles, while I am only able to afford seven
or eight pages. There must of necessity be abbreviations ; but I shall
try to abbreviate by contracting and condensing, not by cutting off
and omitting. Under the circumstances, bibliographical references
have, with rare exceptions, been sacrificed.

Cephalocele.

Definition. — Consists in the protrusion of part of the cranial
contents through a more or less rounded opening in the cranium ;
the swelling thus produced is covered with skin, altered or unaltered.
The chief synonyms are "cerebral hernia," " encephalocele," "cranial
spina bifida," and " congenital hernia of the head " ; the first of these
is too closely identified with one theory of origin, the second is better
reserved for the naming of a variety, the third is awkward, and the '
fourth is too long. " Cephalocele," proposed by W. Heineke, is the
best general term.

Frequency. — It is comparatively rare ; three cases only occurred
in 12,900 labours (Trelat); and in my series of 325 specimens of
antenatal disease and deformity there were only eight instances of
cephalocele in contrast with forty-six of anencephaly, but, of course,
the discrepancy may be due to other reasons than comparative rarity.

352

CEPHALOCELE

353

W

Fig. 59.-

-Occipital Euceplialocele.

No. 26.

Specimen

Historical Notes. — First reported instance by Forestus (1500);

few cases in seventeenth century, and curious errors as to their

nature ("penis at root of nose," "scrotum at Lack of head," etc.);

first illuminating monograph

by J. F. C. Cor vinus (Diss.

inaug., Argentorati) in 1740 ;

since then, a long series of

articles (clinical, pathological,

statistical, pathogenetic, and

therapeutic) by C. F. F.

Buettner in 1832, by A. Spring

in 1854 (a long and learned

monograph of 150 pages, very

worthy of consultation, but not

often consulted, in the original

at least, in these days), by J. Z.

Laurence in 1856 (good tabular

analysis), by G. Reali in 1874

(in a Dissertation of Zurich,

which Taruffi (mirabile dictu !)

had not seen in the original),

by C. Taruffi himself in 1873

and 1801, by T. Ackermann

in 1882, by P. Berger in 1800

and in 1801, and by G. Mus-

catello in 1804.

Clinical History. — Of the antenatal clinical history of foetuses with

cephaloceles little is known. One of my cases, however, had an

interesting record, which
I give here briefly. This
specimen (Fig. 50) I re-
ceived from Dr. A. A.
Martin of North Shields
in 1800. It was born at
the seventh month ; the
head or rather the occi-
pital encephalocelic sac
presented ; on the rupture
of the membranes there
was a tremendous gush
of waters, for there was
'*** marked hydranmios. The

jA pains were feeble ; forceps

Sk ^^^^m extraction; partially re-

■l -. s^^H tained placenta. The

Jgf ^ffi| foetus, a female, never

breathed. This was the
mother's fourth child ; her
first, born at the eighth

month, was " exactly similar " ; her second and third were born at

VOL. II. 2X

Fig. 60. — Dr. Charles's Case of Occipital
Euceplialocele with Accessory Brain.

354

ANTENATAL PATHOLOGY AND HYGIENE

full time, and are alive and healthy ; with all the four labours there
was hydramnios. Three clinical facts emerge from this history,
namely, premature termination of gestation, coexistence of hydram-
nios, and family prevalence.

Another interesting history was communicated to me by Dr. Etta
Charles of Summitville, Indiana, U.S.A., who sent me notes and
photographs. The mother, who was about thirty-two years old, and
had two living female children (ages fifteen and thirteen years), gave
birth in 1897 to a girl baby (Fig. 60) with an encephalocele arising

from the lower part
of the occiput and in-
volving the foramen
magnum and upper
cervical vertebne. I
shall have occasion to
refer again to the mor-
bid anatomy of this
case ; but it may be
added that after several
operations the child
died. Some eight
months later the
mother was operated
upon for abdominal
tumour, and an ovar-
ian cyst weighing 35
lbs. was removed ; at
the operation the
uterus was found to
be two months preg-
nant. The pregnancy
went on and the
patient greatly feared
she might give birth
to another monstrosity.
Labour came on about
the sixth month; there
was hydramnios ; an
anencephalic (pseuden-
cephalic) female foetus
was born ; and a post-
mortem examination revealed transposition of the thoraco-abdominal
organs (heart, liver), and other anomalies. In the former pregnancy
the mother was in good spirits and had no fears ; in the latter she
lived in terror that she might give birth to another monster.

In one of Vannoni's cases (1850-51) a woman gave birth, first,
to an infant with an occipital encephalocele, and her next pregnancy
ended in the birth of one with a cephalocele of the vertex (left
parietal region) ; in another of his cases the mother suffered violence
during pregnancy. Twin brothers affected with occipital cephalocele

Fig. 61.— Occipital Encephalocele. Specimen No. 22.

CEPHALOCELE 355

were seen by Meckel (1822), and Carli (1863) relates the story of a
woman who gave birth to two anencephalic foetuses in her first two
pregnancies and to an infant with a cephalocele in her third. It may,
therefore, be concluded that the history of the pregnancy that ends
in the birth of an infant with a cephalocele resembles in several
characters that which is accompanied by anencephaly.

The postnatal clinical history of the deformed infant differs in
many respects from that of the anencephalic foetus. The child may
not only survive birth, but may live for weeks, months, or even
years ; and the malformation may even spontaneously disappear.
When, however, the cephalocele is large, life is usually measured by
weeks, and the cause of death is inflammation due to traumatism or
operative interference. The small cephaloceles, on the other hand,
may be met with in adults (cases at seventeen, twenty, twenty-three,
thirty-three, and even at fifty-eight years having been reported) ;
the hydro-meningoceles are less fatal than the encephaloceles and
yield slightly better results if operated upon (by compression,
ligature, or, best, by excision). Of my eight cases, four died at birth ;
three lived for some weeks ; and one (a fronto-nasal cephalocele of
small size) was five months old when seen by me. Two died after
operation ; but in one of these cases excision was performed only for
cosmetic purposes and not with the hope of cure.

Infants with large cephaloceles are usually born with difficulty.
The diagnosis is not generally made before labour, or, indeed, till the
os is fully dilated, and even then they are generally mistaken for
cases of hydrocephalus and treated as if they were such. After
birth they may in certain cases require to be diagnosed from
dermoids and serous cysts, from cephallnematomata, and from intra-
natal hernias.

Morbid Anatomy.

In considering the morbid anatomy of this monstrosity I shall
take up (1) the position of the cephalocele, (2) the sac and the
opening through which it protrudes, (3) the contents, including the
varieties founded upon the nature of the contents, (4) the state of
the rest of the cranium, and (5) the associated malformations.

(1) Position of the Cephalocele. — Most commonly it is in posterior
part of cranium (occipital), when it may be (1) in middle line below
occipital protuberance, or above it (in position of ossicle of Kerckring),
or at posterior fontanelle ; or (2) lateral, at postero-lateral fontanelle,
or in the lambdoidal suture. The commonest of all is the first, and
then the opening may extend to the foramen magnum and even to
the cervical vertebra?. Next in frequency, it is in the anterior part
of cranium (sincipital), when it may be (1) in the middle line, in the
naso-frontal, in the frontal, or in the naso-ethmoidal sutures ; or (2)
lateral, between the nasal process of the superior maxilla and the
lachrymal bone, or supra-orbital, or at the outer angle of the orbit.
Less often it is in the part of the cranial vault known as the vertex,
when it may be (1) lateral, in the parietal or temporal bones (right

35 G ANTENATAL PATHOLOGY AND HYGIENE

or left) or in the coronal suture (right or left half) ; or (2) median,
in the anterior fontanelle or in the sagittal suture, Barest of all it
is in the hase of the cranium, when, again, it may be (1) median, in
the sella turcica or between the sphenoid and ethmoid; or (2) lateral,
at the sphenoidal or spheno-maxillary fissures. Save in cephaloceles
of the vertex of the cranium, the opening is more frequently in
the middle line than at the sides ; more often at the posterior and
anterior ends of the middle line than midway, more often at the
posterior than at the anterior end ; and when it is at the posterior
end it is more often below than above the occipital protuberance.

(2) The Sac of the Cephalocele and the Opening in the Cranium. —
The sac varies (1) in size, from that of a pea to that of a fcetal head
or larger; (2) in shape, being spherical, elliptical, pear-shaped, or
cylindrical, with one or two or (rarely) more lobes, and with or with-
out a pedicle; (3) in its consistence, being cystic or soft and doughy
in accordance with its contents; (4) in its coverings, which may lie
unaltered skin, aponeurosis, and cerebral membranes, or (more often)
modified skin (hairless save at margins, red, thick or thin, vascular)
and modified cerebral membranes (no dura ; thickened arachnoid and
pia, often in a myxomatous state). Sometimes the coverings show
cicatrices or amniotic filaments or fringes passing to placenta ; such
specimens are not far removed from instances of mero-acrania, in
fact there is no clear dividing line. The sac may be reducible by
pressure, non-reducible, or partially reducible (depending largely on
the nature of its contents) ; when reduced, strabismus, convulsions,
and coma may follow. The sac may pulsate synchronously with the
pulse or the respiration. The orifice through which the sac protrudes
has usually smooth osseous (rarely membranous) margins, excep-
tionally they are irregular ; it is round or oval, small or large, but
never large enough to allow escape of whole brain or even of a
hemisphere.

(3) The Contents of the Cephalocele. — According to their contents,
cephaloceles may be arranged in four groups (vide infra) :

(a) Hydro-meningocele or Meningocele: consists of a protrusion
of the cerebral membranes (usually altered), containing fluid only;
gives fluctuation, is transparent, and can be diminished by com-
pression ; sometimes its cavity does not communicate with interior
of cranium ; is situated usually posteriorly (occipital), less commonly
at vertex, rarely anteriorly (sincipital) ; and is a comparatively rare
form of cephalocele (16 to 20 per cent.). It may be pedunculated, and
may or may not communicate with an enlarged cerebral ventricle ;
and Bland -Sutton (Tumours, p. 516, 1903) regards an occipital
meningocele as an expanded fourth ventricle or as " hydrocephalus
limited to the fourth ventricle," and thinks the cerebellum is entirely
absent and that the flocculus is enlarged.

(b) Encephalocele : consists in a protrusion from cranium of
part of cerebrum or cerebellum ; is the most common of all the
cephaloceles (54 per cent.) ; has soft, doughy consistence, is opaque,
is little or not at all reducible by compression, and when pressed
upon causes convulsions or syncope ; it pulsates synchronously with

CRANIUM IN CEPHALOCELE 357

the pulse ; is more common posteriorly (occipital), but is not in-
frequent anteriorly (sincipital), rare at the vertex ; and it may
be associated with distension of the cerebral ventricles inside the
cranium. Its contents, when it is posterior, may be cerebellum
alone or with one or both occipital lobes of cerebrum, or one or both
occipital lobes without cerebellum, or unrecognisable part of brain
(a neoplasm) ; when anterior or at vertex the contents are the cerebral
lobes which correspond to the position of the opening, or an unrecog-
nisable mass of brain tissue (a neoplasm or cephaloma).

(c) Hydro-encephalocele or Hydrencephalocele : consists in a
protrusion of brain substance which contains in its interior a cystic
cavity full of fluid ; has characters resembling in some cases those
of the hydro-meningocele, and in others those of the encephalocele,
or partaking of both ; occupies the same positions as the encephaloceles,
and in much the same order of frequency ; is a rarer form than either
encephalocele or hydro-meningocele (11 per cent.); and in its struc-
ture it is said to resemble hydrocephalus when there is no protrusion,
the brain substance being possibly reduced to a very thin external
layer.

(d) Hydro-meningo-encephalocele : consists in the protrusion of
brain substance, along with the presence of fluid between the mem-
branes external to the cerebral protrusion ; is the rarest of all the
cephaloceles (9*8 per cent.) ; it may be situated posteriorly, anteriorly,
or at the vertex, but most ofteii posteriorly (although number of
cases is too small to give value to statistics) ; and it may contain
various lobes of the cerebrum and (when occipital) of the cerebellum.

With regard to the contents in general, it may be added that
in all the varieties which contain cerebral substance the part so
contained may apparently be of the nature of an accessory produc-
tion. In Dr. Etta Charles's case, to which I have already referred,
the cerebrum and cerebellum inside the cranium were apparently
normal and intact, while lying in the sac was an accessory brain
(cerebrum and cerebellum) engrafted on the cord near the medulla
and of the size of a small orange. Something of the same kind was
detected at the incomplete autopsy on a case of large occipital
encephalocele which I saw in connection with the Cowgate Dis-
pensary in 1895 (Specimen No. 143); the child, a male, died on
the eighth day, and the contents of the sac seemed to represent
accessory structures.

(4) The Vault and Basis Granii. — The rest of the head may or
may not be greatly altered in cases of cephalocele. In the instances
of cephalocele in the anterior part of the cranial vault, the head may
not be altered at all save when hydrocephalus exists as a complica-
tion ; when the protrusion is at the vertex the alteration is not great,
being generally a depression of the bones due possibly to the weight
of the extra-cranial portion ; but when the cephalocele is posterior
the shape of the rest of the head may be markedly abnormal. Even
in the occipital cases, however, the deformity is not necessarily great
if the cephalocele be below the occipital protuberance and be of the
nature of a hydro-meningocele. When the cephalocele is in the

358 ANTENATAL PATHOLOGY AND HYGIENE

upper part of the supra-occiput, and when it affects the posterior
fontanelle, and when the occipital lobes enter into its composition,
then the head is much modified : it is of small size, the bones are
close together, the middle part of the vault is saddle-shaped (clino-
cephalus), there is cyphosis at the sphenoidal angle at the basis
cranii, prognathism, and elongation of the nasal and superior max-
illary bones. The cyphosis of the basis cranii is a very striking
character, and there may actually be a dislocation between the basi-
occiput and the post-sphenoid. It will be remembered that these
changes occurred also in anencephaly (p. 342). When, however,
hydrocephalus exists, the whole head is enlarged and the sphenoidal
angle is enlarged, and hyper-orthognathism takes the place of prog-
nathism. Many very interesting but not altogether satisfactory
explanations have been given of these architectonic alterations in the
cranium ; in all of them too much attention is paid to the relation of
parts as they exist at birth and too little to what was the state of
matters in the early period when the monstrosity was produced.

It may be noted, here, that the supra-renal capsules and sym-
pathetic system have been found to be normal in cephalocele ; this
is interesting in relation to their state in anencephaly.

(5) Associated Malformations. — Like all monstrosities, cephaloceles
may be associated with other malformations, among which may be
named spina bifida, hydrocephalus, hare-lip, cleft palate, club-foot,
umbilical hernia, exomphalos, ectopia vesicae, cyclopia, transposition
of the viscera, cystic kidney, amniotic adhesions and bands, and
so-called congenital rickets. In a cephalocele of the occipital region
below the level of the protuberance, cervical spina bifida is so com-
monly associated as to be almost an integral part of the teratological
state. Hydrocephalus, also, exists with great frequency.

(6) Comparative Teratology. — Like anencephaly, cephalocele is a
very rare malformation among the lower animals. When it does
occur, as in the calf, the lamb, the pig, the kitten, the puppy, or the
foal, it is most often found at the frontal end of the cranium. It
may be associated with amniotic adhesions. It may, however, be
met with more frequently in birds than in mammals, and may in
them be the distinctive character of a variety or race as well as a
sporadic malformation; this subject has been dealt with by Darwin,
Virchow, and several others, and constitutes an interesting chapter
in Comparative Teratology.

Teratogenesis.

A great amount of ingenuity has been almost wasted over'
attempts to explain the origin and mode of production of cases of
cephalocele, and that for a reason which will be explained im-
mediately. Let me, first, very briefly indicate some of the theories.
Placing on one side the theories of external or internal maternal
traumatism and diseased states (alcoholism, etc.) of the parents, I
may state, as follow, the alleged causes in the different varieties of
cephalocele.

TERATOGENESIS OF CEPHALOCELE 359

In cases of hydro-meningocele, dropsical distension of the cere-
bral ventricles, and a localised inflammation of the cerebral mem-
branes (with accumulation of serum) are views that have been
entertained ; but as neither of these states may exist, such views
fall to the ground as insufficient. In the combination of cranial
meningocele with encephalocele (hydro-meningo-encephalocele), it
has been assumed that a localised meningitis exists first, and by
weakening the cranium prepares the way for the escape of the
cerebrum ; but apart from other difficulties, there is the fact that
solid encephaloceles may be met with.

Round the causation of encephalocele the greatest controversy
has taken place. It has been ascribed to smallness of the cranial
cavity due to synostosis, a fact which fails to explain encephalocele
when it occurs in a membranous cranium. It has, on the other
hand, been attributed to defective ossification of certain areas of the
cranial vault ; but as every one knows, such spaces may exist without
any encephalocele. It has also been assigned to increased intra-
cranial pressure, which in its turn has been ascribed to ventricular
distension (hydrocephalus), to cerebral hypertrophy (for in many
instances there is no enlargement of ventricles), or to cedematous
swelling of the brain. Finally, hydro-encephalocele has been con-
fidently attributed to localised accumulation of fluid in one or other
of the cerebral ventricles or in a part of one of the ventricles.

Now, in all these theories, it will be observed that it is assumed
that the malformation supervenes upon a normal condition, and even
upon a normal condition of a fully formed structure. It is taken for
granted that there is first of all a cranium (already ossified) con-
taining a brain (developed up to the degree existing at birth) ; that
then a pathological process of the nature of a disease attacks the
parts, enlarges some structures, weakens others ; and that, finally,
enlarged organs push their way through weakened containing struc-
tures and so produce a cephalocele. In other words, it is thought
that the malformation arises in the fcetal period and as the result
of a foetal disease. If this ever occur, it must, I think, be rare.
More reasonable is it to regard all the morbid states found (enlarged
part, dropsical distension, deficient ossification, etc.) as parts of the
malformation, not as causes leading to it. It is absolutely necessary
to form a visual image of the head during its embryological develop-
ment and not after it is formed, and then to try to picture to
one's self its maldevelopment. We must not think of intracranial
parts being pushed out at cranial apertures and so becoming extra-
cranial ; we must imagine parts of the anlage of the brain {e.g. the
cerebral vesicles and undifferentiated membranes) lying originally
outside what is to be the cranium and developing in their ectopic
position. In support of this theory is the fact that occasionally an
accessory cerebral structure is found in the cephalocele ; that struc-
ture, it is reasonable to suppose, was originally outside the anlage
of the nervous system although attached to it. If it be asked, what
is the cause of the accessory part, we, of course, have no satisfactory
answer ; but the idea of a purely germinal (pre-einbryonic) factor

360

ANTENATAL PATHOLOGY AND HYGIENE

suggests itself. To sum up ; it is not claimed that the embryological
theory explains all the facts, but it explains them better than the
pathological hypothesis, and with an extension of our knowledge
of ontogenesis and an acquaintance with the laws of germinal
pathology it may yet be found to be sufficient. The combination
of the idea of an arrested embryonic development and excessive
germinal activity seems to me to contain the explanation.

Hydrocephalus.

In the first section of this Manual I referred to hydrocephalus,
and T again take notice of it here. The reason is not far to seek.

There are two theories of the
origin and nature of hydro-
cephalus : one regards it as a
foetal disease, and on this account
it found a place in Volume I. ;
the other looks upon it as an
embryonic malformation, and so
it appears in the present chapter.
It is probably more correct to say
that there are two varieties of
antenatal hydrocephalus, a path-
ological (or nosological) and a
teratological ; at any rate such is
the current belief at the present
time. I prefer, however, to state
the matter somewhat differently :
I believe that antenatal hydro-
cephalus is generally due to an
embryological arrest of develop-
ment to which may be superadded
in the fretal period a disease
affecting the malformed parts, but
I admit the possibility of hydro-
cephalus arising, without a pre-
ceding malformation, in the foetal
epoch from a disease, just as it sometimes originates in postnatal
life.

The condition itself is well known to the obstetrician, the pediat-
rist, and the surgeon. It consists essentially in the presence of a
fluid accumulation inside the cranium, generally within the dis-
tended cerebral ventricles and their communicating passages, but
occasionally between the membranes.

It is sometimes, but apparently not very often, associated with
hydramnios in pregnancy ; there may also be oligohydranmion
(Bonnaire, Arch, de tocol., xxi. 157, 1894). Its diagnosis is rarely
made till labour is in progress, when the large size of the head, the
width of its sutures and fontanelles, and the parchment-like con-
sistence of the vault bones will lead to recognition. It is most

Fir:. 62. — Case of Hydrocephalus with
Deformity of Right Arm and Hand
(Absence of Thumb). Specimen No. 71.

Plate xix.

Anterior
fontanellt-

Cerebellum

Fluid in
pharynx.

Trachea. 1

Oesophagus... Lj

Rectum.

Vagina

fluid in
Ventricle.

Tongue.

Tooth.
Hyoid.
- Thyroid.

Liver.

Intestines.

Uterus
Bladder.

^-Symphysis pubis.

HYDROCEPHALUS 361

likely to be confounded with a cranial meningocele or encephalocele
or with a large sacral cyst. In breech cases, when the breech has
been born and a lumbo-sacral spina bifida appears, the chances are
that a hydrocephalic head is to follow. If unrecognised, hydro-
cephalus may prove a serious complication of labour.

The reproductive history of the mother is sometimes interesting.
Family prevalence may be met with, several hydrocephalic infants
being born to the same parents. More interesting still are the
records in which hydrocephalus and other malformations have fol-
lowed one another in the same family history. For instance, one of
my specimens of hydrocephalus (Fig. 62) was born to a woman who
afterwards had an anencephalic foetus, and, later still, one with mal-
formation of thumbs, absence of radii, and commencing hydro-
cephalus ; all the three foetuses had deformities of the thumbs, and
the mother, her sister, and her mother had defective thumb muscles.
A frozen section of the third foetus is shown in Plate XIX., and it
will be noted that there is commencing hydrocephalus although no
external indications of it were present. In connection with one of
my specimens of iniencephaly (No. 155) the mother had in a previous
pregnancy had a hydrocephalic child.

Consanguinity of the parents does not appear to be specially
associated with hydrocephalus in the offspring; but parental syphilis
seems to be so frequently met with as to suggest more than a
casual coincidence. E. Founder (Stigmates dydrophiqncs dc Vlure'do-
syirftilix, p. 66, 1898) has indeed collected together from literature
as many as 170 cases in which hydrocephalus occurred in foetuses
and infants who were the subjects of hereditary syphilis. Tubercle,
nervous diseases, and alcoholism in the parents have also been blamed.

The morbid anatomy of hydrocephalus has only been worked out
within recent years, its finer details having been specially investigated
by H. Virchow (Fcst-Schr. Albert v. Kblliker, p. 305, 1887), H. Chiari
(iJbcr Veranderungen des Kleinhirns . . . in Folge von congenitaler
Hydrocephalic des Grosshirns, Wien, 1895), and by M. Karch {Diss,
inauij., Wiirzburg, 1898). As a general rule, the head is greatly
enlarged in all its diameters, and the great round cranium contrasts
in a very striking fashion with the small triangular face ; the sutures
and fontanelles are wide, and in some cases there are little more than
small islands of bone in a sea of membrane. In rare cases there is
not largeness but smallness of the hydrocephalic head. I found, for
instance, in an autopsy which I performed for Dr. Berry Hart on an
infant that died in the Edinburgh Eoyal Maternity Hospital in
March 1895, that although the head was small (so small that the
baby had been known as " The Monkey " in the Hospital) yet the
contents above the tentorium cerebelli were solely fluid ; the infant
had, nevertheless, lived for nine days, and had differed from other
babies only in not opening its eyes and in not sucking properly. It
was cyanosed when born and remained so. It kept constantly
moving its tongue backwards and forwards. The mother was a
I-para, and the labour was at the full term. The child weighed 5 lbs. ;
the occipito-mental circumference of the head was 13£ inches and

362 ANTENATAL PATHOLOGY AND HYGIENE

the occipitofrontal llh inches. The foramen ovale in the heart was
patent, and the anterior fontanelle nearly closed. Cases of this kind
belong to the group of the micro-hydrocephalics ; a somewhat similar
case was described by S. De Sanctis in 1900 (Ann. di neurol., xviii.
265, 361, 1900). In a third group of cases are the infants in whom
the head at birth is normal in size, and in whom, after an apparent
latent period, hydrocephalic enlargement appears. Such cases are
figured in Plates XVIII. , XIX., and XX. ; in all of them hydrocephalus
was not suspected before the sections were made.

The essential character of hydrocephalus, therefore, is not found
in the size of the head but in the dilated condition of the cerebral
ventricles. Liquid in excess is usually found in all the ventricles
(especially in the lateral, but also in the third, and sometimes in the
fourth) ; it is commonly equal on both sides ; the foramina of Monro
and the aqueduct of Sylvius arc distended ; and the liquid itself has
the characters of cerebro-spinal fluid, or may contain much albumen.
It is not always that a communication (foramen of Majendie) between
the fourth ventricle and the sub-arachnoid space can be demonstrated
(J. O'Carroll, Trans. B. Acad. Med. Ireland, xii. 293, 1894). In some
instances the cerebrum may be practically absent, some traces only
of it being found on the basis cranii ; in other cases the malforma-
tion is less pronounced and the walls of the hemispheres may be
thinned, and the fissure of Sylvius may be widely open exposing the
island of Reil. The corpus callosum, trigone, and septum lucidum
may be absent. The dilatation is chiefly at the expense of the
medullary part of the cerebrum, and the cortex may be comparatively
little altered. The ventricular membrane may be thickened, and the
choroid plexus may be variously altered (hypertrophic, flattened,
aneemic).

The changes in the cerebellum have been worked out by H.
Chiari and others. It may be normal ; on the other hand, it may be
absent, atrophic, asymmetrical, or hypertrophic. Further, a curious
change in its form may be found : there may be an elongation of the
tonsils and of the median part of the inferior lobe, and these may
descend into the spinal canal (Plate XVIII.). The fourth ventricle,
also, may be displaced downwards. Chiari has arranged these cere-
bellar and associated changes in four types with subdivisions, and to
his work (op. cit.) the reader is referred for full details. The falx
cerebri and tentorium cerebelli may be absent. There commonly is
great obliquity and shortness of the basilar groove and enlargement
of the foramen magnum ; the floor of the orbital cavity is usually
lowered, giving to the eyes their peculiar appearance. The optic
chiasma may be wanting (H. B. Ferris, Yale Med. Journ., i. 2:!9, 1895).'
The spinal cord may be found intact, but sometimes there is hydro-
myelia, especially if spina bifida coexist.

Associated malformations are common and interesting. Among
the more frequent may be named spina bifida, cephalocele, and club-
feet. Hare-lip and cleft palate are also frequent. Among the less
common are facial fissures (as in A. Heinicke's case, Diss, inane/.,
Kbnigsberg i. Pr., 1890), defective external ears and Polydactyly

Plate

Thyroid. __S

Small
intestines.

Pharynx.

Hydrocephalic
fluid.

Cerebellum .

Suprarenal
capsule.

MICROCEPHALY 363

with facial fissures (as in L. Hirschberg's specimen, Diss, inaug.,
Konigsberg i. Pr., 1891), exomphalos (as in 0. Schmidt's case, Diss.
inaug., Berlin, 1885), and absence of the nose and one eye (in one
of E. Wernicke's specimens, Diss, inaug., Berlin, 1885). I have
examined seven cases : in two there were digital defects, in two spina
bifida, in several the feet were clubbed, and in one there was cyclopia
and absence of the lower jaw. The association of cyclopia with
hydrocephalus is well exhibited in a female foetus figured and de-
scribed by Beveridge {Northumh. and Durham Med. Journ., ii. 51,
1894). It is also met with in cases of so-called foetal rickets and
phocomely. In some instances of hydrocephalus the fluid accumula-
tion may be localised, and then an irregularly shaped head may
result ; if, for instance, there is premature synostosis of the sagittal
suture, scaphocephaly will follow.

Great activity has been shown of recent years in the surgical
treatment of hydrocephalus, but as yet without conspicuous success.
At present most attempts aim not at cure but at cosmetic effects.
To quote from H. J. Stiles' article {Brit. Med. Journ., ii. for 1902,
p. G7-">) : " Repeated tapping (with or without compression of the
head), intermittent or continued capillary drainage of the lateral
ventricles, drainage into the loose cellular area beneath the occipito-
frontalis (Mikulicz), intermittent and continuous lumbar drainage,
capillary drainage into the sub-arachnoid space (Sutherland and
Watson Cheyne), injection of Morton's fluid, repeated and more or
less complete evacuation of the fluid by tapping and simultaneous
introduction of aseptic air (Ewart and Dickinson), and drainage from
the spinal canal into the peritoneal cavity, have all been tried, with,
I fear, it must be admitted, only partial and temporary success."
Stiles himself tried the last-named method, and led some strands of
horsehair from the sub-dural space in the lumbar region into the
peritoneal cavity just external to the descending colon ; but without
success. Probably, in the meantime, the surgeon's efforts will be
best restricted to endeavouring to diminish the great bulk of the
head, and so lessen the distress of the child's parents.

Microcephaly.

The microcephalous individual is one whose head and brain are
abnormally small. Attempts have been made to fix the limits of
cranial size or capacity within which microcephaly lies ; but it is
impossible thus to define the anomaly, for such measurements must
vary with the age of the patient and the dimensions of the body, and
for the additional reason that it is the condition of the brain rather
than of the containing cavity which determines the morbid state.

Very few cases have been described as they exist at or soon after
birth ; for the malformation does not interfere with postnatal life of
a vegetative kind, and it is, therefore, not till some months have
elapsed that it is noticed that the child is mentally deficient. In 0.
Giacomini's fine monograph (Cervelli dei microcefali, Torino, 1890),
which every one who is specially interested in microcephaly ought

364 ANTENATAL PATHOLOGY AND HYGIENE

to study, there was only one observation out of nineteen on a new-
born infant. The condition is truly antenatal, but it is, as I have
said, rare for it to be studied until antenatal life has been left
months or years behind.

Such cases have always created great interest. In olden times
(and in popular opinion now-a-days) they were regarded with a strange
mixture of curiosity and disgust as ape-men (" hommes-singes ") ;
and in recent times they have formed the foundation of many
theories regarding the descent (or ascent) of man from simian
ancestors. It appears to me, however, that the right way to look
upon microcephaly is as an antenatal arrest of growth and develop-
ment ; as such it proves no more in respect to the primal origin of
man than does anencephaly or exomphalos. From antiquarian ob-
servations it would seem to have been observed and recorded far
back in the world's history.

Little is known of the antenatal clinical history of the micro-
cephalic foetuses, for, as I have said, interest in them is not often
excited till some months after birth. At the same time it would
appear that hydramnios is not an uncommon occurrence, and that
family prevalence is frequent. Two microcephalics have been born
to the same mother, as in Giacomini's first case (in which the birth
of the second defective child was ascribed to a maternal impression
received from the first); three microcephalic brothers have been
met with (J. V. Laborde, Rev. scienl. 4 s., iii. 579, 1 895) : four micro-
cephalics and a clinocephalic occurred in the same family (S. N.
Tirrizzi, Atti d, Accad. Gioenia di sc. nat. in Catania, 3 s., xv. 1, 1881) ;
and in the famous Becker family there wTere five individuals affected
with microcephaly (0. Flesch, Verhandl. d. Berl. Gcsellsch.f. Anthrop.,
72, 188."! ; T. L. W. Bischoff, Anatomisches Beschrcibung eines mikro-
ccphalen 8 jdhrigen Madchens, Helcne Becker aus Offenbach, Mtinchen,
187:') ; N. Iiiidinger, Sitzungsb. d. math.-phys. CI. d. k.-baycr. Akad. d.
Wissensch. z. Munchcn, xv. 112, 1885; Stendel, Med. Cor.-Bl. d.
Wurttemb. arztl. TV?-., lvi. :,»."., 1886 ; and others). The so-called Aztec
man and woman (Indians from Mexico), exhibited in various parts of
the world, were instances of microcephaly; they were married in
1867, but had no offspring (F. Birkner, Arch. f. Anthrop., xxv. 45,
1898). It has been affirmed that in one instance only has a micro-
cephalic woman conceived, and that in her case the foetus was born
dead (Ireland, in Hack Tuke's Dictionary of Psychological Medicine,
ii. 807, 1892). One of Shuttleworth's patients (journ. Ment. Sc, xxiv.
438, 1878) had consanguineous parents; and the parents' late marriage
{opsigamy, as it is called) has been alleged to be a causal factor. The
family history in Frey's case {Arch. f. Anthrop., xxv. 33, 1898) was'
interesting ; there was first a normal boy, then a microcephalous boy,
then triplets (a normal boy, and a microcephalous boy and girl), then
a microcephalous girl, and finally two normal children. Into the
matter of the other functions of the body and mind in this form of
malformation it is not my purpose to enter ; it lies outside the sphere
of antenatal pathology, although, of course, it very evidently falls
within that of the postponed effects of antenatal pathology.

MICROCEPHALY 365

The morbid anatomy of microcephaly raises several interesting
problems. I shall, as usual, consider (1) the cranium, (2) its con-
tents, (3) other parts of the nervous system, and (4) associated
malformations.

(1) At birth the cranium may show premature closure of one or
more of the sutures ; but, as this is not a constant character, it follows
that the theory of microcephaly being produced by this untimely
synostosis cannot be maintained. The cranium is small in all its
parts and diameters, but especially in the frontal region; the occipital
region is more expanded, and the occipital condyles are prominent.
The head has, therefore, a peculiar avian character, which has earned
for the malformation such names as " homme-oiseau " and " Vogel-
Kopf." There is prognathism of the face ; the basis cranii is con-
tracted, although not so markedly as the vault ; the ethmoidal fossa
is deep and narrow. Other characters, including the state of the
temporal ridges, mastoid processes, external auditory meatus, etc. etc.,
will be found described in such monographs as those of Cunningham
and Telford-Smith (Trans. Roy. Soc. Dublin, 2 s., v. 287, 1895), and of
Sir George Humphry (Journ,. Anat. and Physiol., xxix. 30-4, 1895).
It may be added that there is nothing in the anatomy of the parts to
support the idea that the defective development of the brain is due
to .cranial compression ; there are, on the other hand, certain char-
acters which may be called " simian," although it does not, of neces-
sity, follow that their presence demonstrates the atavistic origin of
microcephaly.

(2) The brain, which lies within the microcephalic cranium, is also
small in size ; there is microencephaly as well as microcephaly. In-
deed there is every reason to regard the smallness of the brain as the
primary condition, and that of the cranium as secondary to it. The
weight of the brain has varied from less than an ounce (40 grms.)
and nearly 4 oz. (107 grms.) in the new-born infant to a little
more than 2 lbs. in an adult (Giacomini). A brain weighing only
7 oz. from a girl aged twelve years was shown (by Fletcher Beach)
to Ireland.

The subject of the characters of the brain in microcephaly is a
complicated one, but Giacomini (op. cit.) and others have done much
to remove some of the confusion. Giacomini, especially, by arranging
the recorded cases in groups, has thrown some light into this dark
corner of antenatal pathology. He has pointed out that there is no
typical brain of microcephaly, none which constitutes a constant and
characteristic type ; there are only microcephalic brains. These
cannot be satisfactorily arranged in groups either according to weight,
or to volume, or to the capacity of the crania containing them ; it is
necessary to take into account other characters. Giacomini divides
all the cases into two large groups : one contains the true micro-
cephalics and the other the pseudo-microcephalies. He prefers the
words microencephaly and pseudo-microencephaly, but the distinction
is comparatively unimportant. He has also a third group in which
other malformations are present as well as microcephaly ; the mem-
bers of this division he does not regard as microcephalics proper. I

366 ANTENATAL PATHOLOGY AND HYGIENE

think, however, that Giacomini is too insistent upon a clear dividing
line ; I prefer to regard these cases as instances of microcephaly with
associated malformations, and I consider his pseudo-microcephalies as
cases of microcephaly with associated morbid processes of the nature
of foetal or infantile disease. Whether microcephaly occurs alone or
is associated with foetal disease or embryonic malformation depends,
to my thinking, upon the time when the morbid cause comes into
action, and how long it continues to act. If the reader has read the
chapters on Teratogenesis he will understand what I mean. True
microcephaly, as Giacomini considers it, is apparently an arrested
development of the central nervous system at a comparatively late
date in antenatal life ; such an arrest is possible because the brain is
slow in passing out of the embryonic into the foetal state, indeed it is
still in the embryonic condition when most of the other systems are
in the foetal. Hence it comes about that we may meet with micro-
cephaly alone with no other malformations of brain or other part.
In such cases I hold that the causal factor (whatever it was) came
into play late in antenatal life when the other parts were already
formed. In other instances diseases of neighbouring structures, of
the brain itself, are discovered (Giacomini's pseudo-microcephalies);
these I regard as the results of the same cause ; but that cause is
acting on structures no longer in the formative phase, and so diseases
not malformations are produced. In yet other cases there are
coexistent malformations of the brain or of more distant parts : those
of the brain are more fundamental than the slight deviations which
Giacomini allows as characters of microcephaly, and they may be
supposed to have been produced by a cause acting earlier, when the
brain had not assumed its final form and parts ; those of more distant
parts are obviously the result of the action of a cause at a still earlier
date in antenatal life, when other parts of the body than the
nervous system were still in the embryonic or formative stage.
From this standpoint let us now look at the cerebral conditions in
microcephaly.

The brain is simple, poor in convolutions, and those that are
present are coarse and lacking in complexity (microgyry) ; the
secondary and tertiary fissures are wanting, as are also bridging
convolutions. The brain, then, is, in these cases, the human brain in
miniature ; and we may imagine an arrest near the end of antenatal
life or even later. In another group of instances of microcephaly,
however, the changes are more fundamental ; in them there is micro-
cephaly of a high grade. There is greater obliquity of the posterior
limb of the Sylvian fissure; the bends or genua are absent in the
fissure of Rolando ; the fronto-parietal operculum does not develop;'
the external parieto-occipital fissure is notch-like ; and there is
obliquity of the orbital surface of the frontal lobe. In many cases
characters are also to be found which recall the condition of the
brain mantle in the apes ; these are the so-called simian characters.
In the Sylvian region, for instance, part of the island of Eeil is
exposed, and the frontal and orbital opercula are wanting. The
intra-parietal sulcus is much less complicated than normal, and the

MICROCEPHALY 367

so-called " Affenspalte " is present ; the fissure of Rolando, also, is
shallow and has smooth walls. Iu these high-grade microcephalics,
therefore, there is evidence of an earlier arrest in development, about
the third or fourth month of intrauterine life, it is said.

In another group of cases, the pseudo-microcephalies of Giacomini,
there are changes of quite another kind of characters. There may be
marked asymmetry of the brain, due to complete or partial atrophy
of one hemisphere ; there may be evident signs of foetal encephalitis
and marked consequent changes in the appearance of the cerebral
surface ; there may be various degrees of dropsical distension of the
ventricles or hydrocephalus (the only specimen of microcephaly which
I have examined was an instance of hydro-microcephaly) ; and
porencephaly may be present.

The histological appearances of microcephalic brains have,
unfortunately, been little investigated. Giacomini, however, comes
to the conclusion that they are little different from the normal in
typical cases. The cortical substance is smaller in amount ; there are
fewer nerve cells, and their development is less complete, and they
show fewer processes. There is, therefore, according to Giacomini, a
morphological rather than a histological character about the morbid
anatomy of microcephaly ; and this is supported by others, including
P. Bull {Norsk. Mag. f Lcegevidensh, 4 R, xvi. 1106, 1901).

It may be added that through the small size of the occipital lobes
the cerebellum is not well covered by the cerebrum and is therefore
more exposed to view. It is not enlarged ; as a matter of fact it is
commonly found to be diminished both relatively and absolutely.
The various portions which go to make up the cerebellum, however,
are present, and bear normal relations to each other; and their
histological characters are not much altered.

(3) The other parts of the nervous si/stem show deviations from the
normal. As regards the spinal cord, there generally is micromyelia ;
the whole cord is diminished in size and the pyramidal strands of
fibres (pyramidal tracts, columns of Goll, direct cerebellar tract) are
the parts most affected, but the grey matter is also less in amount
(A. Steinlechner-Gretschischnikoff, Arch.f. Psychiat., xvii. 649, 1886).
The state of the spinal cord is used, and with perfect fairness, by
Giacomini as an argument against the local mechanical theories of
origin of microcephaly which regard compression of the brain by the
cranium, diminution in the size of the arteries supplying the
cerebrum with blood, or internal hydrocephalus as causal factors. It
is evident that micromyelia, without obvious changes in the micro-
scopical appearances of the parts of the cord, points to an arrested
growth and development of the whole cerebro-spinal system, and not
to any effect localised in the cerebrum. The same remarks apply to
the medulla oblongata, the pons, and the cerebral peduncles.

(4) Associated malformations occur in connection with micro-
cephalus as with other teratological states ; and I do not agree with
Giacomini in thinking that their presence removes the case from the
group of the true microcephalics. They are no longer uncomplicated
microcephalics ; that is evident ; but it is not, I think, necessary to

368 ANTENATAL PATHOLOGY AND HYGIENE

cease to give them the name of microcephaly. The associated mal-
formations affect, in the first place, the brain itself : they may consist
in almost complete absence of the brain or in its very rudimentary
development ; in fusion more or less complete of the cerebral hemi-
spheres (Rudinger, Munchcn. med. Wchnschr., xxxiir 161, 185, 205,
1885 ; Aeby, Arch./, path. Anah, lxxvii. 554, 1870); in more or less
complete absence of the corpus callosum (Onufrowiez, " Das balkenlose
Microcephalengehirn Hofmann," Diss, inaug., Berlin, 1887 ; Ducatte,
TMsc, Paris, 1880 ; P. Broca, Bull. Soc. d'anthrop. de Paris, 2 s., xi.
85, 1876); and in anomalies of the hippocampus major, fascia
dentata, etc. Outside the central nervous system there may likewise
be malformations : of such are cyclopia (G. Eaineri, Osservatore, xl.
767, 1880), hare-lip and cleft palate (C. Gunther, Diss, inaug.,
Kbnigsberg i. Pr., 1 807), slight anomalies of the kidneys and uterus
(D. Hansemann, Zwci Falle von MiJcrocephalie mit Rachitis, Stuttgart,
1800), etc.

The teratogencsis of microcephaly presents many unsolved
problems. Of late years, however, some things seem to have become
clear : it is not, for instance, now believed that the condition is due
to cranial pressure, or to hydrocephalus, or to narrowness of the
cerebral arteries, or to foetal inflammatory disease (encephalitis). It
is generally regarded as an arrested development. Karl Vogt {Arch,
f. Anthrop,, ii. 120, 1867), writing in 1867, ascribed the arrested
development to a partial atavism, and since then a great deal has
been written and spoken on this subject. It cannot be denied that
certain animal-characters are found in the microcephalic brain, but
whether or not these are really simian reminiscences is very difficult
of decision. Giacomini (op. cit.) thinks that there are some atavistic
characters in microcephaly, but that these cannot be used as argu-
ments for the theory of man's descent from the apes ; the brain as a
whole always retains the human imprint. The question at the
present day has resolved itself into a discussion of the precise
significance of simian or animal-characters in the human subject ; in
other words, it is recognised that it is a problem of germinal rather
than of embryonic pathology. It may be held (as Mingazzini argues,
Bibliotcca antropologico-Giuridica, 1 s., xxii., 1805, cited by Cunning-
ham, loc. cit.) that atavistic characters are latent and only appear
when a disturbing element comes into the ontogeny of the part, but
this does not fully explain their origin, it simply makes them
dependent on the presence of the tendency to be malformed. It
seems to me quite likely that when certain parts of the cerebro-spinal
system do not develop, not only may the parts which remain pursue
new ontogenetic paths, but other (and strange) structures may make'
their appearance ; that they are atavistic in the narrow phylogenetic
sense does not follow, for the plan of human ontogenesis may contain
traces of other and simpler plans without being derived from any of
them. The practical question is to find the cause which produces the
arrest of development and so permits such aberrant structures to
appear.

What lias been discovered regarding the teratogenesis of micro-

CRANIAL PERFORATIONS 369

cephaly, although so limited in amount and in exactness, has
seriously modified our views regarding its surgical treatment. If the
brain be the fons et origo of the whole malformation, it can obviously
be of little use to perform craniectomy in order to give more room
for the cerebrum to expand. In practice this conclusion is supported
by facts, for neither linear nor circular craniectomy, even when
accompanied by division of the dura mater, have been followed by
permanently good results. Surgery may yet devise means of
benefiting this condition ; but at present it is an antenatal problem,
and there is urgent need to discover what it is that arrests cerebral
development at the third month of intrauterine life and later.

Miscellaneous Malformations.

Cranial perforations of antenatal origin are occasionally met
with. I saw a case in the Edinburgh Maternity Hospital in 1902
(Case No. 297). The child was born at the full time, its general
development was good, and the weight was 5 lbs. 15 oz. The head
diameters were fairly normal (O.M. = 5i ; O.F.=4^ ; S.O.B., 4] to 3f).
In the right parietal bone nearer the sagittal suture than the parietal
eminence, but vertically above the latter, was a soft depressed area
about the size of the pulp of the middle finger ; posteriorly there
was an edge of firm bone, but superiorly and anteriorly it faded
gradually away into the surrounding bone. The area had a parch-
ment-like feeling, and became tense when the child cried. A similar
but smaller area was found in the same position on the left side, and
there were two other spots lying posterior to it. The posterior
fontanelle felt larger than usual. There was no history of syphilis
in the mother, who was a I-para. Similar cases of symmetrical and
congenital cranial perforations have been described by I). M. Greig
(Joum. Anat, and Physiol., xxvi. 187, 1892), Broca (Bull. Soc.
d'anthrop. de Paris, 2 s., x. 192, 320, 1875), Larrey (Bull. Acad, de
mM., xxxi. 448, 1865-66), Sir W. Turner (Edinb. Med, Joum., xi. 7,
133, 1865), and J. F. Goodhart (Trans. Path. Soc. Loud., xxxvii. 364,
1886). They may indicate persistence of the parietal foramina.
They do not always close after birth, for Greig's patient was
twenty-five years of age.

Porencephaly is that state in which there is a funnel-shaped
depression in the cerebral hemisphere leading down to and sometimes
opening into one of the ventricles. Externally it usually communi-
cates with the sub-arachnoid space. It may be associated with micro-
cephalus, hydrocephalus, asymmetry of the cranium, and symmetrical
flattening of the cranium. E. Sigmundt's inaugural dissertation
(Strassburg i. E., 1893) gives many useful facts and statistics regard-
ing this malformation, as does also B. Schattenberg's dissertation of
Marburg, published at Jena in 1889. Apparently it may be either
an antenatal condition or be acquired postnatally ; in the latter case
mental processes are not necessarily defective.

Absence of the corpus callosum (complete or partial) may occur with-
out any apparent intellectual disturbances : M. S. Germano (Cervelli
vol. ii. — 24

370 ANTENATAL PATHOLOGY AND HYGIENE

di uomo mancante del corpo calloso, Torino, 187-4) has met with such
a case, and others have been reported by Poterin-Dumotel {Gaz. d.
hdp., xxxvi. 47, 1863), T. 0. Ward (Loud. Med, Gaz., n.s., ii. 575,
1846), A. E. Urquhart {Brain, iii. 408, 1880-81), A. Bruce, with
literature lists {Rep. Lab. Roy. Coll. Rhys. Edinb., i. 70, 1889), and
L. A. Dunn {Guys Hosp. Rep., 3 s., xxxi. 117, 1889). Bruce thinks
that most of the cases can be explained by arrested development at
one time or another in antenatal life, and finds the chief cause in the
primitive falx cerebri and septa lucida. Absence, complete or partial,
of the cerebellum has also been observed, with or without other
malformations (H. Salter, Trans. Path. Soc. Load., iv. 31, 1852-53 ;
D. Fraser, Glasgow Med, Joura., xiii. 199, 1880; E. Fusari, Gazz. racd.
di Pavia, i. 241, 1892 ; A. P. Gould, Trans. Path. Soc. Lond., xxxiii.
6, 1881-82; Hitzig, Arch.f. Psychiat,, xv. 266, 1884).

The name inter -parietal or epactal bone is given to a small acces-
sory bony nucleus found in the sagittal suture near its posterior end ;
other intercalary bones lying further forward in the same suture
have been termed pre-interparietals. Wormian bones also occur in
the lambdoidal suture. They have been regarded by some as due to
arrested development, and by others as atavistic ; and it has been
stated that they are commoner in Peruvian skulls, hence the name
os Lncm given sometimes to the inter-parietal. Several authors have
written upon this subject {e.g., E, Virchow, Ztschr. f. Ethnol., xii. 1,
1880; G. Chiarugi, Atti. d. r. Accacl. di fisiocrit. di Siena, 4 s., i. 31,
1889 ; G. Sergi, Atti. Accad. med. di Roma, 2 s., ii. 57, 1883-86 ; E.
de Archangelis, Ann. di neurol., xiv. 153, 1896).

A third occipital condyle may occasionally be found : it lies
between the other two and in front of the foramen magnum, and
may articulate with the odontoid process. Sometimes there is only
an articular facette in this position. It is described by E. Ardu
{Giora. d. r. Accad. di med, di Torino, 3 s., xl. 408, 1892), who also
gives several references (Sergi, Tafani).

A more or less perfect canal may be met with in the new-born
infant (and even at later ages) leading from the sella turcica through
the sphenoid bone to the pharynx ; this is the cranio-pharyngecd canal,
a remnant of the diverticulum of the pituitary body (Landzert). A
median caned in the basi-occiput has also been noted, or, when it is
imperfect, a slight depression on the basilar part of the bone, called
the foveola pharyngea (Gruber). Earely, an accessory bone, called
the basiotic, is found between the occipital bone and the sphenoid:
P. Albrecht (Presse mkl. beige, xxxv. 121, 129, 137, 1883) described
this bone as he found it in cases of anencephaly, cyclopia, anencephaly
with cyclopia, and idiocy, as well as in otherwise normal crania ; the
bone has a bearing both upon the vertebrate theory of the skull and
upon the occasional presence of a canal in the basi-occiput. Minor
anomalies of the base of the skull (perforation of tegmen tympani,
defect of lesser wing of sphenoid, etc.) have been described by J.
Jamicke {Diss, inaug., Kiel, 1877).

Various deformities of the cranium result from premature closure
of one or several of the sutures. These are not always antenatal in

OMPHALOCEPHALY 371

origin, but some of them are, e.g. K. Forster's case of dolichocephaly
from synostosis of the sagittal suture (Jahrb. f. Kindcrh., vii. 66,
1864). Other types of the dolichocephalic or long head are spheno-
cephaly (wedge-shape, due to compensatory enlargement in the region
of the anterior fontanelle, the sagittal suture being ossified), lepto-
eephaly (narrow-shape, due to synostosis of frontal and sphenoid), and
clinocephaly (saddle-shape, due to synostosis of parietals with sphen-
oidal great wings or with the squamous temporals). The scapho-
cephaly; or boat-shaped head is long and narrow, and the region of
the sagittal suture is raised into a keel. There is marked shortness
(brachycephaly) when the transverse sutures (e.g. naso-sphenoidal) are
early closed : thus there is pachycephaly when there is synostosis
between the parietals and the occipital, oxycephaly (sugar-loaf head)
and acrocephaly (lofty-head) when there is compensatory growth at
the anterior fontanelle, platycephaly (fiat-head) when the temporals
are united with the parietals, trochocephaly (round-head) when the
frontals and parietals are partly fused near the middle of the coronal
suture, and plagiocephaly (wry-head) when the frontal and parietal
are united on one side. The small peaked head is called scillocephaly ;
trigonocephaly is a triangular head, and tapcisocephaly is a low head.
The relation of these malformations to degeneracy is discussed by
E. S. Talbot {Degeneracy, 1898).

Non-union of sutures may occur as well as premature union, and
so lead to cranial malformations; but cases of this kind are rare at
birth, for many of the sutures are normally open then. The frontal
(metopic) suture, however, may be widely open (mctopism), giving rise
to a wide membranous space in this region (vide G-. Vicarelli's case
in an infant born by the Csesarean section, Arch, di ostet. e ginec, ii.
643, 1895, with literature). I have met with an instance in a speci-
men of phocomely ; it is figured in the first section of this Manual,
Fig 51, p. 351.

Omphalocephaly.

There is a most interesting monstrosity, whose exact place in any
classificational system must remain doubtful, in which the head
seems to protrude from the umbilical opening, and the heart (single
or double) is seen on the nape of the neck or in the dorsal region.
Dareste (Production artificielle des monstruosite's, p. 359, 1891) is not
using exaggerated language when he says, " Ce type est l'un des plus
etranges que Ton puisse imaginer." It has not yet been met with in
mammals, but is relatively frequent in chicks artificially hatched.
Dareste noticed it in 1861, but did not then recognise its nature; in
1877 he gave an ingenious explanation of its teratogenesis (Compt.
rend. Acad. d. sc., lxxxiv. 1075, 1877), and he expanded his views
later (op. cit., p. 364, 1891). The anterior part of the intestine or
pharynx is absent, being represented solely by the anterior part of
the abdominal groove, so that there is not really an umbilical hernia
of the head, for the umbilicus can hardly be said to exist. The
anomaly appears to be due to strong inflection of the head (due to

372 ANTENATAL PATHOLOGY AND HYGIENE

pressure, perhaps from the shell), which prevents the union of the
prolongations forward of the lateral mesodermic folds; two hearts
are produced in the same way. S. Warynski (Thdse, Geneve, 1886)
made some interesting experiments upon the artificial production of
omphalocephaly and duplicity of the heart. E. Eabaud (Compt. rend.
Soe. de hiol., 10 s., iv. 327, 1897 ; Journ. de I'anat. et physiol., xxxiv.
247, 496, 545, 1898; These, Paris, 1898) has also investigated the
monstrosity, which he regards as due to the nervous system plunging
towards the endoderm and enveloping itself in it. It is secondarily
surrounded by the alimentary canal. The principal vessels and the
notochord follow the nervous system. In a later paper, Eabaud
(Journ. de I'anat. et physiol., xxxvi. 619, 1900) has described two new
conditions allied to omphalocephaly, and proposes to mal<e a new
group for them all, calling them enenterians, i.e. cases in which some
part of the embryo is abnormally lodged in the alimentary canal,
either anteriorly or posteriorly. When the lodgment is anterior, it is
omphalocephaly ; to posterior displacement Eabaud gives the name
ourcntery ; when the notochord is elongated in the direction of the
alimentary canal and in part clothed by it, the resulting monstrosity
is called cordentery. They all are most curious teratological develop-
ments, and they may perhaps be found to have their homologues in
some of the human amorphous placental parasites or allantoido-
angiopagous twins.

CHAPTER XXI

Merosomatous Terata (runt.) : Malformations of the Face and Month ; Genal
Fissures; Definition, Synonyms, Clinical Details, Morbid Anatomy,
Varieties (Fronto-niaxillarv, Intermaxillary, Anomalous), Teratogenesis,
Comparative Teratology : Median Fissure of the Face, of Upper Lip, of
Nose, of Lower Lip: Hare-lip; Definition, Synonyms, Clinical History,
Morbid Anatomy, Varieties (Unilateral, Bilateral, Simple, Complicated,
Fissure of Jaw, Cleft Palate), other Associated Malformations, Comparative
Teratology, Teratogenesis, Treatment : Fistula of Lower Lip : Fissure of the
Tongue: Miscellaneous Malformations of the Face (Asymmetry, Brachy-
rhyiiclnis, Cranium Progenium, Prognathism) and Mouth (Synchelia,
Synechia1, Tongue-Tie, Microstomia, Microglossia, Distomns, Macrostomia,
Achelia, Brachychelia, Microchelia, Macrochilia, Dicheilns, Macroglossia,
Congenital Teeth).

Having now passed in review the malformations of the Spine (Kachi-
terata) and of the Cranium (Cephalo-terata), we may proceed to the
description of those of the Face (Prosopo-terata). This is a large and
somewhat miscellaneous group of monstrosities and anomalies, for it
includes not only those of the face proper but also those of the organs
of special sense, the mouth, nose, eyes, and ears. If the reader will
also keep in mind the complexity of the ontogenetic arrangements and
rearrangements by which the framework of the face is constructed,
how processes of the most various forms approach towards or recede
from each other, coalescing, disappearing, and transforming in
kaleidoscopic fashion, he will have little difficulty is realising the
almost infinite variety of teratological formations which are to be
met with in this region of the body. At the same time there is in
the midst of this complexity an unexpected simplicity in the mode of
origin of many of the prosopo-terata, for several of them can be
evidently ascribed to the want of union between contiguous parts, to
the persistence of embryonic clefts, or to the delayed arrival of
certain processes and projections.

I have had some difficulty in selecting from so many malforma-
tions the most representative major types for special description.
Thus with regard to the face proper and the mouth, hare-lip suggests
itself as the standard type ; but it is so fully explained and its
characters are so fully made known in so many surgical and medical
works that I have chosen rather to describe in some detail several
malformations, including fissures of the cheek, of the face and nose, as
well as of the lip and palate.

373

374 ANTENATAL PATHOLOGY AND HYGIENE

Genal Fissures.

A genal cleft or fissure of the cheek may be defined as a
malformation of the lateral part of the face consisting in a cleft
affecting the soft parts, or more rarely invading also the bones,
arising during the embryonic period of antenatal life, and caused by
delayed or irregular closure of the fissures between the fronto-nasal
and superior maxillary and the mandibular processes.

It has also been termed "lateral fissure of the face," and this
phrase has been given a classic form by Taruffi (op. cit., vi. 338, 1891)
in the somewhat unwieldy word pleuro-prosoposchisis. It is a much
less common malformation than hare-lip; but Taruffi (loc, cit., p. 339)
has gathered together from medical literature nearly ninety cases,
while in my 325 specimens there were three instances of lateral
genal fissures (Nob. 23, 299, and 300, vide Figs. 25, 26, and 30).

Being a localised malformation, it has no characteristic antenatal
clinical history, and any peculiarities in the pregnancy of the mother
are with greater probability ascribed to the associated malformations,
such as amniotic adhesions and hydranmios, than to the facial fissure.
It is noteworthy, however, that it resembles hare-lip and differs from
many other monstrosities in being much commoner in male than
in female foetuses : two out of my three cases were males, and in
Taruffi's list of sixty-seven instances (in which the sex was stated)
there were forty-one males and twenty-six females. The malforma-
tion does not necessarily prevent postnatal life unless it be com-
plicated with some more serious anomaly (e.g. anencephaly), and it
can very often be surgically dealt with, and improved, even if it be
not quite cured. Before operation it is one of the most disfiguring
anomalies in the whole range of Teratology, and fully justifies such
epithets as " horrible " and " disgusting." In the long list of recorded
cases which have been published, the following may be named as of
special interest : J. Muraltus (Acad. nat. curios. Ephem., cent, iii.-iv.,
304, 1715); C. Mcati (Be labii leporini congenita natura ct originc,
1822); V. Laroche (These, Paris, 1823); E. Eemacly (Diss, inaug.,
Bonn, 1864) ; M. Pelvet (Compt. rend. Soc. dc biol., 3 s, v. 18.1, 1864) ;
H. C. L. Barkow (Beitrcige zur patholog ischen Entwickelungsgcschiclrfe,
iv. Abth., 8, Breslau, 1871); J. Euppersberg (Diss, inaug., Marburg,
1872); C. F. Fritzsche (Beitrcige zur Statistik . . . der angebornen
Missbildungen des Gesichts, Zurich, 1878) ; J. H. Morgan (Broc. Roy.
Med.-Chir. Soc. Bond., ix. 94, 1882); E. Morian (Arch./, klin. Chir.,
xxxv. 245, 1887); J. Dreier (Arch. f. klin. Chir., xxxviii. 269,
1888-89); 0. Niickel (Diss, inaug., Marburg, 1891); W. Bittner
(Brag. vied. Wchnschr., xx. 440, 1895).

The morbid anatomy of these fissures of the cheek in many details
is explicable by a glance at the embryology of the face, but in some
points it shows puzzling divergences. We can, therefore, arrange
all the cases in three chief groups : in one the fissure follows the line
of the space between the naso-frontal process and that of the superior
maxilla (vide pp. 34, 41); in a second it corresponds to the space
between the superior and inferior maxillary processes ; while in a

GENAL FISSURES 375

third it has a direction which resembles none of the known embryonic
facial clefts. Let us look briefly at each of these varieties.

(a) The fr onto -maxillary or ascending fissure of the face has a
general direction from the upper lip upwards towards the eye ; it has
also been called the " oblique " fissure of the face and " prosopo-ano-
schisis " (Tarum). In most instances it begins in the upper lip in a
somewhat wide gap with red margins and floor, and passes upwards
just outside the nose (in the naso-genal fold), to end in or near the
lower eyelid of the same side. It may be unilateral or bilateral, and,
except near its origin and termination, its walls are generally close
together ; it may even be cicatrix-like (antenatal cure ?). In other
instances it begins inferiorly in the ordinary position of hare-lip
(inter-incisor) and extends up into the nasal cavity of one side, and
so on to the inner angle of the eye, leaving in this case part of the
ala of the nose external to it. This form, also, may be unilateral or
bilateral ; and both forms, when unilateral, would seem to show a
preference for the left side. When bilateral, the fissures may be
symmetrical ; but more commonly they differ from each other. One
may be short and wide and be limited to the tissues of the upper lip,
while the other is narrow and is prolonged up to the eye and may
even be continuous with a fissure in the lower eyelid ; all intermediate
degrees of extension may be met with. Again, there may be an
ascending facial fissure on one side, and, on the other, a cleft running
outwards from the angle of the mouth. In a few cases the fissure
is prolonged beyond the eye into the temporal region, and sometimes
there is a cleft of the upper eyelid as well as of the lower.

In most of the reported cases, the cleft affects the bones of the
face as well as the soft parts ; and it may pursue two courses in
agreement with the varieties of direction shown by the cutaneous
defect. It may, for instance, affect the alveolar margin of the upper
jaw in the position corresponding to the union of the external part
of the intermaxilla and the superior maxilla ; then all the incisor
teeth-germs are internal to the fissure, and the canine tooth-germ is
external. Occasionally, what has been called a pre-canine incisor lies
external to the cleft, an occurrence which cannot be satisfactorily
explained by embryological processes. In other cases the cleft
begins, as in ordinary hare-lip, in a space between the internal and
external parts of the intermaxilla (between the inner and outer
incisor tooth) ; now the inner part of the intermaxilla, the vomer, the
nasal bone, the lachrymal, the ethmoid, and the inferior turbinate are
all internal to the fissure, while the outer part of the intermaxilla,
the superior maxilla, and the palatal process lie external. All
possible grades of osseous defect may be met with, from a small crack
in the alveolar margin of the jaw to a wide gap in the bone, passing
through into the mouth and being continuous with a cleft in the
palate. In the grave forms some parts may be actually absent, such
as the intermaxilla or a portion of it.

In association with this variety of facial fissure we may meet with
such anomalies as absence of one or both eyeballs, or cryptophthalmus,
coloboma of eyelid, or amniotic attachments to the eye ; there may

376 ANTENATAL PATHOLOGY AND HYGIENE

also be encephaloceles of various kinds, anencephaly, hydrocephalus,
and the like.

(b) The intermaxillary or commissural fissure of the face starts
at the angle of the mouth and pursues an outward and upward
course, which brings it to the temporal region or near the lobule
of the ear. Various names have been given to it, such as hiatus
buccalis, genal coloboma or hare-lip, commissural hare -lip, and
macrostoma. Taruffi's term ("gonio-cheiloschisis"), although etymo-
logically correct, is unfamiliar. The fissure may be bilateral or
unilateral; and it is more often left-sided, when it is unilateral. It
increases, in a disfiguring manner, the width of the mouth, and
extends towards the lower end of the ear in a curve having its
convexity external. When the fissure is bilateral the mouth may
be equally and symmetrically increased ; but more often there is
asymmetry, and the cleft may reach further back on the one side
than on the other (F. liynd, Dublin Quart. Journ. Med. Sc, xxxii. 45,
1861). The margins of the fissure are usually red, and, during life,
the saliva flows out freely, excoriating the skin ; Stenon's duct may
be seen ; if the individual survive birth the eruption of the teeth
increases the deformity, and lactation, mastication, and articulation
are rendered difficult. The bones of the face are not necessarily
affected, but, if the fissure be directed more upwards towards the
temple, the zygoma may be divided.

Associated malformations are fairly frequent. The commissural
fissure may, for instance, be complicated by a fronto-maxillary one
either on the opposite or on the same side of the face. Deformities
of the external ear have been met with, as well as preauricular
appendages. Less common is cleft palate ; microcephaly, anencephaly,
and exencephaly occasionally occur ; as do also amniotic adhesions.
In Fel vet's patient (loc. cit.) the upper jaw showed hypertrophy of
its alveolar border, while the mandible was twisted and had a mal-
formed condyle on the affected side.

(c) Anomalous fissures of the cheek are those which do not
reproduce, in their direction and relations, any of the embryonic
facial clefts. For instance, a fissure may begin at the angle of the
mouth and pass upwards towards the outer angle of the eye, ending
in the lower eyelid there; it may be associated with a defect in the
bone stretching up between the germs of the canine and first molar
teeth towards the infra-orbital foramen. Again, the fissure may
begin midwTay between the outer angle of the mouth and the philtrum
of the upper lip, and pass to a point in the lower eyelid between the
outer and inner angles of the eye. No satisfactory explanation of
these anomalous fissures is forthcoming, unless wc accept the notion
that the attachment of the amnion may cause a fissure at any spot
whether there exists a natural embryonic fissure there or not.

It is unnecessary to enter in detail into the teratogenesis of
fissures of the cheek. They are evidently arrested developments,
and it is clear that, in some cases at least (as, for instance, in my
three specimens), the arrest is clue to amniotic pressure or adhesion.
The embryonic clefts of the face do not close, and the fissures result.

MEDIAN FACIAL FISSURE 377

It is true that the anomalous fissures do not thus meet with an
adequate explanation; but it must never be forgotten that the arrest
of one part allows other and contiguous parts to develop in new and
unlooked-for directions, and thus irregularities may be introduced
which, it' we knew all the facts, would in the end support rather
than weaken the theory. It is noteworthy that neither the fronto-
maxillary nor the intermaxillary fissure is a common malformation
in mammals other than man ; when, however, they do occur they are
apt to be more extensive, and have been known to open into the
Eustachian tube and tympanic cavity, and to be associated with
absence of the parotid gland and curious displacements of the upper
and lower jaws (e.g. in schistocephalus retrotiexus, Gurlt, op. cit., 1877,
p. 21). Perhaps the greater frequency of genal fissures in the human
subject may be due to a peculiarity in the mode of development of
the amnion in his case.

Median Fissure of the Face.

Early writers described the ordinary form of hare-lip as if it
were median in position ; then it was pointed out that it occurred
laterally, at one or other side of the philtrum of the upper lip, and
it was thought that true median fissures never were met with;
finally, we now know that, although rare, clefts in the middle line,
affecting the upper lip, the nose, and the lower lip, have from time
to time been reported. These three are grouped together by Taruffi
(op. cit, vi. 288, 1891) under the one heading " meso-prosoposchisis."

(a) Median fissure of the upper Lip (Taruffi's name, "meso-
epicheiloschisis," is altogether too cumbrous) is, in one sense, more
truly a hare-lip than is the ordinary form of that defect, for the
natural labial cleft in animals (hare, rabbit, sheep, some dogs) is
mesial and not lateral in position. It is rare, however, in the human
subject, and is not to be confused with the median loss of lip-tissue
(" achelia ") which is found in a case of ordinary bilateral hare-lip
with absence of the structures of the intermediate philtrum. It
consists rather in a triangular notch in the philtrum, or in a shortness
of that part of the lip, so that it has a curved border. Sometimes
there is a vertical groove or furrow in the philtrum, and, coexisting
with it, a sulcus in the intermaxillary bone and palate. Sometimes
there is an actual fissure dividing the whole substance of the lip
in the position of the philtrum; the fissure then has the same
characters as the fronto-maxillary one was found to have; the inter-
maxilla may, at the same time, be grooved, fissured, or incomplete.
Cases are recorded in which both the philtrum and the intermaxilla
were entirely wanting, but these approximate so closely to the
monstrosity termed " cebocephalus " that I shall consider them under
that heading (vide p. 405). Flattening of the nose may be noted ; but
grave defects of that organ, of the brain, and of the deep parts of the
face (the ethmoid, palate) are generally found only in the advanced
degrees of median fissure which almost come under the designation
of " cebocephalus." Associated malformations of a less severe type

378 ANTENATAL PATHOLOGY AND HYGIENE

are preauricular appendages, hypertrophy of the lower lip (macro-
chclia), Polydactyly, and shortness of the limbs. A congenital fistula
of the frsenum of the upper lip has been noted (Lannelongue et
Menard, Affections congenital*, i. 26-1, 1891); it perhaps indicated a
partly cured fissure. Median fissure of the upper lip would appear
to be less common (as a malformation) in animals than in man.

(b) Median fissure of the JVosc ('• meso-rhinoschisis," Taruffi) is
rarer than median fissure of the upper lip, but is rather less rare
in some of the mammals than in man. There is a groove or sulcus,
or simply a cicatrix (antenatal cure?), in the middle line of the
dorsum of the nose, which may affect the whole organ or only a part
of it, and may be shallow or deep ; it may be restricted to the nose
(J. Poland, Pediatrics, i. 101, 1896), or extend on to the forehead, or
be continuous inferiorly with a hare-lip. Two illustrative instances,
with some references to the literature of the subject, are given by
D. Nasse (Arch. f. klin. Chir., xlix. 767, 1895); and some years ago
Dr. Paul Liebrecht of Liege sent me a reprint of his article on a
case of bifid nose with median (not lateral) fissure of the upper lip
(Difformite conge'nitale du nez, Bruxelles, 1 876). Sometimes the fissure
is so deep that the nose is divided into two parts, which may lie side by
side and be in every way symmetrical, or may be separated from each
other and unlike in appearance. Tarutfi's case (oj). cit., vi. 511, 1891)
was particularly interesting : the nose consisted of two longitudinal
halves, of which the left was fairly normal in appearance, but the
right constituted a mobile tube or trunk (like the proboscis so often
found in cyclopia, q.v.), with a circular depression in its free end and
a small opening at the bottom of it ; there were two palpebral
colobomata in connection with the right eye. A still deeper fissure
of the nose may be met with in which the whole face is split
(schistocejyhalus bifid us, Gurlt ; meso-cheilo-rliinoschisis). On the other
hand, the nasal groove may be both short and shallow, and at its
upper end there may be seen a little fistulous opening (J. Hoppe,
Med. Ztej., n.F., ii. 161, 1859). The coexistence of a median fistula of
the nose and a median fissure has suggested to Lannelongue and
Menard (Affections congdnitales, i. 372, 1891) the question whether
when the fistula occurs alone it represents a fissure which has nearly
quite closed. It may be noted that in cases of dermoid cysts of the
nose it is not uncommon to find a fistula leading down to the little
tumour ; it is easy to imagine a fissure which closes, including, as it
does so, some dermal elements and so produces the dermoid, while
the communication with the surface is kept open by the secretion.

With regard to the production of the fissure itself, various
opinions have been expressed. The wide separation of the two '
halves of the nose has been ascribed to the action of an amniotic
band or adhesion, and such a case as that figured by Lannelongue
and Menard (op. cit., p. 371, fig. 33) proves this to the degree of
demonstration. On the other hand, some observations would seem
to show that a mesial meningocele (0. Witzel, Arch. f. klin. Chir.,
xxvii. 893, 1882-83), or a tumour of one kind or another, may prevent
the natural union of the two halves of the nose. The more shallow

HARE-LIP 379

grooves have been regarded as arrested developments produced per-
haps by amniotic pressure, perhaps by excessive thickness of the
nasal septum.

This malformation is not to be confused with duplicity of the
nose, an instance of which, reported by Regnault {Bull. Soc. d'anthrop.
de Paris, 5 s., ii. 333, 1901), was referred to in Chapter XII. (p. 233).
In these cases, parts of two faces are present, while in the subject
now under discussion there is less than one face. At the same
time it may be very difficult from a superficial examination to classify
correctly all cases.

Median fissure of the nose occurs among the lower animals, as a
" normal deformity," so to say, in some dogs {e.g. pointers aud setters),
and as a true teratological state in other mammals. Gurlt {Ueber
thierische Missgeburten, 18, 1877) has subdivided the cases into two
groups, schistocephalus bifid us and s. semibifidus ; the second of these
contains the rarer specimens, but they more closely resemble what is
found in the human subject.

(c) Median fissure of the lower Lip (" meso-hypocheiloschisis,"
Taruffi), little known before the nineteenth century, is even now a
rare occurrence. Sufficient observations have been made, however,
to show that it may exist in all grades from a slight notch in the
lower lip in the median line to a large and complete fissure extending
down through the lower jaw in the position of the symphysis. It
has been known to occur in a family in which there were several
instances of ordinary hare-lip (E. Hamilton, Dublin Joum. Med. >S'c,
lxxii. 1, 1881) ; and its presence may lead to constant loss of saliva
and resulting bad health. Bouisson {Diet, encycl. d. sc. med., viii. 640,
1868) has noted a case in which a slight antero-posterior groove in
the lower lip was the only anomaly ; Parise {Bull. g&n. de therecp.,
lxiii. 269, 1862), on the other hand, has seen a median labial fissure
associated with division of the lower jaw and a bifid state of the
tongue, and continued as a cicatrix to the level of the sternum. In
one of Hamilton's cases {loc. cit.) there was a cleft palate and an
ordinary double hare-lip in addition to the median fissure of the lower
lip ; at each side of the fissure was a small nodule in the lower lip,
and when the mouth was shut these filled up the deficiencies in the
upper lip. Other associated malformations have been club-foot, and
tumours (osteomata, teratomata) in the middle line of the lower
jaw ; and to the latter some importance has been ascribed as tera-
togenic agencies in the production of the fissure. The anomaly has
been met with in the lower animals, both in mammals and birds, and
has generally been complicated with division of the tongue.

All these three varieties of median fissure (of upper and lower lips
and of nose) have been successfully treated by the surgeon ; but the
cases associated with deep-seated osseous defects of the jaws are not
hopeful.

Labium Leporinum (Hare-Lip).

Ordinary hare-lip (labium leporinum, lateral nasal fissure, lateral
labial fissure, cheiloschisis, pleuro-epicheiloschisis) is so fully described

380 ANTENATAL PATHOLOGY AND HYGIENE

in works upon Surgery that I do not enter into many details here,
but from the standpoint of Teratology it is necessary to refer to
certain matters in connection with it.
• It is a malformation which has been known from very early times,
it has a large literature, and lias long engaged the attention of the
surgeon and exercised his skill in devising reparative and plastic
operations. It is also a frequent malformation, and is apt to occur
in several members of the same family (family prevalence). Many
instances of this phenomenon have been recorded: Mercer Adam
{Month. Journ. Med, Sc., Edinburgh, 3 s., ix. 402, 1854) met with the
case of a woman whose five children all had hare-lip ; and Meckel
(Handbuch der pathologischen Anatomic, i. 19, 1812) refers to Anna's
observation of a man who had by his first wife eleven children, nine
of whom were born dead, and two alive, with hare-lip, and by his
second wife four children, two of whom had the same deformity and
one had cleft palate. Direct heredity, however, may also be noted,
as in E. GT. H. Butcher's case (Dublin Journ. Med. Sc., lxiii. 426,
1877) in which both mother and child had double complicated hare-
lip, with entire absence of the palate. C. J. Trew, also, met with a
man whose palate showed a cicatrix ; by his wife, who was normally
formed, he had seven children, three females had hare-lip and cleft
palate, while two boys and two other girls were normal (Nova Acta
phys.-med. Acad. not. curios., i. 445, 1757). Hare-lip seems to have
a preference for the male sex, for if the statistics of T. Bryant (Guy's
Hosp. Rep., 3 s., vii. 3, 1861), C. F. Fritzsche (Ziirich, 1878), and E.
Herrmann (Diss, inaug., Breslau, 1884) be put together we find 180
males and 118 females, or 604:05 per cent, and 39-595 per cent,
respectively.

Morbid A natoun/. — Hare-lip may be simple or complicated, uni-
lateral or bilateral. Simple hare-lip, when unilateral, is generally on
the left side. The fissure passes from the free edge of the upper lip
just outside the philtrum towards the nostril of the same side. It
varies within wide limits as to its extent : it may reach quite to
the opening of the nares and constitute a gaping triangular space
with the apex upwards ; it may stretch as a narrow fissure involving
half the thickness of the lip and reaching half-way to the nose ; or it
may be a mere notch in the edge of the lip. Indeed, in some cases
it may be only a shallow sulcus or cicatrix, and in these instances
it is supposed that there has been antenatal cure of the deformity ;
cases of this kind were reported by Chauviu (Rev. incd. franc, et
c/rau;/., ii. 226, 1838), Cuthbert (Trans. Edinb. Obst. Soc., i. 51, 1870),
M. Bartels (Arch./. Anal., Physiol, u. wissensch, Med., 595, 1872), P.
Seiler (Diss, inaug., Berlin, 1882),Lannelongue and Menard (Affections
conginitales, i. 273, 1891), and several others. It is not, however,
quite clear that this is the real explanation of the anomaly, for no
cicatricial tissue has been found ; it may be simply a less complete
defect of the lip (involving, for example, only the muscular tissue),
and the occurrence of a case of fissure on one side and cicatrix-likc
groove on the other may be regarded as supporting this idea (Fritzsche,
op. cit.). The fissure extends obliquely across the lip to the nostril,

HARE-LIP 381

partly on account of muscular action which draws the outer edge
outwards ; the edges are covered with mucous membrane, which may
show streaks and slight furrows (cicatrices ?), and the inner edge is
shorter and thicker than the outer; and it (the fissure) usually corre-
sponds topographically to the space in the upper jaw between the
internal and external incisor tooth. No case seems to have been put
on record of a fissure affecting only the upper part of the upper lip
and leaving the margin united, and from this occurrence it has been
concluded that the natural fissures of the face close from above down-
wards. The philtrum may lose its natural concavity even in the
unilateral variety of hare-lip ; it does so very completely in the
bilateral form, for it is then usually convex. One nostril may be
flattened, and the nasal septum may show a convexity affecting the
opposite side from that on which the fissure exists.

The bilateral form of simple hare-lip is rare, for when there is a
double fissure the alveolar border of the maxilla is commonly affected.
When it does occur, the fissures have appearances such as have been
already described. Of course there is now an isolated median portion
of the upper lip (median tubercle or lobule), which is somewhat
stunted and leaves the incisor teeth exposed (in postnatal life), and
is attached either to the gum or to the nasal septum. In some cases
the median tubercle may be so reduced in size as to give to the upper
lip the appearance of a median fissure. Its histological characters
have been investigated, and it has been found to consist of skin (with
hairs and sebaceous and sudoriparous glands), connective tissue with
numerous tortuous capillaries, the naso-labial muscle, and a mucous
membrane in which there are no glands (Lannelongue and Menard,
op. cit., p. 271). To bilateral hare-lip Taruffi gives the name dipleuro-
epicheiloschisis.

Hare-lip complicated with fissure of the alveolar arch of the
upper jaw (cheilo-gnathoschisis) may be unilateral or bilateral. It was
formerly maintained that the fissure in the bone corresponded to the
suture between the premaxilla and the superior maxilla, and that
therefore the incisor teeth (after eruption had taken place) lay in-
ternal to it ; but it is now known that most often the cleft runs
through the premaxilla and separates the central from the lateral
incisor tooth. The fissure, therefore, corresponds to the interincisor
suture ; which probably indicates that each premaxilla is developed
from two ossific nuclei lying side by side. The fact that this is the
commonest position for the cleft was established by P. Albrecht
(Centrlbl. f. Chir., xi. 521, 1884; Deutsche Ztschr. f. CMr., xxi. 201,
1884 ; etc.), and although some cases have been reported which cannot
be satisfactorily explained by the theory associated with it, yet it
must be admitted to be a correct observation. For the fissure to run
between the premaxilla and the superior maxilla, outside the region
of the incisor teeth, is quite exceptional. Sometimes a super-
numerary incisor tooth is met with (a precanine incisor), due prob-
ably to cleavage of the dental germ of the lateral incisor ; this may
explain some of the anomalous cases. As a rule, however, the two
central incisor germs are in the central bone between the two fissures

382 ANTENATAL PATHOLOGY AND HYGIENE

(when the hare-lip is bilateral), and one lateral incisor germ is outside
the fissure on each side. Through the presence of the bilateral
fissures (which usually stretch back to the foramen incisivum) the
premaxillary bones are displaced forwards and form part of the
median tubercle. Cleft palate usually but not constantly accompanies
fissure of the alveolar arch. Exceptionally the mucous membrane
may be continuous while the underlying alveolus is split.

Cleft palate (palatoschisis) constitutes one of the malformations
which, when associated with labial fissure, constitutes complicated
hare-lip. It is true that cleft palate may occur alone, but, when it
does so, it shows the same characters as when combined with hare-lip.
It exists in various grades. It may, for instance, consist in a bifid
state of the uvula, in a fissure of the soft palate alone, or in different
degrees of cleavage of the osseous palate and of the mucous membrane
which covers it. In its most marked form and when combined with
double hare-lip and fissure of the alveolar arch it constitutes one of
the most disfiguring of all the teratological states ; there is then a
mesial gap in the roof of the mouth, producing a bucco-nasal cloaca,
hanging down into which is the more or less altered vomer, and
projecting into the sides of which are the inferior turbinated
bones. It may also be unilateral, and then the fissure runs along
by the side of the middle line, and the vomer is attached to the
unaffected side of the palate. Sometimes, while there is a bilateral
fissure, there is asymmetry, the deformity being more extensive
on the one side than on the other. In a few rare cases the osseous
fissure has been covered with intact mucous membrane, or with
cicatricial tissue ; and in one or two instances simply a slight de-
pression or groove may be found in the palate, which has been
interpretated as indicating antenatal cure (H. Chretien, TJrise, Paris,
1873). It has to be borne in mind that in well-marked cases the
defect is not restricted to the actual palate (hard and soft), but may
extend to the nasal septum and neighbouring parts, which may be
longer or shorter or of less or greater dimensions vertically ; thus it
happens that surgical reparative operations may be partial failures,
for something more is needed than merely bringing separated parts
into contact. On the other hand, the cure of a hare-lip may be
followed by an improvement in the state of the cleft palate, showing
that Nature tends to make good deficiencies under improved conditions.
It is unnecessary, in this work, to do more than remind the reader
that grave disabilities attend the child born with a cleft palate and
hare-lip ; lactation and. later, articulation are seriously interfered
with.

Associated Malformations. — Hare-lip and cleft palate may be found
associated with almost every other type of monstrosity. They may,
for instance, complicate anencephaly and cyclopia, and in one of my
cases of anencephaly (Specimen No. 155) there was bilateral labial
and palatal fissure ; club-foot, cranial asymmetry, and hernias of
various kinds are common concomitants ; the rarer deformities
known as cebocephalus and trigonocephalus may also be associated;
and I have met with a case in which there was double hare-lip in

HAKE-LIP

383

Fig. 63.— Uromelic Sympodial Foetus with Double Hare-Lip. Specimen No. 250.

384 ANTENATAL PATHOLOGY AND HYGIENE

a uromelic sympodial foetus (Fig. 63, Specimen No. 250). Again,
hare-lip may be complicated by the presence of genal fissures,
by preauricular appendages, by deformities of the ears, and by
anomalies of the eyes. I have also seen a case of double fistula of
the lower lip (Case No. 221) and another of fcetal ascites (Specimen
No. 229).

Comparative Teratology.- — Hare-lip is somewhat rare in the mam-
malia (other than man) ; it is rarer in them than cleft palate.
Further, median and lateral fissures have not always been dis-
tinguished. Guilt (Lehrbuch der path. Anat. tier ffaus-Saugethiere,
ii. 130, 1832) gave to mammalian hare-lip the name schistocephaltts
fissilabrus, and to cleft palate that of schistocephalus fissipalatinus.
Cleft palate may not infrequently occur alone without labial fissure,
and it would appear to extend forward in the middle line between
the premaxillee and not between them and the superior maxillae or
between the two parts of each premaxilla as in man. As I have so
often pointed out, these peculiarities of Comparative Teratology must
have a meaning and may yet throw light both upon Human Terato-
logy and Human Embryology. It may be noted as an interesting
etiological suggestion that, in the case of lionesses in the London
Zoological Gardens, the absence of bones from the food seemed to be
associated with the birth of young lions with cleft palates.

Teratogenesis. — The notion of maternal impressions and of the
action of the tongue of the fetus and of its nails as factors in the pro-
duction of hare-lip and cleft palate need not lie considered. Further,
the presence of a tumour (teratomatous or other) in the fissure and
possibly producing it, is so exceptional an occurrence that it cannot
be utilised for the explanation of cleft palate in general. Nearly
every one is prepared to admit that these fissures are the lesult of
arrested development, even if all are by no means at one as to
the precise mechanism by which the arrest is initiated. There is
strikingly little evidence that in this particular instance amniotic
bands are effective in teratogenesis ; they are notably so in the genal
fissures (fronto-maxillary, etc.), but are not seen in hare-lip. It is
still open to us to suppose that amniotic pressure may be the arrest-
ing cause ; but it must be admitted that very little light is forth-
coming on this point. Further, although it must be admitted that
the fissure is the result of arrested development, many difficulties
connected with the ontogenesis of the face present themselves;
indeed, the whole question of the nature, origin, and relations of the
premaxilhe or intermaxillary bones is bound up with this terato-
logical problem. Until about twenty years ago it was generally
believed that the fissures in hare-lip indicated a persistence of the'
original divisions between the maxillary processes and the side of the
fronto-nasal process ; and there was a most satisfactory simplicity
about the explanation which was thus apparently established.
Apparently simple explanations of matters embryological and tera-
tological have, however, on more occasions than one turned out to be
fallacious, and it was so with hare-lip. It was pointed out by P.
Albrecht that the fissure in the upper jaw (in hare-lip) did not mark

HARE-LIP 385

off the intermaxilla (or premaxilla) from the superior maxilla as had
been supposed, but passed right through each premaxilla dividing it
into two parts, the inner of which was united with its fellow of the
opposite side to form the osseous part of the median tubercle, while
the outer was conjoined with the superior maxilla. The central
incisor tooth wits commonly carried by the inner half of the pre-
maxilla and the outer incisor by the outer half, so that when the
bilateral fissure was present there were two incisors attached to the
median tubercle and one to each side of the jaw outside the fissures.
The fissure then marked the division between the outer and inner part
of the premaxilla, and it was to be considered that there were in the
human subject not two but four premaxilhe. The cleft in tire bone
indicated not the embryonic fissure between the fron to-nasal process
and the superior maxillary process, but the space between the
internal and external nasal processes of the fronto-nasal process ; it
was a cleft entirely within the fronto-nasal process. The median
tubercle in hare-lip, therefore, represented not the whole of the two
premaxillse but only their inner parts. It cannot, however, be stated
that this question is settled. Many difficulties stand in the way,
most of which are connected with the number and arrangement of
the incisor teeth, which are cut in connection with hare-lip. I think
too much has been made of the morphological significance of these
teeth both in the arguments for and in those against Albrecht's
views. After all, it is not to my mind an unthinkable theory that
the cause, which arrests the development of the face and makes
permanent what ought to be a transitory arrangement of parts, may
also act upon the dental germs in the jaw and lead one germ to
atrophy and another to increase in size and perhaps divide into two.
The facts of embryology must first be thoroughly investigated
(teratological developments being utilised as hints to direct research),
and then the general principles of teratogenesis must be applied to
the scrutiny of the results ; if this be done, I feel sure that the actual
mode of production of hare-lip and all other malformations will be
made plain. Of course this does not mean that the cause which
leads to the arrested developments will be discovered, although we
may be in an infinitely better position to make surmises regarding
its nature ; we shall then, I expect, find we are dealing with the
germinal factor.

Treatment. — As I have pointed out several times already, this
work is not a Manual of Surgery but of Antenatal Pathology and
Hygiene, and therefore it does not fall within its sphere to discuss the
various operative procedures to correct deformity. I may, however,
point out to the surgeons that Nature encourages them by her own
successful antenatal reparative essays, and that they will find it well
worth their while to study all that is known bearing upon the
mechanism of the production of the deformity. Especially ought
the associated anomalies in such neighbouring parts as the vomer,
the premaxilla, and the nasal chambers to be attentively investigated
before operation, and provided for during operation if the final result
is to be good. Since hare-lip and cleft palate have been repeatedly
vol, ii. — 25

386 ANTENATAL PATHOLOGY AND HYGIENE

shown to be hereditary, it may quite reasonably be asked whether
antenatal hygiene ought not to try to prevent their transmission;
possibly lime salts given to the mother may be efficacious ; possibly,
also, they may not. But trial may be made.

Fistulae of the Lower Lip.

I may here refer very briefly to a curious and almost inexplicable
malformation of the lower lip which may be met with sometimes in
connection with hare-lip, sometimes alone. I mean bilateral fistulas
of the lower lip. In February 1899, Mr. Cotterill kindly gave me an
opportunity of examining a case of this kind : it was that of a young
girl who had also hare-lip ; but the most noteworthy defect was not
in the upper but in the lower lip, where there were two fistula?,
symmetrically arranged one on each side of the middle line. The
appearance they presented was exactly like that produced by firmly
pressing the two central incisor teeth of the upper jaw against the
lower lip. It was stated that the girl's grandfather had exactly the
same malformation.

The first case of this peculiar malformation to be reported seems
to have been that of J. N. Demarquay (Gaz. mkl. de Par., 2 s., xiii.
52, 1845) ; in it the fistuke passed downwards under the mucous
membrane of the lip towards the fraenum. Since then a number of
instances have been put on record, including those by Beraud (Bull.
Soc. de cJiir. de Par., 2 s., ii. 233, 1862), J. J. Murray (Brit, and For.
M.-Chir. Rev., xxvi. 502, 1860), Eichet (Bull. Soc. de chir. de Par., 2 s.,
'ii. 230, 1862), Depanl (ibid,, p. 349), L. A. Banvier (Comyt. rend, Soc.
de Hoi., 3 s., iii. 93, 1862), Eose (Vcrliandl. d, Gesellsch. f. Geburtsh.
in Berlin, xxi. 82, 1869), U. Trelat (Journ. de mid. et chir. prat., xl.
442, 1869), Demarquay (Bull. Soc. de chir. de Par., 2 s., ix. Ill, 1869),
Lannelongue (Bull, et mem, Soc. de chir. de Par., n.s., v. 617, 1879),
T. Sympson (Brit. Med, Journ., ii. for 1882, 1145), H. H. Glutton
(Trans. Path. Soc. Bond., xxxviii. 446, 1887), Madelung (Arch, f. liin,
Chir., xxxvii. 270, 1888), 0. Zeller (" Ueber angeborene Unterlippen-
fisteln," Diss, inaug., Berlin, 1891), Lincoln de Castro (Gaz:. d, osp.,
No. 122, 1894), L. H. Miller (Med. Bee, xlix. 87, 1896).

Family prevalence as well as direct heredity have been specially
associated with this anomaly. In the case that I saw, the grand-
father of the patient had the same defect. Demarquay's subject had
four brothers and his mother similarly affected. Lincoln de Castro's
record was a most remarkable one : there were three individuals, a
brother and two sisters, who had each a double fistula in the lower
lip; the father was normal but the mother had a cleft palate;' a
seven months' foetus that lived fourteen days had the same condi-
tion of the lower lip; so had the maternal grandfather: and the
maternal grandmother had given birth to a foetus witli hare-lip
and incomplete closure of the thoracic and abdominal cavities.
It was truly a teratological family ! Murray reported four
cases in the same family, while several other members had hare-lip
alone.

FISSURE OF THE TONGUE 387

The cases closely resembled each other ; but in Lannelongue's
patient the fistulse opened into a single cavity, and in the instances
recorded by Trelat and Glutton there was in addition a fold of
mucous membrane dividing the lower lip into an anterior and a
posterior portion. The part of the lip bearing the fistulse may be
hypertrophied and project almost like a proboscis.

As has been pointed out already, the anomaly is often associated
with double hare-lip, and in its absence with cleft palate. Club-foot
is also common.

Since some fluid resembling saliva exudes commonly from the
fistulous tracts, surgical interference has been occasionally invoked
to remove the inconvenience resulting from this, generally with
success.

The teratogenesis of the condition is obscure. The idea of foetal
inflammation of one or more of the labial mucous glands is unsatis-
factory. The most probable view is that here we have an indication
of the presence of an intermaxillary bone in the lower jaw ; the
fistula.1, therefore, are arrested developments at a stage when onto-
genesis was nearly completed, and the anomaly falls into line with
the minor grades of hare-lip. Bland-Sutton (Tumours, p. 347,
1903) calls them " mandibular recesses," and ascribes them to faulty
coalescence of the intermandibular fissure.

Fissure of the Tongue (Schistoglossia).

Cases of median fissure of the tongue are on record, but they are
rare, and they almost always are overshadowed by the associated
malformations. G. Barling (Brit. Med. Jo urn., ii. for 1885, p. 1061),
for instance, saw a case in which there was a V-shaped cleft in the
anterior part of the tongue in association with cleft palate, and at the
apex of the V was a median lobe which was removed by the surgeon
to allow room for the tongue in the mouth. Another case in which
there was both cleft palate and defective development of the lower
jaw was reported by A. Brothers (Med. Rec, xxxiii. 109, 1888); and
yet another by G. 0. Beaudry (Union nu'd. du Canada, iv. 342, 1875).
In most of the other cases, e.g. Taruffi's anencephalic foetus with two
mouths (op. cit., vi. 527, 1891) and Windle's observation on a calf
(Journ. Anat. and Physiol., xxii. 432, 1888), there are signs of a deep-
seated duplicity affecting the whole head. The anomaly may be
ascribed to excessive development of the two lateral structures which
form the tongue, or to defective growth of the median part (tuber-
culum impar). When the three parts all develop but do not unite
together a trifid tongue may result, as in Gurlt's calf (Ucher thierische
Missgeburten, p. 20, pi. xii., fig. 56, 1877).

Miscellaneous Malformations of the Face.

I have considered with some degree of detail a number of facial
and buccal deformities, characterised by absence of union of
embryonic parts (e.g. fissures of cheek, mouth, palate, and fistuke of

388 ANTENATAL PATHOLOGY AND HYGIENE

lips) ; but there are still many other facial anomalies which I must
briefly refer to.

Facial Asymmetry of various kinds may be met with. I have
already referred to unilateral hypertrophy or atrophy of the face
(p. 204), but it must be borne in mind that the unilateral enlarge-
ment may be due to several different causes, including true hyper-
plasia, neoplasms (such as angiomata), and even teratomata. When
the last-named condition is present the asymmetry may be extreme.
Hemiatrophy of the face, often associated with congenital torticollis
(Lannelongue et Menard, Affections congdnitales, i. 498, 1891) may
occur in the foetal rather than in the embryonic period of antenatal
life, and be due to uterine or amniotic pressure ; there is usually
cranial asymmetry in addition. The resulting facial obliquity has
been termed prosopus varus. Facial asymmetry, however, may also
arise in the embryonic period : of the forms due to marked defect of
the lower jaw I shall speak in connection with otocephalus and
synotia; but I may briefly notice here the results of imperfect
development of the upper jaw. The name brachyrhynchus has been
given to those cases in which the upper maxilla? are short, the nose
and nasal cavities small, and the lower jaw projecting. It is more
common and more characteristic in the lower animals than in man.
It may be met with in birds, in fishes, and in mammals, and is indeed
a racial feature of certain kinds of dogs. In the human subject it is
most often associated with such extreme malformations as cyclopia
and cebocephalus (q.v.), but may occur alone ; and it is possible that
in some grave cases (as in that by W. Eoss, Trans. Obst. Soc. Lond.,
ix. 31, 1868) the superior maxillary bones may be almost absent. In
the deformity known as cranium progenium the appearances sug-
gest increased size of the lower jaw rather than diminished size of the
upper; but there are facts (L. Meyer, Arch.f. Psychiat., i. 334, 18G8)
which make it probable that it is small size of the basis cranii along
with defective growth of the upper jaw that, after all, is the deter-
mining factor.

Congenital Prognathism, although normal in some races of mankind
(African negro, etc.), is a malformation in orthognathous peoples ; the
projection of the jaws and the small size of the forehead are its striking
features. These various malformations are apt to be associated with
cranial anomalies, with microcephaly, nanocephaly, and the like, and
with mental defects when the individuals survive their infancy.

Miscellaneous Malformations of the Mouth.

Astomus, or complete absence of the mouth, will be considered
with aprosopus (in which nose and eyes as well as mouth are
wanting) ; but, at this point, reference may be made to synchrlia or
synchcilus, by which name is meant apparent absence of the mouth or
fusion of the lips. Congenital closure of the mouth due to synchelia
is an exceedingly rare malformation, and in this respect contrasts
strongly with the comparative frequency of anal imperforation. A
case was reported by A. E. Buchner {Acta Acad. nat. curios., ii. 210,

MICROSTOMIA AND MACROSTOMIA 389

obs. xcvi., 1730) in 1730 and described as " infans ore clause- et
concrete- natus " ; it was accompanied by a scholion dwelling on the
question of how this astomatous foetus had been nourished in utero.
Some other cases have been observed, including one by Taruffi (op.
cit., vi. 531, 1891), but generally in association with other defects.
When it occurs alone, surgical interference may prove quite success-
ful in establishing an opening. Combined with it may be various
buccal synechia, such as adhesions between the soft palate and
pharyngeal walls, or between the velum palati and the arch of the
palate, between the tongue and the floor of the mouth {tongue-tie) or
the roof of the mouth (ankyloglossia superior) or the gums, or between
the cheeks, lips, and gums. Union of the gums together is very rare
(Carter, in Garretson's System of Oral Surgery, p. 433, 1873). These
synechias may be malformations developed not in the embryonic but
in the foetal period of antenatal life.

Microstomia or microstomus is the condition in which the oral
aperture is very narrow from side to side, and the lips very small.
When the mouth is reduced to a small foramen or vertical fissure it
may be expected that grave defects of the mandible coexist with it.
It is rare for it to be met with apart from other malformations (W.
Duncan, Trans. Obst. Soc. Lond., xxxvii. 16, 1895); but a few cases
have been reported in which the associated anomalies were com-
paratively slight (F. A. von Amnion, Journ. d. Chir. u. Augenh., n.F.,
iii. 250, 1844). Microglossia may also be met with.

Distomus or " double mouth" is a very rare monstrosity in a foetus
otherwise single : it is, of course, commonly found in double terata.
Even when the foetus is not obviously double, the condition of dis-
tomus may, when fully investigated, show signs of duplicity in
neighbouring parts. This was the case in Tarufh's very remarkable
anencephalic foetus with sympodia (op. cit., vi. 528, 1891), in which
there was a very wide mouth and two lower jaws lying side by side,
the oral aperture was closed in the middle line. In 0. Israel's
specimen (Diss, inaug., Berlin, 1877) the second oral opening was at
the left side of the lower part of the face, where there was a super-
numerary ramus of a lower jaw and a rudimentary tongue; the left
ear was also malformed, but the child was otherwise normal. Such
cases occur in the lower animals, as in the sheep (K. von Kostanecki,
Arch. f. path. Anat., exxiii. 405, 1891).

Macrostomia or macrostomus is an unusual width of the mouth.
It has been already described under the head of commissural fissures
of the face, and is very rare apart from these defects. Taruffi (op.
cit., vi. 534, 1891) refers to a case by G. Friderici as an instance
of non-fissural macrostomus ; but an inspection of the original work
(Monstrum humanum rarissimum reeens in luccm editum, Lipsise,
1737) and a reference to the astonishing coloured plate which
accompanies it (which are in my library) has convinced me that it is
not an instance of large mouth but of open mouth (" os mire aper-
tum "), without a trace of lips (achclia). A famous historical example
of macrostomia was Margaret Maultasche, who married Prince John
of Bohemia in 1342.

390 ANTENATAL PATHOLOGY AND HYGIENE

Brachychelia or brachycheilus is the name given to anomalous
shortness of the lip, usually the upper. Of course this deformity is
often met with in connection with hare-lip when atrophy of the
philtrum is almost constant ; but it also occurs as the sole deformity.
J. Schenckius a Grafenberg (Obscrvationum Medicarum, lib. i. p. 225,
Francofurti, 1609), for instance, refers to the case of a woman (" honesta
matrona ") born with a short upper lip (" labio curto nata ") which
did not cover her teeth. Microchelia is a somewhat synonymous
term. Anomalies of the frsenum of the lip have been reported
(shortening, triple character, etc.).

Macrochclia or macrocheilus, enlargement of the lips, is a more
common deformity than brachychelia. Sometimes it is the upper
lip alone which is affected {ejri-macrochclia, Taruffi) and the tongue
may join in the enlargement {macroglossia) ; only one-half of the lip
may be large and there may then be unilateral hypertrophy of the
tongue (T. Hodgkin, Lectures on the Morbid Anatomy of the Serous
and Mucous Membranes, ii. 228, 1840). Other cases have been reported
by Blot {Bull. Soc. de chir. de Par., 3 s., ii. 332, 1873), Dolbeau and
Felizet {Bull. gen. de tlUrap., lxxxvii. 442, 1874), E. Martel {ibid.,
p. 545), Petrequin {Gazz. med. ital. lomb., 2 s., ii. 169, 1849), and
Lannelongue and Menard {Affections conge 'nitalcs, i. 615, 1891). I
have seen a case of unilateral hypertrophy of the tongue without
participation of the lip (in a man exhibited by Mr. Wallace at the
Edinburgh Medico-Chirurgical Society, November 11, 1903). Some-
times it is the lower lip alone which shows the congenital enlarge-
ment {liypo-macrochelia, Taruffi). G. Buck {Trans. Med. Soc. New
York, p. 171, 1872) has reported a case successfully treated by
operation ; and a hereditary and historical example is found in the
Hapsburg or Leopold lip, so characteristic of the descendants of
Maximilian I. of Austria and Mary of Burgundy. The Austrian
under-lip seems to have come to the Hapsburgs in this way :
Frederick ill., the father of Maximilian I., was the first of the Haps-
burg Kaisers to have it ; to quote from Carlyle {Frederick the Greed,
bk. iii. c. 4), " he got it from his mother and bequeathed it in a marked
manner ; his posterity to this day bearing traces of it ; mother's name
was Cimburgis (or Cymburga), a Polish princess, Duke of Masovia's
daughter," whose almost masculine strength was renowned. Where
she got it, I do not know. Cases in medical literature have been
reported by M. Eichler {Diss, inaug., Bonn, 1883), by Bloxam {Brit.
Med. Journ., i. for 1895, p. 814), and others. The anomaly may be of
the nature of a lymphangioma (Billroth), or of primary hypertrophy of
the connective tissue, or it maybe due to some defect in the development
of the first branchial arch. It may be associated with a mevus of the
skin of the neighbouring part of the face. An anomaly which is prob-
ably closely allied to macrochelia is dichcilus (Taruffi) or double lip ;
it is due to a fold of mucous membrane on the inner aspect of the
lip (upper or lower), which gives to it an appearance of duplicity.
Another malformation, with resemblances to both the preceding, is
macroglossia. This congenital hypertrophy of the tongue is in most
instances of the nature of a lymphangioma, and is usually bilateral,

CONGENITAL TEETH

39]

rarely unilateral. The enlarged organ protrudes from the mouth,
producing an appearance which led the early writers to call it " lingua
vituli." It has heen surgically treated hy the removal of a V-shaped
portion, and with success. There is a considerable literature upon
macroglossia (or megaloglossia), for which the reader is referred to the
Index Catalogue, xiv. pp. 615— Gl 7, Washington, 1893). Unilateral
cases were reported by A. Panlicky {Deutsche mil.-drztl. Ztschr., xi.
212, 1882) and by Wallace; the latter instance I myself examined.

Fig. 64. — Dr. Vargas's Case of Congenital Tooth in Extra-alveolar Dental Sac.

The anomaly may be associated with cystic hygroma of the neck, as
in A. Winiwarter's case (Arch./. Jclin. Chir., xvi. 655, 1874).

Congenital Teeth.— Teeth, generally incisors, may be present at
birth. This antenatal eruption occurs about once in six thousand
new-born infants, and I have reported six cases (Trans. Eclinb. Ohst.
Soc., xxi. 181, 1896; xxiii. 112, 1898). In the report which Dr.
Vargas of Barcelona sent to me, there was an additional anomaly
in the fact that the antenatally cut tooth was in an extra-alveolar
dental sac (Fig. 64). Full details of this curious malformation

392 ANTENATAL PATHOLOGY AND HYGIENE

(which is probably foetal and not embryonic in origin) are to be
found in my articles above referred to. It may be associated with
hare-lip, cleft palate, tongue-tie, and cyclopia. Irregularities in
dental eruption after birth are very common. Occasionally an
additional incisor tooth is present (B. da Costa, Gaz. med. da Bahia,
xxvi. 308, 1895).

CHAPTER XXII

Merosomatous Terata (cunt.) : Malformations of the Eyes and Nose ; Cyclopia :
Definition, Historical Note, Clinical History, Morbid Anatomy (Eye, Nasal
Apparatus, Facial Bones, Cranium and Brain, Associated Malformations),
Comparative Teratology, Teratogenesis, Literature ; Cebocepkalus ; Trigo-
nocephalus ; Cryptophthalmus ; Anophthalmus ; Microphthalmia ; Hydro-
phthalmus ; Coloboma of Eyelid ; Miscellaneous Malformations of the Eye-
lids (Microblepharon, Blepharophimosis, Polyblepharon, Ectropion, Entro-
pion, Distichiasis, Epicanthus, Ptosis Congenita) ; Malformations of the
Lachrymal Apparatus, Cornea, Iris, Choroid, Retina, Lens, and Vitreous ;
Miscellaneous Malformations of the Nose.

In this chapter I shall consider together the teratological states of the
eyes and nose. They are very closely associated, indeed in the very
characteristic cyclopic monstrosity they are constantly combined. In
the succeeding chapter I shall next deal with the anomalies of the
ears and lower jaw, for they likewise are frequently associated. At
the same time it must be borne in mind that both sets of anomalies
may be united in one foetus (e.g. in cyclops hypoagnathus) : this
simply means that here as elsewhere in Teratology there are con-
necting links, which, while they disturb the symmetry of classifica-
tional systems, are of great value in the understanding of teratogenesis.
In this chapter, then, I shall describe first cyclopia, then the nearly
allied cebocephalus and trigonocephalus, and then some miscellaneous
malformations of the eyes and nose.

Cyclopia.

In cyclopia (or synopsia) the two orbits and their contents are
more or less completely fused into one. It does not, therefore,
simply mean a one-eyed foetus, in which the singleness of the eye
may be due to the absence of its companion organ ; but is one in
which, by its position as well as by its structure and relations, the
eye evidently represents two joined in one. For this reason the
synonymous expressions monophthalmus and (less correctly) mono-
culus are not quite suitable.

Historical Note. — Doubtless the cyclops foetus is almost as old as
the practice of Midwifery itself, and it possibly was the origin (as has
been already suggested on p. 84) of the one-eyed Polyphemus of the
ancients and his one-eyed subjects, who forged the thunderbolts for
Zeus at his "almighty nod" in Sicilian caverns. We read also of
the Arimaspi, a one-eyed people, who lived near Arimaspias, a river

393

394 ANTENATAL PATHOLOGY AND HYGIENE

in Scythia with golden sands ; and Lycosthenes in his Chronicon
(1557) has figured one of them for us (p. 8) with single eye above a
normal nose. Licetus (De Monstris, p. 133, 1668) reported what was
probably a real example of the cyclopic monstrosity, for there was in
it a mesial eye with two pupils and a nasal projection or proboscis
(which Licetus compared to a penis) above the eye. Licetus also
reported a Janus double monster with the same cyclopic deformity,
and in the Appendix to the above edition of his work (1668) there
is another instance of it in a dicephalic foetus (p. 296). In the
eighteenth century there were not many cases recorded, but that seen
by Littre (Hist, ct mem. Acad. roy. d. sc. de Paris, Ann. 1717, M4m.,
p. 285, 1719) was noteworthy; in the middle of the face was a single
orbit with an eye evidently representing two (it had two optic nerves,
retime, pupils, lenses, etc., but only one aqueous humour and no
choroid) ; there was no nasal proboscis at all ; there were two palpe-
bral fissures in the normal position; there were six digits on the left
hand (it is the right in the accompanying illustration) ; and the
tongue was adherent to the floor of the mouth, and also to the uvula
at its root. It was indeed a curious case, as Littre thought. J. T.
Eller's specimen (Bee. period, d'obs. de me'd., de chir., et pharm., vi.
347, 1757) differed from Littre's in being a male and having a nasal
proboscis ; Mezeray's case (Hist. Acad. roy. d. sc. de Paris, Ann. 1761,
p. 58, 1763), about which, however, there is some doubt, had two eyes
placed one above the other. A great number of observations on
cyclopia were made in the nineteenth century, and during the past
twenty years marked progress has been achieved in connection with
the embryology of the anomaly. Many of the articles and mono-
graphs that have appeared between 1800 and the present date will
be referred to in the text ; but the following are of great importance
and may be named here. In addition to valuable contributions in
the Text-books on Teratology (such as Saint-Hilaire's, Forster's,
Ahlf eld's, Vrolik's, Otto's, Dareste's, and Tarufn's) there have been
works by A. Hannover (Pen, mcnncskclige Hjcrncslcals Bygning ved
Cyclopia, Copenhagen, 1882), O. Larcher {Diet, cncycl. d. sc. med., xxiv.
538, 1880), C. Pliisalix (Journ. de Vanat. ct physiol., xxv. 67, 1889),
A. Bruce (Proc. Roy. Soc. Edirib., xx. 412, 1895), C. Emery (Anal
Anz., viii. 53, 1892-93), and L. Blanc (Journ. de Vanat. ct physiol.,
xxxi. 187, 288, 1895). I have met with two cases (Figs. 18 and 19,
pp. 85, 86).

Clinical History. — There is little that requires to be said about
the pregnancy which ends in the birth of a cyclops foetus. It may
be accompanied by hydranmios as in the cases reported by H. Vogt
(Norsk. Mag. f. Lcegcvidensk., B. 4, x. 639, 1895), by G. Eustache
(Arch. d. tocol,, xi. 309, 1884), by W. Craig and J. Symington (Trans.
Med. Chir. Soc. Eclinb., n.s., v. 178, 1886), and by G. Beguleas (cited
by Tarufri, op. cit., viii. 379, 1894). In the last-named case the mother
had also frequent attacks of tonsillitis during pregnancy and dropsy
of the limbs. Hydranmios, however, is not constant (Hiibl, Centrlbl.f.
Gyndk., xxii. 21, 1898). Family prevalence may be met with in con-
nection with cyclopia as with anencephaly and other monstrosities;

CYCLOPIA 395

Caradec (Compt. rend. Soc, rfc biol, 4 s., iii., pt. 2, 117, 1867), for
instance, recorded how a woman gave birth to a female cyclops, then
to three well-formed males, and then, seven years later, to a second
female cyclops; G. Sapolini (Ann. univ. di mcd., cciv. 321, 1868),
also, related how a woman gave birth to three well-formed male
infants, and then to three females, of whom one had hydrocephalus,
a second hare-lip and cleft palate, and the third cyclopia. A most
remarkable observation was made by E. Ellis (Trans. Obst. Soc. Lond.,
vii. 160, 1866); it was a case of twins with separate placenta?; one
was a female, and had a nasal proboscis and palpebral fissures, and
the other was of doubtful sex and had a nasal proboscis and no trace
of eyes ; the mother had previously given birth to a female infant
with cleft palate and imperforate anus. We may take it, then, that
this was an instance of dichorionic twins, each with the same type of
monstrosity.

The cyclops foetus does not long survive its birth : it may just
breathe (Hlibl, loc. cit.) or live a few hours, five or six in Blok's case
(Ncdcrl. Tijdschr. v. Gcneesk, 2 E,,xxx.,pt. 2,414, 1894), and seventy-
three in Valenti's (Un caso di ciclopia, Perugia, 1895). Both in the
human subject and among the lower animals (e.g. the pig), cyclopia
is more common in the female than in the male (Tiedemann, Ztschr.
f. Physiol., i. 79, 1842): premature birth has also been noted, and
"maternal impressions," as usual, have not been wanting.

Morbid Anatomy. — The essential features of cyclopia are the
absence of a well-formed nose in its normal position, the presence of
a single eye (often with traces of duplicity in it) in the middle line
of the face, and the defective development of the skeleton of the
upper and anterior part of the face and of the anterior portion of
the brain. The reader has to imagine a developmental failure of the
fronto-nasal process, with a consequent approximation and fusion of
the two superior maxillary processes, and a disappearance of the
parts (nasal fossa?, etc.) which normally lie between them, both in
facial region and in the brain. We may, therefore, suitably describe
its morbid anatomy under — (1) the eye and its appendages : (2) the
nasal organ ; (3) the bones of the face, especially those forming the
single orbit ; (4) the cranium and the brain ; and (5) the associated
malformations in the head and elsewhere.

(1) The Eye. — The eye may be so completely single as to
show no trace whatever of duplicity : this was so in my specimen
(Fig. 18, p. 85), which was carefully examined for me by Dr. J. V.
Paterson, who found only vague traces of duplicity in the retina. On
the other hand, all grades of duplicity may be met with, from two
eyeballs lying side by side in the single orbit up to the full degree
of fusion found in my specimen. These grades were ascribed to
different stages of fusion of the two ocular vesicles (S. Eosenstein,
Arch. f. path. AnaL, vii. 532, 1853). The presence of two eyeballs
each of normal size and form and each with its own muscles can
hardly be said to have been scientifically demonstrated in cyclopia,
but the study of cebocephaly (q.v.) would lead us to believe in its
possibility. Often, however, the two eyeballs have been found to be

396 ANTENATAL PATHOLOGY AND HYGIENE

united into one large globe, with a median groove superiorly and
inferiorly, with slight atrophy of the contiguous margins, hut with
no disappearance of any of the essential parts. Each half has its
cornea, pupil, lens, and vitreous humour ; and the two cornea: are
separated by a strip of conjunctiva. The parts {e.g. the pupils) lie
in the same horizontal plane, with some doubtful exceptions (Mezeray,
loc. cit. ; Gaddi, Gazz. vied. ital. lomb., 3 s., vi. 180, 1855). The two
cornea: may, in other instances, be fused together, the pupils and the
lenses remaining distinct but near together ; again, the pupils may
be fused into one elliptical opening with the lenses either united or
separate ; finally, there is the eye single in all its parts, but larger than
usual (as in my specimen, Fig. 18). Descriptions of the histology of
the eye are few in medical literature ; but A. Bruce (Proc. Boy. Soc.
fidinb.,xx. 417, 1895) found an extraordinarily convoluted appearance
of the retina, so that in places it had assumed an almost tubular
structure. In correspondence with the state of the eyes is that of
the optic nerves: these are separate, or partially united, or completely
fused, or rudimentary (Van Duyse, Arch, d'ojiht., xix. 25, 106, 1899).
The muscles also show various anomalies : most commonly the
internal recti are absent, sometimes the superior and inferior recti
are duplicated ; the external recti are generally present ; and the
obliques are irregular or absent.

It must not, however, be forgotten that the single orbit in cyclopia
may contain a more or less rudimentary eye. Indeed, there may he
no eyeball at all (Caradec, loc. cit. ; Dumon et Savelli, Marseille me'd.,
xl. 455, 1903) ; or it may consist of a vesicle covered with the sclerotic,
but without lens or cornea. In Van Duyse's case (loc. cit.), each
pupil showed a coloboma. When the eye is so defective (anophthal-
mia) the optic nerve is usually little more than an indefinite cord lying
on the under surface of the rudimentary brain and advancing no further
than the optic foramen (Lannelongue et Menard, Affections comjeni-
talcs, p. 450, 1891).

Four eyelids are usually found, two upper and two lower, bounding
a quadrangular or lozenge-shaped opening ; sometimes the two upper
or the two lower are fused into one, and then the palpebral fissure is
triangular in shape ; and there may be other anomalous arrangements.
Sometimes the two upper palpebral do not meet in the middle line ;
the lower ones usually meet and at their junction there is a single
lachrymal caruncula and two papilhe, but there may be only one
(Valude et Vassaux, Arch, d'opht, viii. 51, 1888). The eyelids have
not their normal structure, and the eye cannot, therefore, be closed,
a circumstance which contributes to the peculiar appearance of the
cyclops foetus ; there may be a levator palpebrse superioris, or two of
them (Lannelongue et Menard, op. cit., p. 451). There may be a single
eyebrow over the cyclopia eye: in other cases there may he two
eyebrows in their normal position (Littre, loc. cit.). Again, there may
be no eyelids round the single eye, but they may he discovered in
their normal position. In Valenti's case (loc. cit.) there were two
contiguous but not continuous palpebral fissures ; there was, however,
only one orbit.

NASAL PROBOSCIS IN CYCLOPIA 397

(2) The Nasal Apparatus. — A very characteristic and fairly con-
stant feature of cyclopia is the presence of a nasal proboscis or tube,
situated nearly always above the median eye. Gurlt (op. eit., p. 155,
1832) has utilised its presence or absence to make two types of
cyclopia — -cyclops rhynchaneus and cyclops arhynchus; while I.
Saint-Hilaire (Histoirc, ii. 383, 183G) has separated two genera of
cyclocephalics, one in which the proboscis is present, rhinocephalus,
and one in which it is not, cyclocephalus in the narrow sense. When
the proboscis is wanting it has been found that the single eye is also
rudimentary or practically absent (anophthalmus), indicating a high
degree of absence of development of the mesial structures of the face.
Then, as was noted by Lannelongue and Menard (op. eit., p. 443,
1891), there may be a curious stellate or cruciform depression in the
skin of the forehead ; under this there is a round opening in the
frontal bone, through which the deeper parts of the skin become
adherent to the dura mater.

The proboscis, when present, has the following characters. It is
situated either between the rudimentary palpebral fissures, or, more
commonly, immediately above the single eye-opening in the position
of the glabella. In H. Vogt's remarkable case (loc. eit.), there was a
proboscis above the eye and another below it, a circumstance which
led him to call the specimen cyclops dirrhinus (double-nosed) ;
Taruffi (op. eit., viii. 540, 1894) had an opportunity of examining the
foetus, and confirmed Vogt's description. A sub-orbital proboscis is
very rare but not unknown, for Otto (Seltene Bcobachtungen zur
Anatomic, Physiologic, unci Pathologic gchorig, Hft. i. 35, 1816) saw it
in a cyclopic lamb ; but the presence of a supra-orbital and a sub-
orbital proboscis in the same case is unique. In form the proboscis
is usually cylindrical, but somewhat irregularly cylindrical, a fact
which led early writers to compare it to the penis, and later writers
(A. Bruce) to a miniature champagne bottle ; sometimes it is con-
stricted near its base (e.g. in Ullersperger's specimen, Inaug. Schr.,
Wiirzburg, 1822). At its free end is a small depression, and a single
orifice leading into a small canal or cavity containing mucus and
sebaceous material. Sometimes there are two openings ; and the space
may be divided into two chambers by an incomplete partition. The
proboscis may be nearly the length of the normal nose, or it may be
little more than the size of a pea (Lannelongue et Menard, op. eit.,
p. 493, 1891). It may be pendent over the eye, or project straight
out from the face (when it may be found to possess a cartilaginous
axis) or be turned up on the forehead .(C. Eokitansky, Lchrbuch der
pathologisehen Anatomic, Aufl. 3, iii. 1, 1861). Usually it has a soft
consistence, but, as stated above, it may be rigid from the presence
of a cartilaginous rod in its interior. Microscopically, it is covered
by skin resembling that of the face, under which is adipose tissue,
and below that is loose connective tissue containing vessels and
striped muscular fibres ; in the centre is a tube lined by epithelium
and showing racemose glands, and sometimes there is also a rod of
cartilage surrounded by fibrous tissue. The nervous supply of the
proboscis has not been fully worked out yet ; but no traces of

398 ANTENATAL PATHOLOGY AND HYGIENE

olfactory nerves have been found in three specimens investigated by
Lannelongue and Menard (oj). cit, p. 444, 1891). This curious organ
must be regarded as the soft parts of a nose ; the hard parts are
wanting, and, through the approximation of the superior maxillae and
the fusion of the orbits, the soft structures are in an abnormal position.
(3) The Bones of the Face. — The most striking peculiarity in the
facial skeleton is the presence of a single orbit. This cavity, which
is usually larger than one orbit and not so large as two would be, is
more or less elliptical in shape, and is bounded above by the frontal
bones, at the sides by the malars and greater wings of the sphenoid,
and inferiorly by the fused orbital plates of the superior maxillae
(without their nasal processes). At the fundus of the orbit is a
rounded opening bounded by the malformed lesser wings of the
sphenoid, by the sella turcica, and by the frontal bone ; through this
foramen comes the optic nerve (or nerves). At the sides are the
sphenoidal fissures. Several bones are usually entirely absent : these
are, the ethmoid, the pre-sphenoid, the vomer, the inferior tur-
binateds, the nasals, the lachrymals, and the premaxilla'. As a
result of their absence, the superior maxilla? and malars are approxi-
mated ; the former are small in size and lack the nasal processes.
The septum of the nose is also wanting. The post-sphenoid and
greater wings are generally normal, and so is the lower jaw with the
notable exception of the cases of cyclops hypoagnathus. The frontal
bones are usually fused together and show a median boss just above
the orbit; the single bone, thus resulting, is narrower than usual.
Sometimes there is an opening in the frontal bone in the position
of the metopic suture. The mode of union of the two superior
maxillary bones in the middle line of the face offers some puzzling
anatomical problems. The upper lip shows no philtrum, so that,
although it appears at first sight to be normal, it must really be
regarded as lacking its median part. Lannelongue and Menard
(op. cit., p. 451, 1891) give a mesial section through the skeleton of
the face ; from it we learn that the two palate bones fuse together
into one osseous mass, that in front of this the superior maxilhe are
separated only by a thin membrane which intervenes between them
from the floor of the orbit to the roof of the mouth, and that
anteriorly they (the superior maxilhe) form a single osseous mass
which constitutes the alveolar margin. It is thought that the
membrane separating the upper maxillee represents the nasal mucosa
(all that is developed of it). The vault of the palate is, therefore,
very narrow, but is not fissured ; there is a soft palate and behind it is
a rounded cul-de-sac into which open the Eustachian tubes. Incisor
tooth-germs are usually absent from the upper jaw ; but a median
(double) incisor attached to a rudimentary intermaxilla has been
found. The three upper incisors which have been described (by
Hannover, op. cit.) are doubtless two canines and a median incisor.
It is an interesting and perhaps significant fact that in at least two
cases a cyclops foetus has been born with teeth already cut (Morgagni,
De sedibus, Epist. xlviii., N. 53, 1761, English Translation, B. Alex-
ander, ii. 757, 1709 ; J. H. M'Lin, Med. Standard, xv. 121, 1894).

1 1

BRAIN IN CYCLOPIA 399

The muscles of the eyeball have been already described; it may
be added that some of those of the face are present {e.g., buccinators,
zygomatici, orbicularis oris, levatores menti), while others are absent
or defective (muscles of the nose, levator labii superioris, etc).

(4) The Cranium and the Brain. — I have already referred to
several characters of the cranium in my description of the orbit and
its boundaries, but I may here add some facts relating to the
cranium as a whole. The ossification is not as a rule defective, in
fact it is common to find the bones hard and the sutures firm and
even closed. In my specimen (Fig. 18) the anterior fontanelle was
very small in size, but the metopic suture was still present. The
dimensions of the whole head are below the normal, and the frontal
region specially suffers. The frontal bones (or bone) are small, the
cciput is often malformed, and the parietals are fairly normal.
The anterior fossa of the cranium is very small, the middle fossa is
narrow, and the posterior is not much altered.

The state of the brain is of great importance ; in fact, there is a
cerebral malformation of as great importance as that of the eyes,
orbit, and nose, and as essential to the morbid anatomy of cyclopia.
In my specimen (Fig. 18) there was a hydrocephalic sac found
occupying the greater part of the cranial cavity with a small brain
lying below it ; and this has been met with in other cases. The
posterior parts of the brain may be fairly normal ; thus, the pons,
medulla, cerebellum, and corpora quadrigemina may all be easily
recognised, and the cerebellum may even be larger than usual. The
tentorium cerebelli may be thickened, and is usually displaced up-
wards and forwards, being attached to the cranium at the level of the
lambdoidal suture or even in front of it. The cerebrum is always
greatly modified; It may be incompletely divided into two hemi-
spheres, or they may be united throughout ; the convolutions and
fissures are very slightly marked. The cerebrum leaves the cere-
bellum exposed. The falx cerebri, as can be imagined, is defective
or absent. The olfactory lobes and optic nerves are often wanting ;
but the other cranial nerves can usually be traced, although the third,
fourth, and sixth pair may be very small. In the interior of the
cerebrum the corpus callosum, the falx, and the septum lucidum are
commonly absent, and the third ventricle forms one cavity with the
two lateral ventricles. The optic thalami are fused together, and so
may be the crura cerebri. The pituitary body may be recognised,
but the pineal may be wanting (A. Bruce, loe. cit.). In the spinal
cord there is commonly an absence of the descending tracts ; in my
specimen (Fig. 18, p. 85) there was a small fissure running inwards
in the region of the crossed pyramidal tract, where also myelin ation
was defective, but myelination of the direct pyramidal tracts was
good. All these changes in the central nervous system point to
defective development of the anterior cerebral vesicle.

(5) Associated Malformations. — Cyclopia may occur alone as the
sole teratological state in a given case ; but it is very common for it
to be associated with other malformations. Absence of the nasal
fossoe and contiguous parts, the presence of the nasal proboscis, and

400 ANTENATAL PATHOLOGY AND HYGIENE

the peculiar defects of the brain (to which I have referred above)
can hardly be called associated malformations, for they are almost
as essential as the single orbit and fused eye ; but there are other
malformations of the face and head which are not so bound up with
cyclopia and may yet occur along with it. Some of these terato-
logical states have been used by Saint-Hilaire and others to
constitute separate types of monstrosity : the above writer, for
instance, gave the name stomocephalus to those cases in which, in
addition to the cyclopia, there was smallness of the lower jaw and
of the mouth and a projection forwards of the superabundant soft
parts and skin in the form of a buccal swelling or proboscis. Stomo-
cephalus would seem to be very rare (vide I. Saint-Hilaire, op. cit.,
ii. 403, 1836), but partial or complete absence of the lower jaw with
approximation of the external ears in the middle line anteriorly is
not so uncommon. One of my specimens of cyclopia (Fig. 18) showed
this association with otocephalus, as it is called. Further, the name
edoccphalus has been given to the cases in which there is cyclopia,
a nasal proboscis, and no mouth ; and that of opoccphalus is assigned
to the specimens in which there is neither proboscis nor mouth, but
only cyclopia and the fusion of the external ears in the middle line
anteriorly. L. Blanc (Journ. de Vanat. et physiol., xxxi. 187, 288,
1895) makes a separate division for these two types under the name
cyclotia, representing thus the combined nature of the monstrosity.
Of otocephalus, edocephalus, and opocephalus I shall have something
to say shortly ; I refer to them here only as related to cyclopia.

Anencephaly and cephalocele may likewise be associated with
cyclopia (e.g. in the specimens of Taruffi, op. cit., vi. 372, 1891 ; of
Planchon, Bull. Soc. anat. de Paris, xliii. 541, 1868 ; and of Galloupe,
Boston Med. and Surg. Journ., cii. 135, 495, 1880) ; so may hydro-
cephalus (Beveridge, Northumberland and Durham Med. Journ., ii. 51,
1894) and hare-lip and spina bifida (Panum, Nord. med. Ark., i. No. 1,
1-25, 1869). Malformations in more distant parts of the body and
limbs may also occur: exomphalos (Morgagni, op. cit.), umbilical
hernia (Saussol, Gaz. held. d. sc, med. de Montpelier, viii. 458, 1886),
abdominal hernia and absence of sternum (J. N. Snively, Med. and
Surg. Reporter, xxiv. 383, 1871), club-hand (Morgagni, op. cit.; G.
Eeguleas, Atti d. Acead. Gioenia, 2 s., iv. 123, 1847), atresia ani
(Eeguleas, loc. cit), inversion of the viscera (Heuermann, cited by
Taruffi, op. cit., viii. 374, 1894), absence of the suprarenal capsules
(Eiviera, cited by Taruffi), preauricular appendages, absence of right
kidney, and uterus unicornis (Stybz, Arch, de toeol., xvii. 392, 1890),
Polydactyly (Hiibl, loc. cit.), diaphragmatic hernia (Littre, loc. cit. ; E.
Lacroix, Tr. med., Paris, xiii. 141, 1833), and single umbilical artery
(C. Debierre, Compt. rend. Soc. de biol., 8 s., iii. 184, 1886) are among
the numerous and varied anomalies that have been recorded.

Comparative Teratology. — Cyclopia is a comparatively common
monstrosity in the human subject, not so frequent as anencephaly,
but yet fairly common. Among the lower animals it has a surpris-
ing frequency, and indeed it seems to take the place with them
that anencephaly holds in the human subject. Hannover (op. cit.)

CYCLOPIA 401

collected together the records of cyclopia in man and in the lower
animals, and obtained the following table : —

Human subject . . . 103

Calves
Lambs
•Pigs _
Puppies
Kittens
Foals
Kids
Rabbits
Deer

30

51

130

22

12

10

9

3

1

268

C. Taruffi (op. tit., vi. 433, 1891) collected 43 cases in the human
subject (not including 18 instances of cyclops hypoagnathus), 10 in
calves, 28 in lambs, 47 in pigs, 11 in puppies, 7 in kids, 7 in kittens,
12 in foals, and 1 in the ass — a total of 166 cases, including 123 in
the lower animals. It also has been noted in birds (chicken, goose,
dove, duck), and in fish (salmon). Symington and Woodhead (Proc.
Roy. Phys. Soc. Edinh., ix. pt. i., 208, 1886) described 10 specimens of
cyclopia, 3 in the pig, 2 in puppies, and 1 each in the human subject,
kitten, calf, lamb, and sheep ; they add that Brotherston had met
with about thirty cyclopic lambs, the offspring of one ram. We do
not understand the meaning of these facts in Comparative Teratology,
although it is evident they must have a significance. The morbid
anatomy of cyclopia in the lower animals, especially as produced
artificially in chicks, has many interesting features, into which it
is impossible for me to enter; but I may refer here to Lavocat's
observation (Rev. vdtti-inairr, Toulouse, x. 233, 1885). It was that
of a lamb which had neither median eye nor median orbit, and
there was also absence of the olfactory apparatus and premaxilla.
Lavocat proposed to call it ophthalmocephcdus and to separate it
from cyclopia ; and Taruffi agrees with him in the latter point, but
would call it atrochius (without orbit) arhynchus (without nose),
which is etymologically correct although unfamiliar (op. tit., vi. 379,
1891).

Teratogencsis. — With regard to the origin and meaning of cyclopia
much has been written of recent years, and the reader is directed to
Etienne Eabaud's fine series of monographs (Journ. de Vanat. etphysiol.,
xxxvii. 345, 575, 1901 ; xxxviii. 35, 282, 510, 1902) for a perfect mine
of information on these difficult matters. The theories that have
been adduced to explain its origin are by this time familiar to the
reader ; they are those known as the traumatic, the nosological
(or pathological), the amniotic, and the embryological. I need not
enter into detail regarding these theories, for their general principles,
stated in an earlier part of this volume, apply also in such a special
case as cyclopia. The traumatic or mechanical theory was advanced
vol. ir. — 26

402 ANTENATAL PATHOLOGY AND HYGIENE

in the eighteenth century, and held to some extent also in the
nineteenth ; the idea of the two eyes being more or less completely
fused by some compressing force was, however, a very vague one till
Dareste and others gave it defiuiteness by alleging that the head fold
of the amnion was the compressing agent. Nosological theories may
be said still to prevail in many observers' minds : thus A. Bruce (loc.
cit.) regards a limited pachy- and lepto-meningitis as a separate, or at
least contributing, cause of cyclopia ; and other writers have ascribed
the changes to hydrocephalus. It has, however, been shown already
in the discussion of the nosological theory in teratogenesis that the
idea of diseases as the causes of monstrosities is hardly tenable in the
light of the chronology of antenatal processes. Panum (loc. cit.), for
instance, met with cyclopia in an embryo of six weeks, at which early
date it is scarcely possible to conceive of the occurrence of diseases
in the meninges or lining membrane of the ventricles.

The amniotic theory was strongly stated by Dareste first in 1877
and later in his Production artificicllc des monstruosites (2nd edit.,
373, 1891). He regarded cyclopia as due to amniotic compression
which arrested the development of the anterior cerebral vesicle ;
there is then premature closure of the anterior wTall of the first
cerebral vesicle, the parts of the optic vesicles which form the two
retinae unite in the middle line instead of being separated from each
other, and so interfere with the formation of the cerebral hemispheres
and the parts formed from them. Eabaud (loc. cit.), a pupil of
Dareste, has recently pointed out that in artificially produced
cyclopia in chicks in which there is evidence of amniotic compres-
sion, the morbid changes found are not those of true cyclopia but
rather of states resembling cebocephaly (approximation of the eyes).
Iiabaud thinks there is only an external resemblance to the cyclopic
state in these pressure-produced monstrous chicks.

Apart from questions as to the cause of the disturbed ontogeny
that leads to cyclopia, there is an almost complete agreement among
writers on the subject that the monstrosity is the result of an arrested
development. There is some difference of opinion, however, as to the
primary localisation of the arrest. Dareste is strongly of opinion
that the dominating factor is the defective state of the brain,
and that the facial and other malformations follow upon this. There
is an arrest of the ontogeny of the anterior cerebral vesicle, the groove
from which it normally forms closing too early and so bringing
together the parts from which the retinae of the two eyes are formed
and leading to their fusion. In a similar manner the single condition
of the anterior part of the brain is held to determine the condition ,of
the nose and face. There are, however, some difficulties in accepting
this theory, for it does not seem that the development of the face is
always determined by that of the brain, an obvious exception being
anencephaly, in which, of course, there is disturbance of the ontogeny
of the central nervous system often with no considerable facial
deformities at all. I may mention here that all are not agreed that
the primary defect lies in the nervous system, Hannover (op. cit.), for
instance, ascribing it to imperfect formation of the prechordial part

LITERATURE OF CYCLOPIA 403

of the base of the skull ; but this opinion is exceptional, and it may
be said that the importance of the cerebral defect is generally recog-
nised. Eabaud (loc. tit.) is inclined to regard the cyclopic differentia-
tion as a special evolution rather than as an arrested development, as
a new procedure in ontogenesis which entails novel correlations in
development (" origine adaptative "), and is determined by an external
agency, the nature of which is unknown and about which we are
unable at present to advance a hypothesis. There is much that is
uncertain about the teratogenesis of cyclopia, but that the explanation
is to be found in Embryology is a clearly established deduction from
the data we possess.

Literature. — In addition to the references given in the text, the follow-
ing are important and noteworthy : — Alezais, Marseille med., xxxiii. 487,
1896; R. Allan, Lancet, i. for 1848, p. 227; F. H. Allen, Med. Rec,
1. 249, 1896 ; Arloing, Mem. ef compt. rend. Soe. de sc med. de Lyon, vh.
33S, 1868; Rambaren, Gac. vied, de Lima, v.-vi. 125, 1860-61;
H. G. Rartels, Diss, inaug., Marburg, 1840; E. Recker, Diss, inaug.,
Wurzburg, 1903; Rillaudeau, Rev. phot. d. hop. de Paris, v. 143, 1873;
R. Rramwell, Edirib. Med. Journ., xxvi. 550, 1880-81; AY". Rurns,
Boston Med. and Surg. Journ., Ixviii. 513, 1863; C. J. Carus, Entioicke-
lung der Form tines Angesichts anf einem cyclopischen Auge, 1839;
Clauzure, Rev. mid. franc, et etrang., ii. 59, 1830; Cleland, Journ.
Anal and Physiol., xii. 518, 1877-78; T. Close, Boston Med. Intellig.,
iii. 71, 1825-26; Dantscher, Oesterr. med. Wchnschr., 801, 1847; S.
Delle Chiaie, Istoria anatomico-teratologica intorno ad una bambina rino-
cefalo-monocola, Napoli, 1840; F. L. Fick, Tractatus de Cyclopia, Marburg,
1837 ; 1). J. T. Francis, London Med, Gaz., xxxiv. 580, 1844; J.
Gebauer, Diss, inaug., Dorpat, 1833; E. Gintrac, Journ, de med. de
Bordeaux, s. 2, v. 153, 1860; Gosselin, Compt. rend. Soc. de biol., iv. 28,
1853 ; L. Grosz, Ztschr.f. Nat, u. Heilk. in Ungarn, viii. 34, 1857 ; A. R.
Hall, Boston Med. and, Surg. Journ., lxv. 263, 1861-62 ; Heyman,
Deutsches Arch. f. d, Physiol, vi.r527, 1820 ; C. A. R. Jahn, Diss, inaug.,
Rerlin, 1860; C. Jourdan, These, Paris, 1833 ; P. K. E. E. Koeppel, Diss.
inaug., Rerlin, 1867; E. Lacroix, Tr. med., Paris, xiii. 141, 1833; H.
Laforgue, Journ. de med. chir. et pharm. de Toulouse, 3 s., v. 273, 1860 ;
Lovinsohn, Wien. Idin. Rundschau, x. 271, 1896; A. Malherbe, Bull.
Soc. anat, de Paris, liv. 115, 1879; Mardiere, Bull. Soc. de med. de
Poitiers, xxvii. 48, 1858; Mater, Mag. f. d. ges. Heilk., xvii. 329, 1824 ;
A. Meckel, Arch, f. Anat. u. Physiol., 159, 1828; Xox Monakow, Wien,
med. Wchnschr., xlvi. 2222, 1896: O. Naegeli, Arch./. EnticcTdngsmechn.
d. Organ., v. 168, 1897; J. ISticholls, St. Geor</e's Hosp. Rep., iv. 215,
1869 ; A. Nieden, Arch. f. Augenhlk., xxii. 6l', 1890-91 ; C. Poelman,
Bull, Soc. de med. de Gand, xxiii. 223, 1856 ; xxix. 320, 1862 ; E. J.
Raddatz, Diss, inaug., Rerlin, 1829 ; Rebsamen, Srhweiz. Ztschr.f. Nat. u.
Heilk, ii. 38, 1837 ; W. Roe, Dublin Quart. Journ. Med, Sc, li. 146, 1871 ;
A. Romer, Med. Jahrb. d. A: A: ossterr. Staates, xxiii. 453, 1837 ; V. P.
Roy, Rev. med, franc, et etrang., iii. 36, 1841 ; Sappey, Compt. rend. Soc,
de biol, 3 s., i. 46, 1860 ; J. Scott, Lancet, i. for 1862, p. 633 ; C. G. Speer,
Diss, inaug., Hala?, 1819 ; R. M. Smith and A. J. Parker, Amer. Journ.
Med. Sc, lxxxiv. 132, 1882; J. Schmid, De cyclopia, Turici, 1838; J. A.
Thompson, Trans. Obst, Soc Lond., xv. 35, 1874; Tonglet et Keiffer,
Bull, Soc beige de gynec. et d'obst., ix. 156, 1897-98 ; W. Yrolik, Over

404 ANTENATAL PATHOLOGY AND HYGIENE

den Aard en oorsprong der eyclopie, Amsterdam, 1834 ; J. Wai/tiier, Journ.
d. Ghir. n. Augenh., xxxiv. 345, 1845 ; J. G. Wedekind, Diss, inaug.,
Groningse, 1830.

Cebocephalus.

The monstrosity which is known as cebocephalus has by some
teratologists (e.g. Saint-Hilaire) been placed witli cyclopia proper in
the family of the cyclocephalians ; but it is preferable to regard it as
a type by itself, although it undoubtedly is closely related to cyclopia,
and may even be regarded as an incomplete form of it. Inasmuch,
however, as there are two orbits and not one, it fails to fulfil the
requirements of the definition of cyclopia. In cebocephalus the two
orbits are approximated, and, in consequence of this, the nose is
deformed and the whole face diminished in size. The expression of
the face is ape-like, hence the use of the term cebocephalus (xijjSof,
ape, zspu'kri, head) has some appropriateness. This type is compara-
tively rare, for Taruffi only succeeded in collecting twenty-six case-
records ; but it is possible that some instances have been reported
under malformations of the nose, for the nasal deformity is often
more evident than the ocular.

I have not myself seen a specimen of cebocephalus, but in 1890
Professor A. R. Simpson handed to me a letter he had received from
Dr. G. T. Smith of New Brunswick, containing the description of a
foetus which I have little doubt was an instance of this teratologics!
type. It was a female child, the product of the second pregnancy
of a strong, healthy woman, thirty-four years of age; the first
pregnancy, eight years previously, had ended in the birth of a normal
infant. The labour was full time and there was hydramnios.
The child lived twenty minutes only. The ears and mouth were
normal ; the eyes were absent, but there was a slight depression in
the position of the right eye and a superficial slit, ^ inch long, where
the left eye should be. The nose was a tube, arising between the
two ocular depressions, and standing straight out from the face ; it
was about § inch long, quite flexible, and had two openings at its
free end. The right foot was very narrow, appearing to consist of
one metatarsal bone, and it ended in one well-marked digit (with a
nail) the same length as the toes of the left foot (which was normal).
The right hand was normal, but the left carried only four perfect
digits, with a rounded soft mass, the size of a cherry, springing from
the base of the metacarpal bone, to which it was attached by a
pedicle and carried a nail. The placenta and cord seemed normal.

Dr. Smith was not able to dissect the child, and so no informa-
tion is available as to the state of the orbits, but I think it may be
grouped as a specimen of cebocephalus in which the nasal deformity
took the form of a proboscis such as is often seen in cyclopia. For
this type Isidore Geoffrey Saint-Hilaire created a special genus bearing
the name Ethmocephalus (Histoire, ii. .">7S, 1836); but there is no
special appropriateness therein. In other cases the nose may simply
be flattened, and have one opening (without any septum, and often

CEBOCEPHALUS 405

rather narrow), or more rarely two apertures. The mouth is generally
small, but the lower jaw may be quite normal. Commonly there is
closure of the frontal (metopic) suture, and the frontal eminences form
one median projection. The whole face is smaller, and the palpebral
slits are oblique, their internal angles being lower than the external.

The essential feature, however, is the approximation of the orbits.
This is due to the absence (or incomplete development) of certain
bones, including the ethmoid, the nasals, the superior turbinated, the
lachrymals, and the vomer. The premaxillse may also be small in
size. The inter-orbital space is small in extent, the orbits are wide
and high, and the optic foramina large.

The brain shows the same malformations as in cyclopia : there is
absence of the olfactory nerves, the corpus callosum, and the septum
lucidum; the anterior lobes are malformed, and indeed the whole
cerebrum may be single and small in size, although in other cases
there is the indication of two hemispheres with a median sulcus;
the convolutions are slightly marked ; and the lateral ventricles
communicate freely and widely with the third.

Associated malformations are comparatively common. There was
an adhesion of the placental amnion in A. Charvet's case {Ceboce'phalie,
Grenoble, 1874) ;" bilateral labial, alveolar, and palatal fissures in
Fridolin's cases {Arch. f. path. Anat., civ. 158, 1886; cxii. 536, 1888);
absence of the premaxillse (C. Hecker and L. Buhl, Monatschr. f.
Geburtsk. u. Frauenkr., xxxi. 430, 1868 ; H. Kundrat, Arhinenccphalie,
(Iraz, 1882), absence of the eyeballs in C. Eenner's case (Diss, inaag.,
Halle, 1889), Polydactyly and double uterus and vagina (Renner,
op. cit.), and there were defects of the left hand and right foot in
Smith's case (above related). «

The teratogenesis of cebocephalus need not be specially considered,
for what has been said regarding cyclopia may be applied here. Its
comparative teratology also closely resembles that of cyclopia, for it
is more common in the pig than in any other animal. Owing to
peculiarities in the regional anatomy of the face in animals the
approximation of the orbits is not very striking, and, therefore, it is
not surprising that the maxillary anomaly (brachyrhynchus) is the
feature that specially appealed to the veterinary pathologists who
met with instances of it {e.g. Glurlt).

Trigonocephalus.

Trigonocephaly (triangular head) or oocephalus (egg-shaped head)
is a somewhat incompletely differentiated teratological type ; indeed
it may be said to be a cranial character met with in both cebocephalus
and cyclopia. At the same time it may occur apart from both these
monstrosities (H. Welcker, Uebcr zwei seltnere Difforiiiitatcn dcs
menschlichcn Schddcls, Halle, 1863). Although rare, Taruffi {op. cit.,
vi. 358, 1891) has succeeded in collecting together nineteen instances
of it from literature, including three cases described by Hanns Kundrat
in his admirable monograph {Arhinenccphalie ah typische Art von
Missbildung, Graz, 1882). The anterior part of the cranium is pointed

406 ANTENATAL PATHOLOGY AND HYGIENE

or triangular, the apex of the triangle being the middle point of the
forehead. There is premature synostosis of the two halves of the
frontal bone (J. Fridolin, Arch. f. path. Anat., civ. 156, 1 886), occurring
according to 0. Kustner (Arch. f. path. Anat.Axxxui. 58, 1881) at the
twentieth week of antenatal life. The eyes are obliquely placed, their
inner angles being lower than their outer ; and in some instances the
orbits are approximated. The state of the brain very closely resembles
that found in cebocephalus and in cyclopia : it is arhinencephalic, to
use Kundrat's word. The olfactory nerves are often wanting, and
the anterior part of the cerebrum is single. There may also be
hydrocephalus (as in one of Kundrat's cases). Other associated mal-
formations are microphthalnms, cleft palate, double hare-lip, ompha-
locele, and a perforate condition of the ventricular septum of the
heart. The premaxilhe may be small in size. The malformation is
not incompatible with postnatal life : one of Kiistner's cases was two
years of age. It is to be regarded as a slight degree of the same
series of changes as produce cebocephalus and cyclopia ; and it is
probably due to the same cause, whatever that may be.

Malformations of the Eyes.

Many malformations of the eyes exist, as every student of
Ophthalmology knows: but I can only give them a passing notice
here. A book might easily be written, instead of part of a chapter,
upon the structural anomalies of the eye and its appendages.1 The
multiplicity of its malformations is fully explained by the complexity
of the development of the eye.

Cryptophthalmus.

In this type of malformation the skin passes without a break
from the forehead on to the cheek, and the openings of the orbits
are thus quite closed in. The term cryptophthalmus has been pre-
ferred by recent writers to that of ablepharon. It is not to be
confused with adhesion of the eyelids {ankyloblepharon), which is
hardly a malformation, for it occurs comparatively late in fcetal life,
and is possibly due to antenatal disease (blepharitis); it must also
be distinguished from adhesion of the palpebral and ocular conjunc-
tival surfaces {symblcpharon). A typical instance of cryptophthalmus
was reported by W. Zehender (Einc Missgeburt mit hautuberwachsenen
A a yen, liostock, 1872), with a detailed description of the eyes by
Manz. The child, a female with anomalies of the external genitals,
died at the age of nine months. There were no eyebrows and no
trace of a palpebral fissure; only the skin covering the orbits was
thin and devoid of fat. There was also a small umbilical hernia and
syndactyly of the hands and feet. The optic nerves were present.
The eyes were discovered in the orbits at the necropsy: the con-
junctival sac was absent ; the lens and iris of the right eye were

1 Since this sentence was penned Professor Van Duyse'swork, Elements cfetnbryologie
ct de teratologic de Voeil, lias been published (190 1).

ANOPHTHALMIA 407

absent, the ciliary processes and bodies were rudimentary, but the
cornea was represented ; the left eye was distinctly smaller than the
right, and had a normal choroid and retina. Another typical ease
was put on record by Van Duyse {Contribution a Vetude du crypt-
ophtalmos, Bruxelles, 1899 ; Belgique vied., vi. 705, 1899), with a good
dissection and numerous drawings of the microscopic characters of
the eyes. Yet other cases have been described by Fuehs ( Wien. med.
BUM., xii. 215, 1889), H. Otto {Wien. Hin. Wchnschr., vi. 883, 1893),
and S. Karman (Urvoxi hetil., xxxviii. 139, 1894; Arch. f. Kinderhlk.,
xviii. 206, 1894-95), and in the lower animals by L. Bach {Arch. f.
Augenh.} xxxii. 16, 1895-96), by Van Duyse (op. cit.), and G. de
Grandmont {Arch, d'opht., xiii. 742, 1893). Taruffi (op. cit., vi. 440,
1891) also refers to cases by Chiari (a female foetus with hare-lip,
syndactyly, facial asymmetry, atresia of the larynx, and urino-genital
defects) and Coen (a foetus with absence of the kidneys and uterus).
The eyeballs would appear sometimes to show unmistakable signs
of inflammation, the lachrymal glands may be absent, there is often
a displacement of the parts of the eye, and the lens may be present
but in a degenerated state. It has, therefore, been maintained that
inflammation of the eyes in antenatal life is the causal factor in
cryptophthalmus, and that an amniotic adhesion may be instru-
mental in inducing it ; fusion of the lids would follow. On the
other hand, it is possible that there is in this morbid state a lack
of development of the lids, and that the other changes are secondary
(i.e. a teratological and not a nosological condition). The matter
cannot at present be decided ; but it may be that, after all, crypt-
ophthalmus differs from ankyloblepharon only in the earlier date in
antenatal life at which it is initiated. The changes in the eyeballs
do not hold out much hope of successful operative interference.

Anophthalmus (Anopsia).

Absence of the eyeball either on one side only (monophthalmus)
or on both (anophthalmus) is not so rare as cryptophthalmus. I was
able to examine a case some years ago, which was brought under my
notice by Dr. Bunting. It was that of a female child of five months.
The mother, age thirty-nine, had had eight pregnancies, and the child
with monophthalmus was the product of the fifth. The first three in-
fants died, two of them soon after birth and the third before birth ;
the second and third were born at the seventh month. The other
children were full time and were all alive. The father, age thirty-nine,
was suffering from eye trouble (tertiary syphilis ?) when this child was
conceived. It was the right eye that was absent. The right rima
palpebrarum measured less than h inch (1 cm.) in length, while the
left was nearly an inch (2*3 cm.), and the opening of the former was
nearer the middle line. It was provided with some eyelashes, and
tears came from it. The right ear was a little larger than the left,
and there was cranial asymmetry, the right side of the head showing
very little parietal prominence. There was hardly any eyebrow on
the right side, and some entropion was noted. The left eye was dark

408 ANTENATAL PATHOLOGY AND HYGIENE

grey in colour and reacted well to light. The hands and feet and
the genitals were well formed.

This case (Fig. 65) may be taken as a typical instance of unilateral
anophthalmias. It is apparently rarer than the bilateral variety.
The optic nerves may be absent or atrophic, and the atrophy may
extend to the optic chiasma and corpora quadrigemina. The contents
of the orbit may be simply connective tissue, or the muscles of the
absent eyeball may be discovered. The lachrymal apparatus may be
perfect, or the carunculse defective. The eyelids may also be com-
plete and capable of movement ; the palpebral slit is usually small,
and there may be adhesion of the margins of the lids (ankyloble-
pharon) and some degree of entropion. Associated malformations
have been met with : club hands and feet, cleft palate, Polydactyly,
bifid tongue, hare-lip, cranial asymmetry, and preauricular ap-

FlG. 65. — Case of Unilateral Anophthalmia.

pendages. The malformation, in itself, does not interfere with
postnatal life. The mother of Kamage's patient (Brit. Med. Journ,, i.
for 1894, p. 081) had convergent strabismus. It has been met with
in animals {peroeeplialus anommatus, Gurlt).

Literature. — I may give here some references to important papers.
Vox Arlt, Allg. wien. med. Ztg., xxx. 105, 1885; E. T. Collins, Ophth.
Hosp. Rep., London, xi. 429, ' 1887; Davey, Lancet, ii. for 1836, 293;-
A. D. Davidson, Tram. Ophth. Soc. Unit. Kingdom, i. 216, 1880-81 ;
Forster, Arch. f. path. Anat., xiii. 53, 1858; W. H. Fox, Trans. Med. Soc.
Dist. Golumb., ii. 170, 1898; It. Fusari and B. Mokpurgo, Atti Aecad. <t.
sc. med. e tint, in Ferrara, lxviii. 109, 1894; E. Giovanardi, Riv. sper. di
freniat., vii. 244, 1881 ; 11. J. Godlee, Trans. Path. Soc. Lond., xxxiv.
'230, 1882-83; 8. S. Guekrant, Arch. Pediat., xv. 905, 1898; O. Haab,
Beitr. z. Ophth., p. 131, 1882; R. Hilbert, Arch. f. path. Anat., exxvii.
378, 1892; E. vu.v Hippel, Arch. f. Ophth., xlvii. 227, 1898; J. Hirsch-

MICROPHTHALMOS 409

berg, Arch. f. Augenh., viii. 189, 1879; Hubsch, Gaz. med. d' Orient, xv.
13, 1872; J. Hutchinson, Ophth. Hosp. Rep., Load., v. 326, 1866; L.
W. Kinney, Boston Med. and Surg. Joum., Ii. 25, 1854-55 ; V. Lafosse,
Presse med. beige, xlviii. 193, 1896; ( >. vox Leonowa, Arch. f. Anat. u.
entwcMngsgesch., p. 308, 1893; Arch./. Psychiat., xxviii. 53, 1896; E.
Liebreich, St. Thomas's Hosp. Rep., n.s., iv. 135, 1873; A. Meyer,
Centrlbl. f. prakt. Augenh., i. 221, 1877; J. Michel, Arch. f. Ophth., xxiv.
71, 1878; W. G. Nash, Lancet, i. for 1898, p. 28; J. W. Park, Ophth.
Re<\, Chicago, vi. 23, 1897 ; Poland, Ophth. Hosp. Rep., London, i. 158,
1858; C. Ramage, Brit. Med. Joum., i. for 1894, p. 681; A. Samelson,
Brit. Med. Joum., i. for 1868, p. 401 ; Schenkl, Praq. med. Wchnschr.,
viii. 471, 1883; A. Sissa, Go;:;:, med, Hal. lomb., 3 8., i. 317, 1850; F. A.
Skuhersky, Monatschr. f. Med., Augenh., u. Chir., i. 64, 1838; C. E.
Smith, Brit. Med. Joum., ii. for 1880, 169 ; S. Snell, Trans. Ophth. Soe.
Unit. Kingdom, iv. 345, 1883-84; W. G. Sy.u, Ophth. Rev., xl. 76, 1892;
Voglar, Mag. f. d. ges. Heilk., lxiii. 492, 1844 ; ,). P. Wall, Med. Rec,
xix. 358, 1881 ; L. von Wecker, Klin. Monatsbl. f. Augenh., xiv. 329,
1876; Wordsworth, Lancet, ii. for 1881, p. 875; J. K. Young, Med.
News, lii. 636, 1888; and C. Zimmermann, Arch. Ophth., New York,
xxii. 340, 1893.

Microphthalmias.

Under the name microphthalmus have been grouped together
many cases of diminution in the size of the eye, varying in degree
from those in which the smallness was slight to those in which the
eyeball was not larger than a pea. There can hardly be drawn any
line dividing marked microphthahnus from anophthalmus ; indeed
it has been believed by some that the latter malformation is only the
last degree of the former. At any rate the two malformations have
been met with in the same subject (E. T. Collins, Ophth. Hosp. Rep.,
Lond., xi. 429, 1887).

Microphthahnus is more often bilateral than unilateral, but even
when bilateral it may be of different degree. There may be no
other defect than smallness in size ; but on the other hand malfor-
mations of the eye are very common. In a case which I examined
in September 1895 (it was brought under my notice by Dr. Oliphant
Nicholson, then of Kirkcaldy) there was microphthalmus and aniridia
of the right eye and coloboma of the iris of the left. Other asso-
ciated malformations are corneal defects ; coloboma of the vitreous,
of the choroid, and of the optic nerve ; absence of the lens, iris, and
ciliary bodies ; retinal and orbital anomalies ; blepharophimosis and
blepharoptosis ; and cysts of the lower eyelid or in the interior of
the orbit. Microphthalmus occurs in the lower animals, and received
from Gurlt the name of nanocephalus micrommatus (Lehrbuch der path.
Anat. der Haus-Saugdhiere, ii. 109, 1832).

Like so many other malformations, microphthalmus may show
family prevalence (A. Gescheidt, Ztschr. f. d, Ophth., ii. 257, 1832) ;
it may also affect a father and his sons (P. Gradenigo, Giorn. veneto di
sc. med,, 3 s., hi. 703, 1865), and may even be transmitted to the
third generation (G. Mayerhausen, Centrlbl. f. praht. Augenh., vi. 97,
1882). Other ocular diseases and anomalies may be found in the

410 ANTENATAL PATHOLOGY AND HYGIENE

relatives of individuals suffering from microphthalmus. Some in-
teresting experiments upon animals by J. Samelsohn {Gentrlbl. f. d.
iiml. JVissensch., xviii. .'305, 322, 1880) would almost seem to show
that injuries inflicted upon the eyes of the parent might induce
microphthalmus in the offspring ; but there is grave doubt whether
this theory can be accepted.

There are two chief theories regarding the origin of microphthal-
mus, and they apply also to anophthalmus. The reader, doubtless,
has already guessed that according to some authors the changes are
produced by diseases {e.g. foetal choroiditis, P. Caudron, Rev. gen.
d'opht., vi. 97, 1887) or by cystic growths, while others look upon
them as arrested developments (want of closure of ocular vesicle,
etc.). The greater probability rests with the second theory.

Literature. — Some articles may be enumerated, but many must be left
unnamed. F. Arnold, Monatschr. f. Med., Augerih., u. Ghir., ii. 88, 1839;
Von Arlt, Wien. med. Presse, xxvi. 245, 1885 ; A. H. Benson, Ophth. Rev.,
iv. 230, 1885; W. W. Cooper, OpMh. Hosp. Rep., London, i. 110, 1858;
Desmarres, Gaz. d. hop., 3 s., ii. 13, 1850; A. von Graefe, Arch. Ophth.,
ii. 1 Abtb., 239, 1855 ; C. Hess, Arch. f. Ojjhth., xxxiv. 3 Abth., 147,
1888 ; Hoppe, Arch./. Augerili., xxxix. 201, 1899; J. Jacobi, Klin. Monatsbl.
f. Augenh., xii. 260, 1874; O. Lange, Arch. f. Ophth., xliv. 66, 1897; H.
Magnus, Arch. f. Augenh., xii. 297, 1882-83; W. Manz, Arch. f. Oj>hth.,
xxvi. 1 Abtb., 154, 1880 ; N. Moore, St. Rarth. Hosp. Rep., xiv. 163, 1878 ;
H. Muller, Verhandl. d. phys.-med. Gesellsch. in Wiirzburg, x. 138, 1860;
E. Pfluger, Arch. f. Augenh., xiv. 1, 1884-85; E. Raehlmann, Ueber
Mikrophthalmos, Coloboma ocidi, und Hemimicrosoma, 1897; F. Schilling
und F. Giulini, Miinchen. med. Wchnschr., xxxix. 549, 1892; S. G.
Siiattock, St. Thomas's Ho*p. Rep., xv. 243, 1886; J. Talko, Klin.
Monatsbl. f. Augenh., xv. 137, 1877; D. Webster, Arch. Pediat.,x\. 681
1894; L. Weiss unci M. Goerlitz, Arch. f. Augenh., xxxiii. 101, 1896;
H. W. AVilliams, Roston Med. and Surg. Journ., xlii. 421, 1850.

Hydrophthalmus Congenitus.

If not actually an embryonic malformation of the eye it would
seem that hydrophthalmus congenitus is generally the result of such
a malformation. Under this name or under the synonymous terms,
megalophthalmus, huphthahnus (ox-eye), and congenital glaucoma, a
considerable number of cases have been reported in which the
eyeball is enlarged and has its tension increased. The front of the
eyeball is more globular than normal : the sclerotic is thinned with
the pigment shining through and giving it a bluish appearance,;
the cornea is enlarged ; the anterior chamber is, in most instances,
deep, but may on the other hand be absent through the iris 1 icing in
contact with the cornea; and the optic nerve is cupped. The ob-
struction to the circulation in the eye would appear to be due to a
want of separation of the iris from the cornea in the region of the
ligamentum pectinatum ; a stretching of such an adhesion may occur
as life goes on and so a spontaneous cure take place. In E. von
Hippel's case {Arch. f. Ophth., xliii. 3 Abth., 530, 1807) there were

COLOBOMA OF EYELID 411

signs of former irido-cyclitis and parenchymatous keratitis : the
lens was very small ; and there was nearly complete absence of
Schlemm's canal. I may add here some references to this interesting
ocular anomaly, which lies on the border-line between the embryonic
malformations and the ftetal diseases. F. Arnold, Beitr. z. Augenh.,
216, 1890-91 ; F. R. Cross, Trans. Ophtk. Soc. Unit Kingdom, xvi. 340,
1895-96; Diirr and Schlegtendal, Arch. f. Ophth., xxxv. 2 Abth., 88,
1889 ; M. Dufour, Bee. d'opkth., 3 s., iii. 674, 1881 ; C. Gallenga,
Ann. di offal., xiv. 322, 1885-86 ; C. Grahamer, Arch f. Ophth., xxx.
3 Abth., 265, 1884 ; 0. Hassc, Diss, inaug., Strassburg, 1892 ; Manz,
Amtl. Ber. ii. <l. Versamml. deutsch. Naturf. a. Aerzte, lvi. 252, 1884;
If. Schirmer, Klin. Monatsbl. f. Augenh., ix. 250, 1871; C. Schcene-
mann, Arch. f. Augenh., xlii. 174, 1900: and F. Vianey, Echo mid.,
Toulouse, 2 s., xv. 89, 1901.

Coloboma of Eyelid.

By Blcpharocoloboma or Coloboma of the Eyelid is meant a vertical
fissure or defect of one or other or both eyelids. It is comparatively
common, and it may occur in association with facial fissures (q.v.) or
alone. I have met with several instances of it (Figs. 25, 26, 30), and
Tarutfi collected from literature forty-six cases in which it occurred
apart from fissures of the cheek (op. cit, vi. 456, 1891). When it is
present alone it is more common in the upper than in the lower eye-
lid ; but if the cases associated with genal clefts be included there is
no marked disproportion between the two lids. It usually involves
the whole thickness of the lid ; it varies in size from a small notch in
the palbebral margin to a wide triangular or cpuadrangular cleft ; in
the lower eyelid it usually has the shape of a V, and that of an
inverted V in the upper. Only one lid may be affected ; but two,
three, and even all the four have been found fissured. The coloboma
generally is situated towards the inner side of the middle line of the
eyelid, less commonly in the middle line, and rarely in the outer half.
The margins of the cleft may be smooth or provided with eyelashes,
white as if cicatrised or red, and sometimes a membranous band
(amniotic ?) passes to the eyeball to be attached to the cornea.
Sometimes a dermoid tumour of. the eyeball has been found lying in
the cleft. Among the associated malformations are facial, genal,
nasal, and labial fissures, defect in the eyebrow or in the skin of the
forehead, symblepharon, microphthalmus, coloboma of the iris and
choroid, and preauricular appendages. The teratogenesis of coloboma
palpebral is difficult to understand. It is not a simple arrest, for
there does not seem to be any stage in ontogenesis to which it corre-
sponds, although when it is combined with a genal fissure it may
represent an extension of its upper end. It is probable that it is due
to an adhesion of the amnion to the eyeball which prevents the
growth of the lid at that part. There are difficulties in the way of a
complete acceptance of this view also, but it is supported by a
number of facts, and in one at least of my cases an amniotic band
was actually present.

412 ANTENATAL PATHOLOGY AND HYGIENE

In addition to the numerous references given by Tarufh' (op. cit.)
I may name the following : — L. A. W. Alleman, Ann. Ophth., St. Louis,
vii. 18:3, 1898 ; A. Berthaud, Lyon med,, lxxiii. 288, 1893; A. Brink-
mann. Diss, inaug., Mlinchen, 1887; M. Capel, Sperimentale, Ann.
xlvi. 229, 1892; G. Gowell, Trans. Ophth. Soe. Unit, Kingdom, xi.
214, 1890-91; H. Dor, Rev. gen, d'opht, vii. 529, 1888; W. Gold-
zieher, Centrlbl. f. prakt. Augenh,, xx. 359, 1896; J. Hoppe, Arch. f.
Ophth., xxxix. .".07, 1893; Arch, f. Augenh., xxxvii. 354, 1898; J.
Krischewsky, Diss, inaug., Wiirzburg, 1894; H. Juler, Trans. Ophth.
Soe. Unit Kingdom, xii. 171, 1891-92 ; W. Lang, ibid., vi. 488,
1885-86; J. Nicolin, These, Lyon, 1888; Osio, Rev. de enferm, dc
ninos, i. 115, 1883; E. Owen, Trans. Med. Soe. Lond., xiii. 204,
1889-90; Schanz, Deutsche med. Wchnschr., xxiv. 288, 1898; S.
Snell, Trans. Ophth, Soe. Unit. Kingdom, iv. 348, 1883-84; Zeilen-
dorf, Wien. Bin. Wchnschr., vii. 729, 1894.

Miscellaneous Malformations of the Eyelids.

Complete absence of the eyelids (true dblepharon) is almost
unknown save in association with other grave defects and mon-
strosities; this condition is not to be confounded with cryptoph-
thalmus in which the eyelids are absent only in the sense that they
are represented by a continuous cutaneous covering. Congenital
shortness of the eyelids, especially of the upper ones, is known as
microolepharon ; the palpebral fissure on this account is incompletely
closed (lagophthalmus or " hare-eyed," this animal being supposed to
be unable completely to close its eyes). Blepharophimosis, or small-
ness of the palpebral aperture, is usually associated with and caused
by microphthalmus and anophthalmus.

A supernumerary eyelid (polyblepharon) has sometimes been met
with. To an accessory (semilunar) fold of scleral conjunctiva on the
nasal side of the eye the name of third eyelid (palpebra tertia) or
membrana nictitans has been given ; it is supposed to represent the
structure which is found in a fully developed form in birds and some
reptiles. Another sort of supernumerary eyelid in the human subject
has also been described : it is a conjunctival fold at the outer angle
of the eye, and may be regarded as a fourth eyelid (palpebra quarta).
Case-records have been published by Dubois (Ann. d'ocul, xxxiv. 208,
1855), Fano (ibid., xlix. 23, 1863), and J. Larcher (These,^ Paris,
1889). Congenital ectropion and entropion have been met with, but
are very rare ; the former is associated with ftetal ichthyosis
(ride Vol. I. p. 308); and the latter is peculiar in that the inturned
eyelashes do not rub against the cornea with their points, but
laterally, and so give rise to little irritation. A second row of eye-
lashes (distichiasis) has been found.

Epicanthus is a cutaneous fold found sometimes covering either
the inner or outer canthus or angle of the eye. The internal form is
generally due to a slight nasal defect, and is present almost constantly
in the Mongolian race. It gives to the eye the appearance of con-
vergent strabismus, and it disappears when the skin of the root of the

LACHRYMAL ANOMALIES 413

nose is pinched tip. It may occur, but rarely, over the outer angle
of the eye (Sichel, Ann. d'ocul., xxvi. 20, 1851). Various operations
have been devised for the removal of the deformity (F. A. von
Amnion, Ztschr. f. d. Ophth., i. 533, 1830; Carron du Villards,
Bull. gat. de tUrap., xv. 45, 1838 ; W. Frobelius, Med. Ztg. Buss-
lands, iv. ^\Wo, 1847; St. Pctcrsb. Med. Ztschr., i. 322, 18G1 ; H. H.
Walton, Med, Times and Gaz., n.s., vii. 218, 1853 ; H. Knapp, Arch.
Ophth. and Otol., iii. 48, 1873; E. Berger, Arch. d'opht,, xviii. 453,
1808 ; If. Kuhnt, Ztschr. f. Augenh., ii. for 1800, 160).

Associated with epicanthus it is not uncommon to find a drooping
of the upper eyelid or ptosis congenita : but this anomaly may also
exist apart from an epicanthic fold. I have recently met with an
instance of it in a mother and infant in the Eoyal Maternity Hospital,
Edinburgh ; it was the right eye in both, and it was stated that the
mother's mother had the same anomaly. I have also in my possession
the photograph of a little girl with bilateral ptosis palpebrarum,
which Dr. J. Crawford Dunlop gave me. It may be associated with
microphthalrnus and coloboma as well as with epicanthus. It is com-
monly due to developmental defects in the muscles : it may, as in my
case, be hereditary, and it may also show family prevalence (Du jardin,
Journ, d. sc. mdd. de Lille, xvii. 501, 1804). The bilateral form is
more common than the unilateral. Various operations have been
attempted for the relief of the deformity due to blepharoptosis.
Freeland Fergus {Brit. Med. Journ., i. for 1001, 702), for instance,
i nukes a horizontal incision in the position of the eyebrow, dissects
out a vertical band of the tendon and fascia of the occipito-frontalis,
and secures it to the upper eyelid as near as possible to its margin.
Various articles dealing with congenital ptosis have appeared ; the
following may be enumerated : — G. Ahlstrom, Beitr. z. Augenh., xvi.
51, 1804: V. Caudron, Rer. gin. d'opht, iv. 241, 1885: A. Dehenne,
Bull, et mem. Soc. franc, d'opht., ix. 83, 1801 : A. von Graefe, Arch. f.
Ophth,, ix. 2 Abth., 57, 1803; E. M. Gunn, Trans. Ophth. Soc. Unit.
Kini/dom, iii. 283, 1882-83; C. A. Hall, Arner. Journ. Med, Sc, n.s.,
xii. 143, 1840 ; E. B. Heckel, Interned. Clin., 4 s., i. 307, 1804: J. H.
Jackson, Lancet, i. for 1804, p. 11 ; V. Miller, Brit, Med, Journ,, i. for
1808, p. 1250 ; F. Mitry, These, Paris, 1885 ; E. Pereyra, Sperimentale,
xlvii. 3,84, 1881 : T. Proskauer, Centrlbl. f. prakt. Aue/enh., xv. 07,
1801: E. Eampoldi, Ann. di ottal., xiv. 40, 1885-80; xv. 54, 1880-
87 ; E. Siemerling, Arch. f. Psychiat., xxiii. 704, 1801-02 ; W. N.
Sinclair, Ophth, Rev., xii. 80, 1803 ; Steinheim, Klin. MonatsU. f.
Augenh., xv. 00, 1877; F. Tartuferi, Rir. din. di Boloe/na, 2 s., ix.
337, 1870 ; Thomas, Johns Hopkins Hosp. Bull, vi. l04, 1805 ; S.
Watson, Brit. Med. Journ., i. for 1800, p. 122. For references to
the various operations of Panas, Wolff, Kunn, and Mules, vide the
Index Cedaloejuc, 2 s., v. 410, 1000.

Malformations of the Lachrymal Apparatus.

Malformations of the lachrymal apparatus (gland, duct, sac,
canaliculi) are usually associated with other grave ocular defects

414 ANTENATAL PATHOLOGY AND HYGIENE

(cryptophthalmus, anophthalmia, etc.) ; rarely do they occur alone.
Perhaps, however, absence of one gland is implied by unilateral
absence of tears (A. S. Morton, Trans. Ophth. Soc. United Kingdom,
iv. 350, 1883-84): complete absence of tears has also been recorded
(J. Solomon, Gaz. d. hop., xxviii. 22, 1855), and may mean bilateral
absence of the gland : on the other hand, these anomalies may be
purely functional. Instances of fistula of the lachrymal sac (due to
incomplete closure of the embryonic groove) have been recorded (W.
Rider, Arch. Ophth., New York, xiii. 263, 1884 : J. Dunn, Arch. Ophth.,
xxiv. 251, 1895); fistula of one or other of the canaliculi has also
been noted (Behr, Klin. Ztschr. f. Chir. u. Augenh., i. 456, 1836-37).
There may be absence or imperforation of one or more of the puncta
lacrymalia (S. M. Burnett, Arch. Ophth., xiii. 53, 1884: E. Emmert,
Arch./. Augenh. u. Ohrenh., v. 399, 1876: Magnus, Klin. Monatsbl.f.
Augenh., xm. 199, 1875); on the other hand, supernumerary puncta
have been met with (C. E. Fitzgerald, Ophth. Hosp. Rep., Lond., viii.
427, 1874-76 : A. von Graefe, Arch. f. Ophth., i. 1 Abth., 288, 1854-
55 ; F. Ponti, Gior. d'oftal. ital., ii. 97, 1859 : F. Raab, Klin. Monatsbl.
f. Aivgenh., xiii. 331, 1875). It may also be noted that the lachrymal
caruncle may be the site of dermoid growths.

Malformations of the Cornea.

Complete congenital opacity of the cornea may be found in
association with both hydrophthalmus and microphthalmus, and is
then probably due to an anomalous arrangement of the constituent
layers of the membrane (A. Tepljaschin, Arch. Ophth., xxvi. 64, 1897).
Localised opacities are sometimes of a dense white character and may
be near the corneal margin, where they may form isolated areas or
constitute a ring (" arcus juvenilis," "macula arcuata"). Many
instances of congenital corneal opacity have been reported, among
which may be mentioned those by E. Fuchs ( Wien. med. Bl., xvii.
197, 1894), by R. Hilbert (Arch.f. path, Anal, cxxxi. 182, 1893), by
H. Moulton (Journ. Amer. Med. Assoc., xxx. 247, 1898), by F. Ruckert
(Ztschr. f. vergleich. Augenh., hi. 102, 1885), by J. Santos Fernandez
(Trans. Amer. Ass. Obst. and Gynec, v. 424, 1893), and by 0. Wernicke
(Ann. d'ocul., cxvi. 278, 1896). Congenital clouding of the cornea in
two sisters was recorded by R. L. Randolph (Amer. Journ. Med. Se.,
n.s., xcvi. 570, 1888), and in mother and son by C. A. Oliver (Ophth.
Rev., xi. 349, 1892). G. Saltini's record of three brothers suffering
from the anomaly was remarkable (Boll, d'ocul,, x. 67, 1888) : he
ascribed the corneal clouding to foetal interstitial keratitis, and
possibly the condition is a foatal disease rather than an embryonic
malformation. Of course, we must be careful to exclude instances
due to ophthalmia neonatorum from the category of antenatal cases.

Enlargement of the cornea {macroeoryiea) occurs in association
with hydrophthalmus, and abnormal smallness (microcornea) in
microphthalmus and also as the sole anomaly. Conical (dome-shaped)
cornea may be an antenatal malformation (Gescheidt, Ztschr. f. d.
Ophth., ii. 483, 1832 ; A. Dujardin, Journ. d. sc. mid. de Lille, ii. 629,

MALFORMATIONS OF IRIS 415

1880; S. L. Frank, Maryland Med, Journ,, x. 273, 188:1-84; 0.
Pincus, Diss, inaug., Konigsberg, 1887; and Steinheim, Centrlbl, f.
prakt. Augenh., xxi. 353, 1897); J. Tweedy (Trans. Ophth. Soe. Unit.
Kingdom, xii. 67, 1891-92) is even of the opinion that the cases which
appear in adult life are due to some predisposing embryological
defect. Dermoid tumours of the cornea may be met with alone or in
association with other ocular defects, such as coloboma of the lids ;
long hairs may grow from them in later life.

Malformations of the Iris.

Colour anomalies of the iris are not uncommon ; they are rarely
in existence in antenatal life, although the predisposition to develop
them may be. At birth the colour is in all infants a greyish blue
(due to the thinness of the iris tissue and the absence of pigment
from its mesoblastic part), and anomalies in pigmentation come into
existence later. The two irides may be of different colours (hetero-
chromia): a mulatto with one brown and one blue eye was described
by Bruns (New Orleans Med. and Surg. Journ., n.s., xvi. 110, 1888—
89), and the subject has also been dealt with by W. G. Sym (Ophth.
Rev., viii. 202, 1889) and J. Forrester (Med, Bee, xlii. 399, 1892). An
abnormal extension forward of the uveal pigment may cause dark
nodules at the pupillary margin of the iris; this is "ectropion of the
uveal pigment" (B. Wicherkiewicz, Arch. f. Ophth,, xxxvii. 1 Abth.,
204, 1891). In albinism, that curious state which is sometimes
hereditary and often shows family prevalence, the general absence
of pigment is associated with local absence of it in the iris ; the
eyes of albinos, therefore, appear pink. It has been noted in
twins (J. N. Hyde, Internat. Med. Mag., i. 501, 1892), and among
negroes (P. Topinard, Assoc, frang. pour Variance d. se., eompt. rend.
1878, Paris, vii. 850, 1879 ; Smester, Rev. d'anthrop., 2 s., ii. 675
1879; etc.). The albino eye has been specially studied by Broca
(Bull. Soe. d'anthrop. de Paris, y. 141, 1804), by W. Manz (Arrh.
f. Ophth,, xxiv. 139, 1878), and G. M. Gould (Philadelphia, 1894);
the iris tissue is of a grey colour, and the pink appearance is due
to the interior of the eye seen through it.

The pupil may be slightly or markedly eccentric in position
(eoreetopia) ; it may be associated with ectopia of the lens ; cases are
reported by A. Jaczynski (Diss, inaug., Griefswald, 1889), Badal
(Bull, Soc. d'anat. et physiol. de Bordeaux, xii. 253, 1891), W. M.
Beaumont (Trans. Ophth, Soe. Unit. Kingdom, xiii. 146, 1892-93), A.
Antonelli (Atti di XI Cong. med. internaz., vi. oftal, 73, 1895), and
by J. Brixa (Klin. Monatsbl. f. Augenh,, xxxvi. 432, 1898). The
pupil may be abnormally small {microcoria) ; this may be due to foetal
iritis and the formation of posterior synechias ; or it may be an original
malformation. Again, there may be more than one pupil or polyeoria,
an anomaly which may arise through subdivision of the normal pupil
(e.g. by a band of the persistent pupillary membrane) or by the
appearance of supernumerary openings in the iris especially near its
periphery. Numerous instances have been reported, including those

416 ANTENATAL PATHOLOGY AND HYGIENE

by H. Wilson {Med. Time* and Gaz., i. for 18G0, 392), P. D. Keyser
(Med. and Surg. Reporter, xliii. 30, 1880), W. F. Mittendorf (Trans.
Amer. Ophth. Soc, iii., pt. 5, 735, 188;")), G. E. de Schweinitz (ibid.,
vi. 59, 1891-93), Fouchard (Arch. mid. d' Angers, i. 631, 1897), and
E. Wingenroth (Centrlbl. f. prakt. Augenh., xxiii. 105, 1899). In
Mittendorfs observation the defect was hereditary (father and
daughter).

A greater or less degree of persistence of the pupillary membrane
of antenatal life is a comparatively common malformation of the iris
(about 1 per cent.) ; it is also, as so many of the eye anomalies are,
a malformation arising not in embryonic but in late foetal life. It
usually takes the form of fibres and tags, rarely that of a membrane ;
the fibres may constitute loops, or each may float from the iris with
one end free, or they may pass to the capsule of the lens or adhere
to the posterior surface of the cornea (Van Duyse, Arch, d'opht.,
Avril, 1902, Extrait). A large literature relating to persistence of
the pupillary membrane has grown up since Haller's dissertation on
the subject appeared in 1758 (Med. Chir. and, Anat. Cases, London,
p. 122, 1758); references to many of the articles are in the Index
Catalogue, xi. 860, 1890. Some recent cases are those of F. E. Cross
(Clin/ Sketches, i. 104, 1895), of W. F. ■ Norris (Trans. Amer. Ophth.
Soc., vii. 594, 1897), and of M. Linde (Centrlbl. f. prakt. Augenh., xxi.
81, 1897). The persistent tags vary in colour (grey, black, brown).

Absence of the iris (irideremia or aniridia) is another frequently
recorded ocular anomaly. Long lists of references are in the Index
Catalogue (vol. vii. p. 108, 1886, and second series, vol. viii. p. 343,
1903). The absence may be complete, the blackness of the pupil
extending where the coloured iris ought to be ; it may also be partial,
and nodules or crescentic pieces of iris may be recognised. This
anomaly has been explained in various ways, as by absence of the
anterior ciliary arteries, by absorption (along with the pupillary
membrane) in fcetal life, and by arrested development (due, perhaps,
to strong union of the lens to the cornea from fcetal choroiditis). It is
often associated with other ocular defects (e.g. ectopia of the lens, catar-
act, coloboma), and may show family prevalence (Henzschel, Ztschr. f.
d. Ophth,, i. 52, 1830-31 ; V. Caudron, Rer. gen. d'opht, vi. 16, 1887).
It may also be transmitted hereditarily (Galezowski, Rec. d'ophth.,
.". s., ii. 122, 1880; J. A. Andrews, Ophth. Rec.,n.s.,vii. 546, 1898),
and may be met with in the children of parents with coloboma of
the iris (S. Theobald, Trans. Amer. Ophth. Soc, v. 99, 1888-89; De
Beck, Arch, Ophth., xv. 8, 1886 : xxiii. 264, 1894).

The pupil may be altered in shape by absence of a portion of the
iris (coloboma of the iris). The defect is usually in the lower part of
the iris, but may be in other situations. Like coloboma of the eyelid,
it may show all the degrees, from a slight notch in the margin up to
complete absence of a segment of the iris. It may be associated
with other defects of the eye, as with coloboma of choroid, microph-
thalmus and aniridia (of the opposite eye), as in the case seen by me
in 1895 (vide p. 409). Heredity has been noted in connection with
it (M. Piosskothen, Diss, inaug., Kiel, 1898; F. P. Maynard, Indian

MALFORMATIONS OF CHOROID 417

Med. Gaz., xxxii. 456, 1897; J. F. Streatfeild, Ophth. Hosp. Rep.,
London, i. 153, 1858). Its teratogenesis is rather obscure, as there is
no embryonic fissure in the iris whose persistence might be supposed
to give rise to it ; probably it results from defective closure of the
fissure in the choroid and ciliary body, although this hardly explains
the cases in which the coloboma affects the upper part of the iris ;
it may also be due to partial adhesion or late separation of lens and
cornea.

"Figures on the iris" are due to irregularities in pigmentation or
structure. In the case described by A. Claus (Flandre mM., i. 65,
1804), the figures were "10" in the left eye and "45" in the right.
Doubtless these irregularities in the iris have given origin to the
tales of infants with names (e.g. Napoleon) in their eyes, and to the
curious story related on page 120 of this volume (q.v.).

Malformations of Choroid.

Part of the choroid, usually of an ovoid shape, may be absent,
giving rise to the anomaly known as coloboma of the e/ior<>i<t. No
trace of this defect may be visible from the outside, but by means of
the ophthalmoscope the exposed sclerotic is evident as a pearly white
patch. Its situation is usually in the line of the so-called choroidal
fissure (vide pp. 54, 66); but in C. A. Veasey's case (Arch. Ophth.,
xxiv. 202, 1805) there was a double coloboma with a wide area of
intervening healthy choroidal tissue, an arrangement very difficult
to explain on the theory that such defects are due to want of closure
of the fissure. It may coexist with coloboma of the iris and ciliary
body; it may extend to the sheath of the optic nerve (A. H. Benson,
Dublin Journ. Med. Sc., 3 s., lxxiii. 177, 1882): and it may affect the
macula (S. M. Burnett, Arch. Ophth., xi. 461, 1882 ; Van Duyse, Ann.
d'ocul, xcvi. 139, 1886 ; ibid., xcviii. 108, 1887). Macular coloboma
has been noted also in the cyclopic eye (Van Duyse, Arch. oV ophth.,
Extrait, jan.-fevr. 1800). It may lie present in both eyes (J. Eich-
hoff, Diss, inaug., Bonn, 1878), or there may be a coloboma in one
eye and aniridia in both (A. de Montmeja, Rev. phot. d. hop. d. Paris,
iv. 48, 1872). The malformation has been ascribed with some prob-
ability to adhesion of the retina to the mesoblast (Fuchs). Heredi-
tary transmission has been observed. In albinism the pigment of the
choroid is absent. (For recent literature on choroidal coloboma, see
Index Catalogue, 2 s., iii. p. 623, 1808.)

Malformations of the Retina.

A comparatively common anomaly of the retina is the presence
of an additional artery in the form of a branch from the posterior
ciliary (W. Lang and J. W. Barrett, Ophth. Hosp. Rep., Loud., xii. 50,
1888): and various abnormal arrangements of the vessels on the
disk may be met with (W. A. Frost, Trans. Ophth. Soc. Unit. Kingdom,
vii. 175, 1887; ix. 142, 1880). Pigmentary defects are found in
albinism and in retinitis pigmentosa (which is occasionally congenital).
vol. .ii. — 27

418 ANTENATAL PATHOLOGY AND HYGIENE

With regard to the optic nerve, the direction of the nerve head may
be reversed, the outer or temporal pale portion becoming the inner
or nasal part. Sometimes opaque nerve fibres are discovered in the
retina (L. Monesi, Ann. di ottal., xxv. 603, 1896); they constitute
bright white areas spreading out from the upper or lower margins
of the optic disk ; the anomaly is an arrested development, being
due to the retention of the medullary sheaths around some of the
axis cylinders. It has been said above that retinitis pigmentosa
may be congenital, and several cases have been reported (Bousseau,
Bull. Soc. anat. de Paris, xlii. 740, 1867 ; G. C. Harlan, Amer. Journ.
Med. Sc, n.s., lxiv. 130, 1872; P. Lagleyze, Rev. argent, de oftal. pract.
(Buenos Aires), i. 19, 1883-84); it may be added (as a matter of
peculiar antenatal interest) that it is one of the malformations which
have been attributed to consanguineous marriages (H. Derby, Boston
Med. and Sun/. Journ., lxxii. 149, 1865 ; J. Oakley, Prov. Med. Journ.,
viii. 329, 1889), and it may show family prevalence (W. J. Cant,
Ophth. Rev., v. 245, 1886 : E. Eamboldi, Ann. di ottal., xii. 268,
1883).

Malformations of the Lens.

Aphakia (absence of the crystalline lens) may be found in asso-
ciation with microphthalmus : but it is doubtful whether it occurs
alone, the cases which have been recorded being probably instances
of displacement, not of absence (F. L. Parker, Trans. S. Car. Med.
Assoc., xxxi. 123, 1881). The lens may be unusually small, or it may
show an abnormal curvature (lenticonus) of either its anterior or
(more often) posterior surface, a somewhat rare anomaly (C. E, Agnew,
Arch. Ophth. and Otol., iv. 382, 1874-75). A defect in the margin of
the lens (coloooma lentis) may be met with, but is comparatively rare
(Schiess-Gemuseus, Klin. Monatsbl. f. Augenh., ix. 99, 1871): it is
generally associated with other anomalies, such as coloboma of the
iris and choroid ; and it is probably due to an arrested development
of the suspensory ligament (A. G. Heyl, Rep. Internal. Ophth. Congr.,
v. 16, 1876).

Ectopia lentis or congenital displacement of the lens may be in an
upward or in a horizontal direction, or backward (in microphthalmus).
It is usually bilateral, but may be unilateral. It is rare, but a suffi-
cient number of cases has been reported to enable some conclusions
to be drawn ; among these may be mentioned the records of F. W. T.
Sippell {Diss, inaug., Marburg, 1859), Wecker (Bull. Soc. de chir. de
Paris, 2 s., iv. 42, 1864), Delacroix (Bull. Soc. mid. de Reims, viii. 63,
1869), F. Duval (These, Paris, 1874), C. J. 8. Jeaffreson (Ophth. Hasp.
Rep., London, vii. 186, 1871-73), F. E. D'Oench (Diss, maw/., Wies-
baden, 1879), and J. B. Eoberts (Phila. Med. Times, xi. 280, 1880-81).
It may be hereditary, and has occurred in five successive generations
(A. S. Morton, Ophth. Hosp. Rep.,ix. 435, 1876-79); it may affect
several members of the same generation (family prevalence), as in
B. E. Fryer's record, where two sisters had double and symmetrical
ectopia lentis (Amer. Journ. Ophth., i. 54, 1884-85). The displaced

CONGENITAL CATARACT 419

lens may be small in size, and it may (rarely) show opacities in it ;
it may be associated with colobomg, and also with aniridia, and
corectopia. It is probably caused by defective development of the
suspensory ligament.

Congenital cataracts constitute an interesting group of lenticular
anomalies. The opacity may be complete, when it varies in con-
sistency (tiuid, gelatinous, dense), and it is then probably due to
persistence and thickening of the fibro-vascular sheath of the lens
and of the central artery of the vitreous humour. The cataract,
on the other hand, may be partial. It may then consist of small
scattered opacities (streaks or spots) near the periphery, giving rise
to " dotted " cataract : or it may be localised in the anterior pole of
the lens (anterior polar or pyramidal cataract), a form which is to
be distinguished from persistence of the pupillary membrane. It is
doubtful whether a posterior polar cataract can be distinguished
from persistence of the fibro-vascular sheath of the lens in that
position. Again, zonular (lamellar) and nuclear cataracts of an
antenatal nature are met with. A fairly large number of obser-
vations of congenital cataract (of various types) has now been
accumulated : over one hundred and twenty references are given in
the Index Catalogue (vol. ii. p. 752, 1881; 2 s., iii. p. 232, 1898).
Heredity and family prevalence have both been noted over and over
again in connection with the malformation, e.g. in the cases of
Hiibsch (Gaz. mtd. d' Orient, x. 146, 1866-67), H. W. Williams (Boston
Med. and Surg. Jon rii.y lix. 149, 1858-59), Baudon (Bee. d'ophth., v. 9,
1878), A. D. Williams (St. Louis Med. and Surg. Journ., xxxviii. 368,
1880), G. F. A. Appenzeller (Diss, inaug., Tubingen, 1884), H. Wilson
(J. Opkth. Otol. and Laryngol, iii. 291, 1891), D. Webster (Arch.
Pediat., xi. 905, 1894), H. F. Hansell (Ophth. Bee. (Nashville), v. 488,
1895-96), Purtscher (Centrlbl. f. prakt. Aitgenh., xxi. 198, 1897), and
of many others. In many of the recorded cases successful operations
for the antenatal blindness, resulting from the lenticular anomaly,
were carried out.

Malformations of the Vitreous.

Almost the only important malformation of the vitreous humour
is that due to persistence of the artery (central, hyaloid) which is
present in early antenatal life, and normally disappears completely
before birth. The hyaloid artery in its course through the vitreous
is surrounded in one part of its extent by a hyaline membrane and
in another by a cellular sheath, and various anomalies are produced
by the persistence of the whole or of different parts of it and of its
sheath. When the whole artery persists, the blood passing through
it probably escapes by vessels in the posterior fibro-vascular sheath
of the lens and ciliary body (Van Duyse, Arch, d'opht., Mai, 1902,
Ext rait); but it may also persist but carry no blood, and it then
stretches as a band from the optic disk to the posterior pole of the
crystalline lens, or may end freely in the vitreous being attached
either by its neural or lenticular extremity. The commonest form

420 ANTENATAL PATHOLOGY AND HYGIENE

is that in which the neural end alone remains as a thin narrow cord
lashing freely backwards and forwards in the vitreous humour. It
may be that only a remnant of the hyaloid canal (canal of Cloquet or
Stilling) persists, the artery itself having entirely disappeared (H.
Schindelka, Wien. mecl. Bl.,\\\. 358, 1884: H. Wilbrand, Jahrb. d.
Hamh. Staatskrankenanst., i. pt. 2, 430, 1890 : L. Thilliez, Jovrn. d.
sc. nn'd. de L'dlc, i. 289, 1897). Keferences to about forty articles
dealing with persistence of the hyaloid artery are given in the Index
Catalogue, vols. i. 609, 1880 ; iv. 461, 1883 ; 2 s., i. 699, 1896 : v. 249,
1900. This anomaly may be associated with other ocular mal-
formations, such as coloboma of the choroid (Mitvalsky, Arch. Ophth.,
xxiv. 192, 1895).

Malformations of the Nose.

Some of the malformations of the nose have been already
described, such as mesial fissure (p. 378) and absence of the organ,
or conversion of it into a proboscis, in cyclopia (p. 397) ; but some
other anomalies require to be noticed briefly.

Slight deviations from the normal shape and size of the nose are
common, so common as hardly to be accounted teratological. The
organ may, for instance, be too small at birth, or, very rarely, too
large and too long ; these peculiarities also may show family pre-
valence and heredity. The so-called " eamois nose" includes both
the flattened nose (in which the nasal bones and ascending processes
of the maxilhe may lie absent and the septum very defective) and the
snub or turned-up nose ("ncz retrousse"); it, likewise, may be markedly
hereditary. Paraffin has, of late years, been much used to try to
correct such deformities. The twisted or obliquely directed nose is
generally due to a deviation of the whole septum or of the anterior
(cartilaginous) part of it ; it is generally directed to the right side,
and is accompanied by narrowing of the right nostril and nasal fossaj.
Sometimes there may be a sort of scoliosis of the nose, the bones
being directed to one side and the tip to the other. The palate may
show an increased degree of arching and some unilateral irregularity ;
and there seems to be no doubt that in some instances, at least, the
deviation of the nasal septum is truly antenatal, and that it is
commoner in civilised than in primitive peoples. In the lower
animals obliquity of the nose is a more extensive anomaly, being
(in foals, pigs, and puppies) accompanied by deviation of the nasal,
intermaxillary, and maxillary bones {campylorrhinus, Gurlt): the
crossed beaks of birds are of a different nature (e.g. in the curvirostral
passeres). Inclination of the cartilaginous septum alone to one side
is a common nasal anomaly, and is associated with and perhaps
caused by hypertrophy of the mucosa covering the inferior tur-
binated bone ; but there is no reason to ascribe it constantly or even
frequently to antenatal conditions.

The whole septum (both osseous and cartilaginous) may be
absent in cases of cleft palate and defective premaxilla. In rare
cases it may show in its anterior part a. more or less circular

MALFORMATIONS OF THE NOSE 421

perforation, which would appear to he occasionally antenatal in
origin. About such congenital perforations, A. Eupp (Med. Bee.,
xlvi. 771, 1894) states that they may either be due to perverse or
arrested development (Germs' case) or to a pathological process
implanted on the developing tissues (Klebs). It is possible that the
custom among some savage races of perforating the nasal septum
may have arisen from the birth of an infant with such an antenatal
defect. In the septum above and in front of the recessus naso-
palatine may be found more or less marked traces (blind pouch,
etc.) of the organ of Jacobson (A. Kolliker in F. von Rinecker's
Festschrift, 1, 1877 : G. Romiti, Boll. d. Soe. tore i cult. d. sc. tried, in
Siena, i'i. 1G9, 1884; E. Schmidt, Diss, inaug., Berlin, 1896; M.
Mangakis, Anat. Ariz., xxi. 106, 1902).

Another antenatal malformation of the nose is the stenosis or
atresia of the nares, anterior or posterior, unilateral or bilateral.
The anterior nares may be narrowed or completely closed by
membranous, cartilaginous, or even by osseous tissue on one or both
sides (AV. C. Jar vis, Trans. Airier. Laryngol. Assoc, ix. 222, 1888).
Congenital occlusions of the posterior nares or choanal would seem
to be less rare. The occlusion may lie osseous, as in the cases of
H. Luschka (Arch. f. path. Anat, xviii. 168,1860), Santesson (Fork.
v. Svens. Lak. Sallsk. Sammank., Stockholm, 303, 1866), Schrbtter
(Monatschr. f. Ohrenh., xix. 97, 1885), and A. B. Thrasher (Med. News,
lxvii. 79, 1895); and the bony anomaly may affect only one side, as
in the cases of Somnier (Wien. med, Presse, xxiv. 476, 1883) and E.
Zaufal (Frag. med. Wchnschr., i. 837, 1876). The occlusion may be
membranous, as in Bonaldson's interesting observation (Trans.
Edinb. Obstet. So'c, vi. 48, 1881). Stenosis or atresia at some point
between the anterior and posterior nares has also been recorded ;
thus, there may be an adhesion between the middle or inferior
turbinated bone and the septum (Bryant, Lancet, ii. for 1861,
p. 207).

Median fissure of the nose has been already described, and I have
also referred to lateral nasal fissures in the paragraphs devoted to
hare-lip and oblique facial fissures. A median dermoid cyst of the
nose is now and again met with, associated generally with a fistula
lower down. In November 1900 I saw a case which was probably
of this nature : the child was five months old, and had a small
lump on the bridge of the nose, with a little opening lower down
near the tip, from which some clear fluid occasionally escaped ; the
morbid state was congenital. The little patient was under Dr.
M' Bride's care for suppuration from one ear. AV. Lawrence (Loudon
Med. Gaz., n.s., i. 472, 1837-38), Bramann (Berlin, Tdin. Wchnschr.,
xxv. 1052, 1888), and others have published somewhat similar cases.

I bring this long chapter to an end with a reference to a mal-
formation for which it is difficult to find a place in any system of
classification. In April 1904 Dr. John Thomson kindly asked me
to examine a female child, seven years of age, then under his care
in the Sick Children's Hospital, Edinburgh. She was born with
teeth, and her intelligence was below the average for her age ; but

422 ANTENATAL PATHOLOGY AND HYGIENE

the most striking peculiarity about her was the wide space between
her two e)res and the thickening of the tissues of the nose. There
was also a small coloboma of one eyelid on the left side, and the
mouth was unusually wide and depressed at the corners. It seemed
to me that this little girl possibly showed a minor degree of the
newly established type rhinodyme {vide p. 233), in which there are
two superior maxilla? and two noses, but these are so fused together
as to constitute little more than an unusually wide face. The
condition is the converse of cebocephalus.

CHAPTER XXIII

Merosomatous Terata (cont.) : Malformations of the Lower Jaw and Ear ;
Nomenclature and ( llassification ; Micrognathias ; Agnathus ; Cyclotia ;
Aprosopus ; Absence of External Ear ; Preauricular Appendages ; Fistula
Auris Congenita ; Minor Malformations of the Pinna and of its various Parts
(Helix, Anti-helix, Tragus, etc.) ; Distomus and Accessory Maxillae.

The malformations of the ear and lower jaw fall to be described in
this chapter. From the ontogenetic point of view they are to be
thought of as the anomalies associated with the first branchial arch
and cleft ; they are due to imperfect development or irregular re-
arrangement of the structures composing these transitory structures.
Many of them obviously are arrested embryological processes.

Nomenclature and Classification.

The nomenclature and classification of the monstrous develop-
ments of the first branchial arch are in a chaotic state. Etienne
Geofi'roy Saint-Hilaire (Philos. anat., ii. 97, 1822) saw a case of short-
ness of the lower jaw with conjunction of the ears, with deformity
of the palate bones, and with fusion of the pterygoid processes of the
sphenoid : this was in a sheep. Struck by the curious deformity of
the sphenoid, Saint-Hilaire called the monstrosity sphcnocephalns,
and so initiated a series of terminological and classificational diffi-
culties. Isidore Saint-Hilaire (Histoire cles anomalies, ii. 420 et seq.,
1836) retained the name sphenocephaly for one genus of his family
of otocephalians. The otocephalians, according to this author, are
those in which the ears are approximated or united in the middle
line, there is more or less marked atrophy of the lower part of the head,
and the jaws and a great part of the face may be absent. They are
subdivided into three groups, according as there are two distinct and
separate eyes, a single eye or two united in one orbit, or no eyes at
all. In the first of these three groups is the genus of the spheno-
cephalians, with the ears approximated or united under the head,
and the jaw and mouth distinct. In the second are three genera —
(1) the otocephalians proper, in which, in addition to the characters
of the sphenocephalians, the eyes are fused (cyclops) and there
is no nasal proboscis: (2) the edocephalians, in which there is a
nasal proboscis above the single eye, the ears are approximated
or united under the head, the maxillae are atrophic, and there
is no mouth ; (3) the opocephalians, in which there is no proboscis,

423

424 ANTENATAL PATHOLOGY AND HYGIENE

no mouth, atrophy of the maxillae, a single eye, and approximation
or fusion of the ears under the head. Finally, the third group (no
eyes) contains the single genus of the triocephalians, in which the
ears are again approximated or united under the head, the jaws are
atrophic, there is no mouth or nasal proboscis, and the eyes are
absent. As Tarurn (Storia del/a teratologia, vi. 389, 1891) has pointed
out, this system of arrangement is defective : too much attention is
paid to the state of the ears and too little to that of the lower jaw :
the name edocephalus also is absurd, for although the nasal proboscis
had been compared to the penis, there was no need to make use of
such a comparison in framing a word. Gurlt (Zehrb. d. path. Anat. d.
Haus-Saugethiere, ii. 110, 1832), meanwhile, being more impressed
by the state of the jaw than of the ears, had constructed a place in
his classificational system for nanocephalus brachygnathus (dwarf-
head with short jaw), and had recorded an instance of it in a sheep.
In 1877, Gurlt (Ueber thierische Missgeburten, 14, 1877) went a step
further, and made a new species (micrognathus) to contain the cases
in which there was apparent (not real) absence of the lower jaw, a
small longitudinal fissure to represent a mouth, and approximation of
the tympanic bones. The chief difference between brachygnathus
and micrognathus was one of degree, the lower jaw being less de-
veloped in the latter than in the former. In agnathus the lower
jaw is entirely absent.

Into this chaos of discordant classifications and names Taruffi (op.
tit., vi. 391, 1891) has tried to bring some order. He makes one group
to include all the malformations showing defective development of
the lower part of the face, and calls it hypoprosopo-aplasia, and sub-
divides this into hypomicrognathus (small lower jaw) and hypo-
agnathus (absence of lower jaw). He, also, has two separate groups :
one for cyclops and hypoagnathus combined in the same specimen (fused
eye and absence of lower jaw), and the other for the foetuses whose
faces have no eyes, no nose, and no mouth (aprosopus). Taruffi's and
Saint-Hilaire's classifications cannot be entirely reconciled, for, while
hypomicrognathus corresponds to sphenocephalies, aprosopus to trio-
cephalus, and cyclops hypoagnathus to opocephalus and edocephalus,
hypoagnathus and otocephalus are left unrelated. Recently, L. Blane
(Journ. de I'anat. et physiol., xxxi. 187, 288, 1895) has expanded the
Hilairean system into a still more complicated arrangement, has
added three new types, and has endeavoured to show that the genus
otocephalus of Hilaire's family of the otocephalians does not belong
to that family at all, but is an intermediate type (cyclotia) between
otocephalus and cyclopia. Blanc, like all modern teratologists, finds
an embarrassment in intermediate types, very disturbing to attempts
at exact taxonomy. I append Blanc's classificational system, which
applies to the lower animals largely.

I. Transitional Types, including specimens of (1) defect of formation
of the lower jaw (membranous state) without arrest of development
of the first branchial arch, and (2) arrest of development of the
first arch without other deformities than nasal and buccal occlusion.

OTOCEPHALUS 425

II. Otocephalus —

Group 1. Drain well formed.

Type 1. Ageniocephaly — absence or rudimentary state of
lower jaw, occlusion of isthmus of fauces, pharyn-
geal sac, approximation or fusion of middle ears,
cranium and sense organs well formed, mouth exists.

Type 2. Sphenocephaly- — absence or rudimentary state of lower
jaw, occlusion of isthmus of fauces, pharyngeal sac,
cranium and organs of vision well formed, mouth
exists ; but the superior maxilla?, squamous bones, and
zygomatic arches are bent towards the middle line
inferiorly, and the palate and pterygoid processes of
the sphenoid and the tympanic cavities are approxi-
mated or fused in the middle line. Three sub-
varieties according to state of ears.

Type 3. Agnathocephaly — absence or rudimentary state of lower
jaw, occlusion of isthmus of fauces, pharyngeal sac,
cranium and brain well formed, eyes carried to lower
surface of head, external ears approximated or
united, no mouth, upper maxillae folded and united
by their alveolar border, zygomatic arches much
approximated or fused, squamous bones, approxi-
mated or fused by their bases, tympanic cavities
approximated or fused.

Type 4. Strophocephaly — absence or rudimentary state of
lower jaw, occlusion of isthmus of fauces, pharyngeal
sac, cranium and brain well formed, eyes displaced
to lower part of face, external ears approximated or
united, no mouth, superior maxillae fused and atro-
phied, no zygomatic arch, one large orbit under
anterior sphenoid, squamous bones united and fused
under the posterior sphenoid, tympanic cavities
approximated or fused.
Group 2. Brain vesicular.

Type 5. Edocejihahj— absence or rudimentary state of lower
jaw, occlusion of isthmus of fauces, pharyngeal sac,
external ears approximated or united, no mouth,
superior maxillae fused and rudimentary, squamous
bones fused under sphenoid, nasal proboscis present,
eyes close together or fused under the anterior
sphenoid, brain vesicular, no zygomatic arch, tym-
panic cavities approximated or fused, auditory
ossicles deformed.

Type 6. Opoeephaly — absence or rudimentary state of the
lower jaw, occlusion of isthmus of fauces, pharyngeal
sac, external ears approximated or fused, squamous
bones fused under sphenoid, a single eye or one
more or less double under anterior sphenoid, no
proboscis, no bones of the face, very reduced frontal
bone, vesicular brain, tympanic cavities approximated
or fused, auditory ossicles deformed.

Type 7. Spheroceplialij — absence or rudimentary state of lower
jaw, occlusion of isthmus of fauces, pharyngeal sac,
external ears approximated or united, no bones of

426 ANTENATAL PATHOLOGY AND HYGIENE

face, no eye, no anterior sphenoid, squamous bones
and frontal very reduced, brain vesicular, tympanic
cavities approximated or fused, ossicles deformed.

Group 3. No Brain.

Type 8. Trioceplialy — cranium reduced to the occipital, the
petrous temporals (approximated or fused in middle
line), and to a rudimentary parietal ; no mouth, no
eye, no nasal proboscis ; external ears much approxi-
mated or united ; brain reduced to medulla or pons
and medulla.
HI. Transitional Type. — Cyclotia — shows combination of essential

characters of both cyclopia and otocephaly.

I have given Blanc's arrangement in full in order that the
reader may, by perusing it, gain some notion of the kind of malforma-
tions associated with defect of the first branchial arch, but it is too
complicated as a scheme for use in human teratology, indeed some
of the types described in it have not been recorded in the human
foetus, although, of course, they may yet be. ' I shall employ here
the much simpler classification of Taruffi (op. cit., vi. 388 et seq., 1891),
and shall discuss, in order, micrognathus (sinallness of lower jaw),
agnathus or hypoagnathus (absence of lower jaw), cyclops hypo-
agnathus (cyclops with absence of lower jaw), and aprosopus
(absence of lower jaw and of several other parts of the face).
Thereafter I shall take up some anomalies of the ear which
hardly deserve the designation of monstrosities.

Micrognathus.

The leading character of this monstrosity is, as the name
indicates, sinallness of the lower jaw ; there is a retreating chin of an
exaggerated type, and a state of matters is produced which is the
exact opposite of cranium progcnium in which the chin projects. The
deformity may be bilateral or unilateral ; and it may be of medium
degree (leptomicrognathus of Taruffi) when it is a simple shortness of
the jaw, or of a high degree, when it is usually associated with actual
defects in the jaw.

Lannelongue and Menard (Affection* congdnitales, i. 423, 1891)
have described an interesting series of cases of bilateral micrognathus.
In one of these, a little girl two years old, the upper part of the face
was normal in appearance, but below the mouth there was malforma-
tion. The lower lip lay on a plane posterior to the upper, and, the
lower jaw failing to make the usual projection, the cheek passed
directly into the neck. The mandible was both displaced backwards
and atrophic, and there was a distance (antero-posteriorly) of about
half an inch between the teeth of the upper and those of the lower
jaw. The child was unable to separate the jaws, and even under
chloroform the separation could not be carried out; so in all
probability the glenoid cavity and the condyle of the jaw were
malformed. The clinical result of the malformation was difficulty in
feeding the child. In two other somewhat similar cases in much

MICROGNATHUS 427

younger infants this difficulty was intensified by the coexistence of
cleft palate, and resulted in death (in one case) at the age of twenty-
three days ; the tongue was small in size and was accommodated in
the nasal fossre. In yet another case there were other anomalies
(double club-foot, muscular atrophy of lower limbs, scoliosis, and
cicatrices of intrauterine adhesions over elbows and knees), pointing,
perhaps, to an amniotic teratogenic cause. Sometimes the micro-
gnathy is unilateral, and may then be erroneously regarded as an
instance of congenital dislocation of the temporo-maxillary joint. E.
Canton's case {Trans. Path. Soc. Lond., xii. 237, 1860-61) was an
instance of this : the left external ear was represented only by a fold
of skin, the squamous temporal was flattened, zygomatic process was
absent, the glenoid cavity showed a smooth surface and no cavity,
and the left side of the lower jaw had no ramus and was only of the
size that it ought to be at the age of two and a half years, although the
individual was sixteen years old. I saw a child with a somewhat
similar condition in November 1896 ; he was the sixth child of parents
with eight children, none of whom were deformed save himself ; the left
side of the face was atrophic and the parts affected were the superior
maxilla, the malar bone, and the mandible ; the external ear of the
same side was malformed and showed two fistulous openings, but
there was no proper meatus. The patient was under the care of the
late Dr. George Elder, and was No. 181 in my series of teratological
records. Taruffi {op. cit,, vi. 393, 1891 ; viii. 538, 1894) has collected
together sixteen observations similar in nature to the above, including
a personal observation, and the cases of C. Adam {Diss, inaug.,
Kimigsberg, 1887) in which the external ears and upper limbs were
also deformed, of Maurice {Ann. Soc. de me'd. de St. Etienne et de
la Loire, i. 696, 1861), and of A. Marc {Diss, inaug., Tubingen, 1892)
in which the floor of the mouth was defective and the tongue was
prolapsed under the chin.

Of the more advanced type of micrognathus Taruffi {op. cit., vi.
395, 1891) collected together thirteen cases; to which may be added
0. Burger's observation {Arcli, f. Gyncek., lxviii. 295, 1903) in which,
in addition to absence of the right half of the lower jaw {hemignathus)
and right submaxillary gland, there was congenital amputation of the
right arm at the wrist and of the left arm at the elbow. In A. Duges'
observation {Rev. med. /rang, et etrang., iv. 407, 1827) it wras the left
half of the jaw that seemed to be absent. In two instances reported
by Virchow {Arch, f. path. Anat,, xxx. 221, 1864) there were
associated malformations (encephalocele, spina bifida, absence of
radius, defective ears, preauricular appendages) along with a rudi-
mentary condition of the mandible on one side. Not only is one side
(or both sides) of the lower jaw small in size, but the condyle and
sometimes also the coronoid process show deformities (B. Langenbeck,
Arch, f. Tclin. Chir., i. 451, 1861), as may the glenoid fossa and
zygomatic process of the temporal bone (F. Trendelenburg, Verlet-
zungen und chirurgische Krankheiten des Gesichts, 1886). Other
instances of this malformation have been reported by A. Ogston
{Glasgoiv Med, Joum., 4 s., vi. 289, 1874), by S. Goldenstein {Arrit

428 ANTENATAL PATHOLOGY AND HYGIENE

de dSveloppement de la mccchoirc infe'rieure, Paris, 1879), J. Wolff
(Berl. Idin. Wchnschr., xxxiv. 256, 1897), and E. Kirmisson {Compt.
rend. Soc. d'obst., de gynee,, et de pcediat. de Paris, iv. 153, 1902).
Finally, it may be noted that the lower jaw may be more defective
than in any of the cases described, may have neither angles nor
alveolar borders, and may be fixed or "soldered" on to the superior
maxilla ; this was the state of matters in L. Calori's specimen {Novi
Com ni. lust if, Bonon., ix. 29, 1849), which showed also no mouth, no
uterus, no vagina, no anus, and had some digits wanting in the hands
and feet.

Agnathus.

Agnathus is that monstrosity in which there is absence of the
lower jaw. Taruffi prefers to call it hypoaejnathus, to distinguish it
from absence of the upper jaw, for which he reserves the name epi-
agnathus, but the meaning of agnathus is quite well established and
does not need this elaboration ; further, the term epignathus is
already in use for a type of parasitic twin, and is apt to be confused
with Tarutfi's epi-agnathus.

It is necessary here to refer to an extraordinary fact in Compara-
tive Teratology for which no sufficient cause would appear to be
forthcoming : 1 mean the great relative frequency of both micrognathus
and agnathus among lambs. These monstrosities do also occur in
puppies (Otto, Lehrb. d. path. Anat., i. 184, 1814; I. G. Saint-Hilaire,
Histoire des anomalies, i. 200, 1832), in pigs (P. Eckardt, Diss, inavg.,
Breslau, 1889 ; B. Thompson Lowne, Catalogue, p. 70, 1893), in calves
(G. Jager, Arch./. Anat. u. Physiol., i. 64, 1826 ; iii. 74, 1828 ; Barrier,
Compt. rend. Soc. de biol,, 6 s., iii. 271, 1877 ; E. F. Gurlt, Ucber
thierischc Missgeburten, p. 7, 1877), and in does (G. Jager, Arch. f.
Anat. u. Physiol., i. 64, 1826 ; I. G. Saint-Hilaire, Histoire des
anomalies, i. 259, 1832); but they are numerous, out of all proportion
in lambs. Of micrognathus in the lamb we have records by G.
Jager (loc. cii., supra), by F. Eve {Journ, Anat. and Physiol., xvii. 495,
1883) in a case in which it was unilateral, by E. L. Schubarth {De
maxilla:, inferioris monstrosa parvitate et defectu, 1819), and J. Eeid
{Ann. Anat. ami Physiol., i. 27, 1850); and of agnathus in the same
animal there are the cases described by A. Duges {Bev. me'd. /rang.
<-t Strang., iv. 436, 1827), by C. L. Barkow {Nova Acta phys,.-med. Acad.
mil . curios., xv. 291, 1831), by A. G. Otto {Monstrorum Sexcentorum
Dcscriptio, p. 334, 1841), by E. F. Gurlt {Ucber thierischc Missgeburtc n ,
p. 6, 1877), who recorded as many as forty-five instances, by M. Duval
et G. Herve {Compt. rend. Soc. de biol., 7 s., iv. 76, 1883), and by
Thompson Lowne {Catalogue, p. 71, 1893). Why absence of the lower
jaw should be so frequent in the lamb is not clear, but possibly it
points to an undiscovered peculiarity in the ontogenesis of that animal ;
let comparative teratologists study this instance of predilection care-
fully, for it may contain some fact of illuminating power in this dark
subject.

In the human subject agnathus is comparatively rare. I have

AGNATHUS

429

Since Kere-
case was pub-

not personally examined a specimen of it, save that in which it
occurred along with cyclopia (Fig. 18); but Dr. lingers some time ago
sent me a photograph of a typical case, which I reproduce here
(Fig. 6Q), and in September 1900 Dr. Elizabeth M'Elney sent me a
photograph of a somewhat similar instance which had occurred in
Birmingham. Rogers' case was published in 1898 (Journ. Path, and
Bacterial., v. 137, 1898). The first reported instance seems to have
been that of T. Kerckring
(Spicilegium u t natomicum,
obs. lx.'p. 122, 1717); he
states distinctly that the
lower jaw was wanting in
a five months' foetus, and
that the figure gives a
better idea of the mon-
strosity than his descrip-
tion, but I am inclined
to put it the other way,
for the figure is sadly lack
ing in detail
kring's

lished there nave been
about twenty further in-
stances reported, including
Underbill's specimen in
which hydrocephalus co-
existed (Trim*. Edinh. Obst.
Soc, xi. 79, 1886), F. von
Winckel's twTo foetuses
(Milnchrn. med. Wchnschr.,
xliii. 423, 1896), and S. A.
Ewing's case (Intercolon.
Med. Journ. Australas.,
viii. 229, 1903).

The most striking fea-
ture of the monstrosity
is the complete absence
of a chin, the skin passing
smoothly and continuously
from just below the nose
to the suprasternal region.
There may be no external
indication of a mouth
(astonncs), or there may be a small horizontal opening (micr ostomies),
or a vertical slit (Jiypostomus), as in Eogers' specimen (Fig. 66).
The external ears are displaced downwards and are approximated
towards each other in the intranasal space where the chin ought
to be. The degree of approximation of the external ears and of the
deeper parts varies considerably : the pinnse may be only slightly
displaced downwards, there may be two auditory canals, and the

Fig. 66. — Dr. Rogers' Case of Hypoagnathus with
Synotus.

430 ANTENATAL PATHOLOGY AND HYGIENE

two tympanic cavities may be united ; or the two pinnae may be
furnished with a single median auditory canal, the tympanic
cavities may be fused, and the malleus of one side may be joined
to that of the other ; or, again, there may be the same arrange-
ment of external ears and tympanic cavities, with, in addition, the
fusion of the squamous temporals by their zygomatic processes.
Adolph Hannover {Den menneslcelige Hjerneshals Bygning ved Synotia,
Copenhagen, 1884) figures a typical specimen of the first of these
sub-varieties of agnathus, or, as lie terms it, synotia ; in another
well-reported case (Lannelongue et Menard, Affections congdnitales, i.
487, 1891) the tympanic bones were separated from each other only
by an interval of half an inch.

The upper part of the face may be quite normal. Dissection
reveals the fact that it is the first branchial arch and its associated
structures whose evolution has been arrested. At first it may well
be thought that the lower jaw is truly absent ; but a careful search
usually reveals a small fragment of bone in the middle line which
represents the mandible, and behind which is a narrow pharyngeal
sac communicating with the nasal fossae and lying in front of the
spinal column. F. von Winckel (Munvhen. mcd. Wchnsc hr., xliii. 423,
1896) specially emphasises the presence of the mandibular rudiment
in order to support his theory that the monstrosity is due to pressure
(amniotic or other) acting upon the region of the first branchial arch
and causing arrested growth, and not entire absence of the parts.
The superior maxillae are usually small likewise, the palate is narrow,
and the body and wings of the sphenoid restricted. The buccal
cavity is a cui-de-sac, having no connection with the pharynx, the soft
parts which represent the floor of the mouth being united to the
palate. There is usually a tongue, but it is often of small size, and
may with the larynx lie at a lower level than usual, but the lowness
of level is rather apparent than real. The hyoid bone may be present
or it may be lacking ; in the latter case the tongue also will be absent.
Very marked defects are found in the temporal bones : they are
displaced towards the lower part of the cranium and have no glenoid
fossae ; they may not actually meet in this abnormal position, but
in some cases they do unite so that the pinnae and auditory canals
are in contact or are fused into one (synotus) ; the tympanic cavities,
also, may be conjoined or fused into one, and their ossicles (malleus,
incus, and stapes) may be all present but in a deformed state. The
posterior nares (choanae) may be represented by a small opening or
may be quite closed; in the former case they communicate with the
pharyngeal sac. The pharyngeal sac, it may be added, sometimes
communicates with the oesophagus.

Like all monstrosities, agnathus may occur in association with
other malformations in the head or in more distant parts. Its com-
bination with cyclopia is so important that it is described as a special
type. Transposition of the viscera was noted in A. K. Hesselbach's
case (cited by A. Forster, Die Missbildungen des Alaisehcit,^. 95, 180f>),
and in those of A. W. Otto and Faesebeck (cited by F. Ahlfeld, Die
Missbildungen des Menschen, pp. 164, 166, 1882). In J. Arnold's

CYCLOTIA 431

specimen (Arch. f. path. Aunt., xxxviii. 145, 1867) there was a large
cyst in the front of the neck communicating with the openings into
the Eustachian tubes and larynx. In the case reported by S. Moos
and H. Steinbriigge (Ztschr. /*. Ohrenh., x. 15, 1880-81) there were
interesting anomalies of the internal as well as of the middle ears.
The left suprarenal capsule was absent in Taruffi's specimen (op. cit.,
viii. 402, 1894).

Both micrognathias and agnatlms are most obviously of the
nature of arrested developments of the first branchial arch. Doubt-
less when all the details of the complicated piece of organogenesis
which results in the formation of the lower jaw are understood we
shall know exactly why the characters of agnatlms and synotus are
just what they are ; in the meantime there is in their teratogenesis
much that is obscure. The careful dissection of specimens of the
monstrosity may also throw light upon the embryology of the region.
Dareste, it may be said, regarded otocephaly (including agnatlms) as
due to incomplete development of the medulla oblongata which
allowed the auditory vesicles to come into close relationship with
each other and to hinder the formation of the first branchial arches ;
but Blanc (loc. cit.) and others look upon the aural malformations as
secondary and not primary, and see in arrested development of the
first branchial arches the primary link in the chain of teratogenic
events. The superior maxillary processes by their imperfect develop-
ment or early union " bridle in," as it were, the lower part of the face
and hinder its lateral growth.

Cyclotia (Cyclops Hypoagnathus).

Under the name of cyclops hypoagnathus, Tarulti (op. cit., vi. 408,
1891) describes a type of monstrosity which combines in one the
chief characters of cyclopia and of agnathus (with synotus). It is a
more extensive malformation than either cyclopia or agnathus ; and,
from the embryological standpoint, it may be regarded as represent-
ing aplasia both of the nasal processes (with fusion of the parts
normally separated by these) and of the first pair of branchial arches
(with approximation or fusion of the structures between which they
are interposed). It is a very monstrous type of monstrosity, the
single eye and the displacement of the ears and the absence of the
lower jaw combining to render the facial region most repulsive.
This repulsiveness is well exhibited in the only specimen of this kind
which I have had an opportunity of examining (Fig. 18).

It may be regarded as corresponding to the two genera of Saint-
Hilaire, named edocephalus and opocephalus, and L. Blanc's group of
monstrosities to which he gives the name cyclotia, to denote the com-
posite nature of the teratological state. The outstanding features
are the presence of a single or a double eye in the same orbital cavity,
the union or approximation under the head of the two ears, and
the more or less complete absence of the lower jaw ; but there are
others to which I must refer briefly. For instance, there may be
a nasal proboscis lying above the single eye : this characterises

432 ANTENATAL PATHOLOGY AND HYGIENE

the type named edocephalus by Saint-Hilaire and cyclops astomus
rhynchsenus by Gurlt ; a good specimen of this is in my possession
(Fig. 18). Sometimes, however, the nasal proboscis is quite rudi-
mentary, as in the interesting case reported and figured by Lanne-
longue and Menard {Affections congdnitales, i. 492, 1891). A most
extraordinary arrangement was met by H. Vogt in a specimen of
which he was kind enough to send me a full account and which was
afterwards published (Norsk. Mag. f. Lcegevidensk., 4 R, x. 639, 1805)
under the name of " cyclops dirrhinus hypoagnathus " ; it was a male
foetus, the product of a pregnancy complicated by hydramnios ; the
anterior fontanelle was almost closed ; the two eyes were situated in a
single orbit and had imperfect eyelids, and both above and below the
orbit was a nasal proboscis, each provided with a small opening ;
there was no lower jaw or mouth, the ears were situated horizontally
in the neck, and the external auditory meatus was represented by a
small depression. Vogt's case would appear to be unique in the fact
that it possessed two nasal projections, a supraorbital and a suborbital
(Tarutti, op. cit., viii. 540, 1804). Instances in which the nasal pro-
boscis is wanting have been recorded by G. A. E. T. Knape (Diss.
inaug., Berlin, 182.")), by C. Poelmann(^L;». Soc. ole m<'d. de Gaud, xxv.
220, 1850), by Christian Loven (Anatomisk beskrifning af ett cyklo-
piskt missfostcr nied defekt af ansigtets, Stockholm, 1864), by C.
Taruffi (op. cit, vi. 414, 1801), and by M.H.Fussell (Univ. Med, Mag.,
x. 337, 1808). The absence of the proboscis was used by Saint-
Hilaire as the distinguishing feature of his genus opocepludus, and
Gurlt emphasised it in his nomenclature, calling this type of mon-
strosity cyclops astomus arrhyncus.

There is usually no buccal opening or only a small aperture, but
in Fussell's specimen (be. cit.) there was a large mouth furnished with
lips. Careful dissection will often reveal that the mandible is not
really absent, but is represented by an osseous rudiment (E. Pomey,
Diss, inaug., Konigsberg i. Pr., 1802): this fact, it will be remem-
bered, was noted and emphasised by Wmckel in connection with
agnathus (loc. cit.) ; in two of Thompson Lowne's cases (Catalogue,
Nos. 261, 263, p. 75, 1803), however, there was apparently no trace
of a lower jaw. In Koogler's specimen (Amcr. Journ. Med. Se., n.s.,
lxxxiv. 129J 1882), dissected by E. M. Smith and A. J. Parker (ibid.,
p. 132), the processus gracilis of each malleus turned inwards towards
the median line and was united by a slender splint-like bone with its
fellow of the opposite side ; and this splint-like bone probably repre-
sented an undeveloped lower jaw.

The ears are approximated or fused and often show more marked
deformities than in simple agnathus. There may be two auditory
foramina (Lannelongue and Menard, loc. cit.) ; and the tympanic rings
may be united in the median line by an osseous arch, forming, as
Lannelongue says, a small pince-nez; the cavity of the middle car
usually communicates directly with the pharyngeal sac (no Eustachian
tube) ; and the tympanic ossicles are commonly present but in a mal-
formed state. In some cases the ears may lie so defective as to
consist solely of a single auditory meatus opening directly into the

APROSOPUS 4 33

pharynx (Knape, op. cit); and the external ears may even be entirely
wanting (Poelmann, loc. cit.).

The brain may show numerous anomalies. There may be no
anterior separation into two hemispheres ; fissures and convolutions
may be almost absent (in Koogler's case the only fissure was that of
Sylvius); and the corpus callosum, fornix, fifth ventricle, and septum
lucidum may all be wanting. There may be coexistent hydro-
cephalus (R. Allan, Lancet, i. for 1848, p. 327); the cranium may be
greatly deformed, exhibiting the state known as trigonocephalus
(S. Sebenico, cited by Tarutfi, op. cit, vi. 411, 1891 ; M. H. Fussell,
loc. cit.), or even anencephalus (von Lenhossek, N. Jahrb. d. teutsch.
Med. a. Chir., Bonn, iii. 1, 1821 ; W. Vrolik, Tabulae ad illustrandam
embryogenesin, PI. xxvi., 1840). In Vrolik's case there was the further
associated malformation of exomphalos.

Whatever may be found to be the causes of cyclopia and agnathus,
they will also, it must be supposed, be active in cyclotia. L. Blanc
(loc. cit.) further states his belief that cyclotia is a malformation lying
midway between cyclopia and otocephalus (agnathus), and that there
will yet be discovered a series of types of it running parallel with the
different varieties of cyclopia and otocephaly: from the study of the
specimens at present known it is quite likely that this conclusion
will be established. Blanc also gives interesting details of this
monstrosity as it occurs in the lower animals (lamb, pig, puppy, calf,
kitten), and Tarutfi (op, cit., vi. 416, 1891) adds several interesting
references.

Aprosopus.

Nearly all groups of types of terata have a sort of ultimate
member in which the lowest grade of development along that special
line is reached. In this and in the preceding four chapters I have
been considering the monstrosities of the cranial and facial regions;
in some of them the cranium is more or less defective, and in others
the face ; but I have now to consider a type in which both cranium
and face are defective to the last degree. I modify this statement
to the extent of saying to the last degree possible in the feet us that is
not a twin or a parasite upon another foetus, for we shall find that in
the allantoido-angiopagous twin (paracephalic, acephalic) a still lower
grade of organisation of the head may be reached, when almost every
trace of it is absent. To this very low form of cephalic organisation
the name of aprosopus (a, rrpos^--ov, without a face) was given by
A. Duges (Rev. med. franc, et etrang., iv. 407, 1827) in 1827; but the
type itself was known in animals in the preceding century, and
Etienne Geoffroy Saint-Hilaire had termed it triencephalus to indicate
the absence of three parts, the mouth, the nose, and the eyes (Philos.
Anat., ii. 97, 1822). It cannot, however, be claimed that trience-
phalus of itself suggests any such meaning ; and Isidore Saint- Hilaire's
word triocephalus does not etymologically mark any great advance.
L. Blanc (Juurn. de Vanat. ct pKysiol., xxxi. 296, 1895) retains Saint-
Hilaire's name triocephalus and invents a new name (spherocephalus)
vol. ii. — 28

434

ANTENATAL PATHOLOGY AND HYGIENE

for some allied forms which differ, however, in the possession of a
rudimentary brain. In a certain sense the group of aprosopous
fcfituses as recognised by Taruffi (op. cit., vi. 419, 1891) includes
the triocephalic and most of the spherocephalic monstrosities
(namely, those without a nasal proboscis and even a rudimentary
eye). Another name which is synonymous is Guilt's peroce-
phalus aprosopus ; but it is incorrect to place the cases of facial
fissures, even when the fissures are many and deep, among the
aprosopous foetuses, for in the latter there is a continuous covering
of skin in the region of the face, while in the former, of course, there
is not.

Fig. 67. — Case of Aprosopus, with Deformity of Left Hand. Specimen No. 19.

The aprosopous foetus, then, may be defined as one possessed of
a face, without nose, eyes, or mouth, but with a continuous covering
of skin. It is a rare monstrosity in the human subject ; Taruffi (op',
cit., vi. 421, 1891) has only succeeded in gathering together seven
reported cases (including those of A. G. Otto, Monstrorum Sexcentorum
Description o. lxxxviii. p. 54, 1841; of Vigla, Arch. (jni. d. wed., ii,
1849, p. 25 : of G. Sapolini, 11. 1st. Lotnh. el. sc. c left. JRendic., Milano,
2 s., ii. 415, 1869 : and of B. Thompson Lowne, Catalogue, No. 277,
p. 78, 1893). In my collection of 326 cases I have met with only
one instance of it (Fig. 67). It is more common in animals, Taruffi

APROSOPUS 435

(op. cit, vi. 433, 1891) having been able to collect 72 case-records
of it — 27 in lambs, 17 in puppies, 12 in pigs, 11 in kittens, and 5 in
calves.

The aprosopous foetus has a more or less globular head, something
like the knob of a walking - stick ; the skin covering it passes
continuously into that of the neck. The only sense organs present
are the external ears, which may be situated laterally, as in my
specimen (Fig. 67), or may be approximated to each other in the
middle line anteriorly (C. Pokorny's specimen, figured by Taruffi,
op. cit., vi. 423, 1891). There is usually some hair on the top of the
head, and the circumference of the cranium sometimes does not
greatly exceed that of the neck. Practically all the bones of the
face are absent, save perhaps a trace of the lower jaw ; the cranial
bones, however, may be present and are usually firmly fixed together
by osseous union, but sometimes the ethmoid and frontal bones may
be wanting. The temporals are turned downwards and inwards, con-
cealing the sphenoid ; the glenoid cavities and zygomatic processes
are absent ; and the tympanic cavities are fused into one, the ossicles
being also united together and fixed to the tympanic membrane.
The petrous temporals may be fused or not. There is a pharyngeal
cavity which may be quite closed anteriorly or may communicate
with the external auditory meatus, or with a sac of unknown nature
occupying the anterior part of the neck. There is usually a larynx
as well as an oesophagus, but the hyoid bone and tongue may be
absent or rudimentary. In the cranial cavity nothing else is usually
found than serous fluid and the medulla oblongata ; but in some of
the specimens in animals small parts of the brain have been dis-
covered, and in Vigla's case (foe. cit.) there was a cerebrum without
convolutions but with a central cavity communicating with the
fourth ventricle. L. Blanc (loc. cit.) uses the presence of a brain in
some cases as a means of dividing the spherocephalics from the
triocephalics (which have no trace of a brain beyond the pons and
medulla or the medulla alone). Only the cranial nerves arising from
the medulla are commonly present. Such are the leading characters
of this type of monstrosity as they have been ascertained by Taruffi
(op. cit,, vi. 429, 1891); but it is not easy to say which are essential
and which occasional, for the number of specimens in the human
subject that has been reported is so small, and it is not always safe
to draw conclusions from cases in the lower animals.

In its teratogenesis, aprosopus requires us to imagine a complete
arrest of the first branchial arches on both sides, and not simply
a defect of their mandibular or maxillary processes. According to
Dareste (Production artificiellc dei monstruosites, p. 370, 1891) the
closure of the anterior part of the medullary groove takes place very
early ; the anterior cerebral vesicle does not develop, and the middle
and posterior vesicles either do not develop or remain in the primitive
state of a tubular structure very much like the spinal cord ; in this
way the formation of the head is interfered with. Dareste's views
are founded upon observations on the chick, and it is possible that
the explanation may not hold good for the human subject, and that

436 ANTENATAL PATHOLOGY AND HYGIENE

the facial defects are primary and not secondary to cerebral ones.
It would be necessary to know exactly all the details of the normal
ontogenesis of the cerebral vesicles and first branchial arches, and
especially of Meckel's cartilages in order to elucidate this problem,
and Blanc admits that this is most difficult to achieve.

Absence of External Ear.

The external ear consists of the pinna or auricle and the external
auditory meatus. In the strict sense of the term complete absence
of the pinna and an imperforate state of the meatus constitute a
rare malformation, but I may here conveniently include instances in
which the pinna is rudimentary although not entirely wanting. It
is an interesting fact that in the very ancient Chaldean tablets of
Nineveh, as I have elsewhere pointed out (Tcratologia, i. 131, 1894),
there is a wonderfully complete list of aural deformities with their
alleged prophetic significations ; indeed the diviners of Mesopotamia
seem to have had a knowledge of most of the deformities of the
ears, nose, jaws, and mouth. The records are silent, again, regarding
absence of the ear till the sixteenth and seventeenth centuries, when
a few cases were reported, including that of F. Lachmund (Mixed! .
Acad. not. curios., Dec. i., Ann. vi.-vii., p. 239, obs. 178, 1677); in the
eighteenth and nineteenth centuries several instances, more particu-
larly of rudimentary pinnae, were described.

In 1895, H. J. Stiles showed at a meeting of the Medico-Chirurgical
Society of Edinburgh ( Trans., xv. 54, 1896) an infant of six months,
whose pinnae were represented by nothing more than small uncon-
voluted reduplications of skin, apparently containing no cartilage.
There was no trace of an external auditory meatus on either side ;
the right half of the face was smaller than the left : on the left
cheek there were two preauricular appendages ; and the lower jaw
was also poorly developed, a common accompaniment of auricular
anomalies. This case is fairly typical of this variety of mal-
formation. A somewhat similar case was described by J. Toynbee
(Month. Joiirn. Med. 8c. , vii. 738, 1846-47), three others were put on
record by Allen Thomson (ibid., pp. 420, 729), and one of M. Hoff-
mann's patients constituted yet another (Intcrnat, rncd.-phot. Mon-
atschr., iii. 357, 1896).

What represents the external ear is usually little more than a
fold of skin or a piece of cartilage in a cutaneous covering situated
on the side of the head with its long axis vertical ; sometimes, how-
ever, there are two or more tags of cartilage-containing skin irregularly
placed and not always occupying the normal position of the ear.
The deformity may be unilateral or bilateral: in one of Gradenigo's
three cases it was bilateral (Tarum, op. tit., vi. 551, 1891). The
meatus auditorius is usually obliterated: it may lie represented by
a slight fossa, or there may be no sign of it at all ; the little
openings sometimes seen anterior to the normal position of the ear,
in these cases, do not necessarily indicate the meatus, for they
may be fistulse.

ABSENCE OF EXTERNAL EAR 437

In a small number of cases, post-mortem examinations were carried
out upon individuals with absence or rudimentary development of
the pinna. In several of them a cartilaginous meatus was found,
but it ended blindly, and the tympanic ring and membrane were
represented simply by a small piece of bone. The tympanic cavity and
the osseous part of the Eustachian tube may be found ; but they are
both small in size, and the auditory ossicles may be malformed or
defective. The labyrinth, however, is usually normal, a fact which
accounts for the degree of hearing possessed by some patients suffering
from this malformation, and a circumstance which is einbryologically
explicable by the independence of the ontogenesis of the inner and
middle ears.

Associated malformations are not uncommon. There may, for
instance, be cleft palate (T. Heiman, Ztschr. f. Ohrenh., xxi. 271,
1890-91), absence of nose, imperforate anus, and Polydactyly (W.
Duncan, Trans. Obst. Soc. Loud., xxxvii. 16, 1895), hare-lip and cleft
palate (W. Anton, Prag. med. Wchnschr., xxii. 235, 249, 1897), absence
of a thumb (F. S. Pluskal, Oesterr. med. Wchnschr., p. 1373, 1844),
enlargement of the buccal commissure (Le Briero, Gaz. d. hop., xxxiv.
122, 1861), or preauricular appendages (q.v.). The malar bone may
be absent.

Attempts to improve the hearing powers by operative measures
(cutting down in the position of the meatus, etc.) have not been
successful, a fact which is largely to be accounted for by the
defective state of the middle ear, and by the cicatricial constriction
which follows (Lannelongue et Menard, Affections conge nit ales, i. 522,
1891). For purely aesthetic purposes an artificial ear will probably
be more satisfactory than any plastic operation.

The malformation (called eeto-otus-aplasia by Taruffi) is obviously
to be explained as an arrested development of the first branchial
cleft and adjacent structures. If, as Gradenigo maintains, the pinna
is derived in part from the mandibular process of the first branchial
arch and in part from the second branchial arch, then it is probable
that the cartilaginous tags which are sometimes to be found may
represent one or other of these two portions (mandibular and hyoidal).
The cause of the arrested development may be pressure, e.g. amniotic.

I may add here some further bibliographical references : D. S. St.
J. Eoosa, Trans. Arncr. Otol. Soc, p. 123, 1873 ; Bidder, #£. Petersb. med.
Ztschr., n.F., iv. 388, 1873-74; Chowne, Lancet, ii. for 1860, p. 59;
H. Knapp, Arch. Otol., x. 119, 1881; xxi. 438, 1892; E. B. Corson,
N. York Med. Times, xiv. 50, 1886-87 ; E. Joel, Ztschr. f. Ohrenh., xviii.
278, 1887-88 ; C. H. Vilas, Trans. Amer. Homoeop. Ophth. and Otol.
Soc, vi. 29, 1882 ; A. Bobertson, Glasgow Med. Journ., xxxi. 209,
1889.

Preauricular Appendages.

Preauricular appendages may be defined as skin-covered projec-
tions, of varying number, size, and form, situated either immediately
in front of the pinna or at some point in the line passing from the

438 ANTENATAL PATHOLOGY AND HYGIENE

external ear to the angle of the mouth of the same side, and usually
containing an axial nucleus of cartilage. I have examined two
such cases (Teratologic/, ii. 14, 1895): in one the appendage was
single and was situated immediately in front of the left ear of a hoy
twelve years of age ; in the other, there was a group of appendages
lying in front of the ear and surrounding a small sinus, from which
a little glairy fluid occasionally exuded. To cases like the latter the
name supernumerary auricle (polyotia) has sometimes heen given ;
but it is very doubtful whether they can in any sense be regarded as
true additional ears (e.g. J. Birkett's case, Trans. Path. Soc. Lond.,
ix. 448, 1857-58).

This malformation has long been known to occur, for in the
Chaldean teratological lists, already several times referred to (Tera-
tologic/,, i. 135, 1894), there is the record of an infant born " with two
ears on the right side." The earliest cases reported in recent times
seem to have been those of Saviard (Nouv. Rec. d'observ. chir., obs. 116,
p. 514, 1702), and other instances in the eighteenth century were
those of Sue (Hist. Acad. roy. d. sc., Ann. 1746, p. 42, 1751), of G. H.
Fielitz (Arch. f. d. Geburtsh., ii. 71, 1789), and of J. C. Stark (JV. Arch,
f. d. Geburtsh., i. 415, 1798).

During the nineteenth century many observations were recorded,
of which I have given elsewhere a fairly complete bibliography
(Teratologia, ii. 34, 77, 1895) of over fifty references; specially
valuable papers are those of Virchow (Arch. f. path. Anat, xxx. 221,
1864), of J. H. Morgan (Trans. Med.-Chir. Soc. Lond., lxv. 13, 1882 ;
Illustr. Med. News, ii. 265, 1889; Trans. Clin. Soc. Lond., xxix.
218, 1896), of Lannelongue (Cystes congtnitaux, p. 171, 1886), of
Majocchi (Atenco med. parmense, Ann. i. 75, 1887), and of Bland-
Sutton (Illustr. Med. News, i. 320, 1888 ; vi. 99, 1890).

Various names, more or less synonymous, have been given to these
little appendages, such as " excrescences," " warts," " tubercles,"
" supernumerary auricles," " branchial fibro - chondromata," " pre-
auricular papillary teratomata," " supernumerary tragus," and " ac-
cessory auricles." They are comparatively frequent, for among fifty
thousand children examined by F. Warner (Brit. Med. Journ., ii. for
1894, p. 197) there were thirty-three cases — twenty-one boys and
twelve girls.

The clinical history is sometimes interesting. There was an
alleged maternal impression in the cases of Moxhay(i?n£. Med. Jpum.,
ii. for 1870, p. 521) and C. Apfelstedt (Diss, inaug., Jena, p. 28,
1892). In J. L. Beverdin's observation (Rev. mkl. de la Suisse rom.,
vii. 458, 1887) there was family prevalence (three out of five chil-
dren), and the father had a supernumerary nipple on his chest ; and
in one of J. J. H. Kratz' cases (Diss, inaug., Bonn, 1880) a sister of
the individual with preauricular appendages had a bilateral fistula
amis congenita. Cranial and facial defects have been shown to be
common in the relatives of the subjects of this aural malformation
(Fere, La Famille Nevropathique, 214, 1894). The appendage itself
does not interfere with the health of the bearer of it, except when
there are other malformations, such as absence of the zygoma of the

PREAURICULAR APPENDAGES

439

temporal bone in Walker Downie's patient {Practitioner, lvi. 261,
1890). In two female patients it became larger and more erectile
during menstruation, and was regarded as an aberrant mammilla
(A. Barth, Arch. /.path. Anat., cxii. 569, 1888; P. Eyle, Ueber £11-
dungsanomalien der Ohrmuschel, p. 28, 1891). Hearing was occasion-
ally defective, doubtless from associated malformations (Virchow, loc.
cit. ; J. H. Morgan, loc. cit., 1882). These appendages occur with
equal frequency in both sexes ; they are always truly congenital ;
they grow slightly or not at all in postnatal life ; and they can be
removed easily and with impunity.

With regard to their morbid anatomy, they are more commonly
unilateral than bilateral. In the records collected together by me
(Teratologia, ii. 24, 1895) their number varied from one to four : in
fifty cases there was a single appendage in front of the ear, in twenty
there were two, in thirteen three, and in one four ; and in two
instances (Mignot, cited by Broca, Bull. Acad, de mM., p. 1217, 1879 ;

p -

<•• •-

4^ . > :■■■,.-.
.«v . •»,- -■ - - -

Fig. 68. — Transverse Section of Preauricular Appendage (Low Power).

Apfelstedt, op. cit.) there was a group forming a structure resembling
a whole pinna. Their size varied from that of a pinhead to that of a
pea or bean ; in one of my cases the appendage, cylindrical in shape,
measured 1*3 cm. in length, and had a uniform circumference of 1*5
cm. Although commonly cylindrical, they are occasionally flat,
conical, bifid, trifid, or with a bulbous free extremity. They are
most often situated immediately in front of the tragus ; frequently
they lie in front of the crus helicis, less frequently on the cms itself ;
they may be found on the cheek itself in the line leading from the
auricle to the angle of the mouth ; and they have been seen, but
rarely, on the lobule of the pinna. Their consistence is like that of
the lobule of the ear, but a firmer central part can sometimes be
palpated ; and they commonly stand out from the surface of the
face. In structure they may consist of no more than skin covering
an axial rod of condensed connective tissue (as in one of my cases,
Fig. 68) ; in other instances there is a core of reticular or of hyaline

440

ANTENATAL PATHOLOGY AND HYGIENE

cartilage, which may be inserted into the fascia lying between the
temporal and masseter muscles. The skin has its usual microscopic
characters, and sometimes there is a tuft of hairs at the apex of the
appendage. There may be a marked adipose layer ; but in my case
( Fig. 69) there was no fatty stratum, but simply one of fine areolar
tissue, such as is seen in the foetus before the deposition of the fat.

Associated malformations are common and somewhat interesting.
In some instances there are grave monstrosities ; as in Meckel's speci-
men (Arch./. Anat. u. Physiol., p. 36, 1826), in which there was one
umbilical artery, various deformities of the limbs, absence of one
lung and of one kidney, and a communication between the rectum
and bladder. Generally the ears are defective, and the defect may
take various forms: there maybe absence of the ear (E. Midler,

Fig. 69. — Microscopic Section of Preauricular Appendage (High Power).

Diss, inaug., Kiel, 1893), or a congenital fistula (Hartmann, Compt.
rend. d. IV Gongr. internat. d'otol., p. 15, Brussels, 1889), or occlusion
of the meatus (Billard, TraiU d. mal. d. Enfans nouveau-ne's, 3rd
edit., p. 53, 1835) or narrowness of it (Lesser, Deutsche Ztschr.f. C'hir.,
ii. 311, 1873), with bifidity of the tragus, etc. etc. There may also be
defects in the deeper parts, such as malformations of the tympanic
ossicles (M. Schult/e, Arch. f. path. Anat., xx. 378, 1861) or absence
of them and of the tympanic ring and membrane (G. Mori, Ann. univ.
di med., cexxxii. 250, 1875). Again, there may be associated ocular
anomalies, such as microphthalmus, lengthening of palpebral fissure
(K. Morian, Arch.f. klin. Ghir., xxxv. 286, 1887), and coloboma of an
eyelid. The mouth also may be deformed : there may be cleft palate,
or hare-lip, or a defective mandible, or absence of the temporo-maxillarv

PREAURICULAR APPENDAGES 441

joint, or lenmrian apophyses of the jaw (Fere, op. tit), or (most fre-
quently) macrostoma (Sir W. Fergusson, Lectures on the Progress of
Anatomy, p. 56, 1867; L. Keismann, Arch./, klin. Chir., xi. 858,
1869; Eoulland, Bull. Soc. anal, de Paris, 4 s., xi. 599, 1886; L.
Leplat, Ann. Soc med.-chir. d. Liege, p. 647, 1888). The coexistence
of congenital fistula? has already heen noted ; and there may be cer-
vical auricles (L. Bernacchi, Atti Assoc, med. lonib., No. 1, p. 70,
1892).

In an article which I published in 1895 (Teratologia, ii. 65, 1895)
I discussed with considerable fulness the views that have been held
regarding the teratogenesis of preauricular appendages. I there re-
ferred in detail to the views of Schultze (loc. tit.), of Virchow (loc. tit.),
of Sir James Paget (Traits. Med. Chir. Soc. Load., lxi. 46, 1878), of
Van Duyse {Ann. Soc. dc mid. de Gaud, lx. 141, 1882), of J. H.
Morgan (loc. tit.), of Bland-Sutton (Evolution and Disrate, pp. 82-92,
1890), of Lannelongue and Menard (op. tit,), and of Gradenigo (in
Taruffi's Storia, vi. 566, 1891). The main ideas underlying these
theories are that preauricular appendages are branchial in origin ; that
they are displaced projections which ought to have taken part in
the formation of the external ear ; that they represent, in atavistic
fashion, the opercular skin-folds of animals lower down in the
zoological scale ; that they are inflammatory productions arising along
the margins of the first branchial groove ; or that they arise by
budding from the margins of the cleft in the same ontogenetic way
as the tubercles that form the ear arise. Gradenigo thinks, and his
theory differs essentially from the others, that the preauricular
appendage represents a persistence of the cms prsetragicum helicis,
of which normally there is no trace in the human subject, but which
is well seen in some rodents ; it is to be seen, however, at an early
stage in embryonic life. Now, I think there can be no doubt that
preauricular appendages represent an arrested or diverted or arrested
and diverted state of the ontogenesis of the external ear region in
the embryo. Some six tubercles or colliculi are grouped round the
first branchial cleft, or hyo-mandibular fissure, to form by their fusion
the pinna : but in addition to these there are other fissures and
processes which abound in the peri- and pre-auricular region. Keep-
ing in mind the nature of the associated malformations and the
various positions occupied by the appendages, I do not think that
they are invariably due to an abnormality in the closure of any one
fissure or in the growth of any one process. When they occupy
their usual position immediately in front of the tragus one naturally
thinks of an anomaly in the evolution or involution of the posterior
end of the hyo-mandibular (or first visceral) cleft, or of its adjoining
mandibular and hyoidal arches ; whilst when they occur further
forwards on the cheek one suspects some abnormality in the develop-
ment of one of the other facial processes and fissures, such as the
intermaxillary.

I am inclined to regard the appendages themselves as due to a
sort of budding from the margins of one or other of these fissures, or
from the processes, or in some cases from the colliculi or helices

442 ANTENATAL PATHOLOGY AND HYGIENE

themselves. In fact, the external ear is to be regarded as produced
by a series of buds arising around the posterior end of the hyo-
rnandibular cleft, and it seems to me that the cause which normally
produces the pinna may also, in abnormal conditions, lead to the
formation of the appendages.

What, however, is the nature of this cause ? There can, I think,
be only one answer to this question : the primary factor is the
amnion. Whether it is necessary to predicate also an abnormal
state of the fissures and processes themselves is doubtful, but I am
inclined to believe that the amnion may by an unnatural tightness
or by an unusual agglutinativeness prevent the normal closure of the
fissures, whilst it is also possible that the margins of the fissures
may possess this same agglutinative character, which may be of an
inflammatory nature. But I must refrain from entering again in
detail into the general theory of amniotic action in teratogenesis,
already discussed at length in Chapter XL It may, however, be said
in conclusion, that cervical auricles or appendages will probably be
found to be explicable by the same teratogenetic theory as pre-
auricular ones, for they are apparently of the same nature.

Fistula Auris Congenita.

Fistula auris congenita, which is generally preauricular in
position, is a malformation of little importance ; but it has, however,
a slight interest on developmental grounds. It may easily be over-
looked, for it has a very small opening, and this is often obscured by
a small crust, formed by the drying of the slight secretion which
exudes from it.

Fistuke may occur in various situations on and near the ear, but,
perhaps, the term fistula auris congenita ought to be reserved for
that which lies at the point of junction of the crus helicis and the
crus, or a little anterior to the crus and to the tragus ; at any rate
it is used in this work in that sense. Since it was first clearly
described by Heusinger (Arch. f. path. Anat., xxix. 361, 18G4) and
had its antenatal origin established, many instances have been
reported. According to P. Eyle (Diss, inaiuj., Zurich, 1891) and
others, these fistuke occur in about two per 1000 cases; but, on
account of their strongly developed hereditary character, one
observer may easily happen to have seen six, eight, or ten of them
without having examined many thousand ears.

Their clinical features are comparatively unimportant : they cause
almost no disfigurement, except in the cases in which they give -
rise to a retention cyst (through blocking of the fistulous tract).
They secrete a small quantity of serous or milky fluid, which
occasionally forms a scab over the orifice. They do not appear
to be more common in one sex than in the other ; but, as has been
stated above, they are often peculiarly hereditary. In one of the
case-records of J. J. H. Kratz (Diss, inaug., Bonn, 1880) a mother
and all her sons had this deformity ; in V. Urbantschitsch's inter-
esting article (Monatschr. f. Ohrcnh., xi. 85, 1877) there are instances

FISTULA AURIS CONGENITA 443

of heredity in which the first born seemed specially to participate ;
and Paget (Med.-Chir. Trans. Land., lxi. 41, 1878) saw the deformity
in seven members of the same family, and in some of these and in
their relatives there were also fistula? of the neck. Other instances
were reported by 1). Schwabach (Ztsclir. f. Olvrcnh., viii. 103, 1879),
F. P. Lewis (Journ. Ophth. Otol. and Laryngol., iii. 14, 1891), and
P. Eyle (op. cit.) ; in the latter the defect could easily be traced for
four generations, it affected males and females, and it was bilateral
in some individuals.

In its morbid anatomy the fistula is a small narrow tract from
*5 to 1 cm. iu length, which is directed downwards and forwards
from the crus helicis, and ends blindly, never, apparently, opening
into the auditory meatus. It has a slightly raised margin. It seems
to be as common on the right side as on the left, and it may be
bilateral, although most commonly it is unilateral. I have only seen
two cases of it (unilateral) ; but that is not surprising, for I have not
specially looked for it, nor has my attention been specially focussed
upon it. When the secretion is retained for any cause, a cyst may
result, as in the cases reported by F. Schmitz (Diss, inaug., Halle,
1873), C. J. Kipp (Trans. Amcr. Otol. Soc, ii. 402, 1875-81), V.
Urbantschitsch (loc. cit.), Lannelongue and Menard (Affections con-
gSnitales, i. 221, 1891); ulceration may also occur in the neighbour-
hood (C. Mettenheimer, Mcmoraoilien, xxv. 100, 1880). Occasionally
there is no true fistulous tract, but only a depression or fossa, with
a cicatricial area of skin surrounding it; this, likewise, may be
hereditary, as in one of Gradenigo's cases (in Taruffi's Storia delta
teratologics, vi. 573, 1891). Associated malformations are common in
connection with fistula auris congenita. Among these reference
may specially be made to preauricular appendages (Hartmann, Compt.
rend. d. IV Congr. internal, d'otol., p. 15, 1889; H. Knapp, Trans.
Amer. Otol. Soc., ii. 357, 1875-81 ; Eoulland, Bull, Soc, anat. do Par.,
4 s., xi. 599, 1886); to anomalous states of the pinna, such as absence
or rudimentary development (C. F. Heusinger, Specimen malm con-
formations organorum auditus humani,Jen.dd, 1824; Toynbee, loc. cit.)
or contortion (F. Eohrer, Tagcbl, d, Versamml, dcutsch. Naturf. u.
Aerzte, lviii. 137, 1885); and to cervical fistuhe (Heusinger, Arch.f.
pedh. Anat., xxix. 361, 1864; Urbantschitsch, loc. cit.).

The hypothesis which has been generally accepted regarding the
teratogenesis of fistula auris congenita is the persistence of part of
the first branchial cleft ; but it must be admitted that there are two
difficulties (at least) in the way of a full and free acceptance of this
view. The first is the freedom of the middle ear from participation
in the deformity, and the other is the position of the fistula?.
Gradenigo (in Taruffi's Storia, vi. 580, 1891), from a study of the
development of the crus helicis, comes to the conclusion that fistula?
result from insufficient closure of the sulcus between the crus pre-
or supra-tragicum and the crus helicis, or between the crus supra-
tragicum and the tragus ; he does not think that the coexistence in
some cases of fistula? in the neck (due to branchial anomalies there)
is of any higher value (in argument) than that of a coincidence. In

444 ANTENATAL PATHOLOGY AND HYGIENE

the meantime the question whether fistula auris is due to delayed
union of such a primary fissure as the first branchial or of such a
secondary one as the sulcus between two parts of the external ear,
must be left with a non liquet. It may be added, although the
theoretical value of the statement be obscure, that fistulae in this
position would appear to be commoner in the lower animals than in
man.

Other references on this subject (in addition to those already
given) are as follows: — Pfluiger, Monatschr.f. Ohrenh., viii. 132, 1874;
T. M. Girdlestone, Austral. Med. Journ., n.s., i. 322, 1879 : E. Dyer,
Trans. Amer. Otol. Soc, hi. 468, 1882-86; K. Biirkner, Arch. f.
Ohrenh., xxii. 200, 1885: E. T. Morris, N. York Med. Month., I 169,
1886-87; A. B. Randall, Trans. Amer. Otol. Soc.,iv. 485, 1887-90;
A. Hahne, Diss, inaug., Gottingen, 1889 : J. Gruber, Aerztl. Ber. d.
k. /.'. allg. Krankenh. zu Wien, 185, 1891 ; A. Schwendt, Arch. f.
Ohrenh., xxxii. 37, 1891 ; Peugniez, Gaz. mid. de Picardie, xi. 481,
1893 ; Vauthier, Ann. de la Policlin. de Paris, iii. 95, 1893 ; C.
Grunert, Arch. f. Ohrenh., xlv. 10, 1898.

Congenital fistulte have been occasionally met with in other parts
of the ear, and especially in the lobule (J. Sabrazes, Bull. Soc. d'dnat.
et physiol. de Bordeaux, xii. 124, 1891; Lannelongue and Menard,
op. cit., p. 225; Betz, Memorabilien, viii. 135, 1863; Bland-Sutton,
Evolution and Disease, p. 179, 1890). In Bland-Sutton's case the
fistula was so complete that an earring was suspended in it a few
days after birth. Of course such fistula? have suggested the trans-
mission of an acquired character (ear-boring) ; but it seems to me
that the birth in very ancient times of an infant with this mal-
formation may have suggested to a tribe, or to mankind generally,
the practice of ear-boring. The traumatism may not have caused
the congenital malformation, but, in a sense, the occasional occurrence
of the malformation may have led to the frequent infliction of the
traumatism !

Minor Malformations of the Pinna.

The chief importance of minor malformations of the external ear
is in relation to what are called the stigmata of degeneration. " The
external ear," writes E. S. Talbot {Degeneracy, p. 207, 1898), "is, of
all organs, that most affected by degeneracy." I pointed out (in the
first section of this Manual, p. 97, Fig. 14) that the ears are among
the parts of the body which finish their embryology in the foetal
period of antenatal life ; their morphological differentiation is not
completed in the embryonic period, when that of most of the other
parts of the body is, but is postponed till the later foetal epoch.
Malformations of the ears, therefore, although embryological in
nature are foetal in time. Now most of the stigmata of degeneracy
agree with them in this comparatively late origin in antenatal life
{e.g. the jaws, teeth, skin, eyes, hands, feet, etc.), and, further, it is
about tliis time also that many minor cerebral defects arise. If we
imagine some common morbid cause (toxic, toxinic, microbic) com-

MALFORMATIONS OF PINNA 445

ing to act upon the organism during this epoch it is easy to
understand how mental defects, aberrations of the nervous system,
and minor physical defects of ear, skin, eye, and digits are all found
associated together. Statistics show that in the criminal and in the
insane, the minor malformations of the external ear are more common
than in individuals of normal mental and social qualities.

These minor malformations may now be briefly indicated. There
may be asymmetry of the pinnae, one of them being inserted higher
or lower or more to the front than the other, or one being larger
than the other. Marked displacements, e.g. downwards and forwards
into the neck, are usually associated with other grave defects {e.g.
agnathus) ; but slight displacements may occur, either alone or in
c< mibination with macrostoma, etc. The ears may be more bound down
to the head than usual; and the so-called "jug-handled" (or Morel)
ear is a long and narrow organ attached in its entire length, includ-
ing the lobule. On the other hand, the pinna may stand out from
the side of the head in a prominent fashion ("orecchio ad ansa,"
Lombroso) and to a varying degree ; with this there may be a folding
of the pinna upon itself; so marked may be the deformity that
plastic operative interference may be attempted for its relief (E.
T. Ely, Arch. OtoL, x. 97, 1881; G. H. Monks, Boston Med. ami
Surg. Joum., cxxiv. 84, 1891 ; Haug, Deutsche med. Wchnschr., xx.
776, 1894; Stetter, Arch, f. Ohirnh., xxxix. 101, 1895). Again, the
ears may be obliquely inserted into the head (lower end in advance
of upper) ; or the pinnae may show an exaggerated or a diminished
degree of curvature; or their size may be abnormally increased.
The ears are often defective (crumpled, enlarged, "elephantine") in
anencephalic foatuses.

The various parts of the auricle may show anomalies, some of
which are very interesting. In the helix, for instance, there may be
defects. The Darwinian tubercle, the projection from the free pos-
terior margin of the helix a little above the level of the crus of the
antihelix, may be unusually prominent. The meaning of the Dar-
winian tubercle has been much debated and is not yet clear. In the
so-called " Satanic ear " there is another projection than the Dar-
winian, situated at the highest point in the helix, and this gives to
the organ a pointed and narrow appearance. Other small projections
or folds in the helix may be found opposite the cymba conchas or the
fossa triangularis. The raised outline of the helix may be faintly
marked or absent. Various anomalies of the antihelix have been
reported : it may be absent, or only the inferior crus may be recog-
nisable ; the inferior crus may not be continuous with the rest of
the antihelix ; the main part of the antihelix may be more prominent
than the neighbouring helix (" Wildermuth's ear "), and this anomaly
would appear to be more common in women (Gradenigo) ; rarely the
superior crus of the antihelix is absent ; sometimes the helix and
antihelix are adherent ; occasionally there are accessory antihelices ;
and rarely there are three instead of two crura. The lobule may be
too large or too small or absent ; it may be adherent to neighbouring
parts ; or it may show a fissure (coloboma lobuli). The occurrence

446 ANTENATAL PATHOLOGY AND HYGIENE

of coloboma of the lobule has a special interest from the standpoint
of the question of the transmission of acquired characters : such a
fissure existed in the left ear of a child whose mother's left car had
been torn accidentally by an earring (the mother being then eight
years of age) ; the lobule of the mother's ear never properly closed ;
and thirteen years later she married and gave birth to eight children,
of whom seven had well-formed ears and one (the second born) had
the coloboma referred to (E. Schmidt, Cor. Bl. d, dcutsch. Gfesellsch.f,
Anthrop., Nov. 1888). A somewhat similar case was reported by
Ornstein to the same Society as that to which Schmidt had com-
municated his observation ; and 0. Israel {Arch. f path, Anat., cxix.
240, 1890) has met with two instances in which there had been no
maternal traumatism affecting the ears. B. C. Windle's paper (Journ.
Anat. and Physiol., xxv. 433, 1891) may also be consulted. The
malformation is not to be regarded as a maternal traumatism trans-
mitted but rather as a teratological state of the lobule arising from
arrested union of the original constituent parts of the pinna.
Further, in support of this view, it was noted that the artificially
produced maternal fissure of the lobule and the antenatally de-
veloped coloboma of the child's ear did not correspond in the position
which they occupied. Eecently another case of coloboma lobuli was
reported by J. Boeke (Nederl. Tijdschr. v. Geneesk., ii. for 1902, p. 1034).

The tragus may be smaller than usual or divided into two
(Talbot, op. cit., p. 214); the antitragus may be undeveloped, or it
may lie immediately opposite to, instead of below, the tragus, and so
block the meatus auditorius. The scaphoid fossa may be prolonged
into the lobule, as in an ear with three Darwinian tubercles figured
by Talbot (op. cit., p. 215) ; it may, on the other hand, be absent
(especially when the antihelix is very prominent), or it may be
restricted in extent. Minor malformations may also be found in the
concha. The external auditory meatus may be double, the duplicity
being caused by a septum, as in L. Guranowski's case (Prezeglad chir.,
iv. 217, 1898).

Eeaders who may wish further information on this anthropological
annexe (so to say) of Teratology will do well to consult the following
references: — Binder, Arch. f. Psychiat., xx. 514, 1888-89; H. Daae,
Norsk May./. Lcsgevidensh, 4 R, viii. 824, 1893; H. H. Ellis, Lancet,
i. for 1890, p. 189 ; L. Frigerio, Arch, de I'anthrop. crim., iii. 438,
1888 ; G. Gradenigo (many articles, including Ann. d. mcd. dc Voreille,
du larynx, xv. 279, 1889, and Arch.f. Ohrcnh., xxxii. 202, 1891); M.
Lannois, Arch, dc Vanthrop. crim,, ii. 33G, 389, 1887; J. Julia, These,
Lyon, 1888 ; B. Pailhas, Arch, di psichiat., xviii. 1, 1897 ; G. Schwalbe,
Festschrift E. Virchow, i. 93, 1891 : E. Villi, Orvosi hctil, xxxv. 81,
1891 ; F. Warner, Lancet, i. for 1890, 344.

Distomus and Accessory Maxillae.

Before I close this chapter and pass from the teratological states
of the head to those of the neck, it is necessary that I refer, very
briefly, to some puzzling cases which have been variously described

DISTOMUS

447

as double-mouth (distomus), accessory lower jaw, supernumerary upper
jaw, and teratomata of the face. These are most anomalous mal-
formations. If they are the lowest degrees of double terata, then
their discussion ought to be postponed till the double monsters are
dealt with ; if, on the other hand, they represent a sort of excessive
formation of individual parts in a single foetus, they are exceedingly
difficult to explain by any of the known laws of teratogenesis ; if they

Fig. 70. — Congenital Tumour on the Face of an Infant.

are teratomata or teratoids, then they are allied both to the double
monsters and to congenital neoplasms, and become scarcely less difficult
of elucidation. Oscar Israel's specimen (Diss, inaug., Berlin, 1877) is
one in point : it was obtained from a new-born female child who
showed in the ear a double tragus and a coloboma of the lobule, and
on the left side of the lower jaw a proboscis-like projection, which on
removal was found to consist of a rudimentary half mandible, with
tooth germs, a condyle, and a coronoid process (?), a submaxillary

448 ANTENATAL PATHOLOGY AND HYGIENE

gland, and a cyst. At the free extremity of this proboscis was a
small round opening, supposed to be a month. There was also a
month in the normal situation, which showed some asymmetry.
Israel regarded his specimen as an instance of duplicity of the left
half of the lower jaw. Taruffi {op. cit, vi. 528) met with a somewhat
similar state of affairs in an anencephalic foetus : the nose was large
and broad ; the month was extraordinarily wide, and was turned

Fig. 71. — Congenital Tumour on the Face of an Infant.

downwards at its angles, where there was (on each side) a small
opening into the buccal cavity ; the lower lip in the middle line
passed under the upper one, and was adherent to the palate; and on
the chin was a small round opening with a reddish projection in it.
In this curious case dissection showed that there were two mandibles
lying side by side fused together by their inner halves. In a lamb
seen by P. Gibs (Journ. de I'anat et physiol, xxxv. 707, 1890) there

FACIAL TERATOMA 449

were two lower jaws, four upper jaws (much reduced), and two
tongues, although externally there was no trace of duplicity except
in the great distance which intervened between the two eyes.

Akin to these cases was that of a congenital tumour on the face
of an infant which J. Rutherford Morison asked me to examine for
him in 1896 (Trans. Edinh. Obstd. Sod,', xxi. 254, 1896). The growth
was about the size of a Tangerine orange, and appeared as if it were
growing out of the right side of the nose in immediate proximity to
the right eye (Fig. 70) ; it did not, however, communicate with the
interior of the nose. On its outer aspect were two incisor teeth,
which were cut at the same age (eight months) as the ordinary teeth
inside the mouth (Fig. 71). Mr. Morison regarded the growth as an
accessory upper jaw, and successfully operated upon it ; it consisted
partly of bone, partly of fibrous and mucoid tissue, and partly of
hyaline cartilage. It may, on the other hand, be looked upon as a
teratoma comparable to the intrabuccal growths to which the name
epignathus has been given and which grow from the hard palate.
However it be regarded, it (and the cases narrated above) must be
grouped among the unsolved problems of Teratology.

VOL. ii. — 29

CHAPTER XXIV

Merosomatous Terata (cont.) : Malformations of the Neck : Fistula Colli ;
Definition ; History ; Varieties (Lateral, Median, Supra-hyoidean, Infra -
liyoidean, Complete, Incomplete) ; Morbid Anatomy ; Associated Malfor-
mations ; Teratogenesis ; Literature : Cervical Auricles : Cervical Cysts :
( 'vstic Hygroma of the Neck : Cysts of the Thyroid and of Aberrant
Thyroids : Median Fissure of the Neck : Cervical Patagium : Cervical
Ribs : Malformations of the (Esophagus : Malformations of the Larynx and
Epiglottis : Muscular Anomalies of the Neck.

In studying the teratological states of any region of the body it is
most helpful to keep constantly before the mind's eye the embry-
ology of that region ; normal ontogenesis throwing light upon ab-
normal in a quite remarkable fashion. This is specially true of the
cervical region ; and, if the reader will take the trouble to re-read the
account given (in the early chapters of this volume) of the develop-
ment of the neck, or if he will at least glance at the synopsis of the
organogenetic rearrangements given on pages 68 and 69, he will with
much greater facility comprehend the nature and mode of origin of
the malformations now to be described. The neck is the region of
the branchial arches and clefts, and it follows that the malformations
of the neck are mainly branchial. Thus, I shall have to refer to
various projections and fistulas (with their frequent concomitants —
cysts), resulting from the imperfect closure of the branchial recesses
or from anomalous budding from the arches. Further, the neck is a
region which in early embryonic life can hardly be said to exist, and
it is only clearly marked off when recession of the heart into the
thorax is accomplished ; these facts also have a bearing upon the
origin of certain cervical malformations.

It has also to be remembered that in ontogenesis the lower
branchial clefts open into one sinus (the precervical) ; imperfect
closure of this sinus may lead to fistulous openings in the neck.
The malformations of the cervical region have a strong resemblance
to those of the ear and lower jaw, which have just been described
(Chapter XXIII.) ; the reason is again supplied by embryology, for
they are both branchial in origin. With these introductory sentences
in his mind, the reader may now study with more profit the anomalies
of the neck. Let us take as our type-malformation the condition
known as fistula colli.

Fistula Colli.

The name fistula colli is given to those fistulous openings situated
in the neck, either along the anterior margin of the sterno-mastoid

450

CERVICAL FISTULA 451

muscle or (less commonly) in the median line, which represent
imperfectly closed branchial clefts or traces of the sinus praecervicalis,
and which, although insignificant in appearance, are often very
difficult to close and so to cure.

Their history does not extend far back. C. H. Dzondi {De fistulis
trachece congenitis, Hahe, 1829) was practically the first to draw
attention markedly to them ; but he thought they were tracheal in
nature ; he reported four cases. F. M. Ascherson (De fistulis colli
congenitis, adjecta fissur arum oranchialium in mammalibus avibusquc
historia succincta, Berlin, 1832) described eleven cases, discovered
that the fistula' were connected not with the trachea but with the
pharynx, and so made clear their branchial origin ; eight of the
eleven individuals seen by Ascherson belonged to the same family, in
three generations. Since 1832 many new observations have been
made, so that the bibliography of the subject is now very large ; to
some of the more outstanding articles reference will be made in the
text and others will be referred to at the end. It may be said that
Heusinger (Arch./, path. Anat., xxix. 358, 1864) was able in 1864
to collect forty-six German observations, that J. Gusset (These, Paris,
1877) had brought together sixty cases from French, German, and
British sources, that G. Fischer (Deutsche Chirurgie, Lieferung 34,
1880; Deutsche Ztschr.f. Chir., ii. 570, 1873) increased the number to
100, and that K. von Kostanecki and A. von. Mielecki (Arch. f. path.
Anat., cxx. 385; cxxi. 55, 247, 1890) increased it still further to
151. Taruffi has reported, in addition, a score of Italian observations
(op. cit., vii. 7, 1894).

Fistulas of the neck may be found either at the sides or in the
middle line, and they may be above or below the level of the hyoid
bone. The lateral are more common than the median, and those
below the level of the hyoid are more common than those above it.
Apparently they are more common on the right than on the left side,
for some undiscovered reason. Occasionally they are bilateral, and
are then symmetrical or asymmetrical ; rarely there is a fistula on
one side and a cyst on the other.

Lateral fistula: in the supra-hyoidean region of the neck, between
the external auditory meatus and the larynx, are rare. Lannelongue
and Menard (Affections conge' nitalcs, i. 226, 1891) report the case of
a girl, eight years of age, who showed a small fistulous opening about
half an inch below the angle of the jaw on the right side, in the
line of the anterior border of the sterno-mastoid muscle ; a sound
passed in upwards and backwards towards the ear for a distance of
about an inch ; a little fluid occasionally exuded from the opening.
In Berg's case (Arch. f. path. Anat., xcii. 183, 1883) the orifice lay a
little above the thyroid cartilage on the left side, and was thought to
communicate with the ear. In Virchow's specimen (dead-born infant)
there was a complete fistula opening externally in the neck and
internally into the pharynx ; and it communicated with the external
auditory canal, which was closed at the meatus (Arch. f. path. Anat.,
xxxii. 518, 1865). In one of Heusinger's observations there was a
double and symmetrical fistula in the supra-hyoidean region associated

452 ANTENATAL PATHOLOGY AND HYGIENE

with malformations of the outer ear (Arch. f. path. Anat., xxix. 358,
18G4).

Lateral cervical fistulce in the infra-hyoidean are commoner and
more typical. They may have a free external opening and end
blindly in the tissues of the neck ; they may, more rarely, open
freely into the pharynx, and have no external cutaneous orifice at all ;
or they may be complete tracts opening both externally in the neck
and internally in the pharynx. A few words may be said regarding
each of these varieties.

About fistula? which open only into the pharynx, which are in
fact pharyngeal diverticula, little is known. M. Watson's observation
(Journ. Anat. and Physiol., ix. 134, 1874-75) and Heusinger's three
instances (loc. cit.) stand almost alone ; but it is possible that some of
the complete fistula; which have been reported were at first in com-
munication with the pharynx alone. It must always be difficult to
bring forward proof that these diverticula were really antenatal in
origin ; at the same time there is no reason to affirm that a persis-
tence of one or other of the embryonic pharyngeal pouches is an
impossibility. Complete lateral infra-hyoidean fistula' are also
somewhat rare. It has been supposed by some that cervical fistuhe
never open into the pharynx, or at least not save by the incautious
use of the probe; but Bland-Sutton (Evolution and Disease, p. 81,
1890) has seen milk swallowed showing itself in the neck in the case
of individuals whose fistula had never been sounded at all. G.
Cavazzani (Riforma med., vii. pt. 4, 31, 1891) has reported a case of
complete fistula ; so has M. Neuhofer (Med. Cor.-Bl. layer. Aerzte,
viii. 561, 1847; Diss, inaug., Mlinchen, 1847).

Lateral infra-hyoidean fistula?, which communicate only with the
exterior, are the most typical and the commonest of these anomalies.
The opening lies somewhere along the anterior edge of the sterno-
mastoid muscle, generally not much above the level of the sterno-
clavicular joint, more rarely in the neighbourhood of one side of the
thyroid cartilage of the larynx. It is usually very small in size ; it
may lie at the top of a little eminence, or inside a depression, or may
be covered by an operculum. It usually admits only the finest
probe, and is explored with difficulty. A cicatrix has sometimes
been found inside the pharynx, a fact which has been ascribed to
closure of the internal opening of what was originally a complete
fistula (H. Kehn, Arch.f. path. Anat., lxii. 269, 1874). In direction,
the fistula is usually ascending, towards the hyoid bone (as in a
patient under the care of Dr. John Thomson, Fig. 72), or (sometimes)
towards the angle of the jaw ; its walls are often thick and hard ;
and its lumen may be irregular and may even show diverticula in
various directions (H. Eibbert, Arch. f. path. Anat., xc. 536, 1882).
The canal varies in length from a fraction of an inch up to two or
three inches. When the fistula is bilateral, it is often, but not
invariably, symmetrical in position. Sometimes fibro-cartilaginous
nodules lie in close proximity to the fistulous tract ; these perhaps
represent traces of developments in the third and fourth branchial
arches similar to the cartilages of Meckel and Eeichert in the first

CERVICAL FISTULA

453

and second. These nodules have been observed by many authors,
including P. Buttersack (Arch. f. path. Anat, cvi. 206, 1886), S.
Duplay (Arch. g6n. dc via/., i. for 1875, p. 78; Progres mtd.,v. 321,
1877), and F. W. Zahn (Deutsche Ztschr. f. Chir., xxii. 399, 1885).
The fistulse are lined with epithelium, columnar and sometimes ciliated
in the deeper part, stratified and squamous in the outer part ; the
epithelium lies on a layer of connective tissue which shows many

Fin. 72. — Dr. John Thomson's Case of Cervical Fistula.

embryonic cells. A transparent secretion sometimes exudes from
these fistula?, and may irritate the surrounding skin often forming
a crust over the opening ; it contains columnar and squamous
epithelial cells : it resembles saliva, and this resemblance caused
some of the earlier observers (e.g. Manry) to regard the fistulas as
communicating with the salivary glands. The retention of the
secretion may lead to the formation of a cyst. It may be noted that
practically the only clinical character of fistula1 of the neck is the

454 ANTENATAL PATHOLOGY AND HYGIENE

secretion : and when the malformation occurs in the horse attention
is commonly drawn to it by the effect of the discharge on the harness
(Heusinger, Deutsche Ztsehr. f. Thicrmed., ii. 1, 1875-76). Perhaps,
also, pharyngitis is more common in such cases (Hyrtl, Oesterr. mcd.
Wchnschr., p. 53, Wien, 1842 ; E. Munchmeyer, Hannov. Ann, f. d.
ges. Heilk., n.F., iv. 12, 1844); attacks of asthma may likewise be
associated (F. S. Pluskal, Oesterr. mcd. Wchnschr., p. 902, 184G).

A comparatively common associated malformation of the lateral
fistula colli is the cervical auricle or fibro-cartilaginous appendage ;
to this growth attention will be immediately directed. The same
association was noted, it will be remembered, in connection with the
fistula auris congenita and the preauricular appendage. The sub-
cutaneous nodules of cartilage found sometimes in connection with
the fistula have been already referred to.

Sex seems to have little or no influence on the occurrence of
lateral fistuke colli ; but heredity has been shown to be a powerful
factor (Ascherson, op. tit.; V. Oliva, Biv. vcneta d. sc. rued., xv. 312,
1891 ; S. L. M'Curdy, Columbus Med. Journ., ii. 244, 1883-84; A. E.
Vaughan, Brit. Med. Journ., i. for 1899, p. 148).

Treatment, if resolved upon, had better take the form of excision
of the whole fistulous tract ; but great care must be taken not to
wound important structures (nervous and vascular) in the neighbour-
hood (G. M. Lefferts, Med. News, xlii. 489, 1883).

Median cervical jistu Ice are rare, and above the level of the hyoid
bone (supra-hyoidean) they are almost unknown. When they are
present they may be discovered at any level between the hyoid bone
and the manubrium sterni : thus they may be situated in the thyro-
hyoid space (L. Le Fort, Bull. yen. dc the'rap., cix. 49, 1885); or
under the thyroid cartilage (B. Hill, Lancet, ii. for 1877, p. 842); or
on the cricoid cartilage (J. J. Jenni, Schweiz. Ztsehr. f. Mcd. Chir. u.
Gebartsh., p. 1, 1854; Kuhler, ChariU-Ann., iii. 43,3, 1878); or
over the trachea (M. Eoth, Arch,/, path. Anat., lxxii. 444, 1878: C.
Orecchia, Osscrvatore, Torino, xxxix. 289, 1888). They have charac-
ters closely similar to the lateral fistuke, but they are very rarely
complete. At first it was thought that they opened, when complete,
into the laryngotracheal tract ; but it has generally been found that
the internal opening is into the pharynx. Their general direction is
upwards towards the hyoid bone. The following references on median
fistuke may be consulted : — H. Luschka, Arch. f. physiol. Heilk., vii. 25,
1848; E. Arndt, Bert. klin. Wchnschr., xxix. 53,2,1892; E. Barwell,
Trans. Clin. Soc. Bond., xxix. 51, 189;~>-96 : B. Dalla A^edova, Clin.
Chir., vii. 828, 1899: A. Durham, Clin, Journ., iii. 150, 1893-94: F.
Huber, Med. News, lxxiii. 054, 1898: J. Bland-Sutton, Lancet, ii. for
1895, 1161 : P. Elgeti, Diss, inaug., Greifswald, 1892; M. Yolkenrath,
Diss, inaug., Bonn, 1888.

The mode of development of cervical fistuke (both lateral and
median) has been the subject of much discussion. All writers are
agreed that the lateral fistuke are branchial in origin ; but a more
complete knowledge of the ontogenesis of the neck is necessary
before all the varieties met with can be explained. It is known that

CERVICAL FISTULA 455

in the human subject the branchial fissures are not really fissures but
grooves ; a membrane always separates the outer groove or sulcus
from the inner or pharyngeal pouch. This fact makes it easy to
understand the incomplete fistula; (internal or external), but does not
explain the complete, unless indeed we imagine a simultaneous arrest
of the closure of both the internal and external grooves and a break-
ing down (or non-formation) of the septum. The position of the
fistula1, their varying length, and other characters also present difficul-
ties not easily explained away. Perhaps some of them are got over by
suggesting that the fistula? are due not so much to want of closure of
any one of the branchial grooves as of the whole sinus pnecervicalis
in which they are buried. The median fistula presents peculiar
difficulties of its own. It may be that it too is to be regarded as due
to imperfect closure of the sinus praecervicalis (with rupture of one
of the occluding membranes) or to want of union of the branchial
arches anteriorly ; but there is another possible explanation — persis-
tence of the thyro-glossal duct or canal of His. The last-named
view is favoured by C. F. Marshall (Journ. Anat. and Physiol., xxvi.
94, 1892). W. His, from his study of the ontogenesis of the thyroid
gland, has established the fact that the median part of the gland is a
tubular outgrowth from the ventral wall of the pharynx ; this he
terms the thyro-glossal duct. It consists of an upper portion leading
from the foramen caecum to the hyoid bone (lingual duct), and a
lower portion from the hyoid bone to the isthmus of the thyroid
(thyroid duct). If the middle part become occluded it is conceivable
that persistence of one or other of the terminal portions of the canal
might open anteriorly, and so cause a median fistula. Its terato-
genesis cannot, however, be regarded as settled.

Bibliography.- — In addition to the references in the text, the following
may be noted : — (1) J. Auzilhon, Montpel. med., 2 s., xiii. 566, 1889. (2)
Baerens, Monatschr. f. Med. Augerih. //. Chir., iii. 97, 1840. (3) P.
Berger, Bull, et mem, Soc, de chir. de Paris, n.s., xviii. 75, 1892 ; xxiii. 99,
1897. (4) Broca, Bull. Soc. anat. de Paris, lxiv. 385, 1889. (5) K.
Budinger, Wien, hlin. WclmscJtr., vi. 717, 1893. (6) 0. Bull, Arch.
Otol., xvii. 36, 1888. (7) H. De Stella, Ann. Soc. de med, de Gan<l,
lxxvii. 174, 1898. (8) Dubois, Gaz. med. de Paris, 3 s., ii. 1011, 1847.
(9) A. Eder, Aerztl. Ber., p. 23, 1884. (10) F. Eklund, Hygiea, xxxvii.
137, 493, 1875. (11) A. Faucon, Gaz. d. Up., xlvii. 427, 1874. (12) C.
Fevrier, Bull, et mem. Soc. de chir. de Paris, n.s., xviii. 80, 1892. (13)
M. C. T. Gass, These, Strasbourg, 1867. (14) E. E. F. Gorron, These,
Bordeaux, 1888. (15) S. D. Gross, Med. and Surg. Reporter, xi. 247,
1879. (16) S. Guzman, These, Paris, 1886. (17) B.'Hamm, Diss, inaug.,
Bonn, 1891. (18) L. Hektoen, Chicago Med. Pec, ii. 128, 1891. (19) C.
Horiox, Bull. Acad. roy. de med. de Belg., 3 s., xiv. 414, 1880. (20) A. A.
Kanthack, Jotirn. Anat. and Physiol., xxvi. 197, 1891-92. (21) F. Konig,
Arch./. Tdin. Chir., Ii. 578, 1895. (22) B. Koslowsky, Arch. f. path. Anat.,
exv. 547, 1889. (23) Ltchtwitz, Gaz. held. d. sc. med. d. Bordeaux, xvi.
471, 1895. (24) F. Mobitz, St. Petersb. med. Wchnschr, n.F., iv. 308,
1887. (25) J. Mygge, Ugesk. f. Laeger, 3 R, xx. 345, 1875. (25a) E.
Naegele, Diss, inaug., Speyer, 1866. (26) E. Neumann and P. Baum-
garten, Arch. f. Idin. Chir., xx. 819, 1877. (27) C. Nieny, Beitr. z. /din.

456 ANTENATAL PATHOLOGY AND HYGIENE

Chir., xxiii. 89, 1899. (28) Nutten, Med. Ztg., xxv. 114, 1856. (29) A.
Paci, Sperimentale, Mem. orig., xlv. 425, 1891. (30) A. Paulicky,
Detdsche md.-aerztl. ZtscJn:, ii.' 260, 1882. (31) W. Pye, Proc. Med. Soc.
Lond., xi. 310, 1887-88. (32) E. Rosenbaum, Diss, inaug., Giessen, 1895.

(33) H. Schlange, Arb. a. d. chir. Klin. d. A: Univ. Deri., viii. 60, 1893.

(34) A. Thompson, Month. Journ, Med. Sc, v. 637, 1845. (35) F. Trende-
lenburg, ArcJi.f. /din. Chir., xiii. 404, 1872. (36) E. Tricomi, Riforma
med., vii. pt. 3, 541, 1891. (37 and 38) AYeinlechner, Jahrh. f. Kinderh.,
v. 172, 1861-62; Wien. med. Presse, xvi. 518, 1875. (39) P. \Yinge,
Forh. Norske med. Sel*L: i Kristiania, p. 276, 1868. (40) E. Zeis,
Monatschr. f. Med. Augenh, u. Chir., ii. 351, 1839; Arch. f. Idin. Chir.,\'\\.
777, 1866.

Cervical Auricles.

Under the name of branchial fibro-chondromata or cervical auricles
certain congenital growths on the surface of the neck have been
described which have, in their structure, close affinities with preauri-
cular appendages (q.v. antea). In fact the cervical auricle stands in the
same relation to the fistula of the neck as the preauricular appendage
does to fistula auris congenita. Many characters are common to the
two growths, and it is unnecessary to describe fully the cervical
appendages.

With them, as with the preauricular appendages, the influence
of heredity is manifest (Eeverdin's case, Rev. mid. de la Suisse rom.,
vii. 458, 1887) ; and one member in a family may suffer from fistula
auris and preauricular appendage while another has a cervical
auricle. The two growths may also exist in the same subject
(L. Bernacchi, Atti d. Assoc, med. lorrib., i. 70, 1892). Their only
clinical importance is the deformity they cause ; and they may be
easily removed by operation.

Their usual situation is the anterior margin of the sterno-mastoid
muscle, more often low down in the neck (sterno-clavicular joint)
than higher up. They have usually a core of reticular cartilage ami
are skin-covered ; in form they tend to become pedunculated. They
are usually associated with fistuke and sometimes several of them
surround such a fistula and give rise to an appearance suggesting
an auricle in the neck ; but the association is not constant. A good
description of their histological appearances is given by E. Kirmisson
(TraiU des medadies chirurgicales d'origine congSnitale, p. 168, 1898).

It is with regard to their teratogenesis and their relationship to
the pendulous bodies in the necks of goats, pigs, etc., that cervical
auricles have excited most interest. Bland-Sutton {Evolution and
Disease, p. 85, 1890) has summarised much of our knowledge on these
subjects. He has shown that the pendulous bodies (" pendeloques,"
"breloques") in the goat contain elastic cartilage like that of the
pinna, that there are some small muscles attached to them, and that
they are situated at spots in the neck corresponding to the points
where fistula' open. L. Blanc (Journ. de I'anaf. et physiol., xxxiii. 283,
1897) has fully investigated the subject of their comparative anatomy
and teratology in the case of the goat, pig, and puppy ; he finds that
in the goat the " pendeloques " are supplied by the second cervical

CERVICAL AURICLES 457

nerve and by a filament from the hypo-glossal, that they are not
present in the goat embryo of the sixth week, and that they are,
therefore, not part of the primitive branchial apparatus but some-
thing superadded. In the pig they are often associated with a
cervical fistula ("canal du soyon") which Blanc regards not as an
imperfectly closed branchial cleft, but as a cutaneous invagination ;
they are most common in autochthonous (indigenous) races, dis-
appearing under the influence of breeding. In the embryo puppy
the appendages are represented by little warts. Blanc looks upon
the pendulous bodies as rudiments of a branchial ear, homologous with
the pinna, and developed at the orifice of the second (?) branchial
cleft. Bland-Sutton has expressed the same opinion regarding the
cervical auricles in man, regarding them as of the same nature as
the pendulous bodies in goats ; he also points out the interesting fact
that in the ancient statues and representations of fauns and satyrs
there are often pendulous skin tags in the cervical region in addition
to other goat-like characters (spina bifida tails, etc.). There is some
doubt as to which of the branchial clefts the cervical auricle, in the
human subject, is related to ; but, most probably, it is to the third
or fourth. A possible difficulty arises if we wish to accept the above
explanation of the origin of cervical auricles : if they are really
rudimentary branchial pinnae or opercula, as Bland-Sutton and Blanc
believe, then they ought to have the same origin as preauricular
appendages : but it will be remembered that there was considerable
doubt regarding the primitive branchial origin of these appendages ;
and, therefore, it would seem that the theory of the branchial origin
of the cervical growths is weakened. At the same time, I think it is
quite possible that the two structures may have different modes of
origin. Wherever atavism is in any measure invoked in teratogenesis,
difficulties of this nature arise.

Cervical Cysts.

Although cervical cysts are not malformations in the teratological
sense, it may be well if I devote a few sentences to their description
here. The cystic growths which occur in the neck may be of the
nature of spina bifida, and to these reference has already been made
(vide p. 292) ; they may be due to cystic elephantiasis, a foetal
disease described in the first volume of this Manual (p. 298) ; they
may form the so-called cystic hygroma of the neck, which is closely
related to cystic elephantiasis ; or they may constitute branchial
cysts.

Branchial cysts, as they are called, occur in the same positions
in the neck as do the fistula?, indeed their branchial origin was
suspected by W. Boser (Handbuch d. anat. Chir., Aufl. 3, p. 170,
1859) from a study of the fistula; and from the topography of the
cysts. They are fully described by Lannelongue and Menard
(Affections congenitales, i. pp. 136-210; 1891) and by C. Taruffi
(Storia della teratologic, vii. pp. 38-59, 1894), and to their works
I refer the reader for full details. The most generally accepted

458 ANTENATAL PATHOLOGY AND HYGIENE

theory of origin of these cysts is the persistence of some of the
epithelium lining the branchial clefts or grooves after the clefts
themselves have closed ; although shut in, the epithelium continues
to grow and so initiates a cyst. Attempts have been made to group
the cysts according to the branchial fissure from which they arise,
but without complete success; the best classification yet available is
the topographical or regional one. Thus there are median cysts lying
above the level of the hyoid bone and often known as sublingual
dermoid cysts or as supra-hyoidean dermoids ; others are lateral in
position although supra-hyoidean, and of these I met with an
example by accident when making a coronal frozen section of the
head of a foetus (vide Fig. 19 in the first volume of this Manual).
Others are median and infra-hyoidean, being either thyro-hyoidean
or infra-laryngeal ; and yet others are infra-hyoidean but lateral in
position. All these cysts, as a rule, consist only of one cavity ; they
are generally rounded, less often cylindrical ; they are nearly always
superficial ; the cavity is most commonly lined by stratified squamous
epithelium like that of the skin, sometimes with papillae, hairs, hair-
follicles, and sebaceous and sudoriparous glands; less often it is
lined by columnar ciliated epithelium ; and the contents vary much,
being sometimes mucus, sometimes serum, and most often an
atheromatous material containing degenerated epithelial cells. As
we shall see immediately, these characters are quite different from
those met with in hygroma of the neck. From their mode of origin
it is not to be expected that all cervical cysts will be present at
birth although some are ; it is quite easily understood that the
functional activity of the buried epithelium may not have long
enough time or be great enough to produce a cyst in antenatal life.
The differences in the character of the epithelium lining the cysts
have been explained by supposing that in some of them the included
epithelium came from the outer (cutaneous) aspect of the neck and
in others from the internal (pharyngeal) aspect.

Cystic hygroma of the neck (hygroma cysticum congenitum colli)
differs in several respects from the true branchial cysts. They are
apparently not so often met with : I have seen only one case in 327
instances of antenatal morbid states, that shown in Fig. 73, an infant
born in the Maternity Hospital, Edinburgh, in Dr. Underbill's
quarter in May 1900. They are often deep seated, and even when
apparently superficial are apt to have diverticula passing far in
among the important vessels and muscles of the neck. They are
often largo enough at birth to cause serious delay in labour, and may
fill up all the space between the angle of the jaw and the chin above
and the upper end of the sternum interiorly. They have usually a
quite irregular outline and an elastic consistence : and they are made
up of several cyst-cavities. The lining membrane is an endothelium,
like that of a lymphatic vessel, and their contents are generally clear
serum, which may sometimes be brownish in colour as the result of
haemorrhage into the cyst, in all these respects the hygroma colli
differs from the branchial cyst. Its treatment is often very difficult,
for complete excision involves prolonged dissection among vascular

CERVICAL CYSTS

459

and nervous structures which may easily be wounded ; perhaps, in
many cases, the best plan is to open the cyst, break down the septa
between the loculi, and stitch the cyst wall to the edges of the
skin incision (marsupialisation). With regard to its origin, various
opinions have been advanced. It cannot always be associated either
with the salivary glands or the intercarotid ganglion, for it may be
situated far from both these structures. There are no sufficient
reasons for looking upon it as of the nature of an angioma, and the
only satisfactory theory is that it is due to an anomaly of the
lymphatic system (Lannelongue and Menard, op. cit., i. 632, 1891).
Cystic enlargements of the thyroid gland occur, but they are distinct

Fie 73. — Cystic Hygroma of the Neck. Case No. 254.

from cystic hygroma. It is then, in all probability, to be considered
as a lymphangioma ; and it is, therefore, related to the foetal disease
known as general cystic elephantiasis, which has been described in
the first volume of this Manual (p. 297).

Cystic enlargement of the thyroid gland may cause a congenital
swelling in the neck. Such growths are usually regarded as con-
stituting one of the varieties of congenital goitre {struma congenita),
a morbid state already described in Vol. I. (p. 374). They are to be
looked upon as foetal diseases rather than as embryonic malformations,
and, therefore, do not fall to be considered here. The closely allied
cysts developed in aberrant lobules of the thyroid (supra-hyoidean,

460 ANTENATAL PATHOLOGY AND HYGIENE

hyo-thyroidean, intra-laryngeal, and intra-thoracic) have more claim
to be regarded as teratological, for they take origin in structures
which ought, in all probability, entirely to disappear. An interesting
account of them is to be found in Tarum's Storia della teratologia, vii.
pp. 76-105, 1894 ; Bland-Sutton also describes them fully in his work
on Tumours (3rd ed., p. 366, 1903). Many bibliographical references
are in the Index Catalogue (Washington) under the heading " Cysts
and Cystic Tumours of the Neck" (vol. ix. 665, 1888).

Median Fissure of the Neck.

When we consider the comparative frequency of failure of union
of the abdominal walls in the middle line it is a little surprising to
tii id that in the cervical region a similar failure is very rare. Median
fissure of the neck (tracheloschisis or schistocormus fissicollis) is among
the rarest of teratological states, and when it occurs it is associated with
upward displacement of the heart. G. Breschet (Repert. gen. d'anat.
et physiol. path., ii. 1, 1826) has, for instance, reported the case of a
new-born infant in which the heart, lungs, and thymus gland were
all extra-thoracic and lay in the neck above the clavicles and under
the jaw. The two halves of the lower jaw were separated from
each other, the apex of the heart lying between them and being
attached to the tongue. From the base of the heart the aorta took
origin and passed directly into the thorax. A somewhat similar
displacement of the heart (named by Breschet " cephalic ectopia
cordis") has been seen in the lamb (C. Weese, Diss, inaug., Berolini,
1818). Another form of median fissure of the neck is associated
with displacement downwards of the tongue {tracheal ectopia of the
tongue). This teratological state is also of extreme rarity, almost the
only known instance being that described and figured by Ahlfeld
(Die Missbildungen des Menschen, p. 167, 1882 ; Atlas, plate xxviii.
figs. 16, 17); it was that of a foetus of four or five months, in which
the whole floor of the mouth was wanting and the tongue was dis-
placed downwards into a vertical fissure in the neck. K. Kostanecki
has met with a somewhat similar state in the chick {Arch. f. path.
Anat., exxiii. 427, 1891), and is of opinion that such malformations
are not due to defects in closure of the branchial clefts, but represent
the persistence of the more primitive arrangement in which there is
a wide mesobranchial space in which lies the tuberculum impar of
the future tonmie.

.-v

Cervical Patagium.

I have already (p. 183) referred to the weblike structure
{patagium) which may be found stretching from the neck to the
shoulder on one or both sides, as in 0. Kobylinski's case (Arch. f.
Anthrop., xiv. 343, 1883). Bland-Sutton (Evolution and Disease,
p. 143, 1890) places these weblike expansions among the instances
of spurious atavism, and refuses to see in them any attempt to
reproduce in the human subject the parachute of Hying mammals

CERVICAL RIBS 4G1

or the wings of birds. I quite agree with him. Possibly the
patagium may be due to amniotic pressure which keeps adjacent
parts of the body abnormally in contact during development ; but
it must be admitted that there is a degree of mystery about it in
whatever part of the body it may be found. To regard it as a
" spontaneous variation or sport " (Bland-Sutton) does not throw
much light upon it.

Cervical Ribs.

Supernumerary ribs may be met with in the lumbar or in the
cervical region, but those in the latter locality have the greatest
clinical importance. It is seldom that a cervical rib is observed at
the time of birth, indeed they are not recognised, as a rule, till the
individual has reached the age of between fifteen and twenty years.
I have had an opportunity of examining one patient with this
deformity : she was a girl of twenty-two, under the care of Dr. James
in the Edinburgh Eoyal Infirmary, and, in addition to a cervical rib
on each side, she had an imperforate condition of the vagina with
distension of the uterus. I examined her in April 1899.

As a rule, the cervical rib is attached to the transverse process
of the seventh cervical vertebra ; it may be present on one side only,
but is more often bilateral, although never exactly symmetrical.
Barely there are two supernumerary ribs on the same side (M.
Borchardt, Berl. Jclin. Wchnschr., xxxviii. p. 1265, 1901). The rib
usually ends freely or is united to the first true rib ; it is rarely
supplied with a cartilage, and still more rarely reaches the sternum.
Sometimes it is a mere rudiment consisting of little more than the
head.

Cervical ribs may be met with in some mammals as well as in the
human subject. Thus they have been found in the dog (W. Gruber,
Arch./. Anat. Physiol, u. wissensch. Med., 542, 1867; S. Puchkovski,
Uchen. Zapiski Kazan. Vet. Inst., xiii. 227, 1896), in the sheep, in the
horse (C. Taruffi, Mem. Accad. d. sc. eli Boloyna, 3 s., x. 92, 107, 1879),
and in the whale (W. Turner, Joum. Anat. and Physiol., iv. 130,
1869-70 ; v. 348, 1870-71 ; xvii. 384, 1882-83). They are generally
regarded as an atavistic phenomenon, it being supposed that the
seventh cervical vertebra was originally supplied with a pair of ribs.

No symptoms may result from the presence of cervical ribs ; on
the other hand, curious nervous and vascular phenomena may occur,
which are apparently clue to the pressure exercised by the rib upon
the subclavian artery, the brachial plexus, and the cervical sympathetic
system. The circulation in the arm of the same side as that on which
the rib lies becomes disturbed, the limb is white, or may show appear-
ances like those in Eaynaud's disease ; various nervous anomalies,
such as neuralgia, hyperesthesia, and deficient nutrition of the nails
may also be noticed. A good description of the clinical phenomena
which may be associated with cervical ribs is given by T. E. Gordon
{Brit. Med. Joum., i. for 1901, p. 1395); in his opinion many of the
results may be due to pressure exercised by the rib not at its distal

462 WITWI'Al PATHOLOGY W'U HYGIENE

hut at its proximal end. The anomaly may be well demonstrated
by the Rontgen raya Operative interference may be required on
account of the severity of the Bymptoms; and it is well for the
surgeon to bear in mind that the pleural cavity of the same side mav
bo abnormally prolonged upwards into the root of the nook.

The anomaly has boon known since the time oi Yesalins, and
many observations have born made, one hundred and fourteen of
whioh were oollected by 0. Bngel (V ffalsrippen him Mensehen,
Mnnohon, 1887). 1 append some bibliographical references,

(1) 11. b. Ci u;k. Glasgow M ■'. Journ,, 5 -. vi. 361, 1874, (2) s. K.
C vkk. Brit. Med, J . ;.i. for 1895, p. 1162. (3) K. Coras, S

inbridge), iii. 61, L884, (4) Danlos, BuU. et mSt s nSd\ d, hop. de
Pa s, 3 - . xiv, 845, 1897. (5) A. Dymock, Edinb. M dSurg.Joum,,

xl, 304, 1833, (6) V. Ehrich, Diss .. Rostock, 1895, (7) iuiiKiiK,

if, tU Paris, xliv. 7.. 1869. (8) b. K. HsRTSLKrandA. kruu.
Jour*, Anat. and PhvsioL, \\\. 562, 1895 96. (9) K. Hirsch, IPi
Rundschau, ix. 793, 1895. ^10^ Hinitmu i fk. Ztsc) ./, trf. .1/ •".. ;'> K.,
wi\. 149, 1867. (11) C. Jswsrr, Ann. A $ ■ ■ . > .. L [01,

1878 79, (12) A. Lank, (.':.. f& Hosp. Rep., xlii. 109, 1883 84. (12a)
H. Lkboucq, Am S •• (,',r. ;', l\iv. 34, L885, (13) K. W,

brio. Proc, - Qr. Brit, and Ireland, p. iii., 1889-90. \\\] Kr

SBOBTTI, <>'./::. •'.'•'. di Torino, \'\.i. 207, 1896, (15) Gr, KoMin. (Horn.

■'. .<o. med., ii. 674, 1880. (16) V. J, Shxphskd, .1 tm,

.i/r ■•. >'-.. clxix. 112, L883. (17) 11. J, Stiles, Frans, Med.~Chir. S
Edinb., n.s.. xv, 54, 1896, (18) Stiflsr, M when, med. Wehnsehr., xliii.
145, L896; (19) b. Stirda, Arch, f. pat h. Ana , xxxvi. 425, 1866, (20)
,1. C. Warren, Boston Med. and S ' .■...> xxxiv, 258, L896. (21)

lb Au'kkson. Brit. Med. Journ., ii. for 1897. p. 1638, (22) L Groun
aikk, lr ■. mSd. d« hi m ss . xviii. 19, 1895, (23) 11. b. Barnard,

(V*. './ . xxiii. L90, 1903-04, (24) Knoch, Deutsche mil-arztl Ztschr.,
xxxii. 59, 1903, (25) b. Ranm, Wien. Min, Wehnsehr., xvi 274, L903,
(26) K. Mrnocal, s ma mSd. (Buenos Aires), x, 269, 1903, (27) A.
Wrissrnstbtn, 11 it. Win. Rundschau, xvii. 373, 394, 1903.

Malformations of the (Esophagus.

Although the oesophagus is only in pari a cervical organ, it will
be convenient to refer here to its malformations These may be
enumerated as follows:— (1) Complete absence of the oesophagus, its
place being taken by a thin muscular band stretching from tho
pharynx to the cardiac end of the stomach; (2) termination of the
oesophagus iu a simple cul-de-sac; (3) termination of the oesophagus
in a cul-de-sac, tho lower rudiment of the canal communicating with
the trachea or bronchi ; {4) tracheo-cesophageal fistula, the oesophagus
being otherwise normal; (5) membranous obstruction oi the oesophagus :
(6) presence of oesophageal diverticula; and (7) duplicity oi the
oesophagus. All these anomalies are rare, with the exception of the
third; I. therefore, choose this as the type for special description.

iijaiittil i iii per/oration of the aisophagtts, as it is railed, is in-
dieated by no external signs. The infant thus affected may be born
prematurely , but as a rule it comes at the full term, and is plump and

\\o\l \urs OF THE (ESOPHAGI S 463

well nourished. X" surprise aeed be fell on this account, the um-
bilical vein and nol the oesophagui being, as we know, the gullet of
the foetus. When the infanl is put to the breasl it is soon evidenl
thai something is wrong: the milk is returned with or without
violent vomiting, there is coughing, the face is congested, and it
Beems as if suffocation is impending; but the attack Boon passes off ;
then the child again takes the breast, only to reproduce the ame
Beries of alarming phenomena. No anomaly is found in the mouth,
the breathing is not affected, and ii is onl) when the phj ician tries
in pass ;i bougie into the Btomach thai the oesophageal iniperforation
is discovered. The rest of the clinical history of the case maj be
summarised a rapid emaciation and death usually within the first
week. Some meconium may be passed from the bowels, and some
nourishment may reach the infanl in the form of nutrient enemata,
Imi the fatal issue invariably come , No operative interference vet
devised is applicable, and the cause is evident when we consider the
morbid anatomy of Buch cases.

The pharynx ends, as a rule, a Little belov the level of the cricoid
cartilage in b blind extremity showing sometimes a Blight dilatation.

There may be i isophageal canal helou this point : but it is more

common t<> find an oesophageal rudiment passing upwards from the
cardiac end of the Btomach and ending blindly at the level of the
bifurcation of the trachea. From this lower or gastric pari of the
oesophagus a small opening establishes a communication with the
trachea, bo thai a Bound passed from the stomach into the oesophageal
pouch emerges in the air passages. The tracheo o sophageal fistula is
a practically constant arrangement, whose meaning has nol been dis-
covered; but its presence explains how ii is thai small quantities of
milk may be found in the stomach in some of these cases- the fluid
has regurgitated from the upper oesophageal sac into the mouth, it
has then found its way into the larynx and trachea, and thence
through the fistula into the lower end of the oesophagus and bo into
the stomach. Sometimes a band of muscular fibres, lying posterior to
the trachea, has been traced from the upper pharyngeal to the lower
oesophageal pouch. Normal stratified squamous epithelium has been
tumid in both the upper and the lower pouches; and while striped
muscular fibres have been noted in the upper part, only unstriped
have been seen in the lower (H. von Luschka, Arch.f. path. Anat.,
xlvii. 378, L869).

Associated malformations are uncommon, but have sometimes
been noted. Thus Polaillon (Bull, et rrUm. Soc. de chir. di Paris, n.s.,
i. 613, 1875) has recorded a case of anal imperforation with recto-
uretbral fistula, with absence of radius and club-hands, in addition to
an imperforate state of the oesophagus. In G. M. Davis' case there
was also imperforate anus (London Med.Gaz., \\.\i. 543, 1842 43); in
J. de Bary's there was transposition of the viscera (Arch. /. path.
Anat., xxxi. 430, 1864); and in 0. Ward's there was an open fora-
men ovale and ductus arteriosus (Trans. Path. Soc. Lond., viii. L73,
L856 57). Graver defects and monstrous states maybe met with,'//.
pseudencephaly (Pinard, Bull. S<>r. anat, de Paris, xlviii. 685, 1873).

404 ANTENATAL PATHOLOGY AND HYGIENE

Very little is known regarding the causation and teratogenesis of
imperf oration of the oesophagus. It has been found associated with
hydramnios and with fibro-fatty changes in the placenta (C. Perier,
Bull. Soc. de chir. de Paris, 3 s., ii. 587, 1873 ; Bordeaux mM., iii. 75,
187-4; F. Boisvert, Joum. de mSd. de Bordeaux, xvi. 125, 1886-87);
but it is difficult to see any connection between these states and the
oesophageal anomaly, unless, indeed, it be maintained that the excess
of liquor amnii is due to the foetus being unable to swallow that fluid.
It is to be noted that this teratological state is not to be confused
with imperforation of the pharynx such as occurs in agnathus and
otocephalus ; that is the persistence of a normal phase of develop-
ment (vide p. 30), while imperforation of the oesophagus, so far as is
known, is not. It is remarkable that it is practically constant in its
position (level of lower end of trachea) and that the gastric portion of
the oesophagus should always open into the trachea ; from these two
circumstances some light upon its teratogenesis ought to shine forth,
but if there be any rays they are of a kind invisible to the eye of the
teratologist and embryologist. It is possible that the fistula between
the trachea and the oesophageal pouch may be the means by which
liquor amnii reaches the stomach and intestines in the antenatal life
of these deformed infants ; at any rate it is commonly reported that
they pass meconium during the first few days of life. It may be
added that the tracheo-cesophageal fistula may exist without im-
perforation (Tarnier, Bull. Soc. de chir. de Paris, 2 s., ii. 475, 1873).

A very typical case of imperforate oesophagus was shown to me
by Dr. John Thomson in 1899 ; it was afterwards fully reported by
him (Trans. Med.-Chir. Soc. Edinb., n.s., xviii. 112, 1899). Several
other observations are on record, among which I may refer the reader,
interested in collecting references, to the following : —

(1) T. Annandale, Edinb. Med. Joum., xiv. 598, 1868-69. (2) P. B.
Ayres, Trans. Path. Soc. Lond., iii. 91, 1850-52. (3) Boucher, Bxdl. Soc.
anal, de Paris, xliii. 127, 1868. (4) F. Brentano, Bull. Soc. anat. de
Paris, 5 s., viii. 638, 1894. (5) C. K. Bowes, Brit. Med, Joum., i. for
1897, p. 586. (6) W. Bruel, Diss, inaug., Giessen, 1892. (7) J.
Houston, Dublin Hosp. Rep., v. 310, 1830. (8) L. Lehmann, Nederl.
Tijdschr. v. Geneesk., 2 E., iv., 2 Afd. 142, 1868. (9) C. E. Levy,
N. Ztschr. f. Geburtsl:,, xviii. 436, 1845. (10) T. Mellor, Lond. Med. Gaz.,
xxvi. 542, 1840. (11) J. W. Ogle, Trans. Path. Soc. Lond., vii. 52,
1855-56. (12) Padieu, Bull. Soc. anat. de Paris, x. 95, 1835. (13)
Pagenstecher, Joum. f. Geburtsh., ix. 113, 1829. (14) E. Porro, Ann.
univ. di med., cexvii. 421, 1871. (15) Schoeller, N. Ztschr. f. Gebtirtsh.,
vi. 264, 1838. (16) Sonderland, Joum. compl. du diet. d. sc. med., viii.
369, 1821. (17) F. E. Sundewall, Upsala Liikaref. Forh., v. 385,
1869-70. (18) Willett, Trans. Path. Soc. Lond., xlv. 78, 1894. (19)
B. van de Water, Diss, inaug., Leyden, 1857. (20) Morell Mackenzie,
Manual of Diseases of the Throat and, Nose, ii. 218, 1884 (Full Bibliography).

Congenital narrowness of the oesophagus, with or without an
oesophageal diverticulum, constitutes an anomaly which cannot be
absolutely affirmed to be antenatal in origin unless it be met with at
birth, and since it does not prevent postnatal life (as does imper-

MALFORMATIONS OF LARYNX 465

foration of the (esophagus) it is seldom that it is discovered at an
early age.

Malformations of the Larynx.

Malformations have rarely been reported ; but it is not safe to
conclude that they are therefore less common than many other
anomalies. Probably many of them pass unrecorded because un-
discovered (e.g. in stillborn infants).

A typical instance of imperforation of the larynx was met with
and reported by J. Cousin (Zaire me'd., xviii. 44, 1899). It was in a
well-formed male infant, born with the help of forceps. The child
made inspiratory efforts, but it could be seen that he did not respire,
although he was really living and moving his arms and legs. His
skin remained white. The heart continued to beat, so after ordinary
efforts at resuscitation had been made with no success, an attempt
was instituted to introduce the insufflator of Eibemont into the
larynx. It was then discovered that there was an imperforate con-
dition of the larynx ; the epiglottis was completely ossified and
adhered in its whole circumference to the subjacent cartilage. The
infant died twenty minutes after birth. A tracheotomy would have
been necessary to have assured the establishment of respiration, and
further interference would doubtless have been required to keep the
child in life.

Narrowing of the glottis has been described by several writers ; it
is nearly always due to a tense membrane stretching between the
vocal cords ; and this membrane, fibrous in nature, begins at the
anterior commissure of the glottis and ends posteriorly in a semi-
circular margin. Cases have been reported by T. A. de Blois (Trans.
Ainer. Laryngol. Assoc, vi. 42, 1884), 0. Chiari (Wien. Min.
Wchnsehr., x. 607, 1897), L. Elsberg (Trans. Amer. Med. Assoc., xxi.
219, 1870), P. Fraenckel (Deutsche med. Wchnsehr., xxviii. 909, 1902),
M. Mackenzie (Trans. Path. Soc Lond., xxv. 35, 1873-74), G. V.
Poore (Trans. Interned. Med. Gongr., 7 sess., London, iii. 316,1881).
G. Scheff (Allg. Wien. med. Ztg., xxiii. 280, 289, 1878), and by
F. Sippel (Med. Cor. Bl. d. Wilrttemb. drztl. Ver., lxxiii. 133, 1903).
The clinical manifestation of this malformation is complete aphonia ;
to cure this, tracheotomy has been performed ; and thereafter bougies
have been passed through the larynx by the tracheal wound with
good success in P. Bruns' case (Arch. f. Laryngol. u. PJiinol., i. 25,
1893).

Various anomalies of the epiglottis have been, from time to time,
put on record, such as absence or duplicity (F. Donaldson, New York
Med. Journ., xliv. 149, 1886 ; T. B. French, Ann. Anat. and Surg. Soc,
ii. 271, 1880; etc.): but special interest attaches to the question
whether congenital laryngeal stridor in infants is due to some
structural anomaly of the upper end of the larynx or to ill co-
ordinated spasmodic action of the muscles of respiration. J. Thomson
and A. Logan Turner (Brit. Med. Journ,, ii. for 1900, p. 1561) favour
the latter view ; but according to others the epiglottis is malformed,
VOL. ii. — ^o

46 G ANTENATAL PATHOLOGY AND HYGIENE

being folded together, and herein is the cause of the stridor (H.
Befslund, Miinchen. med. Wchnschr., xliii. 1182, 1896; D. B. Lees,
Trans. Path. Soe. Lond., xxxiv. 19, 1883: P. Bruder, Stridor laryngi
congenital des nourrzssons, Paris, 1901). As the child grows older and
the larynx becomes tinner the malformation and the stridor dis-
appear.

Muscular Anomalies of the Neck.

Various muscular anomalies of the neck are met with (Yeau,
Bull. Soe. anat. de Paris, 5 s., vii. 168, 1893). Absence of the left
sterno-mastoid in a case complicated by oligohydramnios was put on
record by Jaggard (Anier. Journ. Obstet., xxix. 433, 1894), while
Thomas (Brit. Med. Journ., ii. for 1894, p. 1178) noted an instance of
absence of the left complexus producing pseudo-torticollis. There
may be unilateral defect of the platysma myoides (E. Kemak, Neurol.
Centrlbl., xiii. 248, 1894), and the digastric muscle may show anomalies
(H. Morestin, Bull. Soe. anat. de Paris, 5 s., viii. 653, 1894 ; Siraud,
Province med. (Lyon), ix. 219, 1895). E. Juvara described a thyro-
glandular muscle in the neck (Bull. Soe. anat. de Paris, 5 s., viii. 728,
1894), and L. Wilmart reported an anomalous auriculo-stylo-glossus
(Journ. de med. chir. et pharmacol., p. 788, 1895). Many other
instances might be cited.

CHAPTER XXV

Merosoniatous Terata (<ont.) : Malformations of the Thorax : Anomalies of the
Clavicle (Absence, Incomplete Development, Bifurcation) ; Anomalies of
the Scapula (Foramina, Projections, Elevation) ; Anomalies of the Bibs
(Bifurcation, Incomplete Formation); Anomalies of the Mammary Glands
(Absence, Hypoplasia, Athelia, Polymastia) ; Anomalies of the Muscles
(Pectoralis, Sternalis, Trapezius, etc.) ; Congenital Diaphragmatic Hernia,
Illustrative Cases, Varieties, Clinical Characters, Morbid Anatomy, Tera-
togenesis, Literature ; Malformations of the Sternum ; Fissure of the
Sternum ; Ectopia Cordis ; Pleurosomus.

Malformations of the thorax include those of the osseous and
muscular framework of the chest as well as of its contained organs.
They constitute a group of very diverse anomalies related to each
other almost solely by regional proximity. In this chapter I shall
study the teratological states of the thoracic framework, and in the
next the malformations of the viscera.

Malformations of the Clavicle.

Absence or defective development of the clavicle has rarely been
recorded. One of the bones may be entirely absent (P. Niemeyer,
Tagebl. d, Versamml. detitsch. Naturf. u. Aerzte, Hannover, xl. 08,
1865); but entire absence of one or both clavicles would appear to
be rarer than incomplete development. The form which the latter
anomaly takes is absence or non-ossification of the outer or acromial
end of the bone, which has the clinical effect of allowing the shoulders
to be closely approximated in front of the thorax. L. H. D. van den
Bussche (Diss, many., Freiburg, 1890) described the case of a man
who was quite ignorant of his malformation and yet had bilateral
absence of the corpus and acromial part of the clavicle ; the state of
the platysma, pectoralis major, deltoid, trapezius, sterno-mastoid,
omohyoid, rhomboideus, and serratus anticus muscles was found to
agree with the osseous defect {e.g. the clavicular part of the sterno-
mastoid was poorly developed). N. Giannettasio's patient {Arch, di
ortoped., xvi. 65, 1899) was a man, fifty-four years of age ; his clavicles
were represented by two sternal fragments, the ends of which, instead
of reaching the scapula, floated free among the soft parts of the supra-
clavicular triangle. A similar condition of the shoulder girdle was
noted by C. Gegenbaur {Jenaische Ztschr. f. Med. u. Naturw., i. 1, 1864)
in a woman who had been twice married and who had had children
similarly affected by both husbands. Heredity was also evident in

467

4G8 ANTENATAL PATHOLOGY AND HYGIENE

G. Carpenter's case {Lancet, i. for 1899, p. 13), in which a little girl, her
father, and a brother had defective development of the clavicle. A
striking feature of all these cases is the small amount of inconvenience
which the deformity seems to have caused (G. Schorstein, Lancet,
i. for 1899, p. 10). Little is known of the teratogenesis of the
condition : the clavicle is a peculiarly human possession and it is also
the first bone in the body to ossify, therefore atavism or arrested
development or both may be invoked ; on the other hand, foetal
rickets in the second month of antenatal life has been alleged. J.
Comity {Traite des maladies de Venfance, 2nd edit., i. 973, 1904)
adopts for this malformation the name cleido-cranial dysostosis (pro-
posed by Marie and Sainton in 1897); it is true that cranial de-
formities have frequently been found associated with clavicular
defect, and that these take the form of persistence of the fontanelles
(pseudo-hydrocephalus) ; but it does not seem that the association is
sufficiently constant to establish a new name for the malformation,
nor to throw much light upon its mode of origin. If it is as rare as
the records would lead us to suppose, it is remarkable how many
eases have been reported during the past few years. I append some
references in addition to those already mentioned : G. Scheuthauer,
Ally. Wicn. med. Ztg., xvi. 293, 301, 309, 1871 ; 0. Kappeler, Arch.
d. Heilh., xvi. 265, 1875 ; F. Stahmann, Ztschr. f. Med. Ghir. u.
Gcburtsh., xi. 433, 1857 ; T. S. Dowse, Trans. Path. Soc. Lond., xxvi.
166, 1875 ; A. Guzzoni degli Ancarani, Boll, scient, Pavia, ix. 72,
1887; Todd, St. Louis Gour. Med., xix. 373, 1888; K. Preleitner,
Wien. Idin. Wchnsehr., xvi. 70, 1903 ; H. M. Sherman, Amer. Med.,
v. 569, 1903; A. Gross, MilncJien. med. Wchnsehr., 1. 1151, 1903.

Bifurcation of the clavicle would appear to lie excessively rare,
but E. H. Bennett {Dublin Journ. Med. Sc,, lvi. 413, 1873 ; lx. 160,
1875) has recorded a case in which the bone had two articulations
with the scapula of the same side, the coraco-clavicular joint being
very imperfect. In A. de Giovanni's observation (cited by Taruffi,
Storia delta teratologia, vii. 160, 1894), a projection passed from the
body of the right clavicle upwards in the neck to join the vertebral
column between the fifth and sixth cervical vertebra'.

Malformations of the Scapula.

Congenital foramina in and projections from the scapula have
from time to time been reported (W. Gruber, Arch.f. Anat. Physiol,
n.wissensch. Med., 3,00, 1871; Arch.f. path. Anat.,\\ix. 387, 1877);
the suprascapular notch may exist as a foramen; and there may be
a separate acromion process (Struthers and Ponaldson, Trans. Med.
Ghir. Soc. Edinh., xv., Appendix i. pp. 42, 52, 1896). Within the
last decade, however, congenital elevation of the scapula may be said
to have attracted more attention to itself than all the other anomalies
of that bone. This deformity seems first to have been described by
M'Burney in 1888 {New York Med, Journ., xlvii. 582, 1888), but it
was more particularly brought before the profession by Sprengel in
1891 {Arch. f. klin. Ghir., xlii. 545, 1891), and has been sometimes

MALFORMATIONS OF SCAPULA 469

called " Sprengel's deformity " in consequence. It consists in the
abnormally high position occupied by one scapula, usually the left;
there is close approximation of the upper border of that bone to the
clavicle of the same side, so that the supraclavicular space is
abolished ; and the lower angle of the bone is drawn closer to the
vertebral column, a character which distinguishes this anomaly from
the upward displacement of the shoulder blade seen in scoliosis.
Although usually unilateral, the deformity may be bilateral (J. G.
Milo, Nederl Tijdschr. v. Geneesk, 2 1L, d. 2, 695, 1897). In some
cases a cartilaginous or osseous bar has been felt passing from the
scapula towards the seventh cervical vertebra (E. Kirmisson, Traite
des maladies chirurgiealcs d'origine congenitcde, p. 490, 1898; J. H.
Waterman, Arch. Pediat., xix. 455, 1902). Associated malformations
may be met with, such as defect of the pectoralis major muscle (L.
Wolffheim, Ztschr.f. ortlwpcid. Chir., iv. 196, 1896), absence of radius
(Bolten), and anal atresia and ectopia (Kirmisson). The sister of
one of (I. Joachimsthal's patients suffered from congenital dislocation
of the hip joint {Die angeborenen Verbildungcn dcr oberen Extremi-
taten, Hamburg, 1900). In passing I may say that the reader will
find a good account of this scapular anomaly with a bibliographical
list in Joaehimsthal's work above mentioned.

As might be expected, considerable mystery surrounds the origin
of congenital elevation of the scapula. Some authors {e.g. Schlange,
Deutsche med. Wchnschr., xvii. 1380, 1891) have placed it in the
same category with torticollis as a result of primary muscular
defects ; others {e.g. M'Burney, loc. cit.) looked for an intranatal
cause, such as traumatism during labour; others (e.g. Kirmisson, vp.
cit.) suspected a malformation of the scapula, a suspicion strengthened
by one of Bar's specimens examined by Kirmisson : and yet others
(Sprengel, loc. cit. ; J. Bolten, Milnclun. med. Wchnschr., xxxix. 671,
1892) found it in deficient quantity of liquor amnii and dorsal dis-
placement of the arm during fo?tal life. It may be said, therefore, that
there is a postnatal, an intranatal, and two antenatal theories of this
abnormality. With regard to the antenatal hypothesis, that which
regards it of foetal origin is supported by the facts that deficiency
of the liquor amnii has actually been observed, and that the arms
tend to be dorsally displaced after birth in some instances. In a
case of labour at the Eoyal Maternity Hospital (Edinburgh) during
last winter (1903-04) I had my attention drawn to a case in which
the left scapula of the foetus could be very distinctly felt through the
maternal abdominal walls ; so marked was the displacement and
prominence of the bone that we expected to find Sprengel's deformity
at birth, but there was no sign of it, although, of course, it may
possibly have developed later. The theory that the high elevation
of the shoulder is sometimes at least of embryonic origin is supported
by the association of the deformity with undoubtedly embryonic
defects, such as absence of the radius, and by the presence of scapular
malformations (bar passing to spinal column, etc). We are, there-
fore, warranted in placing it among the embryonic malformations,
although there is no certainty that some cases may not be due to

470 ANTENATAL PATHOLOGY AND HYGIENE

anomalies arising in foetal life or even during and immediately after
labour. It may be added that spinal scoliosis appears to be some-
times associated with it simply as a complication.

Treatment has consisted generally of gymnastics, and occasionally
resection of the exostosis passing from the scapula to the vertebral
column has been performed.

Malformations of the Ribs.

Various anomalies affecting the ribs may be met with. I have
already referred to supernumerary ribs in the region of the neck
(cervical ribs), but an additional rib may also occur in connection
with the first lumbar vertebra (C. B. Lockwood, Trans. Path. Soc.
Lond., xxxvi. 359, 1884-85). Two-headed or bicipital ribs are also
occasionally discovered, generally in the dissecting room; the com-
monest rib to show this anomaly is the first, and there is sometimes
a doubt whether it represents a fusion of a cervical rib with the first
thoracic or of the first with the second thoracic ribs. Several
interesting contributions to our knowledge of these bicipital ribs
have been made by Sir William Turner (Journ. Anat. and Physiol.,
xvii. 384, 1882-83), W. Gruber {Arch. /.path. Anat., lxx. 139, 1877;
lxxviii. 97, 1879 ; lxxx. 82, 1880), A. Lane (Guy's Hosp. Pep., xlii.
109, 1883-84), K. L. MacDonnell (Journ. Anat. and Physiol, xx. 405,
1885-86), J. H. Scott (ibid., xviii. 339, 1883-84), and others. Some-
times a bifurcation of a costal cartilage occurs (J. Ferrier, Journ. dc
med. dc Bordeaux, xiii. 607, 1883-84). Hunauld seems to have
been the first to describe these numerical anomalies in the ribs (Hist.
Acad. roy. d. sc. de Paris, Man,, 534, 1744).

A more important, because more evident, costal anomaly is met
with in imperfect development. In some cases the ribs are simply
small in size or flattened or depressed, with the result that the chest-
wall loses its normal rounded outline ; there may even be a gutter-
like depression into which one of the arms appears to fit (Taruffi,
op. cit., vii. 162, 1894). Again, some of the ribs may be incomplete,
with the result that the osseous framework of the thorax is imperfect.
One, two, three, or four of the true ribs with their costal cartilages
may fail anteriorly, producing a hiatus in the chest-wall ; this defect
is usually unilateral, and when it is situated on the left side the
heart as well as the lung may be covered only by the soft structures
and so be brought more completely within the range of clinical
inspection, palpation, and auscultation (T. Smith, Trans. Path. Soc,
Lond., xix. 41, 1867-68). There is no constant position in the chest-
wall at which these defects are found : they may occur at any level
and at any distance from the sternum anteriorly; but, as a rule, the
first rib is not affected, the defect is anterior rather than posterior,
and the costo-chondral junction is involved. Sometimes the lung
protrudes through the opening constituting a pulmonary hernia, as is
recorded by E. J. M. Trape (These, Bordeaux, 1893), by Sabrazes
(Rev. de med., xiv. 1010, 1894), by J. Schlozer (Diss, inaug., Erlangen,
1842), by C. Seitz (Arch.f. path. Anat, xcviii. 335, 1884), and by H.

MALFORMATIONS OF RIBS 471

Haeckel (ibid., cxiii. 474,1888). Associated malformations, such as
absence or defective development of the pectoral muscles or of the
breast and nipple, are not uncommon, and the association is ex-
plicable if the teratogenic cause be pressure acting upon an area of
the side of the chest; since, also, the hand is often malformed in
these cases it is easy to understand how the theory has arisen that
constant pressure by the hand in antenatal life might lead to defec-
tive development of it and of the part of the chest against which it
pressed. To this matter I have already referred (p. 148), but I may
again draw the reader's attention to John Thomson's article (with
literature list) in Teratologia, vol. ii. p. 1, 1895. The cause that
presses the hand against the thorax may be amniotic narrowness or
bands ; in one of my specimens (Fig. 34) the arm was attached to the
thorax by a cutaneous band, and there was an extensive defect in the
side of the thorax and abdomen, but the defect was apparently much
greater than could be accounted for by the manual pressure. The
specimen, however, did not agree exactly with those which I have
been describing, for in it all the layers of the thoracic wall were
wanting and there was an aperture, a more advanced degree of defect
than that under discussion at present (vide " Pleurosomus," p. 492).

Malformations of the Mammary Glands.

Amazia or absence of the mammary gland is comparatively rare.
In 1890 I exhibited to the Edinburgh Obstetrical Society (Trans., xv.
160, 1890) the uterus from a patient who had no mammary glands and
no hair on the symphysis pubis ; she was twenty-eight years of age,
married, but sterile, and her uterus was small in size (1| inch in length)
and malformed (bicornis septus). The vagina, ovaries, and. Fallopian
tubes appeared to be normal. Defective development of the pelvic
organs does not, however, constantly accompany absence of the breasts,
for W. Wylie's patient (Brit. Med. Journ., ii. for 1888. p. 235) menstru-
ated regularly and gave birth to a well-formed male child whom she
was, of course, unable to suckle, for she had no trace of breasts, areola;,
or nipples. The defect may be bilateral, as in the cases referred to,
or, more often, unilateral ; generally the nipple is wanting as well as
the gland, but sometimes a rudiment of it has been noted (F. Paull,
Lancet, i. for 1862, p. 648). Incomplete ribs and defective develop-
ment or absence of the pectoral muscles may occur as associated mal-
formations (King, Med. Times and Gaz., i. for 1858, p. 527). In L.
Itemfry's patient it was clinically determined that the uterus was
absent (Trans. Obst. Soc. Lond., xxxvii. 12, 1896). As may be sup-
posed, the mammary defect usually passes unnoticed till adult life,
but it may be detected much earlier if attention happen to be drawn
to the chest (J. Thomson, Teratologia, ii. 3, 1895); it occurs in males
as well as in females ; and amniotic pressure suggests itself as a cause,
but cannot, of course, be absolutely affirmed.

Hypoplasia of the mammce (micromastia, Taruffi) also occurs; but
it is not antenatal but postnatal in its manifestation, becoming
apparent, as a rule, at puberty or later. At the same time, the fact

4:7-2 ANTENATAL PATHOLOGY AND HYGIENE

that it is often associated with absence of the pectoral muscles (W.
Gruber, Arch. f. path. Anat., cvi. 501, 1886), which is undoubtedly
antenatal, makes it probable that it also is predisposed to before birth.
Further, it may be associated with absence or imperfect formation of
the nipple and areola, and with defective development of the uterus
and ovaries. Like amazia, its clinical importance begins in adult
life when the mammary glands are called upon to perform their
normal function.

Athelia or absence of the nipple may be met with as the only
mammary malformation. In T. Chambers' patient (Trans. Obstet.
Soc. Lond., xxi. 257, 1880) the position of the nipples was indicated
by a small rose-coloured spot ; there were two bodies in the inguinal
regions regarded at first as herniated ovaries but afterwards found to
be testicles ; there was also a rudimentary vaginal canal. Athelia
may also occur apart from genital anomalies (W. E. Williams, Journ,
Anat. and Physiol., xxv. 304, 1890-91), and it may, of course, be
found in micromastia and in amazia.

Polymastia.

Two mammary glands are normally present, but it is not a very
uncommon anomaly to find supernumerary mammae (polymastia) or
nipples (polythelia) either in the neighbourhood of the normal glands
or in some distant part. This malformation raises several interesting
questions and requires a separate paragraph for its description.

Doubtless, polymastia was observed very early in the world's
history, and the many-breasted gods and goddesses of the East (e.g.
the Ephesian Diana) may owe their origin to real instances in human
archetypes. Eecords of actual cases go back to the middle of the
sixteenth century ; and the literature of polymastia is now very exten-
sive, Taruffi giving seventy-seven references (Storia delta tcratologia,
iii. 335, 1886), a number which has recently been greatly augmented.

Supernumerary mammae occur in males as well as females, and
may take the form of polymastia or of polythelia. They have been
more often observed in females, possibly because in them attention
is specially directed towards the mammary region in connection with
lactation. W. F. Adams {Lancet, ii. for 1895, p. 462), K. Bardeleben
(Verhandl. d, anat. Gcscllsch., v. 247, 1891, vi. 199, 1892, vii. 171,
1893; Anat. Anz., vii. 87, 1892), M. Barters (Verhandl. d. Berl.
Gesellsch.f. Anthrop., 201, 1894), E. Evelt (Arch. f. Anthrop., xx. 105,
1891), H. Handford (Trans. Path. Soc. Lond., xxxvii. 568, 1885-86),
E. Hilbert, Memorabilien, n.E, xii. 129, 1892-93), W. E. Williams.
(Trans. Path. Soc, Lo7id.,xlii. 224, 1890-91), G. Paravicini (Arch, di
psichiat., xxiv. 249, 1903), and F. Griffith (Med. News, lxxxii. 21,
1903) have all reported cases of polymastia in the male; P. D.
Handyside (Journ, Anat. and Physiol,, vii. 56, 1871-73) noted quad-
ruple mammae in two brothers, and A. Petrone (Progresso Tried., Napoli,
iii. 516, 1889) saw an instance of six mammae in a male.

In situation, supernumerary breasts show several varieties.
Usually they are found on the anterior aspect of the chest below the

POLYMASTIA 473

normal glands and nearer the middle line. Less common situations
are the middle line (Percy, Diet, d. sc. med., Paris, xxxiv. 525, 1819),
at a point external to the mammillary line (Charcot and Le Gendre,
Compt. rend. Soc. de biol., 3 s., i. 164, 1860), a point above and outside
the mammary region (P. Shannon, Dublin Quart. Journ. Med, Sc., v.
266, 1848), or over the acromion process (Klob, Ztschr. d. k. k. Gesellsch.
d, Aerzte zu JVien, xiv. 815, 1858). In February 1895 I saw a case of
supernumerary mamma (without a nipple) in the right axilla of a primi-
para in the Edinburgh Maternity Hospital (under Dr. Berry Hart's
care) ; the gland gave rise to some trouble during the puerperium and
was thus discovered. The axilla and the anterior axillary border are
among the more common situations (N. Perreymond, Union med., ii.
864, 1874; M. Notta, Arch. d. tocol, ix. 108, 1882; A. Neve, Lancet,
i. for 1894, p. 801 ; E. Martin, Ann. d'ocul. et gyndc, i. 185, 1838 :
Lichtenstern, Arch. f. path. Anat., lxxiii. 222, 1878; etc.). Super-
numerary mammae also occur outside the limits of the thorax, and
although they do not then strictly fall to be considered in this
chapter, I may briefly refer to them. They have been found, for
instance, upon the back, as C. F. Paullinus records (" Mammae in tergo,"
Miscell. Acad, nat. curios., Dec. ii., Ann. iv., Appendix, p. 203, 1686),
on the thigh (Chaussier and Magendie, Journ. de physiol. exper., vii.
175, 1827 ; L. Testut, Bull. Soc. d'anthrop. de Paris, 4 s., ii. 757,
1891 ; C. D. Homans, Boston Med. and Star/. Journ., lxxiv. 181, 1866),
in the vulvar labia (E. Hartung, Diss, inaug., Erlangen, 1875), on the
cheek (A. Barth. Arch. f. path. Anat, cxii. 569, 1888), on the upper
part of the anterior abdominal wall (Centralbl.f. Gynak.,x. 730, 1886),
and within ovarian dermoids (Bland-Sutton, Ovaries and Fallopian
Tubes, 58, 1891 ; Tumours, 405, 190.'!). Supernumerary mamma- in
the anterior abdominal wall below the level of the umbilicus are
apparently very rare ; Windle, however, in his eleventh report on
recent teratological literature, refers to a case (in a man), apparently
well established, reported in 1899 by Braquehaye, in which there was
an accessory gland midway between the navel and the penis ; T. J.
M^Gillicuddy (Med. Bee, New York, xl. 446, 1891) described another
case, also in a man, where there were two mammae, one on each side
of the umbilicus.

As a general rule there is only one accessory breast, and when
this is the case it is usually on the left side (Lichtenstern) ; there
may, however, be two, and then they are commonly but not invari-
ably symmetrical; three have been observed (Percy, Journ, el, rued.
chir. pharm., ix. 378, 1805) ; and Neugebauer some years ago sent me
the photograph of a woman having the large number of eight
(Oentralbl, f. Gynelk., x. 729, 1886). The latter case, however, is
rather an instance of multiple nipples (polythelia) than of super-
numerary mamma'. R. E. Fitzgibbon saw a man who had four
accessory nipples (Dublin Quart, Journ. Med, Sc., xxix. 109, 1860).

In many cases the accessory gland has a distinct nipple and
areola, but these may both be absent; on the other hand, the nipple
or the areola may be the only representative of the breast that is
found. At the present time (June 1904) I have under my care a

474 ANTENATAL PATHOLOGY AND HYGIENE

pregnant woman witli an accessory areola under the left one ; it has
become darker during pregnancy and distinctly shows little spots
like Montgomery's follicles; it is about one-third the size of the
normal areola. The supernumerary mamma is generally much smaller
than the normal one, and is sometimes connected with it by a canal
of communication.

I have not here to deal with the postnatal history of polymastia,
but it may be said that sometimes such accessory glands become
functionally active during lactation, and secrete milk which can be
drawn off through the nipple. In other cases the milk exudes through
a sinus which takes the place of the mammilla, and in others it is
retained, setting up a painful swelling of the breast and leading to
the formation of an abscess. Accessory breasts are nob exempt from
cancer (F. T. Paul, Brit. Med. Journ., ii. for 1894, p. 1309).

Associated malformations seem to be rare in polymastia, a tera-
tological fact which no doubt has a meaning if it could be discovered.
J. P. Priickel (Miscell. Acad. nat. curios., Dec. i., Ann. ix. et x., obs.
viii., p. 45, 1680) describes very briefly a case of polythelia accom-
panied by Polydactyly (six digits on each extremity) ; and Anne
Boleyn,one of the wives of Henry vin.,is said to have been deformed
in the same way.

The Comparative Teratology of polymastia, as might be expected,
is full of interest, and of interest which has not yet been exhausted
by research. Since mammary glands are confined to the mammalia,
it is only in these animals that their anomalies come under considera-
tion. When there are normally many mamma' in an animal they
are usually arranged in two parallel rows along the ventral aspect of
the body ; and when they are less numerous either the abdominal or
the thoracic members of the series may be absent ; in man and the
monkeys, for instance, it is supposed that the inguinal mammae have
aborted leaving only the pectoral pair. In the lemurs almost all the
possible mammary arrangements may be noted in the different species.
J. Bland-Sutton (Evolution and Disease, 162, 1890) gives an interest-
ing account of this subject, and records two cases of polymastia in
monkeys, and refers to instances in the cow, the goat, the sheep, and
the female zebu.

From the comparative teratology of polymastia we pass naturally
to its teratogenesis, for the most widely accepted view is to regard it
as an atavistic phenomenon, a reversion to an original type possess-
ing more than two mammary glands. When additional mamma' or
nipples occur on the thorax or abdomen along the lines of the deep
and superior epigastric arteries, the atavistic theory has strong claims,
upon our belief, and it seems reasonable to regard them as represent-
ing the mammary glands of other mammals. The occurrence, however,
of erratic mammae, their presence, for instance, in the axilla, on the
thigh, or over the acromion process, introduces difficulty and doubt
into the question. These can hardly be looked upon as atavistic, and
Darwin himself admitted this. Ahlfeld's theory of transplantation of
surface tissues to other parts of the body by amniotic pressure scarcely
commends itself as a reasonable explanation (Die Missbildungen des

POLYMASTIA 475

Menschen, p. 110, 1880). The view which seems best supported at
the present time is to look, with Bland-Sutton, upon polymastia as
explicable in some cases by atavistic reversion, in others by dicho-
tomy of a single gland (as when there are bifid nipples), and in others
as a spontaneous variation or sport. When, however, we come to
look more closely into the embryological aspect of the matter we get,
as we nearly always do, some fresh light upon the subject. H.
Schmidt (Anat. Anz., xi. 702, 1896), being struck by O. Schultze's
discovery of a milk line or ridge in embryo pigs (Sitzungsb. d. phys.-
med. Gesellsch. zu Wilrzburg, 77, 1892), made careful observations
regarding its possible presence in the human subject. At first his
researches gave negative results, but later (Morphol. Arb., vii. 157,
1897) he found in human embryos of about 15 mm. long a line
of elevated epithelium on the side of the thorax and abdomen ; this
he termed the " milk streak." Within or along this streak were the
anlagen of the normal mamma-, but in longer embryos (26 to 60 mm.)
there were also epithelial anlagen of various sizes and positions, which
he regarded as the earliest stages of supernumerary milk glands ;
they occurred in the axillae, on the anterior and lateral walls of the
thorax, and in the inguinal region, the positions where hyperthelia
and hypermastia (polymastia) most often are seen in the adult.
There is, therefore, no necessity to invoke atavism or dichotomy or
" sport," for there are in the embryo rudimentary mamma', and it is
sufficient to say that here as in other malformations an arrest of
development serves to account for the anomaly. Instead of entirely
disappearing, as is normal, one or more of the supernumerary anlagen
remain and give rise to polymastia and polythelia. Dauthuile (These,
Lyon, 1899) holds similar views and meets the difficulty of explain-
ing the erratic mammas of the back, thigh, and face, by insisting upon
the close histological relationship between mammary and sebaceous
tissues ; pseudo-mammas may originate from sebaceous glands in any
part of the body. It seems safe, therefore, to conclude that when
supernumerary breasts or nipples develop in the " milk streak " they
represent the persistency of structures which ought to be transitory,
and that when they occur elsewhere they are not real mammas but
giant sebaceous glands. Bland-Sutton's observation upon the ten-
dency of protected cutaneous surfaces (e.g. the skin in the axilla) to
become glandular may be held as supporting this view of the origin
of pseudo-mammai outside the " milk streak." The fact that poly-
mastia is not infrequently hereditary (Tiedemann, Ztschr.f. Physiol.,
v. 110, 1835; Petrequin, Gaz. med. de Paris, v. 197, 1837; J. C.
Saunois, These, Paris, 1812; Garland, Edinb. Med. Joum,, xxiii. 45,
1877-78) does not form an argument against the theory of arrested
disappearance of primitive anlagen, for heredity exists in many ano-
malies which are capable of this explanation. It simply suggests the
presence of a germinal factor which in some way leads to the per-
sistence of states which are normally transitory.

476 ANTENATAL PATHOLOGY AND HYGIENE

Muscular Anomalies of the Thorax.

Absence or imperfect development of the pectoral muscles is one
of the most characteristic of the muscular anomalies of the thorax.
It has already been alluded to in connection with costal and mam-
mary defects, and requires only a short notice here. .John Thomson's
article (Teratologia, ii. 1, 1895), besides containing records of three
cases, brought together a wealth of information on the subject of
this anomaly ; and although many papers have appeared since its
publication, they have not altered appreciably the conclusions con-
tained in it.

Both the pectoralis major and minor may be absent or defective,
and yet, as was noted also in connection with absence of the clavicles,
there may be very little consequent inconvenience. The power of
using the arm may not be appreciably diminished (H. M. Bowman,
St. Barth. Hasp. Rep., xxx. 125, 1894), and, as a matter of fact, the
muscular anomaly may be discovered accidentally, as in the examina-
tion of recruits. At the same time the appearance of the chest is
characteristic. There is a marked depression in the pectoral region
on one or both sides ; when it is unilateral, the one side of the chest
is flatter and has smaller measurements than the other ; the mam-
mary gland and nipple may be absent or poorly developed ; it was
frequently noted that a fibrous band (patagium) passed from the
ribs to the humerus ; and several neighbouring muscles have in
certain instances been found to be defective {e.g. serratus magnus,
latissimus dorsi, intercostals, teres major and minor, etc.). When the
pectorals were only defective and not entirely absent, it was generally
the lower or sterno-costal part that showed the anomaly. The right
side was apparently more often the seat of this deformity than the
left, and males were five times as often affected as females (Thomson,
loe, cit.\ In some cases in adults the axillary and mammary hair
(M. Schein, Arch, f. Dermat. v. Syjih., lxviii. 323, 1904) was observed
to be absent, there was very little subcutaneous fat in the chest-wall,
and the skin occasionally showed a congenital scar. Other associated
malformations were absence of parts of the ribs and sternum (q.v.
p. 470), and of the hand and arm (e.g. syndactyly, absence or short-
ness of digits, curving of the forearm, etc.). It is possible that both
the pectoral defect and the associated malformations may be due
to prolonged pressure of the arm against the chest in antenatal
life : this may, in its turn, lie caused by narrowness of the
amnion. Heredity seems to be very seldom associated with this
anomaly.

Thomson gives the literature of the subject up to 1894: the
following later references may be added : — Cruchet, Journ. <le mid. de
Bordeaux, xxv. 509, 1895; B. Prinz, Diss, inaug., Wiirzburg, 1894;
Overweg, Deutsche mil.-arztl. Ztsclvr., xxiv. 440, 1895; Hofmann,
Arch. f. path. Anat,, cxlvi. 163, 1896; Joachimsthal, Berl. klin.
Wchnschr., xxxiii. 804, 1896; M. Kaiser, Ztschr. f. Jclin. Med., xxxii.
Supplhft., 174, 1897: H. Morestin, Bull. Soc. anat. de Paris, 5 s., x.

HERNIA OF DIAPHRAGM 477

144, 1896 ; J. Azam et J. Casteret, Presse mid., 53, 1897 ; J. Hutchin-
son, Arch. Surg., viii. 355, 1897: A. Schmidt, Deutsche Ztschr. f.
Nervenh., x. 400, 1897; G. A. Sutherland, Clin. Journ., xi. 173,
1897-98; Fallot, Mar* tile mid.,xxxv. 54, 1898: E. W. Sclmeller,
Med. Ree., liii. 282, 1898: J. Harold, Lancet, i. for 1898, p. 499: M.
Lengsfelden, Wien. Hin. Wchnschr., xv. 1306, 1902: W. E. Williams,
Lancet, i. for L903, p. 61.".; J. Martirene, Rev. d'orthop., 2 s., iv. 209,
190:: : Pxitticher, Deutsche med. Wchnschr., xxix. Ver.-Beil., 158, 1903 ;
Grisel, Per. d'orthop., 2 s., iv. 359,1903; 1). Zeiliger, Praht. Vrach,
ii. 611, 6:'^, 1903; H. W. Smith, Amer. Med.,\i. 734, 1903: L. A.
Earriehausen, Diss, inaug., Gbttingen, 1903; and E. Joiion, Rev.
d'orthop.,2 s., v. 71, 1904/

Other muscular anomalies of the thorax are found in the presence
of a musculus sternalis or presternalis, as has been noted in connection
with auencephaly (q.v. p. 347), and regarding which various writers
have stated their views (Samiac, Toulouse mid., 2 s., v. 99, 190.*! ;
Halipre et Cauchois, Normandie mid., xviii. 453, 1903; Piollet, Lyon
mid., ci. 832, 190.*!): other supernumerary muscles are considered by
Cr. S. Huntington (Proc. Assoc Amer. A mil., p. xii. 1 902-03). Absence
of the trapeznis has been described by A. Schiiller {Wien. Min.
Wchnschr., xvi. 516, 1 903) and by II. von Haffner (Internal. Monalschr.
f. Anal. u. Physiol., xx. 313, 1903), and of the deltoid by Fortin and
( haplain (Normandie med., xviii. 299, 1903). "Without doubt, however,
the most important muscular anomaly in connection with the thorax
is defect of the diaphragm leading to congenital diaphragmatic hernia :
that subject requires several paragraphs to itself.

Congenital Diaphragmatic Hernia.

Congenital diaphragmatic hernia is a grave malformation unaccom-
panied by any external sign of its existence. It is essentially an
anomaly in the relations of the abdominal to the thoracic viscera, and
as such gives no indication of its presence until either the abdomen
or the thorax is opened into. It is a comparatively common mal-
formation, as is demonstrated by the fact that 1 have been able to
collect from medical literature no less than a hundred cases published
between the years 1888 and 1900. Many cases had been reported
prior to 1888, and there have been others since 1900, so that it may
be concluded that the bibliography is a large one. There were three
personal observations in my list of a hundred cases : one (Plate
XXIII.) was an infant externally normal in appearance ; another
(Fig. 74) was an anencephalic foetus ; and the third was an inien-
cephalic foetus (Plate XVI.). I shall describe in detail the first of
these three specimens (Plate XXIIL), for it will serve to set forth
the typical characters of the anomaly in a striking fashion.

The infant, a full-time female, was born without instrumental
aid. The confinement took place in the outdoor department of
the Edinburgh Medical Mission Dispensary, in January 1896. The
presentation was the vertex, and the child was large, well formed
externally, and apparently healthy. She was stillborn however,

478 ANTENATAL PATHOLOGY AND HYGIENE

and although efforts at breathing were made, and although attempts
at resuscitation were continued for a long time, respiration was never
fully established. The parents were said to be healthy. Dr. Graham,
the Resident at the Dispensary, began to make an autopsy in the
usual manner: but on opening into the thoracic cavity he found
some coils of intestine ; so, diagnosing a grave malformation, he
replaced the chest-wall and communicated with me. I decided to
make a coronal frozen section of the body, and in this manner
to make clear the displacement of parts winch was evidently
present.

The trunk was frozen the usual way, and a vertical coronal section
was made, which passed through the shoulder joints above and the
hip joints below. I had hoped that the section would have included
the defective part of the diaphragm (for I had made up my mind
that such an anomaly existed), but unfortunately it passed just
posterior to the defect which was afterwards found to occupy the
portion of the diaphragm which normally comes into relation with
the pericardial sac ; in the lower part of the trunk, also, it passed
between the uterus and bladder, showing neither well. The most
striking appearance seen on section was the presence of a large
number of the abdominal organs in the left half of the thoracic
cavity.

The posterior slab of the section (Plate XXIII.) showed very
plainly the misplacement of the abdominal and thoracic viscera :
unfortunately, it passed, as has been said, behind the herniated part
of the diaphragm. It, therefore, showed no break in the continuity
of the diaphragm, either on the right side or on the left ; but it was
clear from it that the left side of the thoracic cavity contained a sac,
afterwards found to be made up of thinned-out diaphragm, which
contained small intestines, stomach, and colon. In the right side of
the thorax was the heart in the pericardial sac, and above it was the
thymus gland. When the heart was lifted up, the right lung, con-
sisting of three lobes and quite unexpanded, was found lying behind
it. In the middle line the trachea was seen twice cut into in this
section ; it was displaced towards the right side. Immediately to
the right of the distended stomach was a part of a gland which
further investigation showed to be a piece of the left lobe of the
liver. It was thought at first that the left lung was absent ; but a
prolonged search revealed it, small in size (1 inch in diameter),
lying in the apex of the thoracic cavity, a little to the left of the
middle line, and in close contact with the upper loop of colon, but
separated from it by the diaphragmatic sac. Below the diaphragm
on the left side was the spleen in section, measuring about If inch
by 1 inch. Below and internal to it was the left kidney and supra-
renal capsule, while in the middle line were the soft structures in
front of the vertebral column. On the right side was the right lobe
of the liver, measuring, as seen in this section, about '2 b inches
vertically and 2 inches transversely. Below the liver was a loop of
large intestine, containing meconium, which was found, on further
examination, to be the commencement of the sigmoid flexure ; below

Plate :

Shoul/ier

Trachea.

Thymus.

Upper border of
diaphragmatic sac.

Colon.

Liver
(left lobe).
Right lung.

Diaphragm

Liver
(right lobe).

Spleen.

Small intestine.
. Stomach.

Small intestine.
Colon.

1_ Diaph ragm.

Suprarenal
capsule
(left).

Kidney (left).

Upper end
of rectum.

Uterus.

Head of femur
deft).

Vaginal
wall.

Anus.

HERNIA OF DIAPHRAGM 479

it, and nearer to the middle line, was another piece of large intestine,
the upper part of the rectum. Still lower down was the uterus, and
below it was the vagina cut into in two places. Quite at the lower
border of the slab, the anal canal had been opened into. The right
kidney and suprarenal capsule, lying behind the right lobe of the
liver, were seen only when it was raised.

A study of the anterior slab of the section brought out several
additional particulars. On the right side of the middle line the
thorax was seen to contain the heart, the thymus gland, part of
the left lobe of the liver, and the left lung ; on the left side were
the small intestines, the distended stomach, and the loop of colon.
Immediately anterior to the parts seen in this section wTas the defect
in the diaphragm, which consisted of a membranous sac extending
up into the thoracic cavity, into which could be seen passing the
loop of colon (found to be the transverse) and the coils of small
intestine. The loop of meconium-containing 1 towel in the upper part
of the thorax turned out to be the caecum with appendix vermiformis
attached to it. Below the diaphragm were seen the same parts as in
the posterior slab, with, in addition, coils of small intestine in the
middle line, and below them the sigmoid flexure and urinary bladder.
In the lowest part was the vaginal canal, for the section had passed
between the bladder and uterus, just shaving off a bit of the cervix
uteri. The tube and ovary of the right side were seen just above the
upper end of the bladder.

Prom the comparison of the two slabs of the frozen section it was
possible for me (with a little extra dissection) to arrive at a conclusion
regarding the nature of the malformation from which this infant was
suffering, and which had proved fatal to her postnatal life, although
it had in no way interfered with her antenatal existence. The
anterior part of the left side of the diaphragm was deficient in
muscular tissue, and projected upwards as a membranous sac, filling
the whole of the left side of the thorax. In this sac were the small
intestines (ileum chiefly), the stomach, the transverse and ascending-
colon, and the greater portion of the left lobe of the liver. Above
the sac was the undeveloped left lung. Through this intrusion into
the left side of the thorax of the structures which normally occupy
the abdomen, the heart was displaced to the right, and the thymus
gland with it, while the development of the left lung was practically
stopped. During its displacement the heart seems to have been
rotated, for the left ventricle lay anterior and the right behind. It
seemed also that the drag of the dislocated abdominal viscera on the
left side had brought the corresponding kidney and suprarenal capsule
forward.

Since the herniated abdominal viscera were contained in a distinct
membranous sac, this case fell to be classed as one of hernia ilia-
phragmatiea vera, or true diaphragmatic hernia, of the left side,
situated in the space between the portio sternalis and the portio
costalis of the diaphragm. But the presence of the sac is not a
constant character, and I have met with a case in which it was
absent, constituting hernia diapliragmatica spuria. This was in an

4S0

ANTENATAL PATHOLOGY AND HYGIENE

anencephalic foetus, sent to me for examination by Dr. Millard. It
was a female, and its birth was preceded by a pregnancy characterised
by excessive vomiting. The gestation had just passed the seventh
month when labour occurred ; there was marked hydramnios, the
quantity of liquor amnii (of a turbid brown colour) being estimated
at -!, gallons. Forceps had to be used to extract the foetus. The
placenta was entirely adherent, and had to be picked off with the
fingers; it was very large, but also very thin, in no part being more
than \ inch in thickness, and generally being \ inch ; there were

fx *

i

&£* C4i£aceA,

... Xtf/>*~f£> f*-*' a-j-4

\

_3._J W)

- T

Fig. 74. — Congenital Diaphragmatic Hernia in an Anencephalic Foetus.
Sectional appearances. Specimen No. 204.

two or three fatty patches in it. The
of age, and had had one previous labour
infant. She came of a healthy family
well developed, but very nervous. The
tion, and, since I had no suspicion that
any anomalies other than those usually
the customary mesial sagittal section.
is shown in Fig. 74.

mother was thirty-nine years
, ending in the birth of a dead
, and was herself healthy and
foetus was frozen for examina-
the internal organs presented
found in anencephaly, I made
The right side of the section

HERNIA OF DIAPHRAGM 481

The section showed very clearly the defect of the cranial bones,
the backward direction of the basis cranii, the extraordinary bending
of the vertebral column in the cervical and upper dorsal regions, and
the condition of spina bifida affecting nearly the whole length of the
spine. In addition, however, to these changes, which are practically
common to all cases of anencephaly, the section showed also that the
internal organs did not bear a normal relation to each other. The
heart lay entirely to the right of the middle line of the body ; for the
vertical sagittal section had passed between it and the pericardium,
leaving the latter in the left slab of the section. The thorax, behind
the heart, contained coils of small intestine, while to the front was
situated the thymus gland. When the heart was lifted up and the
pericardial sac cut through, it was found that the right lung was
lying quite unexpanded in the right side of the thorax. The
diaphragm on the right side was quite normal, and below it lay the
right lobe of the liver. Below it again were some coils of small
intestine and the commencement of the sigmoid flexure, and lying
partly above and partly below the pelvic brim were the urinary
bladder and uterus, the latter in a position of marked anteversion.
When the left slab of the section was examined the following-
additional particulars were made evident. There was no diaphragm
at all on the left side, and the abdominal organs simply passed up
into the left side of the thoracic cavity as if it were a part of the
abdomen. Consequently, above the level of the place where the
diaphragm ought to have been there were found the left lobe of
the liver (somewhat deformed), part of what was probably the trans-
verse and descending colon, the stomach (in a contracted condition),
and several coils of small intestine. Quite in the apex of the left
side of the thoracic cavity was a small lung, the left, in an almost
undeveloped state ; it was not separated from the coils of intestine
by a membranous sac, and in this respect the arrangement differed
from that in the preceding case where there was not actual absence
of the diaphragm but only the projection of a non-muscular extension
of it into the thoracic cavity. I could find no trace of the spleen.
In the lower part of the abdominal cavity on the left side were coils
of small intestine and the caecum and appendix vermiformis, along
with a portion of the ascending colon. The kidneys occupied their
usual position, one on each side of the vertebral column. Only
twenty vertebra- could be counted in the spine, the absent members
being apparently chiefly dorsal.

In the second specimen, therefore, there existed that form of
diaphragmatic hernia to which the name spurious or false has been
applied ; for the diaphragm showed a complete unilateral defect, and
the displaced abdominal viscera had consequently no sac. A some-
what similar specimen was examined by me in 1899. It has been
described on page 279, and is figured in Plate XVI. ; it was an
instance of iniencephaly, and, as in the former cases, the existence of
a diaphragmatic defect was not suspected till a section was made.

From the description of these specimens the reader will have
obtained some idea of the clinical features and morbid anatomy of
vol. ii. — 31

482 ANTENATAL PATHOLOGY AND HYGIENE

congenital diaphragmatic hernia ; but I may add here some further
details on both these matters, making use of my unpublished analysis
of one hundred cases reported in various parts of the world between
1888 and 1900. I append (on p. 486) the literature list and refer to
the cases simply by numbers.

It is a striking fact that little or nothing is stated regarding the
health of the mother in most of the one hundred cases of foetal dia-
phragmatic hernia. In one instance (18) she had syphilis ; in another
(76) she had mitral disease ; but in all the other cases there is nothing
to lead us to think that the mother was an unhealthy woman before
she became pregnant with the foetus which showed this deformity.
In no case is there the record of family prevalence of the diaphrag-
matic defect or of heredity. In only twenty-three cases are we told
the parity of the mother : she was a primipara in five (18, 34, 51, 55,
82), a secundipara in seven (6, 42, 67, 83, 85, 88, 98), a quadripara in
two (68, 96), a quintipara in two (75, 76), a sexipara in two (90, 100),
an octipara in one (81), a nonipara in one (99), and a " multipara " in
three (1, 8, 49). In no less than seventy-five cases no information
was forthcoming regarding the symptomatology of the pregnancy : in
twelve out of the twenty-five cases in which some facts were supplied
to us the pregnancy went to the full term, and, in the remaining
thirteen, labour occurred more or less prematurely. In five instances
the gestation was complicated by hyclramnios (49, 67, 68, 98, 100), in
one by dispncea (76) ; in one the pregnancy was plural (40), and in
one the product was a double-headed monster (49). By far the most
interesting pregnancy-history was that furnished by J. Ikeda of
Fukuoka, Japan (30) : it was the case of Ikeda's wife. She noticed
that at the beginning of the eighth month of her pregnancy rhythmic
movements of the foetus began to be felt, and were most marked to
the left of the umbilicus. Ikeda made an examination at the time,
and made out that the foetus was lying L.O.A., and that the foetal
heart-rate was 152 per minute. He also felt quite distinctly the
peculiar foetal movements : they occurred several times in the twenty-
four hours, consisted of from ten to twenty taps, and lasted from ten
to fifteen minutes with pauses of a few seconds. They were regarded
by Ikeda and his wife as hiccough or singultus of the foetus, a con-
clusion which was borne out by the postnatal history of the infant ;
at the autopsy, the infant having died when eight days old, a dia-
phragmatic hernia was found.

In seventy out of the hundred cases no facts regarding the labour
were stated ; in eighteen it was described as normal ; in the remain-
ing twelve it was prolonged, preternatural, or complex, but even in
them it is probable that it was not the diaphragmatic hernia bait
associated morbid states (hydramnios, placenta prsevia, adherent
placenta) that led to the disturbance of labour. It is unlikely that
such a concealed malformation as hernia of the diaphragm should
have any effect on the progress of the confinement.

The clinical history of the infant was generally stated, and was
strikingly similar and brief. Thus, in fifty-three out of the hundred
cases, the child was stillborn, or gave only a few gasps, dying within

HERNIA OF DIAPHRAGM 483

one hour. In a few cases, however, life lasted for a short time : for
two hours (40), three and a half hours (43), six hours (36), twenty-
four hours (34, 06, 69), forty-three hours (44), fifty-five hours (99),
two days (39), seven days (57), eight days (30), two and a half
months (74), five months (84), and fourteen months (88).

In quite another group of cases the diaphragmatic hernia,
although congenital, was only discovered some years after birth, e.g.,
at the age of three and a half years (5), of eight (71), of sixteen (14,
73), of nineteen (9), of twenty-two (52), of twenty-four (7), and of
twenty-seven (20). In some remarkable cases it was only recognised
at an advanced age, e.g., at fifty years (29, 78), at fifty-five (86), at
fifty-seven (50), and at sixty-seven (53). It is evident, therefore,
that in some of its forms congenital diaphragmatic hernia does not
interfere with postnatal life.

The male sex seems to lie more liable to hernia of the diaphragm
than the female ; thus in the hundred case-records collected by me
there were forty-seven males and twenty females, and in the remain-
ing thirty- three instances the sex was not stated. In nine cases
(1, 2, 24, 30, 41, 42, 56, 58, 90) it was noticed by the medical attendant
that the heart was pulsating on the right side of the chest at birth ;
in one case (68) the presence of epigastric depression was observed ;
but neither in the new-born infant nor in the adult was the diagnosis
of diaphragmatic hernia often made during the life of the subject.

An interesting clinical history was furnished by J. O'Dwyer (5).
The patient was a boy, three aud a half years of age, who had been
sick for four days. He had severe dyspnoea and prostration; his
temperature was 105° F. ; and there had been obstruction for two
days. There was flatness over the posterior part of the left side of
the chest with absence of respiratory sounds, and a moderate dulness
anteriorly with broncho-vesicular breathing at the upper part.
There was resonance of a somewhat tympanitic quality in the sixth
interspace in the axillary line. The heart was displaced to the right,
with the apex beat in the epigastrium. The case was diagnosed as
one of empyema, and a needle was inserted, but with no result. Eight
days later the bowels were freely moved with cathartics, and the
temperature became normal but the general condition remained poor.
Several attacks similar to the above were recorded during the follow-
ing two months. In the last of these no relief followed evacuation
of the bowels with cathartics. The left side of the chest measured
f inch more than the right. O'Dwyer diagnosed empyema with
positiveness ; but the insertion of a large needle and then of a trocar
brought no pus. A large opening was then made in the sixth inter-
costal space, and intestine appeared ; the wound was closed. On the
following day another opening was made into the pleural sac lower
down (tenth interspace), and an opening in the muscular part of the
diaphragm (1| inch in diameter) was found. About 3 inches of the
ninth and tenth ribs were removed, and the floating ribs were drawn
down, and so O'Dwyer got room to insert his whole hand. He had
some difficulty in replacing the intestines in the abdominal cavity,
the csecum being the last part to be reduced. The edges of the

484 ANTENATAL PATHOLOGY AND HYGIENE

wound were then pared and strong braided silk sutures passed; even
after being sutured, the diaphragm bulged greatly into the pleural
cavity. Death occurred six hours later.

Want of space forbids the citing of other instances of the
postnatal clinical history of cases of diaphragmatic hernia ; but
O'Dwyer's patient may be taken as typical of the cases in which the
child survives its birth, although there are many variations in the
symptomatology according to the mode in which the abdominal
organs are displaced into the thoracic cavity.

A great deal has been written regarding the morbid anatomy of
congenital diaphragmatic hernia. The cases which I have described
will have given the reader an idea of the two outstanding varieties,
the form with a sac and that without one, the true and the spurious
as they have been called ; but some additional facts may be stated.
The sac of the true hernia consists of parietal peritoneum and of a
greater or smaller part of the parietal pleura, and sometimes some
fibres of the centrum tendineum of the diaphragm are found in the
wall. The hernia may be on the left side and may take place through
an opening between the portio sternalis and the portio costalis of the
diaphragm, or through % or in the neighbourhood of the foramen
ossophageum, or in the middle of the left half of the tendinous part
of the diaphragm, or posteriorly somewhere in its circumference. On
the other hand, the opening may be on the right side (Lepage, Compt.
rend. Soc. d'ohst., gyndc., paed. de Paris, v. 160, 1903) in situations
corresponding to the above (with the exception of that through the
oesophageal aperture). Again, there may be not so much a hernia
as a dome-ahaped distension of the diaphragm upwards — eventratio
diaphragmatica (Arnheim, 59) ; this is rare, and has only been seen
on the left side ; the wall consists of peritoneum, of pleura, and of
the greatly distended and thinned-out tendinous and muscular part
of the diaphragm. In the spurious form of diaphragmatic hernia
there is, as has been stated already, no sac. The abdominal contents
simply occupy the pleural cavity. There are several varieties : the
whole diaphragm may be absent; the left half may be entirely
wanting, or there may be a large opening in it, or there may be
smaller openings (e.g. through foramen oesophageum, between the
sternal and costal part, in the centrum tendineum, between the pars
lumbalis et costalis, at the entrance of the sympathetic nerves) ; the
right half may be entirely wanting, or may show a large defect, or
may exhibit smaller openings (e.g. in centrum tendineum, between
the pars lumbalis et costalis, posteriorly near the vertebral column,
etc.). With regard to the viscera which may be displaced, all kinds
of varieties and associations may be met with : all the abdominal
viscera including the kidneys and suprarenal capsules may be found
in the thorax ; on the other hand, only a small part of the stomach
or liver may be found therein : and all the intermediate types may be
noted. The displaced organs, especially the liver, may show deformi-
ties ; the lungs are always small and poorly developed ; and the heart
generally lies much to the opposite side.

Associated malformations have been noted : non-descent of testicles

HERNIA OF DIAPHRAGM 485

(6, 28, 65), absence of ulna and presence of a uterus unicornis cum
cornu rudimentario (42), diprosopus triophthalmus (49), facial hemi-
atrophy (55), double uterus and vagina (55), hare-lip and cleft palate
and congenital teeth (81), interventricular opening in heart (81),
congenital goitre (96), iniencephaly (100). There were multiple mal-
formations in P. Nau's case (Bull, ct mem. Soc. anat. de Paris, lxxviii.
594, 1903). Anencephaly, also, would appear to be sometimes
associated with diaphragmatic hernia ; for, in addition to my specimen
(98), cases have been reported by Otto (Mbnstrorum sexcentorum dc-
scriptio, N/os. 38 and 44, pp. 24, 28, 1841), by Gruber (Arch. f. path.
Anat., xlviii. 391, 1869), and by Bischoff (Arch,/. Gynaik., xxv. 445,
1885).

The teratogenesis of congenital diaphragmatic hernia does not pre-
sent many difficulties, and if the details of the development of the
diaphragm were fully and accurately known, even these woidd doubt-
less be capable of explanation. Obviously, non-development of the
great muscular partition between the thorax and abdomen accounts
for the cases of spurious hernia in which there is no sac for the
dislocated abdominal viscera; defective development of muscular
fibres in the diaphragm may explain the cases of hernia diaphrag-
matic vera. So much is clear, but when we come to inquire into the
reasons why lateral defects are more common than mesial, and why
the left side is more often affected than the right, difficulties arise.
In birds, for instance, the diaphragm consists of two lateral parts not
connected together in the middle line, and it has therefore been
thought that in mammals the mesial portion of the diaphragm has
been superadded ; if such be the case, then, according to the theories
of evolution and degeneration, the mesial part ought to be that which
is most frequently absent. Teratology, however, would seem to show
that the mesial part is the first formed, for it persists when the lateral
parts are wanting. The explanation of this and other difficulties
doubtless lies in the ontogenesis of the diaphragm, and in time we
shall know all the stages of this somewhat obscure part of develop-
ment. Already Franklin P. Mall's article (Johns ffojikins Hosp. Bull.,
xii. 158, 1901) has done something to clear up disputed points. One
fact the teratologist must bear in mind in thinking over the origin of
malformations of the diaphragm, namely, that structures which are
internal in the fully-formed embryo are often external in the early
stages, and so come under the influence of teratogenic causes external
to the organism. At the same time it must be freely admitted that
the cause of the defect in the diaphragm is very obscure : it may be
traction upon the vitelline or umbilical duct; primary shortness of
the (esophagus may be a starting-point (E. Eabaud, Bull. Soc. d'obst.
de Paris, vi. 352, 1903); or the presence of a common mesentery for
the large and small intestine may, by permitting greater freedom of
movement to the bowel, predispose to it. The matter is well worthy
of further investigation, and the articles of Otto Grosser (89) and of
E. Schwalbe (91) will prove of service in indicating the lines along
which research may most usefully advance ; Liepmann (Arch. /'.
Gyncck., lxviii. 780, 1903) also gives a good summary of recent views.

486 ANTENATAL PATHOLOGY AND HYGIENE

Literature. — In addition to the one hundred cases, references to which
appear below, the reader may be directed to the articles of H. I. Bowditch
(Buffalo Med. Journ., ix. 1, 65, 1853-54), of L. Lacher (Deutsches Arch. f.
klin. Med., xxvii. 268, 1880-81), and of A. Boursier (Diet, encycl. d. sc.
med., 1 s., xxix. 1, 1884) which contain literature lists ; many references are
also given in the Index Catalogue of Washington, 1 s., vol. vi. 154, 1885 ;
2 s., vol. vii. 15, 1902, under the heading, "Hernia, Diaphragmatic."
(1) Korn, Centrlbl.f. Gyniik., xii. 523, 1888. (2) A. M. Paterson, Brit.
Med. Journ., ii. for 1888, p. 1207. (3) Fleischmann, Centrlbl. f Gyndk.,
xiii. 79, 1889. (4) C. Liman, Handbuch d. gerichtlichen Medizin, Bd. ii.
p. 11, 1889. (4a) A. Monnier, Tliese, Paris, 1889. (5) J. O'Dwyer,
Arch. Pediat., vi. 882, 1889. (6) (4. Schroter, Diss, inaug., Konigsberg,
1889. (7) N. Fried, Internat. lclin. Rundschau, iv. 522, 1890. (8) 6.
Schaeffer, Miinchen. med. Wchnschr., xxxvii. 536, 1890. (9) W.Turner,
Lancet, i. for 1890, 1301. (10) Dehn, Miinchen. med,. Wchnschr., xxxviii.
227, 1891, Three cases. (13) 0. H. J. Frantz, Diss, inaug., Kiel, 1891.
(14) W. A. Hollis, Lancet, i. for 1891, p. 1095. (15) J. Kaup, Diss,
inaug., Kiel, 1891, Two cases. (17) A. Larsen, Hosp.-Tid., 3 R., ix. 287,
1891. (18) O. J. Mayer, Diss, inaug., Berlin, 1891. (19) Sir J. Paget,
Studies of Old Case-Books, p. 149, 1891. (20) C. Reinhard, Deidsche
med. Wchnschr., xvii. 954, 1891. (21) H. R. Spencer, Trans. Obst. Soc.
Lond., xxxii. 132, 1891, Three cases. (24) G. N. Pitt, Trans. Path. Soc.
Lond., xliii. 79, 1891-92. (25) Niemoller, Berl. klin. Wchnschr., xxix.
355, 1892. (26) H. R. Spencer, Trans. Obst. Soc. Lond., xxxiii. 34, 1892.
(27) C. D. B. Hale and J. F. Goodhart, Trans. Clin. Soc. Lond., xxvi.
105, 1892-93. (28) A. E. Giles, Trans. Obst. Soc. Lond., xxxiv. 132, 1893.
(29) P. Guttmann, Berl. klin. Wchnschr., xxx. 33, 1893. (30) J. Ikeda,
Centrlbl.f. Gyniik., xvii. 1089, 1893. (31) B. Thompson Lowne, Descrip-
tive Catalogue of the Teratological Series in the Museum of the R. C. of
Surgeons of England, p. 123, 1893, Six cases. (37) R. J. Marshall,
Glasgow Med. Journ., xl. 34, 1893. (38) K. Abel, Berl. klin. Wchnschr.,
xxxi. 84, 114, 1894. (39) Clozier, Bull, de VAcad. de med., 3 s., xxxii.
646, 1894. (40) C. Debierre and 0. Lambert, Journ. de I'anat. et
physiol., xxx. 609, 1894. (41) L. v. Dittel, Centrlbl. f. Gyniik., xviii.
488, 1894. (42) Gessner, Centrlbl. f. Gyndk., xviii. 824, 1894.
(43) Hochstetter, Bericht Charite-Annalen, xix. 536, 1894. (44) C. S.
Jaffe, Trans. Path. Soc. Lond., xlv. 224, 1894. (45) L. Joly, Bull, de la
Soc. anat. de Par., 5 s., viii. 14, 1894. (46) H. T. Machell, Med. News,
lxv. 389, 1894, Two cases. (48) A. Neumann, Deutsche med. Wchnschr.,
xx. 661, 1894. (49) F. Meola and S. Bakunin, Arch, di ostet. e c/inec,
i. 506, 1894, and ii. 27, 119, 1895. (50) F. A. Fry, Montreal' Med.
Journ., xxiv. 101, 1895. (51) B. Lewy, Deutsche med. Wchnschr., Vereins-
Beilage, p. 11, Jan. 7, 1895. (52) A. J. M'Closky, Lancet, i. for 1895,
p. 1116. (53) A. M. Paterson, Proc. Anat. Soc Gr. Brit, and Ireland,
p. 21, 1895, Two cases. (55) Schauta, Centrlbl. f. Gyniik., xix. 348,
1895. (56) G. G. Sears, Boston Med. and Surg. Journ., exxxii. 107,'

1895. (57) E. Mary P. Stone, Austral. Med. Journ., n.s., xvii. 554, 1895.
(58) Yerdelet, Journ. de med. de Bordeaux, xxv. 128, 1895. (59) A.
Arnheim, Diss, inaug., Giessen, 1896, Six cases. (65) Bonnet, Daiijphine
med., xx. 278, 1896. (66) Ciiambrelent, Journ. de med. de Bordeaux,
xxvi. 483, 501, 1896. (67) A. Cocchi, Ann. di ostet. e ginec, xviii. 497,

1896, Two cases. (69) G. Currado, Gior. di med. leg., iii. 9, 1896.
(70) H. C. Frakek, Columbus Med. Journ., xvi. 400, 1896. (71) A. E.

MALFORMATIONS OF STERNUM 487

Maylard, Glasgow Med. Journ., xlvi. 143, 1896. (72) E. Tekrile, Gazz.
<l. osp., xvii. 591, 1896. (73) C. Ogle, Trans. Path. Soe. Lond,, xlviii.
114, 1896-97. (74) W. I). Booker, Arch. Pediat, xiv. 649, 1897.

(75) II. Botesco, Arch. d. sc. med. . . . de Bucharest, ii. 77, 1897.

(76) Chambrelent and Princeteau, Journ. de med. de Bordeaux, xxvii.
99, 1897. (77) A. H. Cordier, Am. Journ. Surg, and Gynec, x. 114,
1897-98; and Ann. Surg., xxvi. 353, 1897. (78) T. E. H. Fisher, Lancet,
ii. for 1897, p. 1584. (79) P. I. Gautier, Paris, 1897, Five cases.
(84) L. E. Holt, Med. News, lxxi. 769, 1897. (85) H. Hubl, Centrlbl.f.
Gynak., xxi. 1353, 1897. (86) E. Barclay-Smith, Journ. Anat. and
Physiol., xxxii. 341, 1897-98. (87) G. Keim, G. Rosenthal, and Huguier,
Bull. Soe. d'obst., Par., p. 147, 1898. (88) A. J. Wood, Intercolon. Med,
Journ. Australas., iii. 96, 114, 1898. (89) 0. Grosser, IVien. Tclin.
Wchnschr., xii. 655, 1899. (90) S. W. Kelley, Arch. Pediat, xvi. 602,
1899. (91) E. Schwalbe, Miinchen. med. Wchnschr., xlvi. 12, 1899, Four
new cases, and one previously reported in Morpholoq. Arbeiten, Bd. viii.
1898. (96) I. A. Abt, Arch. Pediat, xvii. 261, 1900. (97) J. W.
Ballantyxe, Physician and Surgeon, i. 891, 1900; vide Plate XXIII. and
Fig. 74. (99) L. Fuxck-Bhextaxo, Bull, et mem. Soe anat de Paris, 6 s.,
ii. 537, 1900. (100) J. W. Ballantyne, Case of Iniencephaly with con-
genital diaphragmatic hernia, 1900, vide Plate XVI.

Malformations of the Sternum.

Both cyphosis and lordosis of the sternum occur as congenital
anomalies ; but considerable confusion has arisen as to which is
lordosis and which cyphosis. Taruffi (Storia, vii. 175, 1894), for
instance, defines lordosis as anterior convexity of the sternum, while
cyphosis is anterior concavity; he thus regards the sternum as
similar in this respect to the vertebral column. G. D'Ajutolo
(Mcmoria, Bologna, 1898), however, looking to the etymology of the
words, terms an anterior sternal convexity cyphosis and an anterior
sternal concavity lordosis; but although this nomenclature is more
correct in an etymological sense, I prefer to retain Taruffi's. Lordosis
(anterior convexity of the sternum) is comparatively rare ; but
D'Ajutolo (op. eit.) has described four cases. These were all partial,
and in three of them the lordosis was superior and one inferior ; two
of the three superior cases were in sisters ; they were all present at
birth. Sternal cyphosis (anterior concavity of the sternum) is far
more common. In Germanv this deformity has, since W. Ebstein's
time (Deutsches Arch. f. klin, Med,, xxx. 411, 1881-82; xxxiii. 100,
1883), been termed Trichterbrust ; and it is sometimes known in
English as the funnel-shaped thorax. Cases had, however, been
observed before Ebstein described it; thus Eggel (Arch. f. path. Anat,
xlix. 230, 1870), Flesch (ibid., lvii. 289, 1873), and N. Hagmann
(Jahrb. f. Kinderh., n. F., xv. 455, 1880) had all reported instances ;
within the past twenty years many new observations have been
added, so that the bibliography is now a large one (A. Grunenthal,
Diss, inaug., Berlin, 1888; W. Graefmer, Deutsehes Arch, f. klin. Med.,
xxxiii. 95, 1883 ; E. Coen, Bull. d. sc. med, di Bologna, 6 s., xiv. 7,
1884; F. Percival, Biv. din. di Bologna, 3 s., iv. 401, 1884; G. Klem-

488 ANTENATAL PATHOLOGY AND HYGIENE

perer, Deutsche vied. Wehnschr., xiv. 732, 1888 ; J. Kundinliller,
Deutsche Arch. f. Hin. Med., xxxvi. 543, 1884-85 : H. Ribbert, Deutsche
mcd, Wehnschr., x. 533, 1884 ; H. R. Smith, Norsk Maej. f. Lcegevi-
drnsh, 4 K, i. 236, 1886 ; H. J. Vetlesen, ibid., 4 R., i. 31, 1886,
Gentralbl. f. Hin. Med., vii 745, 1886; K Herbst, Deutsches Arch. f.
Hin. Med., xli. 308, 1887; A. Frey, Deutsche mcd. Wehnschr., xiii. 645,
1887 ; etc.). The concavity is generally in the middle line, and
corresponds to the lower part of the sternum ; in many respects it
resembles the acquired form of the malformation seen in cobblers.
Cyphosis and lordosis may be met with in the same case (sternal
cypho-lordosis of D'Ajutolo). Heredity and family prevalence were
not uncommonly noted (Smith, Vetlesen, Klemperer, Grimenthal,
Herbst). Various causes have been alleged for sternal cyphosis, such
as pressure of the fcetal heel (Hagmann), or chin (Ribbert), or fracture
of the sternum from maternal traumatism in pregnancy (Graeffner),
or shortness of the sternum (Ebstein), or rickets (Griinenthal). It
is difficult to decide upon the most likely theory, but it is evidently
a malformation which, from its nature, may arise in the foetal period
of antenatal life, although it may, as its hereditary character shows,
be predisposed to earlier.

Sometimes suprasterned ossicles are detected along the upper
margin of the sternum near the clavicular facets ; these may be
sesamoid in nature, or, more probably, they represent the epi-
coracoids of some animals. At the other extremity of the bone the
ensiform cartilage may show such anomalies as Infidity, absence, and
outward or inward displacement. Various anomalies in the construc-
tion and ossification of the sternum may be met with ; one or two of
the segments of the bone may remain separate and have fibro-carti-
laginous tissue lying between them ; there may be supernumerary
ossific nuclei ; or there may be one or (rarely) two foramina in the
bone (Bogusat, Diss, inaug., Konigsberg, 1902). More important,
however, than these malformations is congenital fissure of the sternum:
this demands a paragraph to itself.

Fissure of the Sternum (Sternoschisis).

It is difficult to separate partial or complete absence of the
sternum from fissure properly so called ; thus, absence of the bone
and a wide fissure of it (E. H. Martin, Med. Bee, New York, xxxii.
425, 1887) may be practically indistinguishable ; the two anomalies
may, therefore, be considered together. On the other hand, cases in
which there is ectopia of the heart (made possible by the divided
state of the sternum) are best described by themselves.

No case of sternal fissure was ever so widely known as that of
the man E. A. Groux, who travelled about from one European capital
to another exhibiting himself. I have a pamphlet (Ahhandlungcn
unci Notizcn uber E. A. Ghroux's Fissura stcrni congenita), published
by the man himself at Hamburg in 1857, which contains the descrip-
tion of his malformation, with remarks thereupon by Hamernik,
Baumgartner, Wagner, Beclard, Saint -Hilaire, Owen, Williams,

STERNOSCHISIS 489

Ernst, Sundewall, and many other medical men of the time. The
great interest which Groux excited was not, however, due so much
to the malformation as a malformation, but rather to the observations
upon the action of the heart which it rendered possible. With these,
although of great importance, we have not here to do. The fissure
is generally V-shaped, wide above and narrow below, and is bridged
across by fibrous tissue and the integuments. It may divide the
whole bone from the manubrium to the ensiform ; but occasionally
it is restricted to one part, the manubrium being intact in some;
instances (W. Vrolik, Tabulae ad illustrandam Embryogcncsin, PI.
xxiv., Fig. 1, 1849), and the ensiform cartilage in others (0.
Obermeier, Arch.f. path. Anat., xlvi. 209, 1869), while in one curious
case (that reported by L. Eamirez, Gac. med. dc Mexico, i. 217,
1867-68; Gaz. med. dc Paris, •"> s., xxiii. 66, 1868) there was a wide
defect above and below, and a narrow fissure in the middle part of
the bone. In the young man seen by G. A. Gibson and II. Malet
(Journ. Anat. and Physiol., xiv. 1, 1879-80) the fissure affected the
manubrium sterni, and extended downwards to the level of the fourth
costal cartilages ; it measured about 1 J inch transversely in its upper
part ; and a strip of skin stretched upwards towards the sternum from
the umbilicus for a distance of 3 inches. This second malformation
(in the epigastric region) has been noted in other cases (Wittstock,
Monatschr. f. Med., Augcnh, v. Chir., ii. 79, 1839 ; 0. Obermeier, loc.
cit.) ; and sometimes a cicatricial condition of the skin or an actual
defect of the integument has been observed in the same region
(Sebastiani, cited by Taruffi, op. cit., vii. 187, 1894). Possibly both
the sternal fissure and the deformity of the abdominal wall above
the umbilicus originate in amniotic pressure, which arrests the
development of the two lateral folds which grow together to close in
the body cavity anteriorly. Ahlfeld (op. cit., p. 177, 1882) suggests
that the vitelline or umbilical duct may acquire adhesions with the
pericardium, and so keep the original opening open or at least prevent
its complete closure ; he bases his theory on a case observed by him
(Arch.f. Gynask., xii. 154, 1877), in which a filament resembling the
vitelline duct was found attached to the thorax between the two
mammary glands. It does not appear, however, that it is necessary
to invoke such a mechanism, as simple pressure (amniotic or other)
would appear to be sufficient to prevent the union of the two halves
of the sternum.

Sternal Fissure with Ectopia Cordis.

In the previous chapter a brief reference was made to displace-
ment upwards of the heart into the cervical region where there was
a median fissure : as I shall have to point out later, a downward
displacement of the heart occurs sometimes in connection with defect
in the diaphragm and exomphalos (Fig. 28, p. 17-°>); but the heart
may also be dislocated forwards and come to lie outside of the chest
through a defect in the sternum and anterior thoracic wall, and it is
with this teratological state that I am here concerned.

490 ANTENATAL PATHOLOGY AND HYGIENE

In November 1896 I received a letter from Dr. G. F. Barnardo of
Stepney, containing a description of a seven months' foetus which
lived six hours in an incubator. During that period it showed
every sign of life, although the heart was lying outside the thoracic
cavity, being suspended by a pedicle, springing from its base
and running to the thoracic wall. The sternum was divided into
halves, each bar being in relation to the sternal ends of the costal
cartilages and consisting of a thin strip of cartilage, which was
prolonged upward into the neck to articulate with the clavicle of the
same side. The upper ends of the sternal bars were 2 inches apart
and the lower ends 4 inches ; through the opening thus produced the
heart protruded. The pedicle contained the aorta, the pulmonary
artery and veins, and the vense cava;. There was no pericardial sac,
the visceral pericardium simply leaving the wall of the heart and
being continued outwards for some distance, to blend finally with the
skin of the body generally in a raised margin. The upper ends of
the recti abdominis muscles were widely separated, but there were
no other deformities. The ectopic heart had the structure which is
normal at the seventh month of antenatal life ; the lungs were
normal, and each lay in its own pleural sac. It was noted that
during life the apex of the heart at each systole was tilted right
upwards so as to touch the infant's chin. This interesting case of
ectopia cordis (extra-thoracic type) was afterwards figured and
described both in the British Medical Journal (ii. for 1896, p. 1639)
and in the Journal of Anatomy and Physiology (xxxii. 325, 1897-98).

Cases resembling Barnardo's are rare. According to the system
of classification of C. Weese (Diss, inaug., Berolini, 1818) they belong
to the variety ■ectopia cordis cum stemi fissura ; the other types are
ectopia supra-thoracica (displacement of the heart upwards into the
neck) and ectopia sub-thoracica (displacement downwards, by defect
of the diaphragm, into the abdominal cavity or into the sac of an
exomphalos). There has been a further subdivision of the variety
with sternal fissure (also known simply as exocardia) into a superior,
median, inferior, and lateral type, according as the displacement
takes place in the upper, middle, or lower part of the bone, or at the
side of the sternum altogether; but the number of observations is
hardly large enough to justify such detail. Instances in which the
sternal defect was superior, affecting the manubrium, were reported
by E. Sandifort (Acta Hclvet, vii. 56, 1772), by S. Jones (Trans. Path.
Soc. Lond., vi. 98, 1855), and by H. Schlesinger (Berlin. Jdin, Wchnschr.,
vii. .'176, 1870). The heart was destitute of a pericardial sac, and in
one instance (Schlesinger's) had a filamentous band attached to its
apex (amniotic in origin ?). Cases in which the heart protruded
through a space formed by separation of the two halves of the
sternum in their whole extent have been reported by Pecchioli
(Gaz. med. de Paris, vii. 12, 1839), E. Daniell (Brit. Med, Journ., ii. for
1800, p. 770), by H. J. Haan (Piss, inaug., Bourne, 1825), and others.
In these instances, and in others like them, the pericardial sac is
generally absent or rudimentary ; the heart itself may show no
anomalies, but on the other hand it may have a bifid apex, incomplete

ECTOPIA CORDIS 491

interventricular and interauricular septa, two chambers instead of
four, and no ductus arteriosus (Eanvier, Compt. rend. Soc. de biol.,
3 s., v. 93, 1864). Cases in which the heart protruded through an
opening in the lower part of the sternum and of the epigastric region
were seen by Eamel (Journ. de mdd., chir., pharm,, xlix. 423, 1778),
Chaussier {Bull. Fac. de mid de Paris, iv. 93, 1814), J. O'Bryen
(Trans. Prov. Med. and Surg. Assoc., vi. 374, 1838), von Gross and
Heim (Med. Cor.-Bl, d. wilrttemh. arztl. Vcr., xxix. 229, 1859), E.
Kezek (Wicn. mcd. Prcssc, ix. 673, 1868), A. Franc^ois-Franck (Compt.
rend. Soc. de biol., 6 s., iv. 340, 1879), and L. Eanvier (loc. cit.). In
these sub-thoracic cases the pericardium may be present and the
child may survive its birth for some time and even reach adult life.
Sometimes the heart was contained in a thin-walled sac (amniotic or
peritoneal), in which lay also (in some instances) the liver, stomach,
and intestinal coils. Again, there have been cases in which the whole
sternum was divided as well as the epigastrium ; in them the anterior
thoracic wall is open, and so is the abdominal as low down as the
umbilicus. Taruffi (op. cit., viii. 410, 1894) has collected together
nine records of this sterno-epigastric type of ectopia cordis, including
those of Grandi, Hammer, Fracassini, Weese, Locatelli, and Ahlfeld.
Just as fissure of the sternum in its upper part may be unaccompanied
by extrusion of the heart, so even in cases of fissure extending as
low as the umbilicus and sometimes including it, cardiac displace-
ment is not a necessary concomitant (vide p. 520). When we are
considering such specimens as those of Grandi, Hammer, and Ahlfeld,
we are really dealing with the connecting links which join one
teratological type (fissure of the thorax) to another (fissure of the
anterior abdominal wall) ; we pass from one group of cases in which
division of the sternum and protrusion of the heart are the leading
features to another in which defect of the anterior abdominal wall
and protrusion of the abdominal viscera are the leading features, and
between the two groups are the intermediate types which show these
features combined in various ways.

The tc ratogenesis of ectopia cordis presents several difficult prob-
lems. It is a rare monstrosity both in the human subject and in
mammals. It is not indeed unknown to the lower animals, cases
both of schisto-cormus fissistemalis and of sehisto-cormus schistepigas-
trico-sternalis having been recorded by Gurlt (op. cit., ii. 132, 133,
1832) ; but it is uncommon enough to suggest that there must be
some special reason therefor. Considering that there is a stage in
ontogenesis when the heart is outside the body, it is obvious that the
idea of arrested development offers a tempting solution of the
problem of the causation of ectopia cordis. It may further be added
that there is some evidence that this arrest is due to amniotic
influences, traces of filaments and bands having been found in certain
cases. Can we reconcile this theory with the rarity of the mon-
strosity ? At first sight it would appear as if it ought to be a common
instead of a rare type ; but a little reflection and some investigation
of early stages in the formation of the thorax show to us that there
is only a brief period in ontogenesis during which the anterior aspect

492 ANTENATAL PATHOLOGY AND HYGIENE

of the thorax is subject to amniotic influences ; the natural flexed
attitude of the embryo saves its anterior aspect from pressure, and
possibly the presence of the developing upper limb-buds increases the
protection. But, it may be urged, defective closure of the adjacent
abdominal walls is common. This is admitted, but there is in that
region a natural opening, the umbilicus, which does not permanently
and entirely close till after birth, and there is no such complete
protection by flexion of the trunk and the presence of limb-buds as
there is higher up. It is, therefore, quite permissible to accept the
theory of arrested development in ectopia cordis. There may be a
complex action of the amnion to account for the arrest : amniotic
pressure from non-separation of the amniotic membrane may
prevent the fusion of the body walls in the middle line, and amniotic
adhesions may hold the heart forwards. The fact that sometimes, as
in Franoois-Franck's patient (loc. tit.), there is no break in the
continuity of the skin covering the displaced heart, introduces a
difficulty ; but it is possible that in such instances the amniotic
pressure and adhesions may have been temporary and ceased soon
enough to allow the thoraco-abdominal walls to close, albeit im-
perfectly.

Pleurosomus.

Just as fissure of the sternum may be either accompanied or
unaccompanied by protrusion of the heart, so a lateral fissure of the
chest-wall may or may not be associated with prolapse of the lungs
(and heart) through it. I have already considered defects in the
thoracic lateral wall unaccompanied by protrusion of the exposed
viscera (p. 470) ; now the more grave type of defect with visceral
displacement calls for description. Isidore Geoffroy Saint-Hilaire
(Histoire, ii. 267, 1836) placed cases of lateral fissure of the chest
among the celosomic monstrosities, and made a special genus for them
under the name of pleurosomus ; and he defined them as instances of
lateral eventration affecting chiefly the upper part of the abdomen
and extending to the thorax, and being accompanied by absence or
incomplete development of the arm of the same side. Saint-Hilaire
had an opportunity of examining two cases of pleurosomus, one in a
calf, the other in a human foetus ; in both the left side was affected
and in both the corresponding limb was practically absent ; in the
case of the human foetus the umbilical cord, which was short, was
adherent to the head, which was deformed (hyperencephaly). The
left side is not invariably the affected one, as was demonstrated by
the specimen which I showed to the Edinburgh Obstetrical Society
in 1902 {Trans. Edinh. Obst. Soc, xxvii. 112, 1901-02); the right arm
was in this instance adherent to the margin of the thoraco-abdominal
opening in the right side of the body (Fig. 34).

Sometimes the sheath of the umbilical cord can be recognised as
continuous with the skin margins of the thoraco-abdominal opening :
but there may be nu true cord, or the sac walls may have been torn
in labour. The sac may in some instances be covered with skin, or
may be formed by the peritoneal membrane. There does not appear

PLEUROSOMUS 493

to be any known case in which the defect was limited absolutely to
the thoracic wall ; in all of the recorded instances the abdominal
cavity was opened into ; and in some {e.g. Campana's case, cited by
Taruffi, op. cit., vii. 210, 1894) the whole of the left side of the body
from the crest of the ilium to the second rib, and from the margin of
the sternum to the vertebral column, was wanting. We shall see,
however, that a lateral opening in the abdominal wall may occur
independently of any thoracic defect.

The arm of the affected side was generally deformed : it was a
shapeless rudiment in Hertwig's case (Diss, inaug., Landshuti, 1821)
and a stump ending in one digit in Elsholt's specimen (Miscel. Acad,
not. curios, iv.-v., App. p. 80, 1676). In Campana's observation (Joe.
cit.) it was not only deformed (stump-like), but was abnormally placed,
being curiously inserted upon the clavicle; in Heil's case (Illust. vied.
Ztg., iii. 83, 1853) it was fixed to the cartilage of the sixth rib; and
in Forster's specimen (Die Missbildungen des Mcnschen,Jil. xi. Fig. 16)
it was situated near the sternum. It was absent altogether in the
case reported by E. Martin and Letulle (Journ. de Vanat. et physiol.,
xii. 561, 1876). The heart was nearly always extruded and lay on
the top of the abdominal viscera (e.g. in Klein's case, Deutsches Arch,
f. d. Physiol., iii. 391, 1817), and in Campana's specimen (loc. cit.) the
lungs, also, were among the displaced organs. The heart would
appear to have been generally enveloped in the pericardium.

Various associated malformations have been recorded, such as
hare-lip and facial fissures (Campana, loc. cit.), a tail-like projection
(Elsholt, loc. cit.), encephalocele (C. Weese, Diss, inaug., Berolini,
1818), and spina bifida, atresia ani, and absence of genitals (F. W.
Voigtel, Fragmcnta scmeiologiw obstetricice, PL v., p. 77, Hake, 1790).
The associated deformities do not throw much light upon the tcrato-
gencsis of pleurosomus of the thoracic region, which remains very
obscure. It is indeed most difficult to account for lateral openings
in the thoraco-abdominal parietes ; for arrested development fails in
such instances to be an easy method of explanation, there being no
embryonic aperture which by its permanence can become the terato-
logical defect. Any theory to be worthy of credence must take into
account the associated anomalies of the arm of the affected side and
the almost constant left-sided nature of the malformation. Possibly
the primitive left-sided spirality of the embryo may be exaggerated
and made permanent by amniotic adhesion and pressure, and an
actual want of formation of the chest-wall take place in consequence
of the pressure thus brought to bear upon it ; but the causation is
surrounded by great obscurity.

Thoracic Patagium.

Reference has already been made to the occurrence of a web of
skin (patagium) in the cervical region (p. 460) ; and I note here that
the same anomaly may occur in relation to the thorax. It consists
of a fibrous band or fold of skin passing from the ribs to the humerus,
and it is apt to be associated with defect of the pectoral muscles

494 ANTENATAL PATHOLOGY AND HYGIENE

(J. Thomson, Teratologia, ii. 8, 1895 ; Brims and Kredel, Fortsckr. d
Med., viii. 1, 1890 ; E. Johnson, Trans. Clin. Soc. Lond., xxxvi. 259,
1902-03). It is possible that the case reported by Hippocrates
(Littre's CEuvres completes d'Sippocrate, v. 213, 1846) of a dead-born
infant having the right arm adherent to the thorax was an instance
of thoracic patagium ; if so, it is the earliest of which we have any
account {vide my article in Tendologia, ii. 276, 1895).

CHAPTER XXVI

Merosoinatous Terata (cont.) : Malformations of the Thoracic Organs : Malforma-
tions of the Heart : Individual Anomalies of Interauricular and Inter-
ventricular Septa, Pulmonary Artery, Aorta, and Valves ; Associations of
Cardiac Anomalies ; Associated Malformations ; Teratogenesis ; Symptom-
atology ; Malformations of the Lungs, Trachea, Bronchi, and Thymus
Gland.

Malformations of the Heart.

In the first volume of this work (p. 3G9) I discussed the relation of
foetal diseases, and more especially of foetal endocarditis, to congenital
malformations of the heart. I there endeavoured to disentangle the
cardiac diseases arising in foetal life from the cardiac malformations
arising in embryonic life. For various reasons I was not completely
successful, as the reader will realise if he will glance over the passage
referred to. The chief difficulty arose on account of the peculiar
mode of development of the heart. This organ passes through the
phases of its ontogenesis early and quickly ; at the end of the second
month of antenatal life it is structurally as complete as when the
infant is born; its malformations, therefore, must also arise early.
But there is a portion of its ontogenesis which is postponed until
birth ; I refer to the closure of the foramen ovale and the ductus
arteriosus. There is thus produced the unusual phenomenon of
development divided into two phases, an early and a late, by an inter-
mediate resting period of seven months ; the construction of the heart
reaches the stage necessary for the maintenance of healthy antenatal
life at the second month, and it is not till the ninth that further
structural alterations occur to fit it for the requirements of the post-
natal circulation. The malformations of the heart, therefore, are
divisible into two groups : there are those which arise early, such as
absence of the interventricular septum, and are truly embryonic in
time, and those which arise later, such as a perforate state of the fora-
men ovale, and are sometimes postnatal in character. Between these
two periods it is necessary to think of the heart as subject to diseases,
such as endocarditis, but as not in a state to develop malformations.
It is easy, therefore, to see how complicated the question of the
antenatal pathology of this organ becomes, and how foetal diseases,
embryonic malformations, and postnatal anomalies have come to be
combined, not only in the minds of investigators, but in actual
occurrence. Thus it happens that in Volume I. I discussed foetal
endocarditis and the malformations (unclosed foramen and perforate

495

49 G ANTENATAL PATHOLOGY AND HYGIENE

ductus arteriosus) which follow it in point of time, while in this
volume (which deals with the embryo) I have to consider the earlier
and more fundamental malformations of embryonic life ; at the same
time, since this mode of subdivision of the subject is somewhat
unusual, I do not absolutely adhere to it, but permit myself to refer
to postnatal as well as embryonic malformations. Foetal endocarditis,
as I have already explained in Volume I., is related both to the early
and the late malformations : it may possibly occur more readily in a
heart which is malformed in the embryonic phase of antenatal life ;
it probably is to some extent the cause of the malformations which
arise at and immediately after birth.

From the theoretical standpoint it ought to be possible to arrange
all the malformations of the heart in a chronological series according
to the time when they develop. We have some knowledge of the
state of development of the heart at the second, third, fourth, fifth,
and sixth weeks of antenatal life (vide pp. 26, 31, 35, 48, 58, 67), and
we ought, therefore, to be able to arrange the cardiac malformations
in a series according to the week during which they were produced,
for we should be able to recognise the stage of ontogenesis at which
the arrest of development and the resulting structural anomaly
occurred. As a matter of fact this is to some extent practicable : we
know, for instance, that the separation of the ventricles is completed
during the sixth week by the formation of the pars membranacea
septi, and we are at liberty to conclude that perforations affecting the
pars membranacea of the septum arise during this week of antenatal
life. We are, however, prevented from forming a complete chrono-
logical arrangement of the cardiac malformations, partly by a want
of exact knowledge regarding many of the earlier organogenetic
phenomena, and partly by the overlapping of malformations, by the
occurrence, for instance, of anomalies of different ages in the same
heart. I shall, therefore, follow the usual plans of arrangement, and
describe first the individual anomalies according to the parts affected,
and second the most usual associations of these malformations.

Cardiac malformations have been ably discussed by various
authors, among whom I may mention J. K. Farre (1814), J. Paget
(1831), H. Friedberg (1844), T. B. Peacock (1864, 1866), C. F. von
Pokitansky (1875), C. Taruffi (1875), C. Pauchfuss (in Gerhardt's
Handbuch der Kinderkrankhcitcn, iv. 1877-83), E. Theremin (1895),
H. Vierordt (1898), G. A. Gibson (1898), and A. Moussous (in
Grancher's TraiU des maladies dc Venfance, 2nd edit., iii. 708, 1904),
leaving many others unnamed. In this chapter I have not to deal
with all the teratological conditions which may affect the heart ;
for some of them, such as ectopia, have been described already, and
others, such as absence (in acardiac foetuses) and transposition, fall to
be referred to later.

Anomalies of the Interauricular Septum.

The commonest congenital anomaly of this septum undoubtedly is
incomplete closure of the foramen ovale, due to want of union of the

ANOMALIES OF THE INTER AURICULAR SEPTUM 497

membrane with the margin of the fossa. This, however, is not an
embryonic but a postnatal malformation ; it represents a state
which is normal during the whole of antenatal life, but which is
anatomically abnormal as soon as postnatal life begins. Even then
it may not interfere with the physiological action of the heart so
long as the membrane occupies the foramen (although unattached),
for the pressure on one side of it is balanced by that on the other
side ; but in diseased states of the circulation, when the pressure in
the right auricle exceeds that in the left auricle, the interauricular
curtain may swing aside and allow mixing of the venous and arterial
blood.

Other anomalies of the interauricular septum, however, arise at
an early period in antenatal life, and give rise to morbid states which
are truly embryonic and teratological. There is, for instance, com-
plete absence of the whole septum, a rare anomaly ; an example of
it is said to have been reported by Mery in 1700. More common are
the less or more extensive defects in the interauricular septum which
have been described by many writers. In order to understand their
nature and mode of origin it is necessary to remember that the
formation of the septum between the auricles is somewhat complex :
there is first a falciform ridge which runs dorso-ventrally across the
roof of the originally single auricular cavity, and is known as the
septum primum ; it would soon, by its growth, separate the right
and left auricles completely if a foramen (foramen ovale) did not
appear in its dorsal portion ; a second ridge (septum secundum) next
develops in the roof and ventral wall of the right auricle, and
becomes continuous with the fold guarding the opening of the sinus
venosus ; endocardial cushions then grow from the dorsal and ventral
walls of the auricular canal, and uniting together divide this canal
into a right and left half, and also join with the septum primum to
complete the interauricular septum. Arrested development of either
the primary or secondary septum may occur, and will result in the
presence of an opening between the auricles. Thus, the septum
primum may be little more than a ridge, when the opening between
the auricles will be large ; or there may be a less marked defect, the
fossa ovalis may be open or shut, and a semilunar opening be found.
Again, the secondary septum may be incomplete, when the opening
is posterior in position and does not descend as low as the floor of
the auricular cavity ; there may be no traces of the primary septum,
or it may be represented by a ridge, by a fenestrated membrane, by
a band, or by a large open fossa ovalis. Many instances of these
various conditions might be cited ; but I shall refer here to only two
or three. E. Theremin (Etudes sur les affections congenitales du cceur,
p. 154, 1895) has described a case in which the interauricular septum
was entirely absent ; the two auricles formed a single cavity, into
which the two venae cavse opened on the right side, and three pul-
monary veins (of which one was double) on the left side ; the
common auricular cavity communicated with the two ventricles by
a large opening divided into two by the interventricular septum.
In another of Theremin's cases (op. cit,, p. 153), the foramen ovale

vol. ii. — 32

498 ANTENATAL PATHOLOGY AND HYGIENE

was nearly closed by the membrane, and was separated from an
opening in the interauricular septum by the Eustachian valve which
formed a well-developed muscular band ; the opening was triangular
in shape and had the apex turned downwards, reaching the septum
ventriculorum at the lower margin of the posterior wall of the conus
arteriosus. In both these cases the muscular part of the septum was
involved ; but, of course, it is the membranous part in the foramen
ovale that is most commonly defective ; references to cases of the
latter deformity might be multiplied almost indefinitely. A few
references are appended, and the reader will find many more in the
Index Catalogue of Washington (vol. v. p. 962, 1884; 2 s., vol. vi.
p. 855, 1901) : Anger, Compt. rend. Soc. de biol., 4 s., i. 97, 1865 ; Blum,
Bull. Soc. anat. de Par., xliv. 246, 1869 : Bucqoy, Union me'd., 3 s.,
xxx. 698, 1880; E. Caton, Lancet, ii. for 1878, 252; H. Chiari,
Jahrb. f. Kinderh., n. F., xv. 319, 1880; Ford, N. York Med. Journ.,
xxv. 405, 1877; Geffrier, France med., xxviii. 254, 1881; C. Hiiter,
Arch. f. path. Anat., xxx. 587, 1864 ; C. Lacroix, Journ. Soc. de med.
prat, de Montpelier, vii. 200, 1843 ; Landouzy, Arch. gin. de nud.,
xlvii. 460, 1872 : E. Malvoz, Ann. Soc. med.-chir. de Liege, xxviii.
177, 1889: K. Paidolph, Diss, inaug., Kiel, 1900: H. Huge, Diss,
inaug., Berlin, 1891 ; L. Vincenzi, Arch, per le sc. med., Torino, ix. 307,
1885-86; W. W. Wagstaffe, Trans. Path. Soc. Lond., xix. 96, 1868.

Anomalies of the Interventricular Septum.

There is no normal aperture in the interventricular septum at
the end of antenatal life, and all congenital openings here may,
therefore, be regarded as embryonic malformations. The septum
between the ventricles, like that between the auricles, is formed not
of one but of several parts. It consists chiefly of a ridge (ventricular
ridge) which arises from the floor of the single ventricular cavity ;
this grows more rapidly in its dorsal than in its ventral part, and so
comes soon into contact and fuses with the dorsal part of the partition
of the auricular canal, leaving an oval foramen in front just below
the point where the aortic bulb arises. Another ridge, or rather two
fused ridges, closes the above-mentioned opening and completes the
septum ventriculorum ; it is called the aortic septum, and serves also
to divide the bulb into two vessels (aorta and pulmonary artery). A
more detailed description of the interventricular septum recognises
three parts, a posterior segment, an anterior segment (consisting in
its turn of an anterior and a posterior part), and an intermediate
membranous space ("undefended space"); all these parts can lie
seen along the upper part of the septum ; the membranous space
(pars membranacea septi) is derived from the aortic septum, and the
other two (muscular portions) from the ventricular septum. Open-
ings in the septum corresponding to all these various parts have been
put on record, and A. Moussous (Maladies congenitales clu cceur, p. 45,
1895) carefully distinguishes between them. There may, for instance,
be an aperture at the centre or near the apex of the partition ; such
an aperture may be solitary, but more often there are several, and

ANOMALIES OF THE PULMONARY ARTERY 499

they -are then mere pinholes in the wall ; in these positions they are
always rare. More common is it to find a notch at the base of the
septum : it may affect the posterior segment and may include the
membranous space and the posterior part of the anterior segment,
producing a large opening ; or the notch may affect the whole of the
anterior segment (causing a wide aperture), or only its anterior part
(causing a small round aperture), or, most commonly, its posterior
part (producing a semilunar or triangular aperture large enough to
admit the little finger, lying just below the aorta) ; or, finally, the
perforation may lie in the membranous space. It was at one time
thought that the membranous space was the commonest site for inter-
ventricular openings ; but Eokitansky {Die Defecte der Scheidewande
des Herzens, Wien, 1875) has shown that while this membrane is
occasionally affected, either in its aortic or in its mitral part, or in
both, it is more common to find the perforation in the posterior part
of the anterior septum in the immediate vicinity of the membranous
space. It may be noted, as a rare occurrence, that the whole inter-
ventricular septum or a very large part of it may be absent.

I may cite here some bibliographical references to cases of defect
in the interventricular septum ; the list is only representative : C.
Assmus, Diss, inaug., Leipzig, 1877; A. Behier, Bull. Soc. anat. de
Paris, xliv. 171, 1869 ; J. E. Bennett, Trans. Path. Soc. Bond., i. 59,
1846-48; G. W. Callender, ibid., ix. 91, 1857-58: Coale, Boston
Med. and Surg. Journ., liv. 18, 1856 : S. Coupland, Med. Times and
Gaz., ii. for 1884, p. 501 ; G-. Decaisne, Bull. Soc. anat. de Paris, lii.
445, 1877 ; D. Duckworth, Journ. Anat. and Physiol., xi. 183, 1876 :
V. Eisenmenger, Ztschr. f. hlin. Med., xxxii. Supplhft., 1, 1897; E.
Elliot, Journ. Anat. and Physiol, xi. 302, 1877 ; J. P. C. Griffith,
Trans. Coll, Phys., Philadelphia, xviii. 152, 1896 ; S. 0. Habershon,
Guys Hosp. Be})., 3 s., xvii. 443, 1872; Herbst, Munchen. Tried.
Wchnschr., xlviii. 368, 1901 ; L. E. Holt, Arch. Pediat., vii. 81, 1890,
xii. 839, 1895 ; C. Karsten, Diss, inaug., Eostock, 1874; L. Lavergne,
Thhe, Paris, 1886 ; A. Lombardini, Gazz. d. os'p., iv. 660, 1883 ; H.
G. Miihsam, Diss, inaug., Kiel, 1900; O. Mliller, Arch. f. path. Anat.,
lxv. 140, 1875 ; E. M. Olshausen, Deutsche Klinik, xxii. 7, 1870 ;
Passow, Gharite-Ann., xix. 219, 1894; T. B. Peacock, Trans. Path.
Soc. Bond., v. 67, 1853-54; Polaillon, Union med., i. for 1876, p. 819;
E. Quain, Trans. Path, Soc. Bond., i. 60, 1846-48 ; H. D. Eolleston,
ibid., xlii. 65, 1890-91 ; W. H. Stone, St. Thomas's Hosp. Rep., n.s.,
xi. 57, 1882 ; F. Taylor, Guys Hosp. Rep., 3 s., xx. 566, 1875 ; C.
Vinay, Byon med., xciv. 325, 1900 ; F. Willcocks, Trans. Path. Soc.
Tjond., xxxviii. 96, 1886-87 ; Wittcke, Journ. d. pract. Heilk, lxvi. 4
St., 38, 1828; E. Ziegenspeck, Arch.f. Gynaek., xxxii. Ill, 1888.

Anomalies of the Pulmonary Artery.

The commonest of all the congenital malformations of the heart
is narrowing of the pulmonary artery. The stenosis may affect the
valvular orifice itself, and then it is usually due to coalescence of the
cusps ; in this way a sort of diaphragm is produced, perforated at

500 ANTENATAL PATHOLOGY AND HYGIENE

its centre ; more rarely there is complete obliteration. It is also rare
to find pulmonary incompetence ; when it does occur it is usually
due to rudimentary development or absence of the sigmoid valves.
The stenosis, again, may affect the conus arteriosus or infundibulum ;
when this is the case and when the narrowing is low down, a sort of
supplementary ventricular cavity is produced, and when it is in the
middle part the infundibulum takes on an hour-glass shape ; the
defect may lead not only to narrowing but to shortening of the infun-
dibulum, and there may even be complete absence of it. Finally,
the stenosis may be above the level of the valvular orifice : there
may be a uniform diminution in the calibre of the pulmonary artery
up to the point of its bifurcation into its two branches, which may
lie normal in size or narrowed like the parent trunk. B. Zuber
(Jahrb. f. Kindcrh., lix. 30, 1904) has reported a case of marked
congenital dilatation of the whole pulmonary artery. These pul-
monary defects rarely if ever exist alone ; they are generally
associated with septal defects, as will be shown immediately.

I append, as usual, a few out of the many bibliographical refer-
ences which exist. 0. H. Allis, Phila. Med. Times, ii. for 1872, p. 174 ;
A. Bechtel, Diss, inaug., Zurich, 1862 ; G. J. Bozanis, Diss, inaug.,
Wurzburg, 1876; Bohn, Deutsch. Arch. f. klin. Med., v. 436,1869;
A. J. Burgess, Med. News, lxii. 437, 1893; F. Gatti, Ann. univ. di
med. e chir., cexxxv. 305, 1876; A. P. Daniel, These, Paris, 1874;
A. Debely, THse, Montpellier, 1878; A. Hugues.TAese, Paris, 1874;
Kussmaul, Ztschr.f. rat. Med,, 3 B., xxvi. 99, 1866; H. Lebert, Med.
Times and Gaz., i. for 1870, p. 1 ; H. Meyer, Arch./, path. Anctt., xii.
497, 1857; W. E. E. Smart, Lancet, ii. for 1871, p. 288; C. Tarutti,
Gior. d. r. Accacl. di med, di Torino, xxxviii. 257, 1875 ; S. Weiss, Diss,
inaug., Erlangen, 1874.

Anomalies of the Aorta.

Anomalies of the aorta are less common than those of the pul-
monary artery, and when they do occur they are more often of the
nature of dilatation than of stenosis. Congenital aortic dilatation is
practically always associated with other malformations ; and since, as
Moussous {op. cit.) points out, there is generally an increase in the
semilunar cusps as well as in the ring of the vessel, incompetence is
uncommon. Aortic narrowing may exist as the sole malformation or
in association with other defects. When it occurs alone (which is
rare), it may affect the whole vessel and may possibly be an anomaly
predisposing to chlorosis (Virchow) ; or it may be localised to the
part of the vessel lying between the origin of the left subclavian
artery and the insertion of the ductus arteriosus ; or, again, it may
be situated at the part where the ductus arteriosus enters the aorta,
as is well shown by E. Wadstein in an inaugural dissertation of Lund
(Orn stenos och obliteration af aorta rid cller i ndrhetcn of ductus Botalli,
1897). Aortic stenosis may also exist along with other anomalies, and
then it may be situated at the orifice (coalescence of the semilunar
valves to form a diaphragm) or in front of it (conus or pre-orificial

ANOMALIES OF THE AORTA 501

part), which is very rare. Complete obliteration of the aortic orifice
has also been noted, but it, likewise, is rather rare ; instances have
been reported by Canton {Trans. Path, Soc, Lond., ii. 38, 1848-49),
C. Devilliers {Union ma/., 2 s., vii. 338, 1860), A. Meyer {Med. Bee,
New York, xxiii. 428, 1883), S. G. Shattock {Trans. Path. Soc. Lond.,
xxxii. :!8, 1881), and E. Apert {Bull. Soc. anat. cle Baris, lxx. G83,
1895). Theremin {op. cit., p. 125) has given details of seventeen cases
of aortic stenosis ; in all of them the infant was feeble and weakly
at birth ; in five of them there were signs of foetal endocarditis in
both ventricles. It is difficult to exclude fcetal disease as a factor in
the causation of congenital aortic stenosis, and it must be admitted
that some cases are not truly embryonic malformations but are the
results of foetal endocarditis. Theremin {op. rit., p. 14) has also given
notes of twenty-five cases in which the stenosis was limited to the
isthmus of the aorta ; in some of them there were likewise signs of
foetal endocarditis. A few further references to the literature of
congenital aortic stenosis may be appended here. Moutard-Martin,
Bull. Soc. anat. cle Baris, xlix. 737, 1874 : M. Stoll-Krotowski, Diss,
inawj., Berlin, 187-"> : Biilau, Berlin. Idin. Wchnschr., x. 217, 1873;
H. Eppinger, Vrtljschr.f. d.prakt. Ilcillc, cxii. 31, 1871 ; Knoevenagel,
Berlin. Bin. Wchnschr., xv. 525, 1878; T. A. Wise, Trans. Med. and
Bhys. Soc. Calcutta, viii. pt. 2, .'!84, 1842 : Potain, Gaz. held, de med.,
2 s., xxix. 292, 1892 : A. Laboulbene et P. Claisse, Bull, et mem, Soc.
med. d, hop. de Paris, •'! s., vii. 945, 1890; C. B. Lockwood, St, Barth.
Hosp. Rep., xiv. 352, 1878 : E. Leyden, GhariU-Ann., xiv. 151, 1889.

Transposition of Aorta and Pulmonary Artery.

Minor anomalies may occur in the relations of the aorta and
pulmonary artery to each other — the two trunks may lie too exactly
side by side, or one may lie too much in front of or behind the other
— but the most important malformation with which we meet is
transposition, the aorta arising from the right ventricle and the
pulmonary artery from the left. W. Osier (Keating's Cyclopaedia of
the Diseases of Children, ii. p. 760, 1889) describes a fairly typical
instance of transposition of these vessels ; it was an eighth-month
foetus, which had also hydrocephalus, umbilical hernia, spina bifida,
and club-feet ; from the right cardiac ventricle, which was larger than
the left, a vessel was given off, which passed over a vessel emerging
from the left ventricle, and, crossing the left bronchus, descended as
the thoracic aorta ; just above its origin it gave off a small pulmonary
branch to the imperfectly developed lungs ; the left ventricle was
smaller than the right, and from it a vessel passed up on the trachea
without communicating with the vessel from the right ventricle, and
then divided into the innominate and left carotid arteries : there was
a small orifice in the septum ventriculorum. In other cases the
transposition is more complete than what is described above, for the
artery for the lungs may come from the left ventricle instead of from
a vessel which afterwards becomes the descending aorta. A few refer-
ences are added. E. Ducrest, Arch. gen. de me'd., hi. 76, 1840; J. H.

502 ANTENATAL PATHOLOGY AND HYGIENE

Smith, Journ. Anat. and Physiol., xvi. 302, 1881-82 ; I. Etlinger, Med.
otchet Imp. S.-Peterb. Vosp. Doma, 147, 1881 ; E. Termen, ibid,, 218,
1872 ; H. Ashby, Journ. Anat. and Physiol, xvi. 90, 1881-82 ; M. Holl,
Med. Jahrb., 503, 1882 ; T. W. King, Month, Journ. Med. Sc., iv. 32,
1844; D. B. Lees, Trans. Path, Soc. Lond., xxxi. 58, 1879-80: L.
Mazzotti, Bir. din. di Bologna, 2 s., ix. 257, 1879 ; T. B. Peacock,
Trans. Path. Soc. Lond.,v\. 117, 1855; P. H. Pye-Smith, ibid., xxiii
80, 1872 ; O. Ward, ibid., iii. 63, 1850-51 ; F. H. T. Bamm, Diss, inaug.,
Kiel, 1899; A. Ooyon, Bull. Soc. anat. de Paris, lxxii. 519, 1897;
J. Doming, Arch. Pedial, vii. 741, 1890 ; G. Sanders, Journ, Anat.
and Physiol, xxvii. 464, 1892-93 ; H. I). Rolleston, Pediatrics, iv. 108,
1897; Simmonds, Deutsche med, Wchnschr., xxvi., Vcr.-BciL, 81, 1900.

Other Anomalies of the Great Vessels.

Either the aorta or the pulmonary artery may he absent com-
pletely, or, more often, may be represented solely by a fibrous band
or cord. In these cases a single large trunk arises from the base of
the heart ; it may contain three or four semilunar valves ; and it may
or may not be subdivided internally into two passages by a septum.
There is here evidently a persistence of a stage, normally transitory
(truncus arteriosus communis), in which the aortic bulb is single, or,
at any rate, double only in its interior. As a rule, this aortico-
pulmonary vessel, as it has been called, is single only for a short
distance (during which it gives origin to the coronary arteries), and
then divides into an aorta and a pulmonary artery ; in other instances,
two pulmonary arteries, right and left, arise directly from it ; in yet
other cases, the lungs receive their blood supply from the greatly
enlarged bronchial arteries (vide L. Muhr's dissertation of Wiirzburg
(1889) and W. Pitschel's dissertation of Kunigsberg (1897)).

Persistence of the ductus arteriosus as a channel of communica-
tion between the pulmonary artery and aorta is a common anomaly ;
it is to be grouped with patency of the foramen ovale as a postnatal
malformation. In some very rare instances, however, there is no
ductus arteriosus, and the aorta and pulmonary artery simply com-
municate directly (vide L. Cazin's These, Paris, 1897).

Various anomalies affecting the great veins of the thorax may
be met with. Thus, either the superior or the inferior vena cava
may open into the left auricle, while the pulmonary veins open into
the right ; again, either of them may open into both auricles (l)ubrueil,
Memor. d. hop. du midi, ii. 224, 1830). A pulmonary vein may
open into the superior vena cava (W. Gruber, Arch. f. path. Anat,,
cii. 3, 1885). Either the vena cava inferior or the vena cava
superior may be double ; this anomaly has been reported with com-
parative frequency. Instances of duplicity of the superior vena
cava have been reported by (Jhassaignac (Bull Soc, anal de Paris, xv.
106, 1 840), by Eppinger (Mitth. d. Ver. d. Aerzte in Steiermark, xxvi.
144, 1890), by W. Gruber (Ocsterr. Ztschr. f. prakt. Heilk., xi. 1115,
1865, xii. 556, 1866; Arch, f. path. Anal, lxxxi. 462, 1880, xcix. 492,
1885), byR Howden (Journ. Anal and Physiol„\xi. 72, 1886-87), by

CARDIAC VALVULAR ANOMALIES 503

H. Hex (Prag. mcd, Wchnschr., vii. 341, 1882), and by Shepherd (Med.
News, xlii. 694, 1883). Duplicity of the inferior vena cava has been
noted by Lagneau (Bull. Soc, anat. de Paris, xxviii. 344, 1853) and
Broca (ibid., xxvii. 474, 1852), and partial duplicity of it by N.
Nicolai (Diss, inaug., Kiel, 1886) and by J. Walter (Diss, inaug.,
Erlangen, 1884). Again, there may be a persistent left superior
vena cava, the corresponding vessel on the right side being repre-
sented by a fibrous cord (J. J. Charles, Joum. Anat. and Physiol., xxiii.
649, 1888-89) ; or the right superior vena cava maybe absent (W. S.
Greenfield, Trans. Path. Soc. Lond., xxvii. 120, 1875-76). The pre-
sence of a left inferior vena cava has been noted by H. J. Waring
(Joum. Anat. and Physiol, xxviii. 46, 1893-94), by W. H. White
(Trans. Path. Soc. Lond., xxxv. 131, 1883-84), and others. Most of
these anomalies are easily accounted for by persistence of transitory
states, e.g. the left duct of Cuvier. Sometimes the pulmonary veins
are abnormal in number ; three or five have been met with.

Transposition of the thoracic duct has been observed. W. J.
AValsham found it ending on the right side in the confluence of the
internal jugular and subclavian veins (St. Earth. Hosp. Eep., xvi.
99, 1880); and Calori (Mem. d. r. Accacl. d. sc. d. 1st. di Bologna, 5 s.,
i. 189, 1890) noted that it passed into the right subclavian vein by
two tubes, of which the larger was external to the jugular vein and
the smaller posterior to it. G. Cousin's series of cases of anomalous
arrangement of the duct was published in Marseille medicate (p. 334)
in 1898; I summarised his article in the Archives of Pediatrics (vol.
xv. p. 685, 1898).

Valvular Anomalies of the Heart.

Some of the congenital anomalies of the valves of the heart are
doubtless due to foetal endocarditis, but others are apparently of
embryonic origin and of the nature of malformations. To some of
these, allusion has already been made in the description of aortic and
pulmonary narrowness and dilatation. The semilunar valves of the
aorta and pulmonary artery may be diminished in number : they
may all be absent ; there may be two instead of three, and then there
is usually stenosis of the affected vessel, aorta or pulmonary artery.
Sometimes, although rarely, the number of the cusps is increased ;
there may be four or five of them, and they are more often found
in connection with the pulmonary artery than with the aorta ; they
may be all of the same size,, or one or two may be small or rudi-
mentary. Sometimes the cusps are fenestrated. Details of individual
cases will be found in the following references : — E. Albrecht, St.
Petersb. mcd. Wchnschr., i. pt. 24, 1, 1876 ; Bernard, Bull. Soc. anat.
de Paris, lv. 426, 1880 ; C. H. Carter, Trans. Path, Soc. Lond., xxiv.
48, 1873; Duckworth, ibid., xvii. 113,1866; Fletcher, Prov. Med,
and Surg. Joum., p. 270, 1849 ; E. H. Greenhow, Trans. Path. Soc, Lond.,
xx. 98, 1869 ; W. Jenner, ibid., iv. 102, 1852-53 ; Gueneau de Mussy,
Bull. Soc. anat. de Paris, xi. 74, 1836-37; W. Osier, Montreal Gen.
Hosp.Rep.,\. 284, 1880: Paulicki, Deutsche Klinik. xxi. 363, 1869;

504 ANTENATAL PATHOLOGY AND HYGIENE

0. Lindenberg, Diss, inaug., Wiirzburg, 1893; E. Zollikofer, Diss,
inaug., Zurich, 1809 ; H. W. Cattell, Trans. Path. Soc. Phila., xv.
161, 1889-91 ; G. Martinotti, Gazz. d. din., Torino, xxiii. 273, 289,
305, 1886, xxiv. 161, 1886 ; F. A. Packard, Trans. Path. Soc. Phila,,
xiii. 100, 1885-87, xviii. 215, 1898 : H. K. Abbott, Journ, Anat. and
Physiol, xxxviii. 103, 1903-04.

Anomalies of the auriculo-ventricular valves (mitral and tricus-
pid) may also be met with ; but they are rare and are generally
associated with other malformations (e.g. defective septa). Thus the
three cusps of the tricuspid may be fused together, producing narrow-
ing or actual obliteration of the orifice (W. G. MacCallum, Johns
Hopkins Hosp. Bull., xi. 71, 1900); similar changes have been met
with in the two cusps of the mitral. Many of the reported cases
are doubtless foetal in origin (foetal endocarditis), but some may be
truly embryonic. The following references may be consulted : — E. T.
Bruen, Trans. Path. Soc. Phila., xiv. 154, 1891 ; J. J. D. Burns, Brit.
Med, Journ,, i. for 1860, p. 301 ; A. L. Carroll, Med. Pec, xv. 57,
1879 ; W. Ebstein, Arch. f. Anat. Physiol, u. wissensch. Med., 238,
1866 ; W. S. Greenfield, Trans. Path. Soc, Bond., xxvii. 128, 1876 ;
C. Kelly, ibid., xxi. 91, 1870 ; T. Marxsen, Diss, inaug., Kiel, 1886 ;
H. Matthiessen, Diss, inaug., Kiel, 1896 ; H. O. Sommer, Phila. Med.
Journ,, i. 528, 1898.

Associations of Cardiac Anomalies.

I have enumerated the more important individual malforma-
tions of the heart which are produced in early antenatal life ; but
it has to be recognised that these rarely occur alone, being generally
combined together in certain ways. It must not, at the same time,
be thought that they only occur in combination, for perforations of
the interauricular and interventricular septa, narrowing of the pul-
monary artery and aorta, transposition of these great vessels, and
numerical anomalies in the cusps of the cardiac orifices may all be
met with as solitary malformations. So, also, may persistence of the
foramen ovale or of the ductus arteriosus, a fact which is readily
understood when it is borne in mind that these anomalies are
developed late in antenatal or early in postnatal life, at quite
a different time from the others. Nevertheless, the outstanding
character of cardiac anomalies is their tendency to be combined
together in different ways : it is exceptional for them to occur singly.
In many cases the associated malformations make it possible for
both antenatal and postnatal life to be continued, and may restore
the physiological equilibrium of the heart's action. Thus in Coyon's
case (Bull, Soc. anat. de Paris, lxxii. 717, 1897), a child of thirteen
months, both the pulmonary artery and the aorta sprang from the
right ventricle, but then there was very little interventricular sep-
tum. It is indeed often a cause of wonder to the pathologist how far
the heart can adapt itself to circumstances ; thus, in another patient
of Coyon (loc, cit., p. 519) there was complete transposition of the
pulmonary artery and the aorta, so that the right heart supplied the

CARDIAC ANOMALIES 505

greater and the left the lesser circulation, for there was no defect
in the interventricular septum ; yet the child lived to the age of
fourteen months, although it was always more or less cyanosed ;
there was some mixture of hlood, however, through the patency of
the foramen ovale and ductus arteriosus.

Let us look, now, more particularly, at some of the combinations
or associations of cardiac anomalies.

There may, for instance, he the two-chambered or bilocular heart,
in which defect of the interauricular septum is accompanied by
defect of the interventricular septum, and a single large arterial
trunk arises from the organ. This grouping of grave defects may
be met with in markedly malformed twin foetuses {e.g. the foetus
paracephalus dipus cardiacus, Trans. Edinb. Obst. Soc, xviii. 103,
1893), which do not survive birth. On the other hand, it may
occasionally be compatible with a certain degree of postnatal life ;
thus, W. P. Northrup's patient (Med. Bee, New York, xxxiii. 353,
1888) lived for one month in a state of marked cyanosis, although the
heart consisted of no more than two chambers, and the pulmonary
artery was represented by a fibrous string. This grave type of
malformation has sometimes been termed the " reptilian " heart.

A more common variety of association is the complete absence of
the interventricular septum along with incomplete absence of the
interauricular one (or vice vcrsd), and it is not uncommon to find, in
addition, such anomalies as absence or stenosis of one or other of the
great arterial trunks, with, perhaps, transposition of the aorta and
pulmonary artery. Such combinations of grave cardiac anomalies
may lie accompanied by malformations in other parts of the body
(e.g. anencephaly, exomphalos, etc.). Numerous instances have been
put on record, of which I may refer to Epstein's case (Ztschr. f.
Heilk., vii. 308, 1886), in which the interventricular septum was
absent, the interauricular incomplete, the pulmonary veins opened
into the superior vena cava and the right auricle, the aorta was on
the right side, and the spleen and omentum were absent. Notwith-
standing the grave nature of their malformations, individuals thus
affected may survive their birth, dying ultimately from such maladies
as tubercle and cancer.

Associations of defects in which the number of anomalies is more
restricted are more common than any of the foregoing. Thus the
posterior part of the interventricular septum may be absent, when it
is commonly found that the interauricular septum is perforated
inferiorly and that there is some deformity of the auriculo- ventricular
valves, along with dilatation of the right side of the heart and of the
pulmonary artery ; the foramen ovale may be open or closed. Again,
the whole of the anterior part of the interventricular septum may be
wanting, when there may be transposition of the aorta and pul-
monary artery or fusion of these two vessels into one trunk. Yet
again, the defect in the interventricular septum may lie in the
posterior part of the anterior segment (usually regarded as coinciding
with the " undefended space ") ; then there may be various combina-
tions of deformities. For instance, there may be narrowing of the

&

506 ANTENATAL PATHOLOGY AND HYGIENE

orifice of the pulmonary artery or of the whole vessel with dilatation
of the aorta ; or the pulmonary artery may be obliterated, when there
is either persistence of the ductus arteriosus or a special enlargement
or increased number of the bronchial arteries ; rarely the association
is with aortic instead of pulmonary stenosis ; in all these types the
semilunar valves of the aorta or pulmonary artery may be diminished
to two instead of three, and the mitral and tricuspid valves may be
deformed.

Partial defects of the interauricular septum are, as have been
already described, of various kinds, according as the part which is
wanting belonged to the primary or to the secondary septum or to
both ; they may be associated with partial absence of the inter-
ventricular septum, with (very often) dilatation of the pulmonary
artery, and (rarely) with premature closure of the foramen ovale.
When a single arterial trunk arises from the heart, other anomalies
are always present, but they vary so much that no association of
them can be regarded as typical. On the other hand, transposition
of the aorta and pulmonary artery may be found as the solitary
cardiac anomaly.

The associations of the cardiac malformations which are developed
later in antenatal life (namely, patency of the foramen ovale and of
the ductus arteriosus) are fairly typical. The open ductus and foramen
may, for instance, exist apart from other anomalies ; but more fre-
quently an open foramen implies pulmonary stenosis, with, as a rule,
obliteration of the ductus arteriosus. Again, patency of the foramen
ovale may coexist with obliteration of the pulmonary artery, when,
of course, the ductus will remain pervious.

Associated Malformations.

Besides the association together of various kinds of cardiac defect
it remains to be noted that such anomalies are often combined with
malformations outside the heart. It would appear that there are few
monstrosities and malformations which may not be met with in
association with cardiac anomalies, although precise evidence is not
always forthcoming on account of the neglect to examine the internal
organs in all cases. 0. Schaffer (in F. von Winckel's Berichte und
Studien, p. 515, 1892), who made careful dissections of all his
teratological specimens, found, in one case, absence of the inter-
ventricular septum and transposition of the great vessels combined
with umbilical hernia, club-foot, and absence of the right kidney and
suprarenal capsule ; in another instance, there were various defects
(caudal appendage, club-hand, absence of fibula, anal atresia, and
absence of the urinary organs) as well as two openings in the inter-
ventricular septum, the upper of which established a communication
between the right ventricle and the left auricle. While such associ-
ations are common in foetuses so malformed as to be incapable of
postnatal life, they are also frequent in children not so fatally
defective, as is shown by the fact that three out of nine cases of
congenital heart disease reported by J. Thomson and W. B. Drum-

TERATOGENESIS OF CARDIAC ANOMALIES 507

niond (Edinh. Hasp. Brp., vi. 57, 1900) exhibited defects in other
parts of the body {e.g. hare-lip, cleft palate, digital and aural
anomalies, imperforate anus). F. Warner (Med. Times and Gaz., i. for
1882, 61, 90, 144) gave many illustrative instances of the same
association of anomalies in 1882. P. Hebert (Rev. d'orthop. 2 s., v. 57,
1904) has described an infant, one month old, in whom there was
absence of the bile ducts, absence of the radius, club-hand, dislocation
of the shoulder, and a congenital malformation of the heart (open
foramen).

Teratogenesis.

The cardiac anomalies present many difficulties, not the least of
which are concerned with their causation. I do not pretend to be
able to solve these difficulties ; I have already tried to throw some
light upon some of them in the first volume of this Manual (pp.
369-373), although not, I fear, with shining success. All that I can
do here is to throw out some suggestions which may possibly open up
new lines of inquiry which other investigators may profitably follow.

It may be thought that since the malformations of the heart are
internal, lying as they do within the thoracic cavity, they are not
under the influence of and cannot be caused by external morbid
agencies. The same supposition might be made regarding malfor-
mations of the brain and spinal cord. It would, in this latter
instance, be met with the statement that there is a time in the
development of the nervous system when it is external and not
internal. Similarly there is a time when the heart is outside the
body of the developing embryo and not inside it. If therefore such
external anomalies as facial fissures, exomphalos, anencephaly, and
hemimely are to be explained by a teratogenic cause external to the
embryo (although probably internal to its annexa), then there is no
sufficient reason against the possibility of the malformations of the
heart being also produced in their first inception by an external
cause. We are not compelled to look only for an internal causative
agency, such as foetal endocarditis, although we may still regard such
an etiological factor as a possibility. It is, further, unnecessary for
us to think it impossible that the same causes as are in action in
producing external malformations may be also in action in the
development of cardiac defects. There may be a unity in terato-
genesis after all, although the heart lie deeply buried in the interior
of the fully formed embryo. There is a side argument which may be
used in support of this conclusion, although too much stress need not
be laid upon it. I refer to the frequent association in the same
subject of internal anomalies of the heart with external malfor-
mations of the body, head, and limbs.

When we come to study the actual mechanism by which cardiac
anomalies are produced, we are again surrounded by difficulties. If
we have recourse to the most acceptable teratogenic theory, that
which regards malformations as arrested developments, we get an
explanation, fairly intelligible and fairly satisfactory, for such
anomalies as absence of the interventricular septum, the presence of

508 ANTENATAL PATHOLOGY AND HYGIENE

a truncus arteriosus communis, and a rudimentary state of the cusps
of the cardiac orifices. Further, if we examine very minutely into
the details of the various stages of the ontogenesis of the heart, as
Rokitansky did in so illuminating a fashion, it begins to be clear
that even such apparently inexplicable teratological states as trans-
position of the great vessels and anomalies of the large veins may be
explicable in a similar manner. But there are yet other anomalies,
such as pulmonary stenosis, aortic dilatation, and the deformities of
the auriculo-ventricular valves and of the foramen ovale which seem
to offer themselves for explanation by means of the theory of foetal
endocarditis or some other disease of the unborn infant. I have
elsewhere given reasons for doubting the correctness of the notion
that malformations are due to foetal diseases (pp. 164-197), chief
among which is the belief that the malformations precede the
diseases ; but I do not feel that such influences as foetal endocarditis
have no bearing at all upon the malformations which are found in
the heart. The disease may attack the deformed part ; it may even
do so more readily because it is deformed ; it may exaggerate the
effects of the deformity, may prolong its existence, or, possibly,
interfere with its antenatal cure ; and it is not impossible that both
the disease and the deformity may be caused by the same morbid
agency, and that the difference in result may be due to the time
when that agency comes into action. I need not, however, re-argue
to its conclusion this matter of the nosological versus the embryo-
logical theory of teratogenesis, for that has been done once and for
all in Chapter XI. All that I need add is a belief in its applicability
to internal malformations of the heart as well as to external malfor-
mations of the head and trunk.

The ultimate cause of the arrest of development which leads to a
cardiac malformation is obscure. Syphilis, rheumatism, alcoholism,
tuberculosis, and lead poisoning in the parents have all been
suspected of being the ultimate teratogenic cause ; and it is quite
conceivable that such poisons by their presence in the tissues of the
early embryo may so influence its organogenesis as to cause arrests
and disorders which are translated into anomalies and malformations
in later antenatal life. To some extent, clinical evidence supports
this conclusion ; family prevalence has been noted several times in
connection with congenital anomalies of the heart (Eger, Deutsche
med. Wchnsehr., xix. 81, 1893; T. S. Dabney, Proe. Orleans Parish
Mai. Soc. (1896), p. 124, 1898; L. Ferrannini, Arch. ital. di med. int.,
iii. fasc. 3-6, p. 65, 1900; A. Borgherini, Gazz. d. osjj. xxi. 1415,
1900); and in many cases the parents of infants with such malfor-
mations have suffered from syphilis, tuberculosis, and the like.
The supposition is, I think, warranted, that a poison, such as that of
syphilis or tuberculosis, may by its presence in the embryonic tissues
first cause arrested development and consequent embryonic malfor-
mations (defect of interventricular septum, etc.), may then produce
foetal endocarditis in the foetal period of antenatal life, and may
finally interfere with the closure of the foramen ovale and ductus
arteriosus in early postnatal existence.

SYMPTOMATOLOGY OF CARDIAC ANOMALIES 509

Antenatal Symptomatology of Cardiac Anomalies.

I have already (in Vol. I. p. 372) dealt with the possibility of

diagnosing antenatal cardiac anomalies before birth by the detection
of murmurs ; I need not touch upon that subject again, save to add
two references, namely, those of H. Earth (France uied., xxvii. 35:!,
1880) and of C. Audry and E. Lacroix (Lyon mal, lxiii. 341, 1890).
When we come to ask ourselves the question, What effects upon the
foetal circulation and economy does an antenatal cardiac malformation
produce l we enter upon an inquiry which is full of difficulty. I
shall venture no more than a few general statements, which are of
the nature rather of interrogations than of affirmations. In the first
place, it is extremely likely that many antenatal lesions of the heart
interfere little or not at all with the life and growth of the foetus.
Many cases are on record in which, after birth, congenital heart
anomalies have existed for years without being suspected, and it is,
therefore, quite probable that the same thing may occur in utero.
What makes this the more likely is the character of the foetal circu-
lation : even under normal circumstances there is such a mixture of
pure arterial and impure venous blood that no part of the foetus gets
a much better supply than any other part, except, perhaps, in the
case of the liver ; the further mixing which may follow the presence
of a defective interventricular septum is not, therefore, of high
importance. In the second place, there seems to be no reason to
doubt that the heart may attain to antenatal compensation. This
may differ in detail from the compensation of postnatal life, but it
cannot be more difficult of attainment, and may be very much easier
if we consider the protective mechanisms of antenatal existence.
Probably there is evidence of its occurrence in the fact that certain
cardiac anomalies, such as narrowness of the pulmonary artery, are
so often accompanied by associated malformations, such as defective
interventricular and interauricular septa. In the third place, there
is some evidence that cardiac anomalies occasionally have a grave
effect upon antenatal life. Thus, it is no uncommon observation
to note that infants thus affected are puny and delicate at birth.
Further, foetal ascites and general foetal dropsy may sometimes be
traced to such a cause. Hardouin and Moreau (Rev. obstet. internal.,
suppl., p. 184, 1895), for instance, have recorded a case of ascites,
hydrothorax, and general cyanosis in which there was no interauri-
cular septum and only an imperfect interventricular one ; and it has
been maintained by some that premature closure of the foramen
ovale may be the occasional cause of general ftetal dropsy (vide Vol.
I. of this Manual, p. 295). It is quite likely that hydramnios may
sometimes be due to circulatory disturbances in the foetus. There
are many other problems concerned with the effects produced upon
foetal life and health by a malformed heart — there is, for instance,
the bearing of such a state upon the characters and number of the
blood corpuscles, red and white — but I have already led the reader
far enough into a region of surmises. With the much less shadowy

510 ANTENATAL PATHOLOGY AND HYGIENE

subject of the postnatal symptoms, diagnosis, and treatment of con-
genital heart affections I have not, in this work, to do.

Malformations of the Lungs.

Malformations of the lungs are uncommon, and when they do
occur are usually of the nature of accessory lobes (G. Humbert,
Rev. de mid., xxiv. 453, 1904). Normally the left lung consists of
two and the right of three lobes ; but the left may possess three
lobes {e.g. in the cases of L. Humphry, Journ. Anat. and Physiol., xix.
345, 1884-85, and of C. Giorgieri, Gazz. d. osp., vii. 259, 1886), or the
right may possess four (e.g. in the cases of J. Chiene, Journ. Anat.
and Physiol., iv. 89, 1870; of W. A. Edwards, Amer. Journ. Med. Sc,
n.s., xc.'l82, 1885; of W. T. Eckley, Chicago Med. Times, xxvii. 201,
1895 ; and of Apert, Bull. Soe. anat. de Paris, p. 154, 1898) or even six
lobes (Testut et J. Marcondes, Gaz. held. d. sc. med. de. Bordeaux, i.
1045, 1880). In Apert's specimen, which came from a man thirty-five
years of age, the four lobes were produced by the presence of an
X-shaped fissure instead of the normal Y-shaped one. Isolated
accessory lobes may also be met with at the apex (E. W. Collins,
Dublin Journ. Med. Sc, lviii. 252, 1874) or at the base of the lung
(E. Iiektorzik, Wchnbl. d. h. k. Gesellsch. de Aerztc in Wien, xvii. 4,
1861). G. Schaffner (Arch. f. path. Anat., clii. 1, 1898) believes that
the lobus inferior accessorius is a fairly constant structure in both
lungs, that on the right side it is constantly connected with the
cardiac bronchus alone (being, therefore, homologous with the cardiac
lobe of animals), while on the left side it is connected with the inner
branch of the second ventral bronchus ; the cardiac bronchus is an
independent lateral bronchus, only occurring on the right side. The
vena azygos major in a fold of the pleura may be found in the fissure
which separates off a supernumerary lobule of the lung (W. G ruber,
Arch. f. 'path. Anat., lxxxi. 475, 1880; ciii. 484, 1886). An interest-
ing article on this subject is that by E. de Josselin de Jong (Nederl.
Tijdsehr. v. GeneesJc., ii. for 1893, p. 669.

Incomplete development of one or both lungs is not very rare,
and is chiefly associated with congenital diaphragmatic hernia (q.v.,
p. 484). Absence of both lungs is sometimes met with, but only in
grossly deformed twins (acardiac or acephalic) ; but absence of one
lung may apparently occur and yet permit postnatal life. Maschka
(Allg. Wien. med. Ztg., vii. 78, 1862) recorded a case in which the
right lung was absent, the whole of the right pleural cavity being
filled by the heart ; the right pulmonary vessels were absent, and the
right bronchus was represented solely by a small cul-de-sac ; the left
lung consisted of only one lobe ; the infant, a seven-months foetus,
lived for two hours. Hem (Wchnschr. f. d. gcs. Heilk., 536, 1837)
also described a case of absence of the right lung; there was an
opening in the interventricular septum of the heart. Absence of
the left lung has been reported by Munchmeyer (Deutsche med.
Wchnschr., xi. 295, 1885), E. Theremin (Rev. mens. d. mal. de Venf.,
ii. for 1884, p. 554), and W. Gruber (Arch. f. path. Anat., cii. 11,

PULMONARY MALFORMATIONS 511

1885). In the last-named case there were three supernumerary
spleens and an accessory hepatic lobe. L. Fiirst, in his valuable
article on malformations of the lungs (in Gerhardt's Handbuch der
Kindcrkrankheiten, iii., 2 Hefte, 553, 1878), refers to a man seen by
Haberlein who lived to the age of twenty years, although the left
lung was absent. Another case of absence of the left lung in an
adult was reported by M. Tichomiroff {Month. Internal Journ. Anat.
and Physiol, xii. 24, 37, 1895); in this case there also was persistence
of the left superior vena cava.

In connection with pulmonary malformations I may refer here
very briefly to some bronchial and tracheal anomalies. The trachea
may divide not into two but into three chief or stem bronchi. Cases
have been reported by E. Leudet (Compt. rend. Soc. de biol., 2 s., iii.
54, 1857), by D'Ajutolo (Mem. r. Accad. d. sc. d. 1st. di Bologna, 4 s.,
vi. 449, 1884-86),* and by H. Chiari (Ztschr.f. Heilk., x. 470, 1889 ;
Beitr. z. path. Anat. u. z. (dig. Path., v. 329, 1889; Prag. tried. Wchnsehr.,
xv. 567, 1890; xvi. 87, 1891). One of Chiari's cases was specially
interesting. It was that of a child that lived sixteen days. After
death it was found that the interauricular and interventricular septa
were both defective ; the aorta arose from the right ventricle, and
the pulmonary artery (which for some distance was a solid cord) from
the left ; the right superior vena cava (formed by a vena azygos and
the right brachio-cephalic vein) opened into the right auricle ; the
inferior vena cava and the left superior vena cava (formed by a vena
azygos), the left brachio-cephalic vein, and the four pulmonary veins,
united into a common stem, opened into the left auricle. The trachea,
above the normal site of bifurcation, gave off, on the left side, a
separate bronchus, which passed to the upper lobe of the three-lobed
left lung. The right stem bronchus had a double eparterial bronchus.
The spleen was absent, and there was an anomalous arrangement of
the peritoneum in connection with the liver. Chiari's case repre-
sented an unusual form of trifurcation of the trachea ; commonly
there is simply premature origin of the normal eparterial bronchus
to the right side (or to the left in transposition of the viscera).

I have already referred to cases of oesophageal occlusion in which
the lower end of the oesophagus opened into the trachea (vide p. 463) ;
but there are yet other malformations of the trachea and bronchi,
including their absence (when the lungs are attached immediately to
the larynx), congenital narrowness or shortness of the trachea or left
bronchus, defect or excessive number of the tracheal rings, and con-
genital dilatation of a part of the trachea, or the presence of a
diverticulum. Malformations of the pulmonary artery and veins
have been described already.

Malformations of the Thymus Gland.

Carl Hennig, in Gerhardt's Handbuch der Kindcrkrankheiten
(Nachtrag iii. 1893), gives an interesting account of the malforma-
tions of the thymus gland. It may be absent in otherwise well-
formed new-born infants, but more often in acephalic twin foetuses ;

512 ANTENATAL PATHOLOGY AND HYGIENE

it may be abnormally small or abnormally large; or it may show an
increase in the number of gland lobes. Instances of absence were
reported by H. Harrington {Load. Med, Gaz., hi. 314, 1828-29) and
A. Clark (Lancet, ii. for 1896, p. 1077). In the dissection of all kinds
of monstrous foetuses I have been struck by the constancy of the
thymus gland : in a specimen of deformed twin, paracephalus dipus
acardiacus, it was absent (Trans. Edinb. Ohst. Sue., xviii. 48, 1893);
but in other types of monstrosity I always found the thymus, although
in some instances, such as exomphalos, it was somewhat abnormal in
position. I believe, therefore, that this organ plays an important
part in the physiology of the embryo and foetus, although it is not
as yet quite clear what its special duties are.

CHAPTER XXVII

Merosomatous Terata (cont.) : .Malformations of the Abdomen : Gastroschisis :
History, Nomenclature, Classification, Clinical Characters, Morbid Anatomy,
Teratogenesis, Literature ; Hernia of the Umbilical Cord ; Intestinal Diver-
ticula ; Intestinal Stenosis and Atresia ; Hour-glass Stomach ; Malposition
of Intestines ; Anomalies of Appendix Vermiformis ; Congenital Hernia and
Hydrocele ; Anomalies of Liver and Gall Bladder ; Anomalies of Spleen,
Suprarenal Capsules, Pancreas, and Blood Vessels ; Congenital Umbilical
Neoplasms.

There can be no doubt as to which of the many malformations and
monstrosities of the abdomen (gastro-terata) should be selected for
special description : gastroschisis or defect of the abdominal walls
with protrusion of the viscera suggests itself at once as the typical
teratological state. It is relatively common, for out of 331 specimens
and cases of antenatal morbid change examined by me I have met
with sixteen examples of it, not including cases of hernia into the
umbilical cord (of which there have been three) ; it is a striking mal-
formation ; and it raises practically all the questions concerned with
the teratology of the abdomen. It is, therefore, eminently suitable
for consideration as the type-monstrosity. Illustrations of specimens
in my possession are given in Figs. 25, 26, 27, 28, 29, 30, 31, 34, 54,
and 75.

Gastroschisis.

The history of gastroschisis (defective abdominal walls and exposed
viscera) goes back to the time of Lycosthenes, who in his Chronicon
(p. 596, 1557) reports the birth of a monstrous infant at the town of
Ploa in Voitland (Plauen in Voigtland ?) ; this infant, as may be
learned from the description and the illustration which Lycosthenes
gives, had its lowTer limbs bent up on the abdomen with the knees
extended, had a curiously shaped high head ("ad similitudinem
citharis," like a turban), and showed a sac, containing the intestines
hanging from an opening in the chest and abdomen ; this was in the
year 1547. The illustration given in the Chronicon produces the
impression of having been touched up for artistic effect, but there is
no reason to doubt that it represents a real case of gastroschisis, the
first of the long series of specimens with which the archives of tera-
tology are now supplied. Other early instances were reported by N.
Steno {Acta med. et phil. Hafn., i. 200, 1673), L. Schroeck (Miscell. Acad,
not. curios., Dec. i., Ann. vi.-vii. 341, 1677), G. A. Fried (Diss, inaug.,
Argentorati, 1760), Preuss (Acad. not. curios, cphem., Cent, vii.-viii.,
Appendix, p. 117, 1719), E. Sandifort (Acta Hclvet., vii. 56, 1772),
vol. ii.— 33

514 ANTENATAL PATHOLOGY AND HYGIENE

and M. Saxtorph (Acta reg. Soc. mcd. Harn.,\. 191,1783). By the

beginning of the nineteenth century the observations were sufficiently
numerous to permit attempts at classification being made, and now at
the beginning of the twentieth century there are so many cases of
gastroschisis on record that the mere task of collecting all the refer-
ences is arduous.

The nomenclature and classification of instances of gastroschisis
require some words of explanation. " Exomphalos " which is some-
times used as a synonym of gastroschisis is not, in my opinion, so
appropriate, for it concentrates attention too much upon the umbilicus,
and, further, it has been to some extent appropriated for the cases in
which there is an umbilical hernia in the ordinary sense of the word.
In the earlier records no single name was given to the monstrosity,
which was referred to by a descriptive phrase ; thus Fried (op. cit.)
entitled his communication " De foetu intestinis plane nudis extra
abdomen propendentibus nato." He subdivided all the cases into
those in which the intestines were contained in a sac and those
in which they were not. J. F. Meckel (Hanclhuch der path. Ana-
tomic, i. 95, 117, 1812) gave the name of "partial fissure of the
anterior surface of the body " to these monstrosities, and made some
distinction between various specimens according to the region speci-
ally affected. C. Meyer in his dissertation (" De fissuris hominis
mammaliumque congenitis," Berolini, 1835) used the name ventral
fissure, and divided all such fissures into median and lateral and into
complete and partial. A far more extensive scheme of classification
and denomination was introduced soon after by Isidore G. Saint-
Hilaire (Histoire des anomedies, ii. 264, 18.°>G). He gave the name
celosomus to the second tribe of the order of the autositic mon-
strosities ; this tribe contained the single family of the celosomian
terata, but it was subdivided into six genera. These six genera were
arranged in two groups. In one there was eventration of both the
abdominal and the thoracic viscera through an opening affecting both
the abdomen and the thorax ; here was placed the genus celosomus in
the strict sense in which there was a lateral or median eventration
with fissure, atrophy, and even total absence of the sternum and
hernial displacement of the heart ; here also was placed the genus
pleurosomus, in which there was a lateral eventration affecting chiefly
the upper part of the abdomen and extending to the chest-wTall, with
incomplete development of the arm of the same side. In the second
group were four genera which all agreed in the fact that the eventra-
tion affected the abdomen alone and did not extend to the thorax :
the first genus was called asj)alaso)mis,m which there was a lateral or
median eventration, affecting chiefly the lower part of the abdomen,
and the urinary organs, the genitals, and the rectum opened exter-
nally by three distinct orifices; the second genus was agenosomus, in
which, again, the eventration was lateral or median and affected the
lower part of the abdomen, but the urino-genital organs were either
absent or very rudimentary ; the third genus was cyllosomus, in which
the eventration was always lateral and chiefly affected the lower part
of the abdomen, and there was absence or incomplete development

VARIETIES OF GASTROSCHISIS 515

of the lower limit of the same side ; and the fourth genus was schis-
tosomas, in which the whole abdomen was open either in the middle
line or laterally, and the lower limbs were either absent or very im-
perfect. There are defects and complexities and overlapping in
Saint-Hilaire's system, but it has been widely used, and I have
therefore stated it fully ; I do not, however, intend to employ it in
this work, and I may consequently pass from the subject with one
further reference to it. N. -Toly in his Mdmoire sur un noiiveau genre
de monstres c&osom/iens, Toulouse, l<S4S,and in an earlier article, added
three new genera to the already existing six of Saint-Hilaire : to one
he gave the name dracontisomus, because in it, in addition to a median
eventration affecting the thorax and abdomen, the vertebral column
was greatly twisted on itself, and the ribs ran outwards almost hori-
zontally like the wings of a dragon or flying lizard (hence the name) ;
the second he called chelonisomus, and in it the eventration was either
median or lateral and abdominothoracic in extent, and the spine was
so bent that the sacral region and lower limbs were turned backwards
and enclosed within a sort of dorsal pseudo-thorax formed by the
ribs bent backwards ; to the third the term str&ptosomus was given,
because in it the spine was so twisted that the lower limbs were dis-
placed to right or left, and the eventration was again median and
abdominothoracic in extent. The various genera of the Saint-
Hilaire-Jolv scheme are, therefore, as follow : —

Celosomus,
Pleurosomus,

Aspalasomus,
Agenosomus,
Cvllosomus,

SchistosomuSj

Dracontisomus,

Chelonisomus,

Streptosomus.

I do not employ the above system in the present work because I
have adopted the regional method of arrangement, and because I
think that several of the names are confusing or at any rate have a
meaning not immediately obvious. The foetus may be like a dragon
or a turtle, but the likeness is not so striking as to make the name
anything less than an etymological puzzle. In Chapter XXV. I de-
scribed fissure of the sternum with ectopia cordis and pleurosomus
with protrusion of the lungs, and I touched upon chelonisomus on
p. .'U3, so that these monstrosities do not now require to be dealt
with. For these and other reasons I prefer to describe here only the
teratological states which affect the abdominal region (the gastro-
terata) ; and, as I have said, Saint-Hilaire's nomenclature and classi-
fication do not adapt themselves to this plan.

G-urlt's name for gastroschisis was schist ocormiis fissiventralis, and
Forster's was fissura abclominalis {Die Missbildungen des Menschen,
p. 109, 1865); the latter author divided cases of fissura abclominalis
into the complete form, into umbilical hernia, and into extroversion
of the bladder, and under the first heading he subdivided the cases
according to the region of the abdomen affected. Ahlfeld, however,
pointed out that the defect rarely corresponded with any degree of
accuracy to any anatomical region {Die Missbildungen des Menschen

516 ANTENATAL PATHOLOGY AND HYGIENE

p. 204, 1882) ; he separated off ordinary umbilical hernia and extro-
version of the bladder and used ectopia viscerum as a synonym for
fissura abdominalis. 1 think there is no doubt that it is a better plan
to separate hernia of the umbilical cord and extroversion of the bladder
from the ordinary cases of gastroschisis in which there is a greater
or less defect of the anterior abdominal walls with protrusion of the
abdominal viscera ; I have adopted this method here. At the same
time it has to be noted that Taruffi (Storia della teratologia, vii. 406,
1804) retains the idea of a regional classification and nomenclature, and
arranges the specimens of gastroschisis in seven groups as follow : —

1. Epigastro-schisis : aperture in epigastric region.

2. Epi-omphalo-schisis : aperture in the epigastrium in continua-

tion with the umbilicus.

3. Thoraco-omphalo-schisis : umbilical aperture continued into

the sternum.

4. Hypogastro-schisis : aperture in hypogastriuin.

5. Pleurosomato-schisis: lateral aperture- of the trunk.

6. Hypogastro-etro-schisis : aperture of the hypogastric region,

including the symphysis pubis.

7. Hologastro-etro-schisis : aperture of the whole abdomen, ex-

tending to the symphysis pubis.
I do not, as I have said, follow Taruffi in his arrangement ; I prefer
to describe extroversion of the bladder by itself, and to regard the
other types as varieties of gastroschisis not sufficiently clearly marked
off from each other as to deserve separate treatment. No system of
classification is perfect, and it is, therefore, important to avoid com-
plexity both in arrangement and terminology.

The clinical history of cases of gastroschisis differs in no important
detail from that in anencephaly or other grave monstrosity. Post-
natal life is usually very short : in one of my specimens, that repre-
sented in Figs. 27 and 28, the heart, which was outside the body,
beat for fifteen minutes after birth ; the mother, who was quite
healthy herself, had previously given birth to four normal and healthy
infants ; there had been no maternal impression in pregnancy. In
another case (Fig. 75) there was such marked hydramnios that the
woman was thought to be suffering from an ovarian cyst ; she was forty-
one years of age and had had five previous pregnancies terminating in
the birth of four living and one dead foetus. The mother of another
specimen of gastroschisis (Fig. 20) was healthy but suffered from
great pain in the left iliac fossa towards the end of pregnancy ; her
husband was crippled with sciatica and was alcoholic. In 1801 I
received from Dr. W. Paterson a very marked specimen of gastro-
schisis with fissure of the symphysis pubis, anencephaly, spina bifida;
and retroflexion of the vertebral column ; the mother was a primi-
para who during the third month of pregnancy got a bad fright from
seeing a child narrowly escape being run over in the street ; there
was great hydramnios, the liquor amnii " gushing like a mill stream."
In another case (Figs. .°>0, 31) the mother had great pain during
pregnancy in one iliac fossa ; this symptom was also present in A.
K. Simpson's case (Trans. Edinh. Obst, Soc, vii. 80, 1882).

SYMPTOMATOLOGY OF GASTROSCHISIS 517

The diagnosis of the monstrous state of the foetus is usually made
during labour ; for there seems to be a special tendency for the pro-
truding intestines and other abdominal viscera to form the presenting
part, and the palpation of them is usually immediately helpful. A
loop of bowel may be mistaken for a loop of the umbilical cord, but
not if the presence of a mesentery in the former case be kept in mind.
The tendency of the exposed intestines to present at the os uteri in
labour is specially dwelt upon by A. R. Simpson in his article referred
to above {he. cit.); it is to be explained partly at least by the fact
that the normal fcetal attitude of flexion is often replaced by one of
extension, and that in consequence the foetus is so folded that the
narrow end of the ovoid is formed by the exomphalos. In this rela-
tion I may give some notes of the latest specimen of gastroschisis
which I have been able to examine, one sent to me by Dr. Eorie of
Cardenden in May 1904. The mother was a secundipara, twenty-four
years of age ; she fell in labour, and a midwife finding a sac presenting
at the os ruptured it under the belief that it was the bag of membranes ;
on the following day Dr. Uorie was called in and found the fcetal
liver and bowels protruding through a fully dilated os and giving the
sensation as if there was a placenta pnevia ; there had been some
haemorrhage and the foetus was firmly impacted, therefore version was
accomplished and the monstrosity delivered. During version the
liver and a piece of intestine were detached. Five months previously
the mother had a fall while lifting a heavy chest and thought that
" everything came away then." I need not multiply instances of this
kind, but may add that the presentation is not invariably an
abdominal one ; sometimes the head is the lowest part, more especially
when there is anencephaly, a not uncommon complication of gastro-
schisis. Labour often comes on prematurely, and there is usually
delay until version is performed. There is generally some rnemor-
rhage in labour, for the placenta, being closely attached to the foetus,
is apt to be detached early from the uterine wall. The sac con-
taining the abdominal viscera is generally ruptured at some time
during delivery (Glanville Morris, Trans. Edinb. Obst. Soc, xix. 103,
1894).

The morbid anatomy of gastroschisis is a matter of some com-
plexity : I shall first describe certain characters which are fairly
common to all the specimens, and thereafter deal with more excep-
tional details. The most obvious feature of the monstrosity is the
absence of the anterior abdominal walls. There is no umbilical
orifice, because there is absence of the abdominal wall in the region
of the umbilicus. At the sides, and separated from each other by a
considerable distance, are the margins of the ununited walls ; they
are usually continuous with the amniotic membrane, so that the
abdominal viscera which protrude from the abdomen project into a
sac formed by the amnion. There is consequently no properly formed
umbilical cord, but vessels pass from the placenta to the foetus in the
walls of the sac formed by the amnion. These vessels are often much
shorter than usual, and may consist of only one artery and one vein.
The whole placenta is, therefore, situated much nearer to the foetus

518

ANTENATAL PATHOLOGY AND HYGIENE

than is normal, and has often been described as adherent to it (vide
pp. 171-175). The intestines and other abdominal organs which lie
in this sac may be surrounded by a second membrane, the parietal
peritoneum (Fig. 75) ; but apparently this is wanting in most of the
marked cases ; perhaps it is not developed, or it may be fused with
the amniotic sac itself. Sometimes the intestines are adherent to the
walls of the sac by bands or threads.

The protruded viscera vary somewhat in different cases. The

Kuin*

Uterus
Sladder

Vaaina
Symphysis,
Pub,?.

Mouth

Liver

Fir:. 75. — Sectional Appearances of Specimen of Anenccphaly ami Gastroschisis.
Left slab of section shown. Specimen No. '29.

intestines are always present, but the rectum (when it exists) and a
variable part of the descending colon remain in the abdominal cavity.
The stomach may be partly outside the body and the liver wholly so
(Fig. 75), or the liver, stomach, spleen, and even the heart and a lung
may be all external to the abdomen (Figs. 27, 28). The kidneys and
suprarenal capsules are usually intra-abdominal in position ; but they
may be drawn forward out of their place, and, on account of the
dislocation of other organs, they form new relations with surrounding

MORBID ANATOMY OF GASTROSCHISIS 519

parts. The bladder may remain inside the abdomen (Tig. 75) ; but
when the defect in the anterior abdominal wall extends so far down
as to affect the symphysis pubis there may be not only displacement
forward of the bladder, but also defective formation of its anterior
wall (extroversion) ; in other cases the rectum and bladder may form
a cloaca. It is not uncommon to find atresia ani ; and the uterus
(in the female) may be bicornate or entirely double (Thompson Lowne,
Catalogue, No. 310, p. 85, 1893). The diaphragm is usually intact ;
but it may be wanting, when the heart may be among the extruded
viscera. There may be various irregularities in the arrangement of
the vessels : thus the aorta may be continuous with a single artery
passing to the placenta (in T. H. Bryce's case, reported in the Journ.
Anat. and Physiol., xxix. p. 553, 1895, it was the right umbilical
artery that formed this continuation) ; in one of A. Eischpler's
specimens (Arch. f. EntwcJclngsmechn. <L Organ., vi. 556, 1898) there
was persistence of the right umbilical vein with the presence of only
one artery ; and in another case the umbilical vein did not enter the
liver, there was no ductus venosus, and the inferior vena cava and
the superior mesenteric artery were absent.

It is very common to find retroflexion of the spinal column and
absence of certain of the vertebra?. There were only thirty vertebral
bodies in one of my specimens (Fig. 75), and the number was still
further reduced in others. The spinal retroflexion, which may be
combined with torsion, is often very remarkable. Thus, in one of
my cases (Fig. 28), there was an extraordinary sigmoid curve, a very
prominent cervico-dorsal lordosis being compensated by an equally
prominent dorso-lumbar cyphosis ; in another (Fig. 75) the curves
were similar but less marked; and in some other specimens (Nos. 28
and 331 in my collection) the retroflexion was so excessive that the
pelvis could easily be made to touch the back of the head. This
state of the spine may perhaps be regarded as a persistence of what
is normal at the third week of antenatal life (vide Fig. 12, p. 34) ;
but the curves do not always coincide. Backward displacement of
the lower limbs may accompany this spinal deformity, and they may
be found lying posteriorly, a teratological fact which may give rise
to a clinical mystification during the operation of version ; the limbs
may also be rotated (L. Kriwsky, Monatschr. f. Geburtsh. u. Gynaek.,
xi. 895, 1900), somewhat as they are in sympodia, but without being
fused together. There may likewise be spina bifida and anencephaly,
although these are rather of the nature of accidental complications
of gastroschisis than of associated anomalies ; the same remark
applies to malformations of the upper extremities such as club-hand
(Thompson Lowne, Catalogue, No. 308, p. 84, 1893).

The teratological characters which have been described are met
with to some extent in all cases of gastroschisis ; but there are others,
to which I now direct the reader's attention, that are less constant.
Some specimens, for instance, show an opening in the anterior
abdominal wall situated in the epigastric region and limited to it ;
in most of them, however, the opening permits the protrusion of the
heart, with or without its pericardial sac, and accompanied by defect

520 ANTENATAL PATHOLOGY AND HYGIENE

of the diaphragm ; these, therefore, are rather to be reckoned with
the cases of sternoschisis and of ectopia cordis than with gastroschisis
and ectopia intestinorum. There are, however, a few cases in which
there seems to have been protrusion of abdominal contents through
an epigastric opening, and among these Mr. James Calder's record is
quite definite {Med. Essays and Obscrv., Edinburgh, i. 203, 1733). He
wrote, " I was sent for to visit a new-born child that had a large
Share of its Intestines lying without the Teguments of the Abdomen ;
I at first imagined the containing Parts had been torn in the Birth,
but upon Examination found the Navel intire, and a Perforation half
an Inch above it, thro' which the Guts had fallen out, with the Skin
closely united to them. The Child seem'd otherwise to be as lively
and Brisk as any new-born Infant uses to be, and for 12 or 14
Hours it received Milk and Syrups by the Mouth, without any
Appearance of Uneasiness ; but after that time it vomited every
thing till its Death, which happened four Days after, and all the
while it had no Passage by the Anus." At the autopsy it was found
that the protruded intestines consisted of the jejunum, ileum, and
part of the colon and ciecum. " The Mother could not remember
ever to have been surprised, frighted, or hurt during all the Nine
Months of her going with Child." Probably epigastric gastroschisis
is the rarest of all the varieties of this monstrosity.

Less rare are the cases in which an opening exists in the epigas-
trium, extending down to and including the umbilicus, and permitting
the exit of some of the abdominal contents. Tarufn (op. cit., vii. 413,
1894) has collected eight instances, including one by G. Tonelli (Ann.
univ. di mod., lxxxii. 441, 1837) and one by himself. In the latter
case the skin of the abdomen under the ensiform cartilage and ribs
was wanting, and its place was taken by an amniotic sac containing
the heart, stomach, small and large intestine, and the right kidney ;
the rectum and anus were absent.

It is more common to find an abdominal opening such as that
described above continued upwards into a thoracic defect and asso-
ciated with fissure of the sternum. I have already written of such
cases in Chapter XXV. under the heading of sterno-epigastric ectopia
cordis ; but they really belong rather to this chapter, for although
they may be regarded as connecting links between thoracic and
abdominal fissures they are rather to be looked upon as instances of
gastroschisis complicated or associated with sternoschisis. They have
the characters which I have described as common to gastroschisis,
and need not, therefore, be again referred to here.

A more exceptional arrangement is that in which coils of intestine
protrude through an opening in the hypogastric region, often small
in size, and not including the umbilicus. In J. G. Hasenest's observa-
tion ("Foetus mostrosi ex imaginatione deturpati," Acta Acad. nat.
curios., vi. 31, 1742), the gastroschisis was accompanied by marked
torsion of the spine and absence of one lower limb. G. A. Fried's
case (op. cit.) seems to belong to this rare variety. The urinary
bladder is intact, and the urachus may be seen traversing the
abdominal wall at the side of the opening to reach the umbilical

LATERAL GASTROSCHISIS 521

cord ; these characters serve to mark oil' this exceptional type from
the ordinary forms in which there is hypogastric eventration with
extroversion of the bladder.

Somewhat exceptional, also, are the cases of lateral opening in
the abdomen with eventration (pleurogastroschisis). I have met
with a case of this kind in which the fissure, however, was not
restricted to the abdomen, but extended also to the thorax (Fig. 34,
p. 181); in this instance the arm of the affected side was deformed
and fixed by the wrist to the margin of the opening by a band of
skin. In another case which I saw with Dr. D. Milligan in 1897
(No. 199 in my list) there was a defect in the abdominal wall in the
left side of the abdomen through which the abdominal viscera pro-
truded, but the skin was continuous and, therefore, the condition
was rather to be regarded as one of lateral hernia than of gastro-
schisis ; the infant, a male, also exhibited lumbar spina bifida
(ruptured during birth) and deformities of the lower limbs ; he
survived his birth for several weeks. A more typical instance was
reported by A. Bertino {Ami. di ostet. e ginec, xxi. 735, 1899) : it was
a male foetus, normal in all its parts save in the abdominal region ;
there was an opening in the right half of the anterior abdominal wall,
about the level of the umbilicus, through which a packet of abdominal
viscera (with no membranous covering) projected ; the opening was
nearly circular, had a diameter of 4 cm., and was separated from the
normally closed umbilical ring by a tract of normally constituted
abdominal wall ; the extruded mass was made up of three lobes, an
upper (consisting of the lower end of the oesophagus, the stomach,
and duodenum), a middle (made up of small intestines adhering
together by pseudo-membranes), and a lower (constituted by csecum,
ascending colon, transverse colon, and descending colon as low as the
sigmoid flexure) ; and the umbilical cord had its normal appearance,
and contained the usual three vessels, the vein passing in the super-
ficial part of the wall skirting the margin of the opening through
which it passed to be distributed normally to the liver. When the
lateral opening is lower down, as in a specimen which Dr. F. W. N.
Haultain allowed me to examine, and which is represented in Fig. 76,
the lower limb of the affected side may be absent (cyllosomus). A
similar case was reported by H. Stern (Diss, inaug., Kiinigsberg i. Pr.,
1896); and in A. Brabandt's specimen (Diss, inaug., Leipzig, 1896)
both the right os innominatum and the leg were absent. Schaefer
(Diss, inaug., Bonn?e, 1837) and W. Vrolik (Tabulce a<? illustrandam
embryogenesin, Plate lxiii. Fig. 4, Plate Ixiv. Figs. 4, 5, 1894) gave
good descriptions of this condition (gastroschisis and monopodia);
and Tarufti (op. cit., vii. 549, 1894) has included these cases with
the one-legged foetuses in which there wTas no gastroschisis in the
group which he calls mono-anileus apus (absence of one os innomina-
tum and corresponding lower limb).

Gastroschisis occurs in the lower animals as well as in the human
subject : it has been observed with comparative frequency in the calf,
but has also been noted in the pig, the lamb, the kitten, and in
reptiles and birds.

522

ANTENATAL PATHOLOGY AND HYGIENE

The teratogenesis of gastroschisis, as was to be expected, has given
rise to much discussion. The amniotic theoiy has been already dis-
cussed in this volume (pp. 168-175), and I need not again open up
the whole subject. There have been many theories, including those
of shortness of the umbilical cord, injury to the foetal abdomen,
adhesions of the amnion, twisting of the vertebral column, smallness
of the thoracic and abdominal cavities, and simple arrest of closure
of the anterior abdominal walls ; the great difficulty consists in
separating the causes from the effects, for, obviously, a small
abdominal cavity may either be the cause or the result of the even-

^>

Fig. 76. — Specimen showing Absence of Left Lower Limb
with Protrusion of Viscera (Cyllosomus).

tration. In order to account for the frecment association of defects
in the terminal part of the bowel and in the generative organs, T. H.'
Bryce {Journ. Anat. and Physiol., xxix. 553, 1895) brings forward
defective development of the allantoic stalk and hind gut which
interferes with the incurving of the tail end of the embryo and
maintains the attitude of retroflexion of the trunk. This is a. matter
upon which investigations into the vascular supply of the foetus and
placenta may throw some light ; for if it can be shown that the
placental blood supply is furnished by vitelline and not by allantoic

UMBILICAL HERNIA 523

vessels, it would give some support to the idea that the allantois was
defective.

Literature. — There is a large literature bearing on this subject.
The references, however, are scattered, and little care has been taken
in lists and catalogues to separate records of true gastroschisis from
cases of hernia of the umbilical cord and ring. For instance, in
the Index Catalogue (Washington), some references to gastroschisis
appear in vol. ix. under the heading Monsters, from defect or mal-
formation of abdomen and thorax ; others come under Abdomen,
abnormities of (vol. i. 5, 1880; 2 s., i. 7, 1896); and yet others under
Hernia, umbilical (vol. vi. 168, 1886; 2 s., vol. vii. 60, 1902).
Taruffi (op. cit, vii. 403-430, 492-507, 1894; viii. 561-563, 1894)
makes an attempt, with some measure of success, to separate gastro-
schisis both from umbilical hernia and from hernia of the umbilical
cord. I refer my readers to these bibliographical sources, and
simply add here a few references that I have found useful : J.
Lindsay, Trans. Glasgow Obstet. and Gynazc. Soc, iii. 233, 1903; Leith
Napier, Trans. Obstet. Soe. Lond., xxxvi. 302, 1895 ; A. Klautsch,
Centrlbl.f allg. Path. u. path. Anal, vi. 385, 1895 ; H. M. MacGill,
Lancet, ii. for 1894, p. 687 ; P. Ardouin et E. Kirmisson. Rev. d'orthop.,
viii. 104, 1897 ; H. Lockwood and C. Addison, Quart. Med. Journ.,
vii. 231, 1899 ; E. Stangl, Areh.f. klin. Chir., lxxiii. 853, 1904; L. E.
Deshusses, These, Lille, 1903.

Congenital Umbilical Hernia.

Much more common than gastroschisis is the condition known as
congenital umbilical hernia, omphalocele ab ortu (Plater), or schisto-
cormus exomphalus (Gurlt). It is essentially a herniation of some
of the abdominal contents into the umbilical cord, a hernia funiculi
umbilical is; and, like many other malformations, represents a condi-
tion which is normal in early antenatal life, but if persistent becomes
obviously teratological. By some writers it is made to include
hernias through the umbilical ring which come on soon after birth
(e.g. at the fall of the cord) ; but such states, although they may be
predisposed to antenatally, are really postnatal developments in the
great majority of cases ; I do not, therefore, specially consider them
here, although I freely admit that antenatal influences take their
place along with delayed and incomplete closure of the umbilical
ring, increased abdominal pressure, and the like, in their causation.
There is, with all possible care in classification, a certain degree of
confusion existing regarding the cases in which the hernia occurs
into the cord and those in which it is truly umbilical ; for it is quite
possible that in later foetal life, after the intestine has been with-
drawn from the root of the cord and retracted within the abdomen,
a hernia may occur through the ring closely resembling that which
develops during the early weeks of postnatal life, but differing
iu the fact that the umbilical cord arises from it. In this work,
however, I deal rather witli the truly antenatal and embryonic
malformations, those which arise during the embryonic phases of

524

ANTENATAL PATHOLOGY AND HYGIENE

development; on that account it is with hernia funiculi umbilicalis
that I am specially concerned.

Hernia of the umbilical cord is comparatively common ; it is not
indeed seen so often as hare-lip, cleft palate, spina bifida, or anen-
cephaly, but it occurs about once in five thousand births (0. Lindfors,
Nord. med. Ark., xv. No. 25, 1-34, 1883). In '.VM cases of antenatal
disease and deformity examined by myself, there were four cases
{Nos. 27, 39, 185, and 198 in my list) of hernia into the cord;
and Taruffi (pp. tit., vii. 365 ; viii. 413, 1894) has gathered together
from medical literature 130 cases, without completely exhausting the
list.

Fig. 77. — Case of Hernia of the Umbilical Cord.
Specimen No. 27.

The clinical history of hernia funiculi umbilicalis seldom contains
any antenatal indications, with the possible exception of those of
hydramnios. In one of my specimens (Fig. 77) the mother had a
record of habitual abortions ; she had been ten times pregnant, but
had carried only three children to the full term ; she had generally
aborted about mid-term, and the foetus with umbilical hernia (Fig. 77)
was regarded as at four and a half months ; there was a large quantity
of greyish-brown liquor amnii ; and the foetus, which was alive when
born, made lively movements for about five minutes (Trans. Edinb.
Obstel. Sue, xvii. 241, 1892). In the above case the placenta seemed

HERNIA FUNICULI UMBILICALIS 525

healthy ; but in another one (Trans. Edinb. Obstet. Soc, xvii. 243, 1892),
in which the foetus was born with the membranes unbroken, the
placenta showed lesions regarded as syphilitic. In both these cases
delivery took place at mid-term; but in that of an infant (No. 185)
sent by Dr. Waddell of Slamannan into the Sick Children's Hospital,
Edinburgh, in 1896, the labour was at the full time and wras accom-
panied by marked hydramnios ; in this instance (which I examined
with Dr. Joseph Bell) the mother's health and heredity had been
good, but on the father's side there was a very clear history of
scrofula {Trans. Edinb. Obstet. Soc, xxii. 27, 1897). In a case which
I examined with one of my dispensary pupils (Mr. Dick) in 1897,
the infant was the product of a second pregnancy, and the elder
child was quite normal. (Case No. 198.) Sometimes the umbilical
hernia is the presenting part in labour (as in the case of the foetus
shown in Fig. 77) ; at other times the head presents, and there is
delay with the birth of the trunk on account of the large size of the
abdomen (as in Dr. Waddell's case). Male infants are more often
affected with hernia of the cord than females ; G. Buschan (Diss,
inaug., Breslau, 1887) gives the proportion as 43 males to 20
females.

The clinical history after birth has a sad similarity, for most
of the infants perish in a few hours or days, unless they are skilfully
operated upon. The cause of death is commonly rupture, mortifica-
tion, or inflammation and suppuration of the sac, with peritonitis ;
and sometimes there is strangulation of the hernia. The result,
however, is not invariably fatal ; for retreat of the protruded parts
into the abdomen and cicatrisation may occasionally occur (e.g. in
the cases of L. Margariteau, Bull. gni. de Therap., xlviii. 544, 1855,
and Stoltz, ibid., liii. 467, 1857), and treatment by operation or by
methodical compression has sometimes succeeded. Details of the
probable mode of spontaneous cure, and of the various plans of
treatment, are given by E. Kirmisson (Traite des maladies chirurgi-
cales d'origine cdngenitale, pp. 230-233, 1898). The great difficulty
which meets the operator is the fact that the abdominal cavity is not
large enough easily to contain all the viscera when the protruded
parts are replaced. At the same time, the surgery of to-day has
greatly improved the prognosis in all these cases.

In its morbid anatomy hernia of the umbilical cord presents fairly
constant characters. In the umbilical region of the abdomen there
is seen a more or less spherical projection, varying in size from that
of a walnut to that of the closed fist, or even of the foetal head.
It is easy to recognise that the swelling is funic in nature, for the
distal part of the cord may arise from it and pass to the placenta, or
there may be an indication of the umbilical ring between its base
and the abdominal wall. Sometimes the sac is defective or absent,
and the viscera are exposed : this occurs most often when there is no
true cord, when the sac is simply composed of amniotic membrane
passing to the placenta, and when in consequence the birth of the
child can hardly take place without rupture. In one of my speci-
mens, however (that numbered 39, and given me by Dr. W. Fordyce),

526 ANTENATAL PATHOLOGY AND HYGIENE

on account of the birth of the foetus in the membranes this sac was
found unruptured, lying between the infant and the foetal surface of
the placenta. Possibly, also, the sac may rupture in utero before the
commencement of labour. In most cases, however, there is a sac
which can be recognised as consisting of the sheath of the cord. At
first it is smooth and transparent, and allows the contents of the
hernia (intestinal coils and liver) to be seen plainly through it, but
in a few days after birth it becomes opaque (inflammatory changes).
Sometimes two membranes make up the sac- wall, and the inner of
these is to lie regarded as parietal peritoneum; but this is not a
constant arrangement, and it is possible that the single membrane,
when it is found, represents both the covering of the cord and the
peritoneum, in an undifferentiated state. Generally there is a sharp
line of demarcation at the umbilicus where the normal skin of the
abdomen begins and the covering of the sac ceases ; but the sac-wall
may be, as it were, prolonged beyond the margin of the umbilical
ring and only fade away gradually into the surrounding cutaneous
covering.

It has been said that the herniated mass is generally round in
shape ; but it may, on occasions, be conical, and may even, in rare
cases, show projections or diverticula (A. G. Luecke, Diss, inaug.,
Halis Sax., 1854). It gets larger during straining or crying of the
infant. Its relations to the vessels of the umbilical cord have not
always been clear ; at any rate they have not often been clearly
described. A. Scarpa, however, in his monumental work (SuU'ernie,
Pavia, 1819), has given details on this point, and has shown how
one of the umbilical arteries may be absent; subsequent writers
confirmed Scarpa's conclusions in the main. The umbilical cord,
when it is clearly marked off, may arise either under or above the
hernia, in the middle line or to one or other side. From the
descriptions given it seems to me to be quite likely that the vessels
in the cord which run to the abdomen (especially when they consist
of only one artery and a vein) are really vitelline (omphalo-mesenteric)
and not umbilical (allantoic) in nature.

The contents of the hernial sac vary somewhat, but not to the
extent that they do in gastroschisis. Most often the intestines are
met with, and the ileum is very constantly present ; the large
intestine is less frequent. Next in frequency among the extruded
viscera is the liver, and there are also a few cases in which this
organ formed the sole contents of the sac (J. B. Palletta, Excrcita-
tiones patkologicce, pt. 1, Art. x. p. 161, 1820) ; the stomach also may
be found ; and among the rarer structures may be named the spleen,
the pancreas, a kidney, and even the heart (in cases of defect of the
diaphragm). When the liver is either the only or the most obvious
organ in the sac the name Iwpatomphalocrle has sometimes been given
to the condition. This organ may lie partly within and partly outside
the abdomen, and it may show so deep a constriction at the umbilical
ring as to suggest that it consists of two parts or that there is a
" double liver." It is an interesting question whether the malforma-
tion of the liver in these cases is due to the hernia or is the cause

HERNIA FUNICULI UMBILICALIS 527

of it. In K. C. Buist's specimen {Trans. Edinb. Obstet. Soc, xxii. 25,
1897), which I had an opportunity of examining, the projecting mass
of liver substance grew from the quadrate lobe ; this supernumerary
structure may have prevented the normal closure of the umbilical
ring, or it may have resulted from displacement of the liver through
it, by traction upon the umbilical vein passing into the hepatic
substance. The question, however, cannot be settled without a more
thorough knowledge of all the determining factors that have to do
with the closure of the anterior abdominal wall in embryonic life.
Some very interesting speculations have been made regarding lower-
ing of the level of the diaphragm, shortness of the umbilical vein,
and the relation of the umbilical vessels to the duodenum ; but they
are only speculations. The contents of the sac may show adhesions
to the sac- wall and to each other ; when there are no adhesions
the organs may to some extent be returned to the abdominal
cavity.

Associated malformations are not uncommon in connection with
hernia of the umbilical cord. There may, for instance, be anen-
cephaly, spina bifida, club-feet, club-hands, atresia ani, ectopia vesica',
and retroflexion of the spine. As might be expected, the associated
intestinal malformations are of great interest, and demand for theii
full elucidation a knowledge of the ontogenesis of the lower end of
the alimentary canal such as we do not at present possess. There
may, for instance, be varying degrees of defect of the lower bowel,
such as atresia ani and atresia recti ; the whole of the large intestine
may be wanting and the small intestine may end at an opening in
the wall of the hernial sac, possibly but not certainly produced by
antenatal inflammation and ulceration (A. Billi, Ann. univ. di med.,
clxxvii. 492, 1861) ; or, again, the lower end of the intestine may
open in or near the ectopic bladder (P. Bockenheimer, Arch./, klin.
Chir., lxix. 669, 1903). Meckel's diverticulum may be present in an
umbilical cord hernia (e.g. in M. Wollstein's case, Arch. Pediat., xxi.
416, 1904). The pelvic organs, also, may show malformations : there
may be a splitting of the genital tubercle into two (P. Bockenheimer,
loc. cit.), or the uterus may be bicornate or didelphic (J. H. Raymond,
Med. Bee, 1. 514, 1896). Rarer anomalies are sacral tumour (A.
Fleischmann, Diss, inaug., Konigsberg i. Pr., 1894), facial fissure
( W. Jacobs, Diss, inaug., Marburg, 1884), hydrocephalus (R. Lange,
Diss, inaug., Konigsberg, 1891), multiple cysts of the umbilical cord
(W. F. Boggess, Arch. Pediat, xv. 678, 1898), Polydactyly (Ponty,
Bull. Soc. d'anat. et physiol. cle Bordeaux, xv. 113, 1894), and per-
sistence of cardinal vein and absence of ductus venosus (V. V.
Preobrazhenski, Journ. ahush. i jensk. boliez., St. Petersburg, xiv. 141,
271, 1900).

In its teratogenesis, hernia of the umbilical cord illustrates very
clearly the leading teratogenetic theories which have already been
discussed in earlier chapters (vide pp. 129-224). There was, first,
the theory of pressure or mechanical action, showing itself in external
violence to the mother during pregnancy, in backward flexion of the
spine of the foetus, in shortness, essential or acquired, of the umbilical

528 ANTENATAL PATHOLOGY AND HYGIENE

cord, and in overfilling of the abdomen with enlarged organs (e.g.
kidneys) preventing the drawing in of the coils of intestine at the
umbilicus. There was, second, the doctrine of disease as the cause of
hernia funiculi umbilicalis, which practically took always the form
of foetal peritonitis and its effects ; for instance, adhesions forming
between the coils of intestine and the sheath of the cord might
prevent the normal withdrawal of these coils into the abdominal
cavity. Finally, there was the embryological theory, the hypothesis
of arrested development or disordered ontogenesis. This view has
taken many forms, and the changes have been greatly due to the
gradual progress of our knowledge of the mode of development of
the abdominal cavity and its contents. It seemed at first that
nothing needed to be added to the statement that at an early stage
in embryonic life there existed a physiological hernia funiculi, and
that, therefore, the monstrosity was simply a persistence of this
normally transitory phase. But difficulties arose when it began to
be realised that it was not known in what manner the intestinal
coils in the cord were drawn back again into the abdomen, and that,
therefore, the cause of the non-withdrawal of them in teratological
cases remained equally obscure. It was hazarded as an explanation
that shortening of the mesentery might be the indrawing force, and
that, therefore, non-occurrence of the shortening might lead to the
persistence of the herniation. It was also suggested, and with more
probability, that persistent traction on the intestines by the vitelline
duct might be the cause of the malformation (a theory with which
Ahlfeld's name has been specially associated, Arch. f. Gynack., xi. 85,
1877) ; but there are difficulties in the way of a full acceptance of
this theory, including the position of attachment of the cord to the
herniated mass (e.g. lateral or inferior). At the present time there is
a tendency to combine these various theories together in order to
obtain a good working hypothesis to explain the different arrange-
ments of parts which have been recorded. Narrowness of the amnion,
a mechanical cause, has been invoked in order to explain the non-
union of the margins of the umbilical ring (an arrest in development) :
and there have been many other more or less ingenious suggestions.
The general conclusion to be drawn is that the explanation of hernia
of the cord is to be found in embryology, but that all the details of
the mode of origin can only be known when all the steps in the
development of the abdomen and its organs are understood. Possibly,
cases in which the herniation of the abdominal contents occurs
laterally and not in the immediate neighbourhood of the umbilicus
may throw light upon the whole subject. Such cases are rare, but
not unknown. We have also to bring together the knowledge which
has been gained regarding gastroschisis both median and lateral, for
I believe that the causes operating in hernia of the cord and in
gastroschisis differ not so much in nature as in the time in embryonic
life when they come into play.

MALFORMATIONS OF INTESTINES 529

Intestinal Malformations — Diverticula.

Among the many anomalies found in the organs that lie within
the abdomen, intestinal diverticula take a foremost place. Unlike
gastroschisis and hernia funiculi umbilicalis, they are not visible
externally and only come under notice when they establish a com-
munication with the exterior (faecal fistula at umbilicus, etc.) or inter-
fere with the normal functions of the bowel and call for surgical
interference. They come, therefore, under the heading of anomalies
causing pathological manifestations in postnatal and not as a rule in
antenatal life ; they constitute very striking instances of the post-
poned effect of antenatal malformations. Since I have taken gas-
troschisis and hernia of the cord as the type-monstrosities of the
region of the abdomen I shall only touch upon the anomalies now
under consideration ; but the reader who desires further details and
more minute particulars will find them in Tarufn's work (Storia delta
teratologia, vii. 289-330, 1894), where forty pages are occupied with
the discussion of them and with an enumeration of the bibliographical
references to them.

As a matter of fact no part of the alimentary canal is exempt
from diverticula. They may occur in connection with the oesophagus,
and a few cases have been recorded in connection with the stomach.
These gastric diverticula would seem to have been connected with
the pyloric end of the stomach and have usually been observed only
when they established an opening at the umbilicus ; it is probable
that the diverticulum is in the root of the cord at birth, and that,
when the cord falls off, an opening is thus formed putting the gastric
cavity in communication with the exterior (a gastric fistula). A
probable instance was reported by H. Tillmanns {Deutsche Ztschr. f.
Chir., xviii. 161, 1882-83); in it the diverticulum formed a tumour,
the size of a nut, at the umbilicus, and had the structure of the pyloric
part of the stomach.

Duodenal diverticula have also been noted, although they are of
considerable rarity. M. Both (Arch. f. path. Anat., lvi. 197, 1872)
met with five cases ; and other observers have reported examples,
Taruffi, for instance (op. cit., vii. 307), having in his museum a
specimen showing two diverticula arising from the middle third of
the duodenum. Gandy (Bull. Soc. anat. de Paris, lxxv. 691, 1900)
has met with a case on the left side below the head of the pancreas ;
and Letulle (Presse med,, Paris, i. for 1899, 13) deals with the subject
of the " peri-vaterian " diverticula. From the jejunum, also, pro-
jections or appendages may arise : L. Ciniselli (Sopra il gabinetto
anatomo-patliologico esistentc nello Spedale Maggiore di Cremona, p.
54, 1869) has reported a case in which six of these took origin close
together, while a seventh and a larger one was situated about 5
inches away from them. It is not clear, however, that Ciniselli's case
had an antenatal origin. More recently, F. Buzzi (Arch. f. path.
Anat., c. 357, 1885) has described a " congenital " example of a jejunal
diverticulum.

Diverticula of the large intestine also are somewhat rare ; and
vol. ii. — 34

530 ANTENATAL PATHOLOGY AND HYGIENE

when they occur it is not always possible to assume that they are
congenital in origin. G. Fleischmann (Leichertoffnurigen, 28, 1815),
however, saw a case, affecting the colon, in a seven-mouths foetus;
and several probable instances have been recorded by more recent
writers (<i. Durante, Bull. Sac. anat. dc Paris, lxxi. 318, 1896;
L. Scbreiber, Deutsches Arch. / klin. Med., lxxiv. 122, 1902; I.
Tonta, Verhandl. d. Gesellsch. deutsch. Naturf. u. Aerzie, lxxi., pt. 2,
2 Hefte, 132, 1900). The nature of the so-called diverticula of the
sigmoid flexure is not clear (G. P. Biggs, Proc. N.Y. Path. Soc. (1894),
p. 44, 1895).

The most frequent site for intestinal diverticula is undoubtedly
the ileum ; and since the time when -T. F. Meckel (Handbuch dcr
path. Anatomic, i. 553-597, 1812) threw a flood of light upon the
nature of this diverticulum by showing that it was the persistent
vitelline or omphalo-mesenteric duct, no anomaly has been more
thoroughly investigated and reported upon. The literature of the
subject is very large, as may be learned by consulting the Index
Catalogue (vol. vii. p. 27, 1886 ; 2 s., vol. viii. p. 233, 1903). The
names most commonly given to these diverticula are Meckel's diverti-
culum (diverticulum. Mcckclii), persistent omphalo-mesenteric dvet, and
persistent vitelline duct. In form the diverticulum is tubular, very
rarely conical, spherical, or pear-shaped ; it is always narrower than
the ileum from which it takes origin ; and is always single, the rare
cases of multiple diverticula arising from the ileum being in all
probability of another nature altogether. It is attached to the
intestine at one point or another in a portion of the ileum called the
segment of Meckel ; this segment is situated (in the foetus) about 10
inches (25 cm.) above the ileo-c;ecal valve, and measures a few inches
in length ; it is most exceptional to find the diverticulum taking
origin below or above Meckel's segment. Its walls are continuous

*£>*" — " "■*■ «">■'"•" ^.^^^.^v..^ k, ~~a*

with those of the ileum ; and its lumen may communicate freely with
that of the bowel, or (rarely) may have a valve, or (more rarely) may
be shut off from it entirely, when, of course, it will consist of an
appendage blind at both ends. It comes off1 from the intestine
generally on the side furthest away from the attachment of the
mesentery, and at a right angle ; but sometimes it may be situated
laterally or even near the mesenteric attachment, and be directed
obliquely. Barely it possesses a mesentery of its own; very rarely it
lies between the folds of the mesentery. In structure it is made up
of all the layers of the normal ileum ; it possesses Lieberkiihn's
follicles and may show a Peyer's patch, but the glands and villi are
often rather poorly developed ; the muscular coat may be deficient at
the free end and allow the mucosa to form a hernial protrusion there
which gives the diverticulum a peculiar clubbed or hammer-like
shape. On its walls traces of the vitelline or omphalo-mesenteric
vessels may occasionally be recognised.

The outer end of a Meckel's diverticulum may either float free in
the peritoneal cavity or be attached. In the former case it may
either end in a rounded extremity, in a swelling, or in one or more
solid cords or threads; in an extraordinary case reported by E.

MECKEL'S DIVERTICULUM 531

Neumann (Arch. f. Heilk., xi. 200, 1870) it ended in a gland, the size
of a pea, with the structure of the pancreas. Freely floating diverti-
cula may endanger the postnatal life of the individual by forming-
loops round coils of intestine and leading to strangulation ; they may
also come to lie, alone or with a loop of intestine, in the sac of an
inguinal (L. Hendee, Bcitr. z. klin. Chir., xxiv. 542, 1904) or umbilical
hernia, or they may protrude into a hernia of the umbilical cord.
When, on the other hand, the outer end of the diverticulum is fixed
new possibilities arise. It may become attached to a coil of intestine
and so produce obstruction and strangulation in one or other of the
many ways described so clearly by F. Treves (Intestinal Obstruction,
37, 1884). This it certainly may do in postnatal life; and it is
possible that it may also atfect the bowel in a similar way in antenatal
life, although then the results will differ on account of the compara-
tively quiescent state of the alimentary tract before birth. It is a
reasonable hypothesis to think that in this manner some of the cases
of congenital defect of the intestines may be produced. In other
cases the diverticulum acquires adhesions with the umbilical ring : it
may then lie attached to the umbilicus or may even pass for a short
distance into the cord ; the diverticulum may remain pervious or may
be changed into a more or less fibrous cord with traces of the vitelline
vessels in it. Very often, however, the distal end of the diverticle
opens at the umbilicus and forms a faical umbilical fistula or
umbilical anus ; in most cases this is probably the result of postnatal
changes occm-ring at or about the time of the fall of the cord. (The
literature of the subject is extensive and will be found in the Index
Catalogue, vol. iv. pp. 1004, 1018, 1883; 2 s., vol. v. pp. 831, 837,
1900.) If the posterior end of the intestine be pervious the umbilical
anus may gradually close ; at other times it remains open and con-
tinues to discharge fajces and even lumbricoid worms ; at yet other
times, a small tumour, consisting of a prolapsed part of the mucous
membrane of the diverticulum (vitelline prolapse), may form, and
being cylindrical in shape may be described on superficial examination
as a " penis at the umbilicus." Inflammation of the diverticulum
(" diverticulitis ") may develop in postnatal life (L. Picque et A.
(Uiillemot, Bull, mid., Paris, xiii. 517, 1899), or a sarcoma may be
found in it (O. Fried, Diss, inaug., Erlangen, 1902).

Associated malformations are common in instances of intestinal
diverticula, and may take the form of anencephaly, displacement of
the viscera, acephaly (in the omphalo-angiopagous twin), thoracopagous
double monster, and many other teratological states. Diverticula
may also be met with in the lower animals (horse, dog, pig, lamb,
calf); and are not restricted to the mammalia, but occur in birds as
well.

The teratogenesis of Meckel's diverticulum presents no great
difficulty. It is obviously a persistence of the transitory vitelline
duct, and its frequent attachment to the umbilicus is easily explicable
by embryology. Diverticula arising from other parts of the intestine
than the segment of Meckel in the ileum admit of no such simple
explanation ; they might be regarded as distension-diverticula origina-

532 ANTENATAL PATHOLOGY AND HYGIENE

ting after birth, but in some cases at least they would seem to be
indubitably antenatal.

Intestinal Stenosis and Atresia.

Antenatal narrowing or occlusion of the intestines is not very
rare, but, being an internal malformation, doubtless often escapes
discovery. I have already (Vol. I. p. 365) described the blocking
of the pylorus which may cause symptoms and end in death a few
weeks after birth, and need not refer to it again here, save to remark
that it is probably the result of a foetal rather than of an embryonic
morbid state.

Obstruction of the duodenum has been noted, and I had an
opportunity, in 1897, of examining a case of probable antenatal con-
striction of this part of the intestines at the point of entrance of the
bile ducts (Case No. 190 in my list). There may also be a completely
imperforate state of the duodenum causing the death of the infant a
few days after birth. Then the upper half of the duodenum may be
found greatly dilated and a transverse septum may exist just below.
I have elsewhere (Trans. Edinb. Obstct. Soc, xvii. 133, 1892) referred
to a case of this kind which occurred in the hospital practice of the
late Dr. Angus Macdonald : there was no communication between the
first and second portions of the duodenum ; the infant, a full-time
male, developed jaundice, vomited constantly, and died on the sixth
day after birth (Trans. Edinh Obstet. Soc, x. 229, 1885). Durante
(Bull. Soc. anat. de Paris, lxxvi. 593, 1901) has recorded two cases of
occlusion of the duodenum just below the place of entry of the bile
and pancreatic ducts. Other duodenal cases have been reported by
J. B. S. Jackson (Boston Med. and Surg. Journ., lix. 355, 1858-59), L.
Cordes (Arch. Pcdiat., xviii. 401, 1901), C. Hess (Deutsche med.
Wchnschr., xxiii. 218, 1897), F. Heymann (Monatschr. f. GeburtsJt. u.
Gynack., x. 186, 1899), A. Katz (Bull. Soc. anat. de Paris, lxxvi. 471,
1901), and M. O. Wyss (Beitr. z. Bin. Chir., xxvi. 631, 1900). E.
Kirmisson (TraiM des maladies chirurgicales, 412, 1898) has related a
case of anal atresia operated on with apparent success soon after
birth ; the infant, however, died, and it was then found that there
existed a valvular obstruction of the duodenum in addition to the
anal defect.

Congenital intestinal obstruction may be situated in the jejunum.
This seems to have been the case in J. Thomson's interesting specimen
(Trans. Edinb. Obstet. Soc, xvii. 128, 1892) in which a small bit of gut,
blind at both ends, intervened between the distended duodenum and
the rest of the small intestine ; it was fixed in the shape of a horse-
shoe by a little tongue-like flap of mesentery ; the child, from whom
the preparation was obtained, died when ten days old. Other instances
of jejunal obstruction were reported by Pied (Journ. de mat., chir.,
pharm., iii. 227, 1802), Kirchner (Bcrl. klin. Wchnschr., xxiii. 444,
1886), and F. C. Turner (Trans. Path. Soc. Bond., xxxviii. 145,
1886-87). The fa>ces which pass from the anus in these cases are
usually colourless or earthy in appearance, a fact which H. Sal us

INTESTINAL ATRESIA 533

(Pra;/. mcd. Wchnschr,, xxi. 529, 1896) made use of to diagnose an
intestinal anomaly in the case of a foetus which was being born by
the breech. The absence of the dark green meconium, however, is not
a constant phenomenon, for in J. Thomson's patient (loc. cit., supra)
some was passed several days after birth; it is very difficult to
explain this circumstance.

The ileum is apparently a common seat of intestinal obliteration,
a fact which may rind its explanation in the frequent presence of
a Meckelian diverticulum here and in the traction produced by it
(J. B. Sutton, Amcr. Joarn. Mcd. Sc, n.s., xcviii. 457, 1889). Probably
most of the cases reported as instances of congenital occlusion of the
small intestine affect the ileum ; many references to these are to be
found in the Index Catalogue (vol. vii. pp. 19, 61, 1886; n.s., vol. viii.
pp. 207, 277, 1903). The seat of obstruction is frequently the place
where the small intestine becomes continuous with the large. Mul-
tiple constrictions have also been noted (C. Thorel, Milnchen. mcd.
Wchnschr., xlvi. 1202, 1899; F. Gaertner, Jahrl.f. Kindcrh.,\\.Y., xx.
403, 1883 ; etc.).

Obliteration of the large intestine above the sigmoid flexure is
not common ; below that level it is much less rare, and generally
takes the form of atresia ani or of imperforate rectum, anomalies
which are dealt with along with the malformations of the pelvic
organs (p. 563). In some cases of atresia of the small intestine, the
colon may be filiform or absent altogether ; but even when the upper
part of the bowel is normal, the lower part may be absent (Charon
et Vervaeck, Ann. Soc. beige dc chir., viii. 71, 1900), or greatly con-
tracted (E. Kirmisson, Traite des maladies chirurgicales, 415, 1898).

Various opinions have been expressed regarding the mode of
production of intestinal atresia in the foetus. At first sight it seems
probable that the cause may be fcetal peritonitis, and that, in this
anomaly at least, the pathological or nosological theory of terato-
genesis holds good ; but there are difficulties in the way, for, in order
to explain some of the reported cases, it must be supposed that the
peritonitis is developed at a very early date in antenatal life, at a
time in fact when it is not easy to understand how an inflammation
can exist. G. Durante and L. Siron (Bull, et mem. Soc. obst. et gynec.
dc Paris, p. 51, 1897) incline towards the view of arrested develop-
ment, and think that failure of the artery which ought to supply the
affected part of the intestine is the cause of the absence or contracted
state of that part.

Miscellaneous Gastric and Intestinal Anomalies.

In addition to anomalies in the position of the stomach such as
occur in connection with diaphragmatic hernia and transposition of
the viscera, that organ may show hour-glass constriction or contraction
of an antenatal kind (J. H. Musser, Phila. Med. Times, xiv. 331,
1883-84). It has been supposed that this may indicate an atavistic
tendency or return to the condition of the stomach met with in some
of the lower animals ; but it may be accounted for in the same way

534 ANTENATAL PATHOLOGY AND HYGIENE

as is intestinal constriction. Dwight (Amcr. Joum. Med. Sc., n.s.,
exxvi. 581, 1903) lias recently met with a well-marked case; If.
Sievers saw another instance in 1899 (Berl. klin. Wchnschr., xxxvi.
325, 1899). Various anomaly's in the position of the intestines have
been recorded. Guyot (Bull. Soc. d'anat. ct physiol. de Bordeaux, xix.
82, 1898), for instance, found the caecum in the right hypochondrium
attached to the lower surface of the liver by a peritoneal fold ; the
ascending colon was absent. I also have found the caecum displaced ;
it was nearly mesial in one case, and in another was higher up than
normal. In fact this part of the intestine and the appendix vermi-
formis attached to it would seem to be frequently displaced (E. B.
Young, Joum. Aneit. and Physiol., xix. 98, 1885 ; J. L. Faure, Bull. Soc.
anat. de Paris, lxx. 9, 1895 ; Gentes, Bull. Soc. d'anat. et physiol. dc
Bordeaux, xx. 365, 1900; H. A. Sifton, Clin. Rev., Chicago, xvi. 106,
1902 ; etc.). All these malpositions doubtless represent stages in the
evolution of the intestine which are normally transitory, but may
be rendered permanent by some cause (foetal peritonitis (?), arrested
development).

Since the appendix vermiforniis lias come to be studied and
operated upon, its anomalies have also become better known. It may
be entirely absent (e.g. in G. Piquand's case, Bull, et mem. Soc. anat.
de Paris, lxxv. 602, 1900, and in J. M. Swan's case, Univ. Med. Mag.,
viii. 194, 1895-96) ; or it may be unusually large (E. Fawcett, Joum.
Anat. eind Physiol., xxix. 498, 1894-95) ; or it may open into the small
intestine (Thoyer, Bull. Soc. anat. de Paris, lxviii. 205, 1893). In
Altuchoffs case (Anat. Anz., xxii. 206, 1902) the appendix passed
upwards for a distance of 15 cm. behind the caecum; then it turned
to the left and downwards for a distance of 10 cm., lying in the
mesentery of the small intestine ; and finally it ended in a bundle of
fibrous tissue 4 cm. long.

Into the subject of congenital inguinal and femoral hernia I do
not consider it my duty to enter in this work. These anomalies are
fully described in every system of surgery, and their bibliography
may be found in the Index Catalogue (vol. vi. 152, 1885 ; 2 s., vol. vii.
13, 1902). The same remark applies to congenital hydrocele (Index
Catalogue, vol. vi. 548, 1885 ; 2 s., vii. 506, 1902); but I may mention
that I have met with a case of congenital inguinal hernia in an infant
whose father suffered from congenital hydrocele. The mode of pro-
duction of both these anomalies in antenatal life needs no elucidation.
For a good description of them both the reader is referred to E.
Kirniisson's Trevitd des maladies chirurgicales eVorigine conge'nitalc, pp.
342-375, 1898.

Anomalies of Liver and Gall Bladder.

Malformations of the liver have been already alluded to under the
headings of hernia of the diaphragm and of the umbilical cord; but
this organ may show anomalies apart from these states. Accessory
livers (hepatet succenturiata) have been described by W. Gruber
(Arch f. path. Anat., lxxxii. 476, 1880), Simpson (Month. Joum. Med.,

ANOMALIES OF CALL BLADDER 535

Loud., xx. 179, 1855), and Van Buren {New York Med. Times, hi.
120, 1853-54); and accessory lobes or an anomalous arrangement of
lobes are not very uncommon (E. Laget, Bull. Soc. anat. de Paris,
xlix. 42, 1874; Micheleau, Journ. de m6d. de Bordeaux, xxvi. 228,
1896; and N. Moore, Trans. Path. Soc. Lond.,xxxm. 192, 1881-82;
Planteau, Journ. de m4d. de Bordeaux, xiv. 347, 1884-85). In complete
transposition of the viscera, the liver, like the other abdominal organs,
exhibits the characteristic inversion ; and the parts on the under
surface of it may show this inversion as a separate anomaly. The
Spigelian and quadrate lobes may be absent (A. B. Simpson, Edinb.
Med '. and Surg. Journ., vi. 1045, 1860-61). Cystic disease of the liver
has been met with in the foetus (J. C. B. Smallman, Lancet, ii. for
1859,573; Bagot, Bub/in Journ. Med. Sc., xciii. 265, 1892); and cases
of malignant disease, both carcinomatous (E. Noeggerath, Deutsche
Klinik, vi. 496, 1854) and sarcomatous (B. W. Barker, Trans. Path.
Soc. Loud., xxxi. 290, 1879-80), have been reported.

Absence of the gall bladder has been observed in a fairly large
number of cases. Becent instances have been noted by A. A. Eshner
{Med. News, lxiv. 548, 1894), by K. Time (Festskr. . . . Prof. Lfeibergs,
Kristiania, p. 137, 1895), by O. Giese {Diss, inaug., Leipzig, 1896),
and by A. Latham {Proc. Anal. Sue. Gh\ Bed. and Ireland, p. xxxix.,
1898) ; references to the older cases of W. N. Baker, G. H. Bergmann,
H. J. Cholmely, Cuynat, Gaultier de Claubry, Godclier, F. Godfrey,
J. J. Huber, J. Lynch, Bambault and Schachmann, M. Both, H. B.
Sands, A. B. Simpson, G. Targioni, Tozzetti, E. Thomas, D. M. Trimble,
J. H. Vergue and L. O. J. Leignel, Wiedemann, and B. AVolfartus are
given in the Index Catalogue (vol. v. p. 249, 1884). Eshner's case
was that of a rachitic child who died of broncho-pneumonia at the
age of two years ; he never had jaundice and yet there was absence
of the gall bladder ; the liver was otherwise normal, and there was a
supernumerary spleen. In B. Midler's specimen {Diss, inaug., Kiel,
1893), however, the defect of the gall bladder was combined with
atresia of the duodenum, atresia of the left ureter, a cystic condition
of the kidneys, and anomalies of the outer and middle ear and
palate ; and in Gevaert's {Arch, de toeol., xxi. 588, 1894) there was
atresia ani and absence of the right thumb as well as no gall bladder.
Complete absence of the gall bladder is the normal condition in some
animals, and so this arrested development in man has been regarded
as atavistic ; but it may arise apparently from early obliteration in
conjunction with obliteration of the bile ducts. Of this latter state
J. Thomson has reported an interesting case {Trans. Edinb. Obstct.
Soc, xvii. 191, 1892), in which the gall bladder was very small, its
posterior end and part of the cystic duct being entirely obliterated ;
the infant, a fifteenth child, only survived birth a few hours, and
showed other structural defects (hare-lip, cleft palate, and anomalies
of outer ears, of both hands, and of the heart). J. Thomson has also
given a very good account of congenital old deration of the bile ducts
{Trans. Edinb. Obstct. Soc, xvii. 17, 1891-92), with literature list and
tabular analysis of cases. I have referred to the subject in Vol. I.
of this Manual (p. 363), for the anomaly has been ascribed to fcetal

536 ANTENATAL PATHOLOGY AND HYGIENE

disease {e.g. peritonitis); but as it has also been thought to be due
to an arrested development (a view favoured by Thomson, he. eit.,
p. 45) it requires to be mentioned here. It is one of the morbid
states lying on the border-line between foetal pathology and embry-
onic pathology.

Other anomalies of the liver, gall bladder, and associated ducts
have been reported. Griffon {Bull. Sac. anat. de Paris, lxix. 777,
1804), for instance, found the bladder situated in a depression on the
antero-superior surface of the liver; there was a small accessory
right lobe. A. E, Simpson (Trans. Edirib. Obstet, Soc, xx. 13, 1895)
noted extensive fusion of the right and left halves of a foetal liver
below the ductus venosus. E. Cruveilhier (Ball. Soc, anat. de Paris,
xxxv. 66, 1860) saw a double gall bladder, with a single neck, giving
origin to two cystic ducts. Bran (Bull, ct mini. Soc. de chir. de Paris,
n.s., xxiii. 207, 1897) has reported dilatation of the bile ducts,
probably antenatal in origin.

Miscellaneous Malformations of Abdominal Viscera.

The spleen may sometimes be absent, as in C. Taruffi's specimen
(Mem. d. r. Accad, d, sc. d. 1st. di Bologna, 5 s., iv. 73, 1894), in which
there was also absence of the pancreas, suprarenal capsules, genitals,
prostate, colon, and rectum. Other instances have been reported by
F. D. Hirschfeld (Arch, d. Hcilk., xii. 190, 1871), Jelenski (Berl. Mm.
Wchnschr., xvii. 704, 1880), G. Martin (Bull. Soc. anat. de Paris, 2 ed.,
i. 40, 1841), and H. L. F. Robert (Arch. f. Anat. Physiol, u. wissensch.
Med., p. 57, 1842). Supernumerary or accessory spleens (" splenunculi "
or " lienculi ") are not very rare ; I figured one (in an otherwise
normal infant) some years ago (Introduction to the Diseases of Infancy,
PL vi., Fig. 2, p. 79, 1891); P. Claisee (Bull, Soc. anat. de Paris, lxix.
829, 1894) has reported an instance of two spleens and numerous
supernumerary spleens in a child (nine years old) suffering from
congenital heart disease ; and other cases have been noted by L.
Calori (Sulla duplicitd congenita delta milza, Bologna, 1863), W. H.
Bainbrigge (London Med, Gaz., xxxviii. 1052, 1846), W. (Jheselden
(Philos. Trans. (1700-1720), 2 ed., iv.-v. 252, 1831), J. J. Harderus
(Misccl, Acad, na.t, curios., Dec. 3, ix.-x. 127, 1706), Jolly (Bull, Soe.
ana/, de Paris, 5 s., ix. 745, 1895), and L. Eocher (Journ. de meil. de
Bordeaux, xxxiii. 833, 1903). Transposition of the spleen is seen in
general inversion of the viscera.

Anomalies of the suprarenal bodies have been noted. Their absence
or rudimentary state in cases of anencephaly has been already referred
to (p. 346) ; but they may be defective in other types of monstrosity,
as in Taruffi's case of agenosoma (Mem. d. r. Accad. d. sc. d. 1st. di
Bologna, 5 s., iv. 73, 1894), and they may be congenitally absent where
no malformations exist but where the skin is bronzed (J. K. Spender,
Brit, Med. Journ., p. 768, 1858). In cases of Addison's disease where
one of the suprarenals is absent (J. W. Legg, St. Barth. Hasp. Hep., x.
225, 1874), it does not, of course, follow that the defect was con-
genital. H. D. Eolleston (Brit, Med. Journ., i. for 1895, p. 629) has

HYPERNEPHROMATA 537

referred to various anomalies of the adrenals, including the presence
of accessory ones. These accessory suprarenal bodies have considerable
pathological importance : they may be found in the neighbourhood of
the normal organs and under the capsule of the kidney (G-. N. Pitt,
Trans. Path. Soc. Bond., xiv. 141, 1894), or in the semilunar ganglion
(Jaboulay, Lyon med., lxv. 300, 1890), or in the broad ligament (Mar-
chand, Arch. f. path. Anat., xcii. 11, 1883), or in connection with the
vas deferens (F. Friedland, Pray. med. Wchnschr., xx. 145, 1895), or
(most frequently) in the kidney. In these positions the accessory
suprarenals may take on malignant characters and produce tumours,
carcinomatous, adenomatous, or lipomatous in their nature. Iienal
tumours developed from these adrenal " rests " or accessory glands
are called hypernephromata ; they vary from a cherry to a child's
head in size ; and form very interesting evidence in support of the
teratological theory of the origin of tumours. A very considerable
literature has grown up round hypernephromata, and among many
references (the list of which may be found in the Index Catalogue,
2 s., viii. 714, 1903) I may specially single out those of Cullen (Johns
Hopkins Hosp. Bull., vi. 37, 1895), F. Harbitz (Norsk Mag.f. Lcegevi-
densk, 4 R, xiv. 521, 1899), A. 0. J. Kelly (Phila. Med. Journ., July
30, 1898), O. Lubarsch (Arch. f. path. Anat., exxxv. 149, 1894),
E. J. M'Weeney (Brit. Med. Journ,, i. for 1896, p. 323), B. H. Buxton
(Journ. Path, and Bacteriol, vii. 221, 1900-01), A. Jenckel (Deutsche
Ztschr.f. Chir., lx. 500, 1901), T. K Kelynack (Chir. Journ., xx. 335,
1902), and A. Pepere (Monitorc zool. ital, xiv. 261, 1903).

Anomalies of the pancreas are comparatively rare. Accessory or
succenturiate pancreas (Nebenpankreas of the Germans) has been
described by E. Montgomery (Trans. Path. Soc. Bond., xii. 130,
1860-61), C. Apollonis (Gazz. d, osp., viii. 196, 1887), and Beitmann
(Anat. Anz., xxiii. 155, 1903). In a case reported by J. Symington
(Journ, Anat. and, Physiol., xix. 292, 1885) two processes of pancreas
passed round the upper part of the descending portion of the
duodenum, and, with the aid of the head of the gland, formed a
complete ring round the intestine. In some other instances the
pancreas has established relations with the gastric and intestinal
walls (C. Gegenbauer, Arch. f. Anat, Physiol, u. wissensch. Med., 163,
1863; E. Wagner, Arch, d, Heilk, hi. 283, 1862; A. Weichselbaum,
Bcr. d. k. k. Krankenanst. Bud olph-Stif tuny in Wicn, 379, 1884; F.
A. Zenker, Arch. f. path. Anat., xxi. 369, 1861). G. Sangalli (Gazz.
med, lomb., lvi. 31, 1897) has met with a double pancreas, one part
lying in front of the other, and with an accessory lobe arising from
the upper border of the gland. Anomalies of the pancreatic duct also
exist : in a case reported by Bodinier (Bull. Soc. anat. de Paris, xviii.
262, 1843) the duct opened into the duodenum 4 cm. above the bile
duct ; and other malformations were described by M. See (Compt.
rend, Soc. de biol., 2 s., iv. 1, 1858).

Malformations of the kidneys and bladder fall to be described in
the next chapter, for although neither of these organs is pelvic in
position in the foetus it is convenient to discuss their anomalies with
those of the pelvis. I may note here that vascular anomedies in the

538 ANTENATAL PATHOLOGY AND HYGIENE

abdomen are met with, but have been little investigated. M. Cavasse
{Bull. Soc. anat. de Paris, 811, 1897) has met with a case in which
the vena cava inferior terminated below the renal veins in a cone,
and had no communication with the right femoral vein except
through an enlarged spermatic vein; there had been an arrested
development of the right posterior cardinal vein. Lauber {Anat.
Anz., xix. 590, 1901) has seen a specimen in which the two cardinal
veins persisted to the level of the kidneys, where there was a cross
union ; the inferior vena cava came from the right renal vein ; and
the larger trunk passed upwards, taking the course of the vena
azygos major. Other anomalies of the vena cava inferior have been
reported by J. Kallmann (Anat. Anz., viii. 75, 97, 1892-93), by
Lagneau (Bull. Soc. anat, de Paris, xxviii. 344, 1853), and by H. J.
Waring (Journ. Anat, and Physiol., xxviii. 46, 1893-94).

Congenital Umbilical Tumours.

A few words are not out of place regarding antenatal growths
originating in the umbilicus. The so-called granulomata which
form immediately after the fall of the cord do not belong to this
group, although some writers would admit them. No case of
umbilical cancer in a new-born infant has been reported ; but cases
of mevus maternus of the umbilical region may in later years become
malignant ; it is also believed that cancer may develop in a persistent
Meckelian diverticulum attached to the navel. Dermoid cysts may
be found at the umbilicus ; so may cysts developed from Meckel's
diverticulum and having a structure closely resembling that of the
wall of the intestine. These latter growths have been called vitelline
or diverticular cysts, and are closely related to the umbilical adeno-
mata or vitelline tumours (called in a loose fashion entero-teratomata).
E. Kirmisson (Maladies chirurgicales d'originc congdnitale, p. 214,
1898) gives a good description of them, while L. Pernice (Pie
Nobel <j c schw idstc , Halle a. S., 1892) has a valuable monograph (with
bibliography) dealing with them and with all other forms of umbilical
neoplasm. Parasitic foetuses attached to the umbilicus or lying in
the abdominal cavity belong to the polysomatous and not to the
monosomatous terata.

CHAPTER XXVIII

Merosouiatoua Terata (cont.) : Malformations of the Pelvic Organs : Extro-
version of the Bladder ; Definition, Synonyms, Historical Notes, Clinical
Features, Morbid Anatomy (in the Male and in the Female), Cloacal
Variety, Teratogenesis, Literature ; Urinary Umbilical Fistuhe ; Miscel-
laneous Malformations of the Bladder (Double Bladder, Diverticula) ;
Malformations of the Kidneys (Absence, Single Kidney, Displacement,
Lobulation, Accessory Kidney, Cystic Kidney) ; Malformations of the
Ureters ; Malformations of the Penis (Absence, Duplication or Diphallus,
Double Urethra, Penile Canals, Atresia of Urethra, Epispadias, Hypos-
padias) ; Malformations of the Testicles ; Malformations of the Female
Genitals ; Malformations of the Anus and Rectum ; Hermaphroditism.

The regional method of dealing with the subject of Teratology is less
satisfactory in its relation to the malformations of the pelvis and its
viscera than to those of any other part of the body. The bladder and
some of the genital organs are abdominal in position in foetal life
although they are pelvic in the adult, and the question therefore
arises whether their anomalies are to be described among the
abdominal or among the pelvic terata. In the scheme of classifi-
cation on p. 235 I got over the difficulty by grouping the pelvic and
abdominal anomalies together; but for the purposes of chapter-
division it is necessary to separate them, and, for convenience, I
place the malformations of the bladder and the associated organs
(the kidneys and ureters) and of the genitals with the pelvic malfor-
mations (lecano-terata). With this word of explanation I proceed
with their description, taking as the type-malformation extroversion
or ectopia vesicae.

Extroversion of the Bladder.

When the anterior abdominal wall in the hypogastric region and
the anterior bladder wall are both defective, the anomaly known as
extroversion of the bladder is produced. It has also been called
exstrophy of the bladder, but the proper spelling according to the
analogy of the Greek derivatives would be ecstrophy (Murray's New
Engl. Did,, iii. E. p. 453). Other synonyms are ectopia vesica?,
inversion of the bladder, prolapse of the inverted bladder, and
ectropion vesica1 ; but inversion of the bladder has come to be applied
to the acquired condition in which (in females) the bladder is
prolapsed through the urethra, and the other names have no advan-
tages over extroversion. C. Taruffi (Storia della teratologia, vii. 406,

639

540 ANTENATAL PATHOLOGY AND HYGIENE

433, 1804), in order to emphasise the fact that there is fissure of the
abdominal wall in the hypogastric region and separation of the pubes
(to rjrpov), has suggested hypogastro-etro-schisis, a perfectly unmanageable
vocable, which, after all, does not draw the attention to the defect
in the bladder. The German expression, Bauchblasenschainbeinspalte,
cannot be rendered by any suitable English equivalent. For these
reasons I retain the term " extroversion of the bladder."

The historical record* of vesical extroversion go far back. Perhaps
the first known case is that found on the Chaldean tablets (Teratologic!,,
i. 132, 1894), in which a child is described as wanting the penis and
the umbilicus. At any rate, an authentic instance of congenital
aperture of the abdomen and bladder was reported by Ambrosinus
(editor of Aldrovandus' Historia Monstrorum, p. 507, 1641).
Several cases were made public in the seventeenth century, in-
cluding that of Cornelius Stalpart vander Wiel (Zeldzame Aanmer-
kingen, p. 307, 1686) under the title of Ecu Kint sonder Navel
gcboren, and that of C. L. Gockelius (Miscel. Acad. nat. curio*.,
Dec. ii. Ann. v., obs. 43, p. 84, 1687), graphically described as
" vesica spongiosa extra abdomen posita cum defectu penis." In the
eighteenth century the nature of the malformation came to be better
understood, for it was recognised in 1767 by Devilleneuve (cited by
Taruffi, op. cif., vii. 432) that the tumour, seen projecting from the
abdomen above the symphysis pubis, was not a simple hernia of the
bladder but was the posterior vesical wall itself, the bladder as well
as the abdominal walls being defective anteriorly. James Mowat,
writing in 1735 (Med. Essays and Obscrv. Edinb., iii. 276), was
evidently quite at a loss to explain the appearances in a typical case
of extroversion of the bladder, and was careful to entitle his article
" An Account of a Child born with the Urinary and Genital Organs
preternaturally formed." In the nineteenth century many details of
the morbid anatomy of ectopia vesica? were carefully investigated,
and considerable progress was made with the elucidation of its
teratogenesis. A great literature has grown up round the subject ;
how great, the reader has only to consult the Index Catalogue of
Washington to realise (vol. ii. 81, 1881 ; vol. ix. 408, 1888; vol. xv.
128, 1894; 2 s. vol. ii. 371, 1097, etc.).

The clinical history of cases of extroversion of the bladder begins
as a rule with birth. There is, in most cases, nothing during the
pregnancy to cause the mother or her medical attendant to suspect
any anomaly in the infant. After the birth of the child, however,
the mother may recall a maternal impression, a circumstance which,
as we have seen, is by no means peculiar to this special type of
monstrosity. In a case, the details of which were communicated to
me by Dr. W. Iverach Eobertson in 1891, the maternal impression
was the hearing of the birth of an infant about whose sex there was
some doubt (absence of penis), and the woman gave birth to a child
with ectopia vesicae and epispadias seven months later. In Mowat's
case (loc. cit.) the mother, when about two or three months pregnant,
was struck in the abdomen with a cow's horn, but was apparently
more frightened than hurt.

EXTROVERSION OF THE BLADDER

»41

Postnatal life is not gravely interfered with in extroversion of
the bladder, save in the cases in which the intestine opens into the
malformed bladder. In the uncomplicated cases adult life has often
been reached, although the individual suffers great inconvenience
from the constant dribbling <>f urine and from catarrhal inflammation
of the bladder. Possibly on account of the continuance of postnatal
life, possibly also because the anomaly is not very rare, many cases
have been reported, as the extensive bibliographies in the Index
Catalogue abundantly testify. As it so happens I have only had an
opportunity of examining cases in which gastroschisis of an advanced

^tr6r

-#"*<

Fig. 78. — Dr. Michin's Case of Ectopia Aresicse in a Female Infant.

degree was also present, and in which, therefore, the ectopia vesica?
was not the most obvious feature. I reproduce, however, as a
typical instance of the deformity, a photograph which I received
from Dr. Michin of Charkow in 1897 (Fig. 78). The child was born
spontaneously after a thirteen hours labour; it was a female and
showed pelvis fissa and prolapsus uteri incompleta, as well as ectopia
vesica" ; on the twelfth day after birth she died from haemorrhage
from the bladder {Trans. Edinb. Obstel Soc, xxii. 108, 1897). The
frequency of extroversion of the bladder has been placed at 1 in

542 ANTENATAL PATHOLOGY AND HYGIENE

50,000 births ; it is much commoner in males than in females, in the
proportion of six or seven to one.

The distressing circumstances of a patient with vesical ectopia
(constant dribbling of urine, ulceration and excoriation of neighbour-
ing parts, risk of ascending pyelo-nephritis, urinous and ammoniacal
odour, associated genital malformations) have been the cause of the
many attempts, several of them most ingenious, that have been made
to correct the deformity by surgical interference. In the first place,
there have been the methods in which flaps of skin from adjacent
parts have been used to close in the abdomen (plans of Koux,
Thiersch, Wood, Le Fort, and others) ; then there have been the
various devices by which it has been endeavoured to turn the stream
of urine into the lower part of the intestine (Simon, Smith, Lloyd,
Holmes, Turner), or into the urethra itself (Sonnenburg, Zesas,
Segond) ; and in the third place, there have been attempts to close
the bladder itself by direct sutures and to approximate the two halves
of the symphysis pubis (Gerdy, Eigaud, Wyman, Trendelenburg,
Pousson). The difficulties in the way of complete success by any of
these methods are formidable. To use flaps of adjacent skin produces
a urinary reservoir which is not lined by vesical mucous membrane,
and in which concretions are almost certain to form. To turn the
ureters into the rectum or sigmoid flexure has given good results in
some cases, although the lower bowel is not fitted physiologically
to act as a bladder or cloaca. Direct suture of the vesical walls is
the most scientific and the most hopeful procedure, if the difficulty of
the reproduction of the sphincter can be got over. There is still
work for the courageous and resourceful surgeon in this department
of reparative intervention ; and there is pressing need, for the only
alternative to operation is the wearing of a urinal. Some doubtful
cases of antenatal cure or partial cure of this malformation have been
recorded (E. Kuster, Verhandl. d. Berl. vied. Gesellsch., viii. pt. i. 4,
1877); and spontaneous cure may apparently take place also after
birth (L. Lichtheim, Arch. f. Uin. CMr., xv. 471, 1873). Before
leaving the clinical aspects of extroversion of the bladder I may add
that some instances of pregnancy and labour have been recorded
(C. Taruffi, op. cit., vii. 462, 1894), in two or three of which the birth
of the child was rendered long and difficult.

The morbid anatomy of extroversion of the bladder differs con-
siderably according as the patient is a male or a female ; and I shall,
therefore, follow Taruffi's plan (op. cit., vii. 435) and describe it first
in the male subject and second in the female. Thereafter I shall
consider the associated malformations and shall pay special attention
to those cases in which the intestine opens into the bladder (cloaca/
extroversion).

In the male subject the most striking feature is the projection of
a red vascular mass, somewhat rounded in size and elastic in con-
sistence, in the hypogastric region, just above the penis (malformed
or normal). In the lower part of the mass are two openings (lower
ends of ureters) from which urine dribbles away and keeps the whole
surface moist; the openings are situated, one on each side of the

EXTROVERSION OF THE BLADDER 543

middle line, at an equal distance from it. The surface of the mass is
really the mucous membrane of the posterior wall of the bladder, and
by pushing it inwards with the ringer a bladder cavity can be to some
extent produced ; here and there the vesical mucosa may take on
cutaneous characters, and elsewhere it may show granulations and
bleed on handling. The ureteric openings may lie in depressions
between the folds of the mucosa or may form little projections or
even tubular prolongations. Sometimes the ureters lie close together
and have a single opening; sometimes one ureter (with its corre-
sponding kidney) is absent, the other opening by itself at one side ;
at other times both the kidneys and their ureters are absent ; and at
others, they open into the rectum, a circumstance which suggested
one of the methods of surgical interference to which I have referred.
Behind the bladder the ureters may show cyst-like dilatations.

The umbilical cord, in the form of a cord, may be absent, and then
the umbilical vessels may enter the abdomen separately or may run
to it in the membranes ; when this is the case no proper umbilical
scar is visible as the infant otows older. In other instances the
vessels (consisting sometimes of only one artery and a vein) are
collected together in a distinct funis which enters the abdomen above
the level of the vesical extroversion ; then the fall of the cord is
followed by the formation of a cicatrix either in the normal position
or a little lower down. Sometimes the cord is attached to the apex
of an umbilical hernia (P. Bockenheimer, Arch. f. Idin. Chir., lxix.
609, 1903).

There is nearly always want of union of the pubic bones at the
symphysis ; indeed there is usually a wide separation of them, bridged
over by a fibro-muscular structure, the so-called infrapubic ligament.
This separation of the pubic bones is normal in some animals, and it
may occur in the human subject cpiite apart from extroversion of the
bladder (Schauta, Centrlbl.f. Gynak., xxiii. 1047, 1899). There may
be other deformities of the pelvis as well as pubic diastasis : the
pelvic cavity, for instance, may be diminished in size by the upward
displacement of the sacrum and coccyx or by forward inclination of
the promontory of the sacrum due to what may be called sacral
lordosis or (less correctly) pelvic inversion.

A very commonly associated malformation is epispadias, the penis
being divided superiorly and the urethral canal laid open. The
corpora cavernosa are often defective, so that the penis appears as if
it consisted of little more than the glans with its prepuce. The
scrotum may have a single or a double cavity and may contain both
testicles ; but it is not uncommon for the latter organs to be intra-
abdominal in position or to be situated at some point in the inguinal
canals. The vasa deferentia may open directly into the urethra.
The genital organs may be still more rudimentary, the penis and
scrotum being either entirely absent or represented by tags of skin.
There may be two genital tubercles situated at some distance from
each other, as in the specimen of gastroschisis kindly given me by
Professor Stephenson of Aberdeen (Fig. 79).

In the instances of extroversion of the bladder that survive birth

544

ANTENATAL PATHOLOGY AND HYGIENE

the anus is usually normal ; but atresia ani has been several times
noted in those that die in the early days of life. In some cases the
intestine opens into the bladder, and about these something will be
said immediately. In yet other cases there is hernia of the umbilical
cord or in the inguinal and scrotal regions ; even perineal hernia has
been noted in association with extroversion of the bladder (Gr. Kriiger,
Diss, inaug., Rostock, 1872).

*

Fig. 79. — Case of Gastroschisis and Double Genital Tubercle (a). Specimen No. 200.

Iii the female subject, extroversion of the bladder is, as has been
said, much rarer than in the male. Possibly this difference is due to
some peculiarity in the embryology of the hypogastric region in the
female as compared with the male. A similar reason may perhaps
explain why it is that vesical extroversion in the female is often of
an incomplete kind. Well-marked cases present the same features

ECTOPIA VESICLE IN WOMEN 545

as in the male : there is the red projection in the hypogastric region
with the two ureteric openings, there is separation of the pubic bones,
and usually the anterior urethral wall is absent leaving the posterior
wall exposed (epispadias). Some features, however, are peculiar to
the female. The vagina has a transverse opening, is somewhat short,
and may show a degree of prolapse. The uterus may be bicornate.
The perineum usually is short and so the anus is situated further
forward than is normal. The rectum may open into the vagina
(vulvar anus). The clitoris may be absent or may be represented
by two widely separated tubercles lying one at the anterior end of
each labium minus. The labia majora do not usually unite in a
recognisable mons veneris, and may only be represented by skin
tags.

In the male, extroversion of the bladder and epispadias although
often associated can be easily separated from each other ; but in the
female these two malformations are often very difficult of differentia-
tion, for the urethra is short and its malformations are apt to be
insignificant. Further, there would appear to be intermediate cases
having some of the characters of vesical ectopia and some of epis-
padias. Such an intermediate type I recorded under the name of
epispadias in 1896 {Edinb. Hosp. Rep., iv. 249, 1896); but it might
with some show of reason have been called extroversion of the bladder
of a very slight degree, or " subsymphysial vesical exstrophy." In
these cases the symphysis pubis is usually closed, and in this character
they resemble epispadias ; on the other hand, a small part of the
anterior bladder wall in the symphysial region is absent (allowing
prolapse through it of the mucous membrane), and in this character
they approximate to vesical extroversion. In my article above
referred to, I have discussed at some length both female epispadias
and subsymphysial exstrophy, and have given a bibliography of cases ;
to it the reader who is interested in this obscure corner of teratology
may have recourse.

Finally, a few sentences may be devoted to the description of the
morbid anatomy of the cases of extroversion of the bladder which
have been called cloacal. These are the instances in which the intes-
tine ends in the open bladder ; and Taruffi {op. cit., vii. 464, 1894)
has gathered together from literature forty-one of them, to which may
be added P. Bockenheimer's specimen (Arch.f. klin. Chir., lxix. 669,
1903). In this last-named case the patient was a male infant, three
days old. In the middle line of the abdomen between the openings
of the ureters there was a proboscis-like projection which consisted
of the caecum and ileum, prolapsed and split ; below this was another
opening which was found, on post-mortem examination, to be the
end of a short large intestine which ended blindly in the pelvis.
This may be regarded as a typical case of the cloacal form of extro-
version in the male. From the intestinal openings meconium can be
seen protruding at and soon after birth, and the prolapsed bit of
bowel may simulate a penis. There is generally atresia ani in these
cases. In the female subject the uterus is usually double, and there
may also be duplicity of the vagina ; in such circumstances the vaginae
vol. ii.— 35

546 ANTENATAL PATHOLOGY AND HYGIENE

may open within the area of extroversion in the near neighbourhood
of the ureters. Unusual cloacal combinations have been noted, as
when one vagina opens on the surface and the other ends blindly, or
when one vagina opens into a ureter, or when both vaginas end blindly.
In the male, the vasa deferentia have been found opening into the
extroverted bladder ; and in a very remarkable case (P. Gast, Diss,
inaug., Greifswald, 1884) the vagina on the left side and a testicle on
the right side were connected with the open bladder. Sometimes a
peritoneal fold (recto-vesical ligament) has been found passing from
the posterior wall of an extroverted bladder between the two halves
of the double uterus to be inserted on the rectal walls. In these
complicated cloacal forms it is common to find malformations of the
intestine within the abdomen, such as absence of the appendix ver-
miformis, displacement of the small intestine, etc.

As will have been gathered from the preceding description of
vesical extroversion, associated malformations are not uncommon.
Among these may be mentioned spina bifida, especially of the sacral
region (0. Grundies, Diss, inaug., Berlin, 1883), spinal scoliosis, um-
bilical hernia, club-foot (Hamaide, Bull. Soc. de mid. prat, de Paris, p.
310, 1891), hare-lip, non-descent of the testicles (A. Broca, Bull. Soc.
anal de Paris, lxii. 791, 1887).

The teratogencsis of extroversion of the bladder is a subject of
great complexity. The older writers looked, as usual, for a mechanical
or pathological (nosological) cause. Some found it in overdistension
of the foetal bladder with urine ; the result was bursting of the
urethra (epispadias), of the bladder (extroversion), and of the tissues
of the symphysis pubis (pubic diastasis) ; the cause of the overdis-
tension of the bladder was found in blocking of the urethra or in a
dropsical tendency. Others looked for a determining cause in external
violence or injury suffered by the pregnant woman or in mental im-
pressions. Shortness of the cord or of the vessels entering into it
was another alleged cause of extroversion of the bladder, and brevity
of the vitelline duct was added to it to explain the associated in-
testinal malformations. Traction by the vitelline duct has been a
favoured theory, Ahlfeld especially pressing it strongly (Die Miss-
oildungen des Mcnschen, ii. 206, 1882). The duct, as is well known,
arises from the intestine in the region of the ileum ; and it wras
thought, therefore, that in this fact lay the explanation of the cloacal
forms in which it was the ileum that opened into the extroversion.
At the same time it must be admitted that this theory does not
explain all the facts, and it was found necessary to bring forward
blocking of the urethra and overdistension of the allantois to account
for the rupture of the anterior bladder wall. At the present day the
theory of mechanical origin may be said to be merged in that of
disordered embryology (to which I shall refer immediately).

The idea of disease as a cause of extroversion of the bladder was
once popular, and erosions and ulceration of the anterior abdominal
and vesical walls were regarded as sufficient causes ; but with an
increase in our knowledge of the principles of antenatal pathology
the nosological theory has ceased to have a prominent place in

TERATOGENESIS OF ECTOPIA VESICAE 547

teratogenesis. It is no longer necessary to have recourse to such
morbid causes to explain the open state of the abdomen and bladder,
for arrested ontogenesis is sufficient; foetal peritonitis need not be
invoked to account for the opening of the bowel into the urinary
bladder, since the persistence of the entodermal cloaca is a sufficient
explanation.

The only reason why the embryological theory of the production
of extroversion of the bladder has been incomplete lies in our im-
perfect knowledge of all the details of the ontogenesis of the bladder
and surrounding parts. The early writers of the nineteenth century,
including Meckel, although they believed in arrested development as
the great teratogenic cause, were hampered in their efforts to explain
all the features of the vesical malformation by defective information,
or by erroneous preconceptions regarding the development of the
bladder. A great difficulty seemed to exist in the fact that the
bladder cavity was open, for it was not known that there was any
stage in ontogenesis when this organ was in this state, and there was
no evidence that it was derived from two symmetrical halves, for it
was believed to be derived from the allantois, which is a single and
not a double organ. F. Tourneux (Journ. de I'anat. ct physio!., xxiv.
503, 1888; xxv. 229, 1889) and Durand (L'exstrophie vdsicale ct
l' epispadias, Paris, 1894) have, however, thrown some light upon the
subject by their theory of the formation of an opening in the uro-
genital sinus by the breaking down of a plug of epithelial cells (the
so-called " bouchon cloacal") ; and Keibel (Anat. Anz., vi. 186, 1891)
and Berry Hart {Trans. Edirib. Obstct. Soc, xxvi. 308, 1900-01) have
still further elucidated the matter by showing that the bladder is
really a product of the entodermal cloaca, and not (save in a small
part) the result of persistence of the allantois. The development of the
bladder from primitive intestine and from the part of it known as
entodermal cloaca does away with many of the difficulties which
stood in the way of an embryological explanation of vesical extro-
version and epispadias. The entodermal cloaca is closed in front by
the cloacal membrane, which at one time has no mesoblast in its
composition ; this cloacal membrane is believed to be derived from
the margins of the blastopore or neurenteric canal, which, although
primarily dorsal in position, becomes ventral by the changes in
direction of growth of the caudal end of the embryo ; and the
imperfect formation of the cloacal membrane offers a satisfactory
explanation of the various forms of vesical exstrophy and epispadias.
Arrest at an early stage accounts for the cloacal forms of exstrophy ;
at a later stage simple extroversion of the bladder will result ; and
at a still later the minor degrees of exstrophy and the various types
of epispadias will be produced. There are still difficulties to be
explained away, but it is reasonable to hope that a further and more
minute acquaintance with the ontogenesis of the caudal end of the
embryo will clear them up also. In this way extroversion of the
bladder falls into line with so many other malformations as essentially
an arrested development.

Bibliography. — The literature of extroversion of the bladder is

548 ANTENATAL PATHOLOGY AND HYGIENE

very large ; the references given here are simply intended to open
up the subject and lead on to other sources of information.

(1) J. M. Adriani, Diss, inaug., Groningen, 1896. (2) F. Ahlfeld,
Arch. f. Gijnaek., xi. 85, 587, 1877. (3) E. M. G. Ahn, Diss, inaug.,
Leiden, 1892. (4) A. Alquie, Ann. din. de Montpel., iv. 17, 1856-57.
(5) J. Ashhurst, Amer. Journ. Med. Sc, n.s., lxii. 70, 1871, lxv. 135,
1873, lxvii. 421, 1874; Internal. Med. Mag., iii. 735, 1894-95. (6) I).
Ayres, Amer. Med. Gaz., x. 81, 1859. (7) M. Baillie, Trans. Soc
Improve. Med. and Chir. Knoui., i. 189, 1793. (8) A. Banon, Dublin
Quart. Journ. Med. Sc, xxxvii. 182, 1864. (9) E. Barker, Med. Chir.
Trans., liii. 187, 1870. (10) M. Bartels, Diss, inaug., Berlin, 1867 ;
Arch. f. Anat. Physiol, u. wissensch. Med., p. 165, 1868. (11) J. Berg,
Nord. med Ark., n. F., iii., Hft. 3, 1, 1893. (12) C. Beste, Diss, inaug.,
Freiburg i. B., 1889. (13) A. Bonn, Amsterdam, 1791. (14) Browne,
Dublin Quart. Journ. Med. Sc, xxxiii. 489, 1862. (15) E. Canton, Trans.
Path. Soc. Lond,, xiii. 163, 1862. (16) C. J. G. Calmeilles, These,
Bordeaux, 1887. (17) F. H. Champneys, St. Barth. Hosp. Rep., xiii. 81,
1877. (18) W. Conrads, Diss, inauq., Wiirzburg, 1881. (19) E. Cowan,
Glasgow Med. Journ., iii. 329, 1830. (20) L. Cros, These, Lyon, 1890.
(21) Depaul, Bull. Soc anat. de Paris, xxviii. 353, 1853. (22) Des-
granges, Journ. de med. chir. pharm., xci. 30, 149, 1792; cviii. 3, 1829.
(23) A. Duncan, Edinb. Med. and Surg. Journ., i. 43, 132, 1805. (24)
M. Durand, These, Lyon, 1894. (25) H. Duret, Journ. de sc. med. de
Lille, ii. 241, 265, 1891. (26) C Ellis, Boston Med. and Surg. Journ.,
lxii. 477, 1860. (27) B. J. Feinberg, Journ. akush. i jensk. boliez., viii.
967, 1894. (28) G. Friedlander, Monatschr. f. -Geburtsk. u. Frauenkr.,
vii. 243, 1856. (29) M. Ginsbtjrg, St. Petersb. Med. Ztschr., n.s., iii.
525, 1872. (30) C Grandjean, These, Strasbourg, 1868. (31) F. W.
Garvens, Diss, inaug., Halis Sax., 1841. (32) H. von Glan, Diss, inaug.,
Strasburg, 1879. (33) P. D. Handyside, Edinb. Med. and Surg. Journ.,
Iii. 422, 1839. (34) A. Herrgott, These, Nancy, 1874. (35) T. Holmes,
Lancet, i. for 1863, p. 714; Trans. Path. Soc Lond,, xv. 254, 1864, xvi.
169, 1865, xviii. 176, 1867. (36) Humphry, Journ. Anat. and Plug siol.,
iii. 81, 1869. (37) J. Innes, Med. and Phil. Comment., 2nd ed., ii. 437,
1784. (38) H. J. Isenplamm, Diss, inaug., Dorpat, 1806. (39) Jackson,
Amer. Journ. Med. Sc, n.s., xxv. 71, 1853 ; Boston Med. and Sure/. Journ.,
lxii. 124, 1860. (40) S. Jones, Med. Times and Gaz., ii. for 1872, p. 6.
(41) K. Katz, These, Paris, 1895. (42) Kocher, Corr. El. f. schweiz.
Aerzte, vii. 580, 1877. (43) Labbe, Bull. Soc de chir. de Paris, 3 s., i.
115, 1872. (44) G. Lecaze-Duthiers, These, Paris, 1890. (45) A.
Lachmann, Diss, inaug., Berlin, 1883. (46) A. L. Leatherman, Indiana
Med. Journ., xiii. 345, 1894-95. (47) H. Leboucq, Ann. Soc de m4d'.
de Gand, liv. 91, 1876. (48) Lloyd, Lancet, ii. for 1851, p. 370. (49)
G. H. Makins, Med. Chir. Trans., lxxi. 191, 1888. (50) P. Magnussen,
Diss, inaug., Berlin, 1886. (51) J. P. Martin, Diss, inaug., Mainz, 1878.
(52) H. C. Masland, Med, News, lxv. 489, 1894. (53) K. Maydl, Wien.
med. Wchnschr., xliv. 1113, 1169, 1209, 1256, 1297, 1894; xlvi. 1241,
1333, 1374, 1896. (54) A. Hermann, Arch. f. Gynaek,, xxvi. 143, 1885.
(55) J. Horgelin, Diss, inaug., Bern, 1855. (56) P. Heisner, Diss.
inaug., Greifswald, 1873. (57) P. Huller, Diss. inaug., Greifswald, 1883.
(58) L. A. Neugebauer, Pam. Towarz. Lek., Ixiv. No. 6, p. 5, 1870. (59)
Niven, Month. Journ, Med, Sc, xiv. 90, 1852. (60) A. Nota, Arch, di

URINARY UMBILICAL FISTULA 549

ortop., vi. 40, 1889. (61) F. Ogston, Journ. Anat. and Physiol., xvii. 86,
1882-83. (62) A. Paci, Comment, din. di Pisa, i. 545, 594, 1877. (63)
Partridge, Lancet, ii. for 1862, p. 114. (64) F. Paroxa, Arch, di ortop.,
v. 19, 1888. (65) W. J. H. Pilgram, Diss, inaug., Bonn, 1892. (66) J.
Phillips, Trans. Path. Soc. Lond., xix. 272, 1868. (67) C. B. Porter,
Journ. Amer. Med. Assoc, xiv. 529, 1890. (68) C. Rose, Trans. Obstet.
Soc. Lond., xv. 125, 1873. (69) J. Roux, Union med., vii. 449, 453,
1853. (70) A. W. M. Robson, Brit. Med. Journ., i. for 1885, p. 222.
(71) G. Ruggi, Bull. d. sc, med. di Bologna, 5 s., xiii. 335, 1872; Riv.
din. di Bologna, 2 s., iii. 43, 1873, vi. 76, 1876. (72) P. Schmitt, Diss,
inaug., Wiirzburg, 1836. (73) G. B. Schmidt, Beitr. z. Irfin. Chir., viii.
291, 1891-92. (74) J. Schoexexberger, Diss, inaug., Greifswald, 1882.
(75) S. G. Shattock, Trans. Path. Soc. Loncl, xxxviii. 170, 1886-87;
xlv. 117, 1893-94. (76) J. Smith, Brit. Med. Jotirn., i. for 1880, 202,
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xl. 612, 1892. (79) F. Trexdelenburg, Centrlbl. f. Chir., xii. 857, 1885;
Arch. f. Bin. Chir., xxxiv. 621, 1886-87, xliii. 394, 1892. (80) L.
Yiai.letox, Arch. prov. de chir., i. 233, 1892. (81) J. Wood, Trans.
Path. Soc, Lond., xv. 150, 1864, xxiv. 142, 1873 ; Brit. Med. Journ., i. for
1869, p. 174, i. for 1880, p. 278. (82) D. G. Zesas, Arch./. Uin. Chir.,
xxxvi. 753, 1887.

Urinary Umbilical Fistulae.

A somewhat rare malformation is that in which urine flows or is
expelled in a jet from the umbilicus. At the navel there is, in these
cases, a granuloma or fleshy tumour, or a groove from which the
fluid emerges ; this represents the umbilical end of a pervious urachus
communicating with the bladder cavity. The case is known as one
of congenital urinary fistula of the umbilicus, or of persistent urachus,
or of urachal fistula. One of the earliest known instances was that
described by J. Caulton {Gentleman s Mag. and Hist. Chron., xxii.
271, 1752) under the following graphic title: "Account of a negroe
boy, two months old, well and thriving, who made water in a full
stream from the navel as well as from the penis ; the navel stream
was cured by a strong solution of crude alum." Since 1752 many
cases have been recorded, so that Taruffi (op. cit., vii. 346, 1894) had
no difficulty in collecting eighteen of them. Some of these were truly
antenatal in origin, the anomaly being observed at or within a few
days after birth (at the fall of the cord) ; others were developed in
later life. The adult cases could sometimes be traced to an antenatal
cause ; there was evidence that a patent or easily dilated urachus
existed, and that, on account of an obstruction to the normal urethra
or an injury to the bladder, the new route was forced open, and so
urine began to pass from the umbilicus, although it had previously
been expelled by the urethra. It is not, however, with these adult
cases but with the antenatal ones that we have here to do.

Of truly congenital cases Gueniot (Bull. gin. de the'rap., lxxxiii.
299, 348, 1872) collected seven ; L. Levie (Nederl. Tijdschr. v. Geneesk,
2 R., xiv. 501, 1878) increased the number to twenty-one; Naury

550 ANTENATAL PATHOLOGY AND HYGIENE

(These, Paris, 1881) dealt with the treatment of the anomaly ; and
J. Castel (These, Paris, 1884) brought the number of observations up
to thirty-five. The full bibliography of the subject will be found in
the Index Catalogue (Washington) under the titles Fistula, Umbilical
(vol. iv. p. 1018, 1883 ; 2 s., vol. v. 837, 1900), and Urachus, Ab-
normities o/(vol. xv. 39, 1894). At birth, or when the cord falls off,
the infant suffering from this anomaly shows a fleshy growth at the
umbilicus from which urine trickles. The growth protrudes like a
hernia when the child cries or struggles ; but palpation does not
reveal the presence of intestine in the growth. The urethra may be
closed or narrow (Piose, Med. Bee., xii. 516, 1877; Charles, Brit. Med.
Journ., ii. for 1875, p. 486 ; A. Stadfeldt, Nord. Tried. Art, iii., No. 23,
1, 1871); but in many cases there is no obstruction, although, of
course, such may have existed in antenatal life. The anomaly is
more common in the male sex, possibly on account of the greater
length of the urethra and the greater chance of obstruction. The
essential fact in the teratogenesis of the defect is the persistence of
the urachus in an open or easily opened up state ; and the investi-
gations of L. Gruget (Thlsc, Paris, 1872) and of J. B. Wutz (Arch./.
path. Anctt., xcii. 387, 1883) show that this condition is occasionally
found. When the umbilical end of the urachus is closed or becomes
closed a cyst may develop in the abdominal wall, due to distension
of the cavity of the urachus ; this is called a urachal cyst. Many
important articles have appeared dealing with such cysts, among
which may be mentioned those of C. C. Wolff (Diss, inaug., Marburg,
1873), of Lawson Tait (Lancet, ii. for 1888, p. 675), of H. Schnellenbach
(Diss, inaug., Bonn, 1888), and of F. B. Eobinson (Ann. Surg., xiv.
.">.'!7, 1891). Both the fistulas and the cysts are capable of successful
surgical treatment (E. Kirmisson, Maladies chirurgicates, p. 224,
1898).

Miscellaneous Malformations of the Bladder.

Douhle or septate bladder is a rare anomaly. It has been found,
however, in connection with the malformation known as diphallus
or double penis (Ballantyne and Skirving, Teratologia, ii. 92, 184,
255, 1895). In G. Sangalli's case of diphallus in an adult (Gazz. mcd.
lomb., liii. 491, 1894) two bladders lay side by side and communicated
with each other by a canal, 1 cm. long ; and in M. Lange's infant
(Schmidt's Jahrb. gesammt. Med., ccxlv. 215, 1895) the bladder
showed no sign of division externally, but internally was divided into
two equal parts by a median perpendicular fold. Cases of double
bladder may, however, exist apart from duplicity of the penis
(Ehrlich, Journ. compl. du diet. cl. sc, ■me'd., xxxvi. 24, 1830 ; Huppert,
Arch. d. Reilk., vi. 382, 1865; A. a Lebenwaldt, Miscel. Acad. nat.
curios., Dec. ii., Ann. viii., p. 264, 1690 ; F. Ferguson, Broc. New York
Bath. Soe., p. 58, 1890). Probably most of the cases which have
been described as double bladder have really been of the nature of
diverticula of the bladder, and many instances of this latter malfor-
mation have been put on record in recent years. Among these I may

MALFORMATIONS OF THE KIDNEYS 551

mention the inaugural dissertations of C. Blumenthal (Gottingen,
1868) and L. Mitzkuner (Jena, 1878), and the articles of Bimar (Gaz.
held. d. sc. mdd. dc Montpel., viii. 445, 1886), J. Coats (Glasgow Med.
Journ., xix. 72, 1883), I. J. van Gieson (Proc. New York Path. Soc,
p. 171, 1888), Porter (Dublin Quart, Journ. Med. Sc., xxv. 504, 1844),
and F. Zachrisson (Upsala Lakaref. Forh,,n. F., i. 199, 1895-96). In
W. Midler's specimen, a female fcetus, there was a double bladder and
a uterus bicornis ; the two bladder cavities were superior and inferior
(Piss, inaug., Marburg, 1895) ; and in Pean's case, a girl of fifteen years,
there was an accessory bladder arising from the anterior wall of the
other one, from which urine escaped by a second median urethra
lying below the normal meatus urinarius (Pull. Acad, de med.,Y>. 542,
1895). A curious case was described by F. Beach (Trans. Path, Soc.
Lond,, xxv. 185, 1873—74) in which the bladder contained a pouch,
which opened into a dilated coiled tube (a third ureter), which com-
municated with the right kidney by a smooth-walled cavity.

Malformations of the Kidneys.

Since the progress of surgery has brought the kidney within the
sphere of the operator it has become increasingly important that he
understand the anomalies of this organ. This remark applies speci-
ally to what is known as single kidney, but it has also a bearing upon
all renal malformations.

Absence of both kidneys is incompatible with postnatal life of any
but the shortest kind, unless indeed we accept Moulon's case (cited
by H. Morris in his Surgical Diseases of the Kidney, p. 112, 1885) of a
young girl of fourteen years as a genuine instance ; x but it is not of
extreme rarity in antenatal life, and may interfere little with ante-
natal growth and development. In Mayer's specimen (Ztschr. f.
Physiol., ii. 36, 1827), a stillborn, full-time male infant, the kidneys,
ureters, urinary bladder, and renal arteries were all absent, although
the heart, lungs, liver, spleen, and part of the intestines were well
developed, and the suprarenal capsules were twice their normal size ;
the external genitals, sigmoid flexure, rectum and anus, and gall
bladder were also wanting. Absence of both kidneys is not rare in
sympodial monsters (q.v.) and in the malformed twin foetus (acardiac,
acephalic, paracephalic).

The single kidney appears to be a relatively frequent anomaly.
Several widely different states, however, are included under this one
name : these are — (1) entire absence of one kidney, or unsymmetrical
kidney ; (2) congenital rudimentary kidney, when one organ alone is
of any functional importance ; and (3) fusion of the two kidneys into
one mass, the " solitary kidney," including the " horse-shoe kidney."
A few words may be said about each of these types.

The unsymmetrical kidney is not very uncommon, as is borne out

by the long list of references in the Index Catalogue (Washington)

under the heading Kidney, Abnormities o/(vol. vii. 389, 1886 ; 2 s., viii.

635, 1903). It may be situated in its normal position at the side of

1 Possibly the kidneys were misplaced and not absent.

552 ANTENATAL PATHOLOGY AND HYGIENE

the spine, or may lie on the sacrum or in an intermediate position ;
it is often but not invariably of a larger size than normal ; it may
retain its foetal appearance (lobulation) ; and it may have either a
single ureter or two pelves and two ureters. When the kidney
has two ureters, and more especially when these pass to opposite
sides of the bladder, it may be doubted whether it is a case of unsym-
metrical kidney at all ; if, however, both ureters go to the same side
of the bladder it is probable that it is of the unsymmetrical type.
As a rule, two pelves and two ureters mean that the single kidney
is really two organs fused (the " solitary " kidney).

Cases of congenital atrophy or (better) rudimentary development
of one kidney are comparatively rare. Clinically, they have the same
significance as complete absence. The ureter, however, is generally
present in the former anomaly ; and the kidney itself may be a
small cystic, fibrous, or fatty mass, or may be simply small and
lobulated. The ureter may be a fibrous cord, or it may have a lumen
and open into the bladder, or it may be twisted on itself and have no
communication with the bladder. The renal arteries and veins may
show various anomalies ; and, finally, the rudimentary kidney may
be misplaced. Possibly some of the reported cases have been the
results of antenatal or postnatal disease and are not true develop-
mental anomalies.

The " fused kidney " is comparatively common. When both
kidneys are displaced they are generally also united, but the nature
and degree of the union vary much. In some instances the two
organs are united at their lower ends by a thin or thick band of
tissue which crosses the spine ; and the single renal mass thus pro-
duced has a distinct horse-shoe shape (horse-shoe kidney). In other
instances the kidneys are completely united together and form a
single disc-shaped mass, with a double or a single pelvis and two
ureters. Intermediate types lying between these two extremes may
be met with : there may be a single S-shaped renal mass lying on
one side of the vertebral column, the upper end of one kidney being
united to the lower end of the other; or there may be a horse-shoe
kidney, but one in which the union is more extensive than usual ; or
there may be a fusion of the two kidneys in such a way that the
hilum of the one is joined to the outer border of the other. The
fused kidney may be either lateral or central in position.

The single kidney may be found (generally by accident) in the
foetus, or it may lie unsuspected during the whole of adult life. It
may, however, give rise to trouble, as when it lies in the middle line
over the promontory of the sacrum, and is the cause of obstruction
to delivery in women. A fatal result is produced when nephrectomy
is performed and it is a single kidney that is removed ; all surgeons
ought, therefore, to practise catheterisation of the ureters before
carrying out such an operation. G. J. Winter {Arch. f. Idin. Chir.,
lxix. 611, 1903) reports several cases (including a personal one) of
surgical interference when there was a solitary kidney ; his article
is worthy of consultation upon this whole subject. In its clinical
relations the solitary kidney is a perfect example of the teratological

MALFORMATIONS OF THE KIDNEYS 553

state whose pathological effects may be postponed till adult life or
may never be revealed at all. In this respect the anomalies of the
kidney have a resemblance to those of the genital organs.

Sometimes the kidneys may be normal in number but abnormal
in form, and they may then be misplaced as well. Sometimes the
lobulation may be very deep in the kidney at birth (as in a specimen
given to me by Dr. Graham in 1894), or the fcetal degree of lobula-
tion may persist into adult life or even into old age (L. L. M'Arthur,
Ann. Surg., xxxiv. 203, 1901). A kidney may show two pelves and
one ureter or two pelves and two ureters ; the hilum may be absent ;
or the ureter may lie in front of instead of behind the renal artery
and vein at the hilum. Anomalies of the renal arteries have also
been noted, either in association with other malformations or alone ;
the most common anomaly is the presence of more than the usual
number of arteries (Micheleau, Journ. de med. de Bordeaux, xxviii.
188, 357,445, 487, 1898; xxix. 39, 1899), and the supernumerary
ones may arise from the aorta, or the lumbar, suprarenal, iliac, or middle
sacral arteries. A " floating " kidney possesses a mesonephron and
abnormally long vessels ; it is due, therefore, to an antenatal anomaly
of the peritoneum. Barely the kidneys are increased in number to
three (J. Hyrtl, Oesterr. med. Wchnschr., 965, 1841) or four (M. L.
Amick, Cincin, Mcd. Bepcrt.,\y. 551, 1871).

The various anomalies of the kidney are apt to be associated with
malformations of other organs, among which must be specially men-
tioned the genital organs and the anus. There may, for instance,
be atresia ani (B. Langenbeck, Deutsche Klinik, x. 77, 1858), or a
bicornate uterus (S. H. Scheiber, Med. Jahrb., 260, 1875), or a uni-
cornate uterus (F. C. Turner, Trans. Path. Soc. Lond., xxxvii. 284,
1885-86).

" Cystic " kidney is hardly to be regarded as an embryonic mal-
formation ; indeed I have already considered it in the first volume of
this Manual among the foetal diseases (p. 383). It has, also, been
regarded as a neoplasm. I have recently met with a case of labour
in which a large cystic kidney on the left side was the cause of great
delay (specimen No. 327 in my list) ; in this instance there was also
hypospadias. Similar cases have been reported by Gailleton and
Oilier (Gaz. d, hop., xxvi. 433, 1853), Borak {France med., ii. for 1885,
p. 1441), C. W. F. Uhde (MonatscJir. f. Gcburtsk. u. Frauenkr., viii.
26, 1856), and W. Wolff (Berl. Bin. Wchnschr., iii. 269, 1866 ; iv.
480, 1867).

In describing renal anomalies I have referred also to malforma-
tions of the ureters (absence, duplication); but I may further note
here that cases of apparent low termination of these canals have
been observed by F. Colzi (Sperimentale, Sez. biol, p. 37, 1895), A.
Wolfler (Prog. med. Wchnschr., xx. 231, 253, 1895), and others. The
ureters opened in the vestibule a little below the normal meatus
urinarius, the patients being women. D. Berry Hart (Trans. Edinh.
Obstet. Soe,, xxvi. 313, 1901) does not think that the tubes which thus
open so low down are altogether ureters ; he regards them as due to
persistence of the Wolffian duct and of the original communication

554 ANTENATAL PATHOLOGY AND HYGIENE

between it and the ureter. Incontinence of urine is the clinical
feature in this anomaly. Somewhat analogous cases are met with in
the male subject, when the ureter opens into the vas deferens or
seminal vesicles (Tangl, Arch. f. path. Anat., cxviii. 414, 1889). For
a list of references to the literature of ureteric malformations the
reader may again consult the invaluable Index Catalogue (vol. xv.
p. 53, 1894).

Malformations of the Penis.

Complete absence of the penis is very rare save in combination
with such grave monstrosities as sympodia and acephaly. In 1897,
however, I received a letter from Dr. K. P. Harris of Philadelphia in
which he told me of two cases in adults which he had met with in
Philadelphia. Dr. Harris afterwards published these two instances
(Phila. Med. Joitrn., i. 71, 1898), and gave a summary of five others
(those reported by Goschler. Eauber, Nelaton, Mathews, and Collier),
drawing the conclusion that there is one male infant born with this
defect in about 30,000,000. The rarity of complete absence of the
penis is in striking contrast with the frequency of rudimentary
development (as in extroversion of the bladder, hypospadias, etc.).
In five of the seven cases collected by Harris the urine was passed
into the rectum apparently by an opening in the anterior rectal
wall, and in the other two the urethra opened in the perineum just
outside the anus. J. Bloomfield's patient, a man, thirty-five years of
age, was a, twin. (Columbus Med. Journ., p. 133, 1895); so was Lemke's
infant (Arch.f. path. Anat., exxxii. 181). In all the instances cited,
there was a scrotum containing testicles, and the characters of the indi-
vidual were distinctly male. The urine did not cause much irritation
in the rectum unless the individual happened to develop haemorrhoids ;
it was this fact that suggested to surgeons the possibility of turning
the ureters into the bowel in cases of ectopia vesicae, a proceeding the
feasibility of which was experimentally determined by E. H. Eeed
(Ann. Surg., xvi. 193, 1892).

Duplication of the penis or diphallus is one of the teratologieal
states that lie on the border line between the single or monosomatous
terata and the double or polysomatous terata. When the double
penis is associated with one or two supernumerary lower limbs the
case is by everyone regarded as belonging to the double terata or
united twins ; but when diphallus is the solitary external anomaly it
becomes much more difficult of classification and is obviously one of
those connecting links which are so disturbing to all systems of
classification.

In 1895 Dr. Scot Skirving and I described an instance of diphallus
in the tenth child of healthy parents (Teratologia, ii. 92, 1895). The
scrotum was divided into two lateral lobes by a deep groove in the
middle line, and these lobes merged together in the middle line
anteriorly but were quite distinct posteriorly (Fig. 80). Each of the
scrotal pouches contained an apparently normal testicle. In the
middle line of the perineum was an ill-defined swelling from which
two short but perfectly recognisable penes arose ; each of them had

DI PHALLUS

555

a prepuce so short as to expose the whole glans ; and eacli had a
meatus nrinarius, but that on the left side appeared to end blindly.
Lehind the penes and in front of the anus (which was normal), there
was an area in which the skin was defective ; in the centre of this
was a mound of red, ill-formed material, apparently granulation tissue
(k in Fig. 80), and on each side, between it and the healthy skin,
was a groove or sulcus (g, h in Fig. 80). During the act of micturi-

FiG. 80. — Sketch of Genital Organs in Case of Diphallus, with Infant in
Lithotomy Position (Case No. 137).

a, sjnnphysis pubis ; b, c, right and left halves of scrotum ; d, c, right and left penises ;
/, common swelling at root of penises ; k, granulation mass, with urinary fistula in
centre, and sulci at each side (g, h) ; j, normal anus.

tion, a gush of urine came from the centre of the granulation mass
(urinary fistula), while at the same time a few drops came from the
rio;ht meatus urinarius.

I have elsewhere (Teratologia, ii. 182, 255, 1895) gathered together
the literature of diphallus up to the year 1895, finding records of
twenty observations in all ; but since then a few more instances have

556

ANTENATAL PATHOLOGY AND HYGIENE

been reported (H. Kiittner, Beitr. z. kiln, Chir., xv. 364, 1896 ; M.
Lange, Beitr. z. path. Anat. u. allg. Path., xxiv. 223, 1898 ; J. H.
Morgan, Trans. Clin, Soc. Land., xxix. 216, 1896; Hofmokl, Win*.
mcd. Presse, xxxvii. 1396, 1896; Arch. f. Idin. Chir., liv. 220, 1897;
F. Neugebauer, Gaz. leh, 2 s., xvii. 561, 1897, Pam. Towarz. Irk., xciii.
327, 1897, Ccntrlbl, f. Gynak., xxvi. 1395, 1902). One of the most
interesting and typical cases was that described by Jenisch {Med.
Cor.-Bl. d, wiirttemb. drztl. Vcr., vii. 129, 1837). The patient was a

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Fig. 81. — Case of Double Penis (Diphallus).

full-time male infant, well formed save in the following details.
In place of one penis there were two, situated near to each other, of
which one (the right) had the appearance and size of that of a normal
infant, while the other (the left) was larger and was not furnished
with a prepuce. Each penis had a patent urethra, from which urine
along with a trace of meconium was expelled ; each had its own
scrotum, but each scrotum contained only one testicle (Fig. 81).
Behind the scrotal sacs was an elevation made up of spongy folds of

DI PHALLUS 557

skin darker in colour than the surrounding integument ; near to this
was attached a steatomatous tumour in the position of the absent
anus. A sound could be passed through both urethral into the
bladder, and thence through an opening in the posterior wall of the
neck of that viscus into a cavity behind it (the intestine). The
atresia ani was operated on unsuccessfully, and the infant died.

In the article to which I have referred {Tcratologia, ii. 92, 184,
255, 1895) considerable attention was paid to the clinical history
and morbid anatomy of the recorded cases of diphallus, and it is
unnecessary to repeat the details here. It may, however, be stated
that the malformation of the penis did not in itself interfere with
postnatal life, although it was usually productive of sterility (physio-
logical incompetence). Generally the two penes were situated
close together ; but in a few instances they were widely separate,
recalling the double genital tubercles of the specimen of gastroschisis
shown in Fig. 79 (p. 544); the two penes may be so close together
as to justify the term bifid or bifurcated penis. In a few instances
the bladder appears to have been double, or at least consisted of two
compartments ; and in one case, at least, there were said to be three
testicles. In a number of the records it was stated that the anus was
imperforate or opened in an abnormal situation. From the point
of view of teratogenesis it is unfortunate that little is known regard-
ing the state of the lower end of the spine in diphallus ; but in some
instances there was unusual breadth of the pelvis, pointing to some
tendency to duplication of the sacrum. In Pigne's case {Bull. Soc.
a/riat. de Paris, xxi. 110, 1846), an anencephalic foetus, there were
two stomachs, two pericardial sacs (each containing a well-formed
heart), four lungs, and two aortae ; yet the foetus was externally
single save for the presence of two penes. The symphysis pubis
may be defective and the lower limbs shorter than normal.

The diphallic monstrosity presents problems of the greatest
difficulty when we try to classify it or to explain its origin and
nature. With regard to classification, it would seem to be best to
look upon it as a slight form of duplication of the foetus, and to
consider it (as Taruffi does, Sull'ordinamento della teratologic/,, Bologna,
1896, 1898) as really two pelves joined together, so that each ilium
represents one pelvis and the sacrum is really two fused together.
Taruffi admits the hypothetical nature of this view ; and, of course,
if it had been well established I should have placed diphallus with
the polysomatous terata and not with the monosomatous. As it is,
I retain the type among the single monsters just as I did polymastia
and some other malformations by excess. At the same time I am
inclined to accept Taruffi's hypothesis, especially as two penises are
actually met with in cases of undoubted double monster, such as the
tripod (three-legged) monstrosity exemplified in Jean Battista dos
Santos (W. Acton, Med.-CMr. Trans., Lond., xxix. 103, 1845-46;
E. Hart, Lancet, ii. for 1865, p. 124; P. D. Handyside, Edinb. Med,
Journ., xi. 833, 1866). I do not, however, regard it as advisable to
adopt Taruffi's name (" Syncephalus dilecanus dipleurus dipus "), and
retain the shorter " diphallus." It is only fair to state that, instead

558

ANTENATAL PATHOLOGY AND HYGIENE

of regarding this monstrosity as a minimum degree of duplication of
the individual, it is competent to look upon it as a reversion to a
lower type, for a double penis is a character of some of the lower
animals. Yet another view is possible, namely, that the penis is
developed from the fusion of two lateral genital tubercles, and that,
therefore, diphallus represents an arrested development, each penis
being really only a half organ.

Duplication of the urethra in the penis has been observed by
J. Englisch (IVien. mecl. Presse, xxix. 985, 1888) and others (J. Wood,
Trans. Path. Soc. Zonal., x. 201, 1858-59); and three urethra? in one
glans penis were noted by Szymanowski (Vrtljschr. f. d. prakt. Heilh,

b

c
d
e

Fig. 82. — Transverse Section of Penis-like Body, x 10, in
case of Fcetal Ascites.

a, skin surface ; b, connective tissue ; c, unstriped muscle ;
d, cavernous erectile tissue ; e, canals lined l>y cubical
epithelium ; /, nerve in section ; g, area of denser
connective tissue.

lxxxiii. 23, 1864). Various anomalous canals may also be met with
in the penis, which communicate neither with the urethra nor with
the ejaculatory ducts : or there may be diverticula of the urethra
itself ; or, finally, there may be fistuke extending from the rectum to
the urethra and opening into it (usually in cases of atresia ani).
All these varieties of penile canals are described by C. Taruffi in his
work entitled Sui canali anomali del pene, Bologna, 1891. Perhaps
some of these canals are due to incomplete antenatal cure of
epispadias. Some years ago Dr. W. Fordyce published in my
journal (Teratolof/ia, i. p. Gl, 1894) a case of foetal ascites in which
there was a curious penis-like body (Fig. 82) ; this body, on transverse

EPISPADIAS AND HYPOSPADIAS 559

section, showed two canals, which communicated not with the bladder,
but with two canals (vaginal ?) which passed to two uterine horns.
The exact nature of the penis-like body is doubtful, but Fordyce
regarded it as the united labia minora containing the hypertrophied
and irregularly developed clitoris.

Various degrees of atresia of the urethra in its course through
the penis have been recorded. In some of these the bladder became
greatly distended with urine in antenatal life (vide Vol. I. of this
Manual, pp. 379-382). Sometimes the obstruction is valvular, and
then a diverticulum of the urethra may be developed behind it,
giving rise to the curious penile pouches described in detail by
Johann v. Bokay (Jahrb.f. Kindcrhlk., 3 F., ii. 181, 1900) and others
(e.g. S. Grosglik, Monatsber. f. Urologie, vi. 577, 1901). Congenital
phimosis is an anomaly so well known and so frequently and so fully
described that I need do no more than mention it here. Absence of
the prepuce at birth has also been reported (M. Pioth, Cor.-Bl.f. schweiz.
Aerztc, xiv. 441, 1884), sometimes in association with occlusion of
the meatus urinarius (H. J. Garrigues, Amer. Journ. Obstet., xii. 615,
1879). Numerous references to articles on these various anomalies
of the male urethra are to be found in the Index Catalogue (Washing-
ton), vol. xv. 57, 1894.

Epispadias is a well-known malformation of the penis. Through
the presence of a groove or fissure of varying extent the urethra is
laid open along the dorsum of the penis. It is most commonly
associated with extroversion of the bladder, being indeed very rare
as a single malformation. E. Kirmisson (Maladies chirurgicales, p.
282, 1898) has reported and figured two typical cases, and others
are to be found in the list of references in the Index Catalogue (vol.
iv. 306, 1883 ; 2 s., vol. v. 84, 1900) ; but it is of much less frequent
occurrence than hypospadias. Sometimes the fissure affects the
whole penile urethra ; in other (very rare) cases the glans alone is
affected (balanic epispadias). The penis itself is generally small in
size, and the prepuce is well marked on the lower aspect. Such
malformed individuals often survive birth, and may, as F. Mayr has
shown (Diss, inaug., Munchen, 1892), belong to the criminal class ;
there may or may not be incontinence of urine, and impotence is
common. The teratogenesis of epispadias is probably the same as
that of extroversion of the bladder, namely, breaking down or arrested
formation of the cloacal membrane in the lower part of its extent.
Fpispadias may be surgically corrected either by the procedure of
Thiersch or of Duplay (vide A. Zoller (Diss, inaug., Heidelberg, 1893),
C. Bayer (Ccntrlbl.f. Kinderh., iii. 1, 1898), and others).

Hypospadias (the presence of a groove along the lower aspect
of the penis opening into the urethra) is comparatively common.
I have met with it in a number of cases, two of which I have
published (Teratologic, i. 95, 1894; ii. 119, 1895). The male urethra
may be divided into three parts, which may be named membrano-
prostatic, spongy, and balanic or glandular. When the canal is not
closed in at all anterior to its membrano-prostatic part, the hypo-
spadias may be called perineal or scrotal; this is the most severe

5 GO ANTENATAL PATHOLOGY AND HYGIENE

form of the deformity ; and it constitutes a comparatively common
type of pseudo-hermaphroditism, for the scrotum is usually cleft and
the testicles have often failed to descend. When the deficiency in
the lower urethral wall exists somewhere in the spongy portion, the
penile variety results ; and when it is in the glandular portion we
have to do with the balanic or glandular form. Intermediate types
also exist, such as F. C. Schaefer's, in which the hypospadias was
partly of the penile and partly of the scrotal type {Chicago Clin. Rev.,
p. 434, 1894). In the balanic form the urethra, instead of opening
in its normal position, does so at the base of the glans ; various
minor varieties of it may exist, as where the meatus urinarius is
found in its normal place, but leads into nothing more than a cul-de-
sac. A good description of the minor varieties of all the three
leading types of hypospadias is given by C. Kaufmann ( Verletzungen
a ikI Kranklieitcn der milnnlichen Hamrohre und des Penis, Stuttgart,
1886). Associated malformations, such as flattening of the glans,
torsion of the penis, atrophy of corpus spongiosum, atresia ani, non-
descent of the testicles, webbed penis, and a pseudo-vulvar orifice,
are not uncommonly met with. Hypospadias, also, may show family
prevalence, two or more brothers in the same family being affected
with it, as in T. H. Kellock's observation (Trans. Clin. Soc. Lond.,
xxxii. 242, 1898-99), and in several other instances which have been
carefully collected by F. Neugebauer (Monatschr. f. Geburtsh. u. Gynazk.,
xv. 281, 1902). Hereditary transmission has also been noted (E.
Lesser, Arch. f. path. Anat., cxvi. 537, 1889). There may be in-
continence of urine, but commonly there is only the inconvenience
arising from the abnormal direction in which the urine flows.
Sterility is common, but nor constant, as Dombrovski's case shows
(Vrach. Gaz., St. Petersburg, viii. 962, 1901). The mode of pro-
duction of hypospadias is arrested closure of the groove along the
under surface of the genital tubercle, and E. Loumeau (Arch. prov. de
chir., iii. 320, 1894; Journ. deme"d. de Bordeaux, xxiv. 121, 1894) has
shown how different minor varieties are accounted for by arrested
development at different stages. The anomaly may be met with in
the lower animals, as in the dog (Eetterer et Eoger, Journ. de Vaunt,
et physiol., xxv. 113, 1889). Treatment, which is almost of necessity
surgical, may consist solely in separating the penis from its fixed
position (as in webbing of it to the scrotum), or may be of the nature
of a separate operation. In the latter case the reconstruction of
the urethra may require to be performed by successive operations.
The literature of hypospadias, somewhat extensive, is to be found in
the Index Catalogue (vol. vi. 747, 1885 ; 2 s., vol. vii. 770, 1902).

I have only space to name some other malformations of the
penis : such are webbing of it whereby it is enclosed in the skin of
the scrotum (P. Strassmann, Ztsehr. f. Geburtsh. u. Gyndk., xxviii.
183, 1894); a supernumerary glans penis (Dannie, Arch. prov. de chir.,
i. 518, 1894); shortness of the fraenum (C. Fere, Rev. de chir., p. 333,
April 10, 1895); and that very rare anomaly, antenatal paraphimosis
(P. Strassmann, Ztsehr. f. Geburtsh. u. Gyndk., xxviii. 181, 1894). An
individual with a vulva and a pseudo-penis lying posterior to it

ANOMALIES OF TESTICLES 561

(implanted on the perineum) was described by F. Neugebauer in 1899
(Centrlbl. f. Gyndh., xxiii. 139, 1899) ; I know of no other modern
instance like this.

Anomalies of the Testicles.

A few sentences must suffice for a description of testicular
anomalies. The commonest is displacement or ectopia : this is gene-
rally due simply to non-descent of the testicles, and is therefore
fostal rather than embryonic in origin ; it may, however, be due to
causes which are embryonic. The missing glands may be either in
the abdomen or in the inguinal canals ; rarely is a testicle found
displaced into the perineum (Hoeck, Centrlbl. f. Gyndk.,xv\\\. 99,1894).
When one testicle alone is absent from the scrotum, the individual
is called a monorchid, although it is more than probable that he
really possesses both testicles ; when both testicles are displaced, he
is called a cryptorchid, and this name more truly expresses the state
of matters. Monorchids may be sterile ; cryptorchids are almost of
necessity so, for it would appear that the testicle does not usually
produce spermatozoa until it reaches the scrotum, although it may
influence the growth of the body at puberty (J. Griffiths, Journ.
Anat. and Physiol,, xxviii. 209, 1894 ; W. M. Eccles, Brit. Med, Journ.,
i. for 1902, pp. 503, 570).

Anorehidij or true absence of the testicles is a very rare terato-
logical state ; probably most of the recorded cases were instances of
rudimentary development of the glands and non-descent. Both
testicles may be found in one half of the scrotum {ectopia testis
transversa), as in the case of the eight years old boy seen by M.
Jordan (Deutsche med. Wchnschr., xxi. 525, 1895). Fusion of the
testicles (synorchidy) may be abdominal or scrotal ; it has been re-
garded as an atavistic phenomenon, the condition being normally found
in some of the lower animals ; it is very rare in the human subject.
Increase in the number of testicles (e.g. triorchidy) has occasionally
been reported, and some references may be found in the Index Cata-
logue (vol. xiv. 319, 1893) ; but it is always doubtful whether the
supernumerary glands are really testicular in nature, unless an
autopsy and microscopical examination can be made. F. Parona's
case of several testicles in the scrotum {polyorcliidy) is a most anom-
alous one (Policlinico, iii. 203, 1896).

Before I leave the anomalies of the testicle, I must refer to the
frequency of the development of malignant disease in the misplaced
testicle in later life ; to the association of this abnormity with con-
genital hernia : to the hereditary transmission of ectopia testis ; and
to the enlargement of the mammary glands (gynecomastia) in later life,
when the testicles are abnormal and the individual is also a hypo-
spadiac.

Malformations of the Female Genitals.

The anomalies of the genital organs in the female form a large
and important group ; but although most of them are developed in
vol. ii. — 36

562 ANTENATAL PATHOLOGY AND HYGIENE

embryonic or in early foetal life they have little or no effect on the
physiology of the individual until late in postnatal life. In fact, if
the woman never become pregnant most of her genital anomalies
are never discovered at all, or are only accidentally revealed. The
genital anomalies, therefore, are good examples of the postponed
effects of antenatal morbid states. For this reason we find very full
accounts of them in the text-books of Gynecology and Obstetrics, and
for the same reason I do not intend to discuss them in detail in this
work. Those of my readers, however, who may wish to obtain such
detailed knowledge will find my articles in Allbutt and Playfair's
System of Gfyncecology (pp. 6.3-112, 1896) and in Eeed's Text-Book of
Gynecology (pp. 118," 131, 274, 473, 560, 1901, 2nd edit., 1904) suf-
ficiently full to open up the subject to them. I shall content myself
here with some general remarks.

In the first place, the anomalies of the female genitals have the
peculiarity that some of them are produced comparatively late in
antenatal life and some in postnatal existence. The reason is to be
found in the late development of the genital organs. While some of
the ontogenetic processes are finished in the early weeks during the
embryonic stage, others are long delayed and are perhaps only com-
pleted some years after birth. For instance, the development of a
well-marked fundus for the uterus only takes place at puberty, and
the absence of such a fundus, leading to the anomaly known as the
uterus infantilis or planifundalis, is, therefore, a morbid state which
arises far on in postnatal existence. Again, the septum between the
two halves of the uterus may be found as late as the fifth month of
antenatal life ; if, then, part of this septum persist, producing the
anomaly known as the septate uterus, the date of production of this
anomaly may be regarded as the fourth or fifth month. A little
earlier in antenatal life there are external indications of the double
state of the uterus in the presence of two horns or cornua : an
arrested development at this stage (third or fourth month) produces
the uterus bieornis or bicorn uterus. Still earlier, the two halves of
the uterus are quite separate, and if they remain so the anomaly
known as the uterus duplex or uterus clidelphys is formed, or if, still
earlier, one of the two halves fail to appear at all, the uterus unicornis
results. Finally, failure of both halves of the uterus (Miillerian
ducts) to develop is a very early anomaly, and gives rise to absence
of the organ (defectus uteri) or to a very rudimentary state of it
{uterus rudimentarius, membra n ace us, etc.). It is evident, therefore,
that the uterine anomalies (and the same might be said of the vaginal
and tubal and vulvar) originate at various times in antenatal and
postnatal life ; they may be embryonic, foetal, or infantile in their
teratogenesis. In this respect they differ from most of the malfor-
mations described in this volume, for the latter are embryonic in the
time of their production.

In the second place, most of the anomalies of the female genital
organs very obviously are arrested developments. The embryological
theory of teratogenesis explains them satisfactorily and as a rule
completely. This has been shown conclusively by many writers,

MALFORMATIONS OF ANUS 5 63

among whom F. von Winckel deserves special mention ( Ueber die
Eintheilung, Entstehung und Benennung der Bildungshemmungen dcr
weiblichen Sexualorgane, Leipzig, 1800). Now and again, as in the case
of the trifid uterus, female epispadias, and atresia an i vaginalis, we meet
with teratological states which are with difficulty ascribed to arrested
development ; but in most of these instances evidence is not wanting
that if we had a more perfect knowledge of the early embryological
phenomena of the formation of the genitals, and more especially of
the formation of the associated urinary and alimentary systems, we
should have little trouble in satisfactorily explaining them all. Thus,
in vaginal and hymeneal anomalies it is clear that there are facts
not explicable on the hypothesis that the vagina and hymen are
developed solely from the ducts of Miiller, but which become capable
of explanation if the theory of origin of the canal from the lower
ends of the Wolffian ducts (D. Berry Hart, Trans. Edinb. Obstet. Soe.,
xxii. 18, 1897) be accepted. Hypospadias in women is not compar-
able to the anomaly of the same name in the male sex ; for it really
represents a persistence of the urogenital sinus ; in this malformation
the urethra appears to open into the vagina at a higher level than usual.
Since I wrote the articles on malformations of the female genitals
to which reference has been made, I have met with a variety of
anomaly quite new to me and of which I can find no notice in
literature. It was the case of a girl, two years of age, whom I saw
in December 1800 in Professor Annandale's Ward in the Edinburgh
lioyal Infirmary. The anomaly consisted in a congenital deficiency of
the perineum, with some prolapse of the rectal wall anteriorly and
on the left side. Had there existed a vulvar anus the condition
would not have been so peculiarly anomalous : but faeces were passed
through the anal opening lying behind the rudimentary perineum.
The hymen had the usual pouting form seen in the infant, and there
was no spina bifida and no other malformation. At first I thought
the child might have been born as a breech presentation and have
had the perineum torn accidentally ; but there was no history of any-
thing of the kind having occurred. The case is No. 241 in my list.

Malformations of the Anus and Rectum.

Stenosis of the rectum and anus is comparatively rare, although
possibly it sometimes escapes notice and undergoes natural cure by
dilatation. It may consist simply in slight narrowness which is first
noticed when the child begins to pass solid fa?ces ; or it may be very
marked and give trouble soon after birth. In the latter case the
anal aperture may be occluded by a hymen-like membrane with a
tiny opening in it. In other instances a valvular obstruction may
exist in the rectum above the anus. A curious form of obstruction
which may be classified with the cases of stenosis is that in which a
cutaneous band bridges over the anal aperture passing from the skin
over the coccyx to the perineum (J. H. Morgan, Lancet, ii. for 1881,
p. 705). Such forms of malformation are capable of successful
surgical treatment (dilatation, incision, excision).

564 ANTENATAL PATHOLOGY AND HYGIENE

Imperforation or complete occlusion of the anus or rectum or
both is more common. A thin membrane may entirely close the
anus ; or there may be no indication at all of the anus or of the lower
part of the rectum ; or, again, the anal opening may be present but
the rectum may be absent higher up. I have met with several
instances of these anomalies. To one of these I may specially refer
as it was of a somewhat unusual kind. It occurred in a 51-months
male foetus, the product of the fourteenth pregnancy of a healthy
woman, who had, however, had three miscarriages. There was no
external indication of an anus, but a marked postanal dimple
existed ; the internal organs of the foetus showed no abnormality
save that the greater part of the large intestine was contracted and
that the rectum ended blindly at a distance of 1 cm. from the peri-
neum, its terminal cul-de-sac being in close contact with the posterior
surface of the urethra at its point of origin from the bladder ( Trans.
Edirib. Obstet. Soc, xix. 49, 1894). Differences exist as to the level at
which the intestine ends, a fact which the surgeon must keep con-
stantly before him in his efforts to relieve those abnormalities by
operative procedures.

Abnormal terminations of the rectum are occasionally met with,
and show many differences, chiefly as regards position. In some
instances the bowel ends on the skin surface at the root of the penis,
behind the scrotum, at the symphysis pubis, in the gluteal region,
in the loin, or through an opening in the sacrum. In other instances
the bowel may end in one or other of the surrounding mucous
canals or viscera. Thus there may be atresia ani vesiealis, a narrow
canal passing from the rectum to open into the bladder near the
trigone or at the fundus ; there may be the closely allied atresia ani
urcthralis, in which the bowel communicates with the male urethra,
usually in its membranous portion ; there may be atresia ani vagineilis
or vulvar anus, in which the rectum empties itself by an opening
immediately within the fourchette ; or, finally, there may be the
extremely rare form in which the rectum has a communication with
the vaginal canal high up.

Many of these rectal and anal anomalies can be explained by the
embryological theory of teratogenesis, and had we a more perfect
acquaintance with all the details of the ontogenesis of the caudal end
of the embryo we should, I believe, be able to account for them all,
even for the most anomalous of them. I need hardly say that the
ingenuity of surgeons has for long been exercised in the treatment of
these defects, and with no small measure of success.

The literature of the subject is very large, and the reader is referred
to the Index Catalogue of Washington (vol. i. 460, 1880 ; vol. xi. 1054,
1890; 2 s., i. 559,1896).

Hermaphroditism.

In treatises on teratology much space is commonly given to
the consideration of hermaphroditism and pseudo-hermaphroditism.
Isidore G. Saint-Hilaire, for instance, in his Histoire des anomalies

HERMAPHRODITISM 565

(ii. 30-173, 1836) gave 143 pages to the discussion of the subject.
This work, however, is not a treatise upon teratology but a book
dealing with antenatal pathology, and there is a difference between
the two things. Nearly all the importance of hermaphroditism is
centred in its postnatal phenomena : there is the matter of registration
of the infant, there is the social and political position of individuals
of doubtful sex, and there are numerous medico-legal questions, such
as nullity of marriage and the like, which may arise in any given case.
These, however, all are the postponed effects of antenatal morbid
states ; they are due to states which do not themselves interfere with
antenatal life and health. Such phenomena are of the greatest
interest, and involve problems not easy of settlement ; but, at the
same time, from the standpoint of antenatal pathology, they have
just the same degree of importance as attaches to the fact that a man
with deformed fingers may be incapable of performing certain mani-
pulations, such as playing the piano or firing a gun. We have to
study their characters and mode of origin at and before birth ; but
with the complications and consequences which are set up by them in
adult life we have not specially to do. In this way the subject of her-
maphroditism is, for the antenatal pathologist, considerably narrowed.

Let us consider in the first place what is the antenatal aspect of
pseudo-hermaphroditism. This morbid state is due to the existence of
a malformation of either the male or the female organs of generation ;
this is the essential fact from the antenatal pathologist's standpoint.
This malformation may lead to the individual who is really a mal-
formed male being brought up as a girl ; he may thus acquire feminine
habits and possibly some of the secondary sexual characters of the
female may appear ; but true menstruation does not occur, and so an
anomalous condition arises in which there is a certain degree of
mixture of the characters of both sexes, often with troublesome and
even tragic residts. Still the individual is just what he or she was
at first — a malformed male or a malformed female. To the former
case, that in which testicles are present, the name of pseudo-herma-
phroditismus masculinus or androgyny is given; the latter, in which
ovaries are in existence, is called pseud 'o-liermaphroditismusfcmininus
or gynandry.

One form of androgyny is that in which the testicles have not
descended into the scrotum at the time of birth ; since the penis may
remain small at puberty while the breasts may enlarge, it is easy to
understand how doubts as to the proper sex of the individual may
arise. The essential antenatal fact, however, is the want of descent
of the testicles. Again, the condition of hypospadias (penile or scrotal)
may be present at birth, with the result that the child is registered
as a girl and brought up as such ; in this instance there may be a
trace of a uterus and the mammas may enlarge (gynecomastia) and a
pseudo-vulva be formed. Nevertheless, the individual is nothing
more than a hypospadiac male, and the antenatal phenomena are
simply the existence of a defective penis (hypospadias) and the per-
sistence (in a male) of the parts of the Mullerian ducts which form
the uterus (in the female).

566 ANTENATAL PATHOLOGY AND HYGIENE

In gynandry the ovaries are present, bnt some malformation
obscures the true sex. The clitoris, for instance, may be enlarged,
and the labia united behind it (atresia vulvae superficialis) ; or there
may be a single or a double inguinal ovarian hernia, simulating
descent of the testicle ; or, at a later date, hair may develop on the
face. In these instances, the individual although really a female,
takes on some masculine characters ; but, again, from the antenatal
standpoint the case is one of embryonic or foetal malformation.

The early writers dealt with another form of pseudo-hermaphro-
ditism, that in which the external organs of both sexes were present.
Modern teratologists have been inclined to doubt the correctness of
the old observations ; but I think the old authors were not wrong in
their descriptions, they were only in error in their interpretation of
them. I believe the cases were of the same nature as those reported
in recent years by Moostakov (Meditzina, i. 32, 1894 ; abstract in
Teratologic/,, ii. 234, 1895) and by Neugebauer (Monatschr. f. Geburtsh.
u. Gyncck., vii. 550, 1898); they were in other words instances either
of diphallus or of double vulva. In the former case one of the
penes was probably hypospadiac and so caused the appearance of
the two sexes ; while in the other, one vulva might be closed and its
clitoris enlarged so as to produce the same effect. The antenatal
aspect of the subject is the fact that two penes or two vulvas1
may exist in an individual who is otherwise single, and that one of
them may be so malformed as to resemble the organ of the opposite
sex.

Besides these cases of pseudo-hermaphroditism which are, after
all, simply antenatal malformations, there are also some recorded
instances which have been classed as true hermaphroditism, and
which are of a quite peculiar nature. They are the cases in which
the essential reproductive organs of both sexes (ovaries and testicles)
are present in the same individual. Of the bilateral form of true
hermaphroditism (that in which an ovary and a testicle are present
on both sides of the body), no absolutely conclusive example in the
human subject has yet been recorded; C. L. Heppner's case (Arclt.
f. Anat. Physiol, u. wissensch. Med., 679 , 1870; St. Petersb. Tried. Ztsehr.,
n.s., iii. 87, 1872), in which there was a rudimentary uterus and
vagina, and, on both sides, a normal ovary, parovarium, and tube,
with near to each ovary a body resembling a testis, may have been a
genuine instance ; but the complete identification of the bodies near
the ovaries with testicles cannot be regarded as accomplished.
Lateral hermaphroditism, in which there is an ovary on one side and
a testis on the other, has been occasionally reported as in the cases
of Schmorl {Arch. f. 'path. Anat., cxiii. 229, 1888) and Obolonsky
(Ztsehr. f. Heilk., ix. 211, 1888). Unilateral hermaphroditism is that
state in which there is an ovary or testis on one side and an ovary
and testis on the other. Blacker and Lawrence (Trans. Obstet. Soc.
Lond., xxxviii. 265, 1896) have reported such a case: the foetus
possessed a uterus unicornis, a normal ovary and tube on the right

1 As in J. Suppiger's case {Cur. Bl.f. schweiz. Aerzte, vi. 418, 1867 ; viii. 744, 1878)
and in <!. Chiarleoni's (Ann. di ostet., xvi. 469, 1894).

HERMAPHRODITISM 567

side, and on the left side an ovo-testis, with a vas deferens and epidi-
dymis. The left sexual gland (ovo-testis) in one part showed cell
columns, cell nests, and Graafian follicles with a large quantity of
stroma (ovarian portion) ; and in a second part exhibited an abundant
stroma, with definite tubules filled with cells, and forming at the
hilum a rete-like structure (testicular portion). In 1901 I received
for examination a foetus with hypospadias, which one of my students,
Charles E. Whittaker, dissected for me. Besides finding various
muscular and vascular anomalies in different parts of the body, Mr.
Whittaker found a testicle and an ovary on the right side and an
ovary (with tube) alone on the left side.

The foregoing cases prove that anatomical hermaphroditism exists
in the human subject ; but there is as yet no evidence that in one
individual two functionally active glands of the opposite sexes can
exist. We need not, therefore, consider physiological hermaphro-
ditism ; but anatomical hermaphroditism presents us with a practically
insoluble problem. It cannot be explained on the theory of arrested
development ; at least I can think of no way in which to account for
the facts on such a hypothesis. It is, of course, open for us to regard
such cases as instances of fusion of two individuals, one male and the
other female, the fusion being so complete that only the left genital
gland of the one and the right of the other have been left to give a
hint that such a fusion has taken place. But this theory is not
available until twins in the same amnion and of different sexes have
been met with, or until united twins of different sexes have been
clearly proved to exist. If, again, we maintain that the genital glands
are originally neuter and that one may develop into an ovary and
the other into a testicle, then we are met by the difficulty of the
cases in which an ovary and a testicle have been found on the same
side of the body. The only other explanation which seems feasible
is to regard hermaphroditism as a phenomenon of the germinal
period of life, as a reversion to a lower form of animal construction ;
this, it need hardly be added, is a most unsatisfactory view.

Literature. — Eeferences to articles bearing on hermaphroditism
and pseudo-hermaphroditism are to be found in the Index Catalogue
(vol. v. 345, 354, 1884; vol. vi. 84, 1885; 2 s., vol. vi. 138, 159, 1045,
1901).

CHAPTER XXIX

Merosomatous Terata (cout.) : Malformations of the Limbs : Sympodia (Derini-
tiou, Frequency, Synonyms, History, Clinical Characters, Morbid Anatomy,
Teratogenesis) ; Monopodia ; Ectromelus or Amelias ; Hemimelus ; Phoco-
melus ; Absence of Individual Bones ; Miscellaneous Defects ; Double Hand
and Double Foot ; Polydactyly and Hyperphalangy : Malformations of the
Fcetal Annexa ; Anomalies of the Placenta, Umbilical Cord, and Vessels.

The malformations of the limbs form a long and varied series of
anomalies. Few of them seriously affect postnatal life, but most of
them require surgical treatment in order to remove the inconvenience
they cause: they do not prevent the individual surviving his birth,
but they greatly interfere with his usefulness, and they may, as in
cases of amelia, so limit his activities as to make it impossible for
him to earn his living save by exhibiting his deformity. I shall do
with anomalies of the limbs as with those of other parts of the body ;
I shall describe one type in detail, and shall do little more than
indicate the others. The type chosen for detailed description is
sympodia or fusion of the lower limbs, for, in many ways, it has the
greatest interest from the antenatal standpoint.

Sympodia.

Sympodia may be defined as a state of inversion of the lower limbs
with a greater or less degree of fusion of the parts, and with an
imperfect development of them and of the neighbouring pelvic organs
and pelvis. This definition excludes the cases of rotation of the
lower limbs without fusion, but it includes those of rotation with
membranous adhesion such as my specimen (Fig. 35, p. 182) ;
theoretically, also, it includes sympodia as it occurs in the allantoido-
angiopagous twin (vide my article on paracephalus acardiacus, in
which I describe a sireniform variety, Trans. Edinb. Obstct. Soc., xviii.
210, 1892-93) and in united twins or double monsters, but I shall not
specially consider these forms here.

Sympodia is relatively somewhat rare. It is true that a fairly long
list of cases can be collected — I gathered together 116 instances up
to 1897 (Trans. Edinb. Obstet. Soc,, xxiii. 74, 1898)— but then it must
be remembered that it is a striking and arresting and clearly denned
monstrosity, and that, therefore, it is most likely to be reported and
preserved in museums or private collections. I have personally been
able to examine three specimens (Nos. 121, 209, 250), and these are
represented in Figs. 20, 35, 56, 63, and 83 ; since my list of cases of

568

SYMPODIA 569

antenatal diseases and deformities numbers 331, sympodia may,
therefore, be looked for about once in every hundred cases, and it is,
therefore, not nearly so common as anencephaly (of which I had
forty-six specimens). The proportion of cases of sympodia in general
obstetric practice can hardly be even approximately stated.

Synonyms of sympodia are " cuspidate foetus," " monopodia,"
" sirenomelia," " sirenia," " symelia," and " sireniform foetus." The
terms " mermaid foetus " and " foetus with a tailed appendage " have
also been employed. None of these terms is cpiite so good as
sympodia, which indicates the fusion of the lower limbs as the
essential feature of the anomaly. Symelia, for instance, simply
means fusion of limbs, upper or lower, and, as a matter of fact, fusion
of the upper limbs is only known in cases of double terata (Fig. 90).
To adopt the terms " sirenia " or " sireniform," and thus to liken the
monstrosity to a fabulous creature (the siren), is scarcely scientific.
" Cuspidate fcetus " is a fairly correct expression, indicating as it does
that the fcetus " ends in a point," but it is somewhat pedantic.
" Sympodia " is, therefore, the name I have chosen. " Monopodia,"
meaning one-legged, is quite inappropriate, for in cases of sympodia
there is always evidence that two limits have been more or less com-
pletely fused together to form the single lower extremity ; there are,
moreover, true cases of monopodia to which reference has already
been made (p. 521).

The history of the early cases of sympodia is rather obscure. The
earliest case seems to have been that of N. Eocheus (Dc mortis
mulierum curandis, Paris, 1542): this I have not been able to see in
the original, but Schenkius (Otservationum .... volumen, lib. v.
737, 1609) described the same specimen as "monstrum superna parte
hominis effigie ad umbilicum usque inferna syrenum caudam, pedum
loco referens," and went on to state that Christian baptism had been
given to it as it lived for at least an hour after birth (" tinctum fuit,
hoc monstrum Christianorum lavacro, horamque a partu duntaxat
vixit"). Another case was reported by J. Fincelius (De miraculis
sui temporis, 1556-57). According to Taruffi (op. cit., vii. 509, 1894),
neither of these cases is referred to by C. Lycosthenes in his Chroni-
con (p. 142), published in 1557 ; he simply related a tale from Livy
(Bk. xxxiv., par. 45) about an amelic fcetus ; and, in Tarulii's opinion,
he had an imperfect knowledge of sympoclial foetuses, since he con-
fused them with examples of amelia and figured one with a penis
and scrotum. I think, however, that Taruffi has, in part at least,
misjudged Lycosthenes, for in my copy of his Chronicon, on page 623,
is the picture of a fcetus sympus with arms and what looks like a
vulva (which is evidently different from the specimen shown on
page 142), and this instance is ascribed by Lycosthenes to Fincelius.

Aldrovandi (Monstrorum historia, 519, 1642) recorded a well-
marked case, as may be gathered from the description he gave — " Ab
umbilico autem, relicta hominis figura, in formam pyramidalem
terminabatur, referens in cuspide similitudinem caudae suis inflexse."
J. Scultetus (Armamentarium chrnirgictim, p. 172, 1656) reported a
dead-born foetus which had neither anus, nor urethra, nor external

570 ANTENATAL PATHOLOGY AND HYGIENE

genitals, but showed a perineal hernial protrusion and had the lower
limbs closely united ; the other parts of the body were well formed.
Towards the close of the seventeenth century P. J. Hartmann (Miscel.
Acad. not. curios., Dec. ii., Ann. x., obs. 162, p. 258, 1692) described
his specimen, and was the first to give an account of the dissectional
appearances; and Du Cauroi {Joum. cles. sgavans, xxiv. (for 1696,
p. 81) was told by a midwife about an infant, normal in its upper
parts, which was without any appearance of distinction of sex and
had a leg arising from the middle of the hypogastrium. Mery's
"enfant monstrueux" (Hist. Acad. roy. d, sc, Ann. 1700, 2nd edit.,
Paris, 1719, p. 42), although placed by Taruffi among the sympodial
foetuses, was really a case of inversion without fusion of the lower
extremities.

In the eighteenth century nine new examples of sym podia were
put on record, including two British instances, those of Superville
(Philos. Trans., xli. 302, 1744) and of Easter (ibid., xlvi. 479, 1752).
Hottinger described a case in 1706 (Miscel. Acad. nat. curios., Dec. iii.,
Ann. 9, 10, p. 413, 1706), and Sue (Hist. Acad. roy. d. sc, lxiv. p. 42,
for 1746, Paris, 1751) met with one in which there were two uteri
and a double thumb ; but the most important contributions of the
century were the two which came from the pen of A. K. Boerhaave
(Hist. anat. infantis cujus pars corporis inferior monstrosa, Petropoli,
1754; Hist, altera anat. infantis, Petropoli, 1757). There were more
than one hundred pages of description accompanying each of Boer-
haave's records, and there were in all twenty-four plates illustrating
very fully their anatomical peculiarities ; even at the present time
the observations of the St. Petersburg professor are well worthy of
perusal. In this century also the long series of inaugural disserta-
tions dealing with the subject of sympodia was begun with that of
F. I. A. Eossi (Diss, inaug., Jena?, 1800).

The contributions of the nineteenth century were very numerous,
and their bibliography up to the year 1897 will be found in my
article already referred to (Trans. Edinb. Obstet. Soc, xxiii. 74, 1898);
but I may perhaps name those of Cruveilhier, Saint-Hilaire, Bardinet,
Otto, Vrolik, Wolff, Julliard, Gebhard, Labougle et Eegnier, Bening-
ton, Buge, and Larcher as of special value. Since 1897 there have
been several articles of merit, including those of Opitz (Ztschr. f.
Geburtsh. u. Gh/ndJc., xl. 319, 1899), A. Guzzoni degli Ancarani (Ann.
di ostct., xxi. '609, 1899), L. Bolk (Gcncesk. Bl., vi. 301, 1899), E.
Babaud (Bull. Hoc. philomat. dc Paris, 9 s, v. 25, 1902-03), W. K.
Stokes and B. L. M'Neer (Maryland Med. Joum., xlv. p. 1, 1902),
Audebert (Toulouse mdd., 2 s., v. 129, 1903), Abramoff and Biezanoff
(Arch, f path. Anat., clxxi. 284, 1903), M. 1ST. Poroskin (Joum. ahu.sh.
ijensh. boliez., xviii. 89, 1904), and Cichorius (Arch.f Gynaeh, lxxii.
571, 1904). My three cases were published in 1898 (Scott. Med. and
Surg. Joum., ii. 296, 385, 1898), 1899 (Trans. Edinb. Obstet, Soc, xxiv.
18, 1899), and in 1900 (ibid., xxv. 144, 1900). A representation of
the posterior appearances of the lower part of my first specimen is
given in Pig. 83, while Figs. 20, 35, 56, and 63 give other views of it
and of the two other cases.

SYMPODIA

571

Fig. 83. — Posterior Aspect of Lower Part of a Foetus
Sympus Dipus, showing fused lower limbs and
sinus over sacrum. Specimen No. 121.

572 ANTENATAL PATHOLOGY AND HYGIENE

The clinical history, antenatal and postnatal, of sympodial foetuses
requires no lengthy consideration. None of the recorded infants
survived its birth for more than a few minutes, or at most, hours ;
but it is not quite clear to what cause this non-viability is to be
attributed. The kidneys are frequently absent, and this may, perhaps,
explain the early death of such monstrosities. No certain facts are
forthcoming regarding the antenatal symptomatology of sympodia.
In many cases a maternal impression (sight of a one-legged man, etc.)
was referred to, generally after the event; in one case, curiously
enough, the father had only one lower limb, the other having been
amputated, but his other children were normal (Saint-Hilaire,
Histoire dcs anomalies, ii. 246, 1836). The pregnancy seems some-
what frequently to have terminated prematurely, and reference has
been occasionally made to the weakness of the foetal movements or
to the occurrence of an abdominal traumatism. In Ehrmann's case
(Musec d'anatomic . . . . de Strasbourg, 1852) the father committed
suicide during his wife's pregnancy which ended in the birth of a sym-
podial foetus. In Gerrard's record {Month. Joiirn, Med,, xx. 348, 1855)
there was hydramnios. All these occurrences, however, are quite
exceptional, and the pregnancy was often absolutely uneventful. The
labours, also, were often easy, delay, when it did occur, being commonly
due to an associated malformation (e.g. hydrocephalus, as in my case)
or to a malpresentation. When the fused lower limb presented, as it
sometimes did, the diagnosis of the presentation was thrown into
doubt.

Owing to the nature of the malformation the sex of the infant
was often in doubt ; but it was ascertained in a number of cases, with
the result that neither sex could be said to preponderate.

The morbid anatomy of sympodia is a somewhat complicated
subject. I shall consider, first, the external appearances, and, second,
the conditions discovered by dissection.

The fused lower limb usually is directly continuous with the
lower (sub-umbilical) part of the trunk, and it is commonly flattened
anteriorly (Fig. 20) and curved posteriorly (Fig. 83). It is generally
capable of being folded upwards upon the anterior surface of the
trunk, so that, in one of my specimens, the fused foot lay over the
right shoulder and near the right ear. In exceptional cases the lower
limb arises at an angle from the trunk ; sometimes, also, one segment
of it moves freely upon the other, but, as a rule, the movement is
chiefly at the hip joint. The lower part of the abdomen shows a
tendency to narrow as it approaches the lower limb, and this tendency
along with the form of the fused limb gives to the foetus its cuspidate
appearance. On the anterior aspect erf the trunk near its junction
with the lower limb there may be an indication of a penis (Fig. 20)
or, rarely, of a vulva ; generally there is no trace of external genitals.
Posteriorly there is no indication of gluteal folds or anus ; or a linear
depression with radiating furrows may be visible (Fig. 83), which
possibly indicates a rudimentary anal aperture. Not uncommonly a
small tailed appendage is found situated posteriorly in the lumbo-
sacral region ; this caudal projection is due to a backward tilting of

SYMPODIA 573

the lower end of the sacrum (Fig. 56); and the " tail" thus produced
is not to be confused with the caudiform fused lower limbs.

The external appearances of the lower limb vary considerably,
and the differences have been used as a means of classification.
There are three varieties : according to the system of Saint-Hilaire
{Histoire des anomalies, ii. 239, 1836) these are the genera, symelus,
uromelus, and sircnomclus ; according to that of A. Forster (Missbil-
dungen dcs Menschen, 66, 1865) they are the types known as sympus
dipus, sympus monopus, and sympus ajnis. In sympus dipus there are
obvious signs that the single lower extremity is made up of two
lower limbs: the digits number from 6 up to 10 or even 11, the foot
is divided more or less deeply into two halves by a groove or fissure ;
three or four bones (2 tibiae and 2 fibulas, or 2 tibiae and 1 fused
fibula) can be felt in the leg; and 2 femora, more or less united, can
be palpated in the thigh. The whole lower limb is inverted, so that
the patella? are situated posteriorly while the sole of the foot looks
forwards (Fig. 20) and the dorsum backwards (Fig. 83). In sympus
monopus there is more intimate union of the two conjoined lower
limits : the thigh and leg are narrower than in s. dipus ; the two
femora are fused into one ; the leg contains only one or at most two
bones ; there is one patella situated posteriorly ; there are rarely 5
digits, more commonly 2 or 1 (Figs. 56, 63). In sympus apus, the
fused lower limbs end simply in a tapering point or in a stump like
that seen after an amputation ; there is here an even greater degree
of fusion of the extremities, and a very defective state of the pelvis
and its organs ; no recognisable digit or digits exist. To these three
distinct types Taruffi proposes to add a fourth : sometimes the lower
limbs are rotated and united simply by membrane, as in one of my
cases (Fig. 35) ; for this the name epi-sympus dipus has been pro-
posed; the case of J. Labougle and F. C. Begnier (Journ. de mid. de
Bordeaux, xviii. 438, 1889) ought probably to be placed in this
group.

The body of the sympodial fo3tus above the umbilicus is usually
normal in its external characters. There may be associated mal-
formations such as hydrocephalus, hare-lip, Polydactyly, which alter
the appearances of the head and limbs ; sympodia itself, however,
consists essentially in the sub-umbilical anomalies that have been
noted.

The dissectional appearances are very interesting, and have been
described in great detail by several authors, among whom I may
name Boerhaave writing so long ago as 1754. The sacral and
coccygeal vertebrae may be defective or excessive in number ; and
the tip of the spinal column may be directed backwards, or to
one side, or even upwards, a circumstance which accounts for the
occasional presence of a caudiform projection in the sacral region.
The whole pelvis indeed is usually malformed. The ilia, in cases of
absence of the sacrum, are united to each other and to the last
lumbar vertebra by ligaments ; these bones are also spread out
laterally, so that the anterior superior spines are directed to the
sides and downwards ; their fossae are flattened or even converted into

574 ANTENATAL PATHOLOGY AND HYGIENE

convex surfaces. The tuberosities of the ischia are generally turned
inwards, and their ascending rami are joined together in a horizontal
plane with disappearance of the obturator foramina. In front of the
ischia there is a projecting crest formed by the horizontal rami of
the pubic bones. Sometimes the ischial and pubic bones unite to
form a mass which may almost block the outlet of the pelvis, and
may show a wide cotyloid cavity for the reception of the large head
of the fused femora. These are the more usual anomalies found in
the pelvis ; less common malformations are the existence of a figure
of 8 pelvic outlet and of membranous union of the symphysis pubis
with outward displacement of the pubic bones. It is on account of
these conditions that Taruffi (op. cit., vii. 508, 1804) places sympodia
among the pelvic monstrosities (" lecano-terata ").

It is unnecessary to describe in detail the appearances revealed
by a careful dissection of the lower limb. All degrees between
complete fusion of the constituent bones, muscles, vessels, and nerves,
and a simple membranous union of two outwardly rotated limbs may
be met with. Where two acetabular cavities exist the femora have
separate heads and shafts, and their great trochanters are situated
internally, and are separate or united in part or completely. The
femora are concave anteriorly and convex posteriorly, and they
articulate usually with two tibia? which are placed posteriorly or less
commonly at the sides. In other cases the thigh bones are more
or less fused, and have a single large head fitting into a single
acetabular cavity ; four condyles may be found inferiorly or three
(two lateral and one large median one which represents the two
external ones fused together) ; again, the shafts of the femora may
separate inferiorly, showing two lower extremities, each articulating
with a tibia. The femora in all these cases show rotation, external
parts becoming internal and anterior parts posterior. The tibia? also
exhibit rotation, and so the " knee " flexes anteriorly. There may be
two fibulae situated internal to the tibiae ; or, more often, there is a
single large fibula in the middle line of the leg. When there are
only two femoral condyles there is generally a single tibia and no
fibulas. Nearly all the bones of both feet may be present, but, far
oftener, the foot is represented by only two or three tarsal and
metatarsal bones and the phalanges of one digit. When both feet
are indicated, the heels are internal and anterior and the digits
lateral or posterior.

Many of the internal organs are malformed in cases of sympodia,
Those which are most affected are the genital and urinary organs.
The kidneys, ureters, and bladder are generally absent, although the
suprarenal capsules may be present. In forty out of fifty-two cases
in which there was any reference to the state of the bladder it was
declared to be wanting ; and in the remaining twelve it was generally
rudimentary, having no orifices. The urachus, although seldom re-
ferred to in descriptions of sympodia, was generally stated to be
absent when any allusion was made to it (Trains. Edinb. Obstrt. Soc,
xxiii. 64, 1898). The genital organs are usually very defective. A
uterus has sometimes been found, but commonly only rudimentary

SYMPODIA 575

ovaries and tubes or testicles without vasa deferentia are to be
detected. There may be a cloaca opening posteriorly or not at all ;
in other cases the descending colon ends blindly. The diaphragm
may show a defect causing hernia ; the liver may be malformed ;
and the gall bladder may be absent, and so may the ductus venosus.

The state of the abdominal and pelvic blood vessels was fully
investigated by me in my research already referred to (Trans. Edinb.
Obstct. Soc, xxiii. 54, 1898). In it I showed that in most cases of
sympodia only one artery existed in the umbilical cord ; in one
instance (F. T. Majer, Diss, inaug., Tubingse, 1827) there were four
vessels, regarded as an umbilical vein, an umbilical artery, and two
omphalo-mesenteric vessels. In no case were there the three usual
funic vessels. Further, out of thirty-five cases in which details of the
abdominal vessels were given, there were twenty-five in which the
single artery of the cord arose directly from the descending aorta,
and passed towards the anterior abdominal wall in the middle line,
carrying the peritoneum with it, and then turned upwards to the
umbilicus. In most cases the absence of the hypogastric arteries
was noted. Putting all these facts together I came to the conclusion
that, in sympodia, the derivatives of the allantois (urachus and part
of bladder) and its vessels (umbilical arteries and vein) were usually
absent, and that the vessels of the cord were omphalo-mesenteric or
vitelline in origin. The further conclusion seemed to me warranted,
that in such cases the vascularisation of the placenta must be by the
vitelline vessels.

The teratogenesis of sympodia is a subject which has been fruitful
in ingenious explanations, and yet cannot be regarded as in any
degree settled. Indeed, it is far from being settled. I have not, for
instance, met with any explanation which explains the extraordinary
rarity, the almost complete absence, of any cases of sympodia in the
lower animals. Probably this is to be explained by differences in
ontogenesis, and possibly by peculiarities in the comparative embry-
ology of the allantois and placenta, but there is as yet little light
upon the subject.

The two leading characters of sympodia for which an explanation
has to be found are the fusion of the lower limbs and their rotation
from within outwards and from before backwards ; but the state of
the pelvis, the absence of the bladder, and the presence of one artery
in the umbilical cord also call for investigation. No theory yet
advanced satisfactorily accounts for them all. Of course a mechanical
compressing force suggests itself : it must drive the lower limbs
together and at the same time rotate them outwards, and it is
difficult to find in the muscles of the extremities, or in the uterine
walls, or in the surrounding amniotic membrane such a peculiarly
acting force. Further, it must be so exact in its action that only
like parts (bone, muscle, vessel, and nerve) shall unite with like.
Some light is thrown upon the problem by the study of the state of
the lower limbs and caudal end of the embryo in early antenatal life.
If the reader will look at Figs. 10 to 14 in this volume, it will
become perceptibly less difficult for him to understand how the

576 ANTENATAL PATHOLOGY AND HYGIENE

anatomical peculiarities may be produced ; and if he further keep in
mind that we have as yet no complete knowledge of the normal
ontogenesis of these parts of the embryo, it will become apparent to
him that with the acquisition of such knowledge some facts at
present inexplicable might become easily explicable. I refer
especially to the mode of the formation of the bladder, to the way in
which the lower limb buds evolve and rotate, to the relation of the
allantois and umbilical vesicle to the development of the placenta,
and to the meaning of a single artery in the umbilical cord.
Although I do not feel inclined to accept any one theory as a
satisfactory explanation, I am yet constrained to think that Dareste's
idea of an interrupted or arrested evolution of the caudal fold of the
amnion and consequent pressure upon the underlying parts of the
embryo produced thereby is essentially correct. Alt the same time it
seems to be a possible hypothesis to affirm that the lower limb-buds
may in a case of sympodia never actually exist separate, but are
there from the very first as a single limb-bud or process. In other
words, it is to my mind quite thinkable that the fused lower limbs
of sympodia develop fused, possibly because the parts that normally
lie between do not develop at all. It is unnecessary here, however,
to open up this subject further ; readers who are interested in these
most difficult problems of teratogenesis will do well to re-read
Chapters X., XL, and XII. with the special case of sympodia in view,
and to consult some of the more recent monographs on the subject,
such as those of Dareste (pp. cit., 2nd edit. p. 419, 1891), L. Bolk
(op. cit.), and Eabaud (Bull. Soc. philomat. de Paris, 9 s., v. 25-62,
1902-03; Bull, scient. de la France ct de la Belg., 6 s., vi. 436-460,
1903).

Monopodia.

The term monopodia has sometimes been used as synonymous
with sympodia, but if it be employed at all it ought to be restricted
to the cases in which there is a single lower extremity which shows
no indication that it had been derived from the fusion of two limbs.
Some cases of this kind occur in association with gastroschisis of the
lower lateral part of the abdomen ; to them I have referred in
Chapter XXVII. (p. 521). There are, however, other instances in which
there is no such imperfect condition of the abdominal walls. Taruffi
(op. cit., vii. 549, 1894) has placed these two groups (with and with-
out lateral gastroschisis) together, and, having found that in both
there was absence of one os innominatum, has called them instances
of mono-anileus apus. The cases in which there was no gastroschisis
were those reported by Ahlfeld (Arch./. Gynotk., xiv. 276, 1879), by
I. B. Budenz (Diss, inauy., Marburg, 1862), by A. F. Tassani (Gazz.
med. ital. lonib., 2 s., i. 274, 1848), by G. Sacchero (Ann. univ. di mcd.,
lv. 95, 1830), by G. Sangalli and by V. Colomiatti (cited by Taruffi,
Storia delta teratologia, vii. 551, 552, 1894). In this teratological state
the presence of a single artery in the umbilical cord would seem to
be a constant character ; the artery, also, which is present would
appear to rise from the aorta, and may. therefore, be vitelline rather

AMELUS 577

than allantoic in nature. Ideas regarding the teratogenesis of
monopodia have given rise to theories resembling those referred to
under sympodia.

Ectromelus or Amelias.

Under the names ectromelus and amelus are placed the cases in
which one or more of the four limbs is absent or reduced to a rudi-
mentary stump. Some teratologists would restrict the term amelus
or amdia to the cases in which all the four limbs were absent,
retaining ectromelus for those in which one, two, or three of the
extremities were affected ; but it is difficult to carry out the dis-
tinction. H. Meunier {Nouv. iconogr. de let Salpetr., x. 15, 1897) has an
interesting article on amelus, in which he gives bibliographical
references to a number of cases. He divides all the recorded
instances of it into perfect and imperfect specimens : in the former,
neither the pelvic nor the thoracic girdles show the slightest trace of
limbs, while in the latter there are indications of one or more limbs,
of the nature of stumps containing one or more bones and varying in
size and shape. Sometimes the stump can be seen to represent the
upper segment of the aborted limb ; at other times it represents the
lower segment (hand or foot).

Cases of perfect or complete amelus are comparatively rare
(Meunier has collected ten from literature), while incomplete amelus
is less rare, although still far from common. Good instances of the
former type are those reported by G. Joachimsthal (Die angeborenen
Verbildungen der obercn Extremitaten, p. 5, 1900) and S. Cholmogoroff
(Centrlbl.f Gyntih, xii. 819, 1888). In 1898 I had the opportunity of
examining Dr. Gemmill Thomson's case of incomplete amelus shown
at a meeting of the Glasgow Obstetrical Society. This infant, as may
be seen from Fig. 84, had practically no arms, while its lower limbs
were markedly dwarfed. Dr. Lindsay afterwards published a dis-
section of it, for the child died when six months old (Trans. Glasgow
Obstet, and Gyncec. Soc., ii. 16, 1898-1901). The dissection revealed
the presence of two fragments representing the upper limbs : there
was a humerus about 1 inch long on the right side, which fitted by a
concave head on to a convex glenoid facet ; while on the left side the
humeral rudiment measured If inch in length and had a normal
joint. The lower extremities were also anomalous. There was no
acetabulum on either side ; the femur consisted of two small car-
tilaginous nodules (the great trochanter and the lower epiphysis)
united by a fibrous band (the head and shaft) ; the fibula was absent ;
the tibia was strongly curved forward ; the tarsus was made up of
one large cartilage (astragalus — os calcis) and two small cubes of
cartilage ; and there were three metatarsals and phalanges in series
with them. There were many anomalies in the muscular system,
and these occurred not only in connection with the limbs but also at
a distance from them ; there were likewise vascular variations and
the sciatic nerve divided high up.

I have met with two other instances of incomplete amelus ; in
vol. n. — 37

578

ANTENATAL PATHOLOGY AND HYGIENE

both the subject was a kitten. In one the right forelimb was
apparently entirely absent, but dissection revealed a round mass of
cartilage in a small glenoid cavity (Interstate Med. Journ., vii. 367,
1900) ; this specimen I received from my friend, Dr. J. K. Drysdale.
Of the other case I was able to examine only the scapula and rudi-
mentary forelimb bone which were sent to me in 1902 by Dr. W. J.
Stevenson of London, Ontario ; the defective limb had been attached
to the side by skin and fascia.

Little is known of the antenatal symptomatology of amelus ; but it
is noteworthy that in Gemmill Thomson's case the mother felt no
foatal movements during her pregnancy, and a glance at Fig. 84 will
explain the reason. The malformation is not incompatible with

Fig. 84. — Case of Ectromehis or Thoracic Amelus.

postnatal life ; indeed many amelic infants have lived to advanced
ages, and have earned a living by the exhibition of their malfor-
mation and by the marvellous way in which they have adapted a
foot or a hand to do the work of all the limbs. In this relation I
need only refer to Miss Biffin, to Mr. Buckinger, to Thomas Pinning-
ton (" born without hands, feet, or legs — to be seen at the Goat -at
Charing Cross — he makes his own Pens, writes well, beats a Drum,
plays at Cards — he stands upon the Crown of his Head on the Bottom
of a Drinking-( Uass "), to Thomas Eoberts, to Antonio Morretti (" the
astonishing Podothaumanist "), and to Thomas Schweicker. In
recent years there have been the famous cases of John Chambert,
of Unthan, and of Charles Tripp (" the armless man " of Barnum's
Show).

H EMI M ELLIS 579

Hemimelus.

Hemimelus is the name given to those cases in which one or more
of the four extremities ends in a more or less tapering point or
stump resembling that left after an amputation at the ankle, wrist,
elbow, or knee; the proximal end of the limb is generally well
developed and complete, while the distal is absent or greatly reduced.
It includes nearly all the instances of so-called sjiontaneous amputa-
tion {vide Vol. I. of this Manual, p. 396 ; also pp. 150 and 186 of the
present volume). A typical example was brought under my notice
several years ago by Dr. T. B. Darling ; it is represented in Fig. 37
(p. 137); and I have already (ride Chapters X. to XII.) dealt at
some length with the whole subject of these malformations and their
mode of production.

Hemimelus is a comparatively common defect, at any rate in its
minor degrees, and it has a very large literature. Several hundred
cases might without much difficulty be collected together, the only
difficulty being that they are scattered and that indexes and lists
have been in the habit of entering them under various headings.
The Index Catalogue, for instance, gives some references under
Extremities (Abnormities and Deformities of) in vol. iv. pp. 439. 440,
444, 1883, and in 2 s., vol. v. pp. 231, 236, 240, 1900 ; others are found
under Amputations (Intrauterine), in vol. i. p. 244, 1880, and in 2 s.,
vol. i. pp. 318, 321, 1896; and others under Monsters, from defect or
malformation of limbs, in vol. ix. p. 420, 1888.

Hemimelus may affect one or more limbs in the same patient, and
while causing no disturbance in antenatal life may be the cause of
much inconvenience after birth. The rounded or conical stump, which
not infrequently carries several rudimentary digits, is of little value
as a hand or foot, and has to be supplemented by various mechanical
devices to ensure a certain degree of usefulness. In this deformity,
as in amelus, the individual usually acquires great dexterity with
the normal extremities (e.g. with the feet when the hands are
deformed).

Since I have already considered at some length the teratogenesis
both of amelus and hemimelus (Chapters X. to XII.), I need not
return to the subject here ; but I may refer to one or two recent
cases which throw some light upon the subject. Gasne's case, for
instance, was the child of a man who was in the active secondary
stage of syphilis at the time of his conception (Nouv. iconogr. de la
Salpetr., x. 31, 1897) ; in a case reported by E. Huet and C. Infroit
(ibid., xiv. 128, 1901) there was seen one of those intermediate types
between hemimelus and phocomelus, which are so troublesome when
we try to classify instances of deformity of the limbs ; and E.
Fournier (Stigmates elystrojjJiiques ele llieredo-syphilis, p. 158, 1898)
recorded several examples of hemimelus in individuals the subjects
of hereditary syphilis.

.80

ANTENATAL PATHOLOGY AND HYGIENE

Phocomelus.

The phocomelous foetus is one in which the proximal and not the
distal segments of one or more limbs is aborted. It contrasts
markedly on this account with hemimelus, in which the distal seg-

Fig. 85. — Specimen of Phocomelus, showing also an Umbilical Hernia.

ment is the affected part. The result is that a foot or a hand seems
to spring directly from the trunk, giving to the extremity or extremi-
ties the appearance of the flippers of the seal (phoca). This character
is well displayed in Fig. 85, which represents the specimen of phoco-

PHOCOMELUS 581

melus, affecting all the four extremities, which was described by-
Baxter Tyrie and J. W. Haigh in my journal in 1894 (Tcratologia,
i. p. 89, 1894) ; I have also reported and figured a case of the same
type of monstrosity in the first volume of this Manual (p. 351).

Possibly there is proof of the occurrence of phocomelus as far
back in the world's history as the early times of Babylonian rule, for
in the teratological records of Chaldea (vide my article in Tcratologia,
i. 133, 1894) there is the account of an infant whose hands and
feet are like four fishes' tails or fins. Of course, this may have been
nothing more than an instance of the more common malformation
known as syndactyly or webbing of the digits. At any rate, many
cases of phocomelus have been reported, and since the deformity does
not interfere with postnatal life, and may be used for exhibition pur-
poses, it is easily understood that some of them have gained notoriety.
I need only refer to Marc Cazotte or Pepin, to the " turtle woman "
of Demerara, and to Nicodemus, " the Nondescript Negro from Texas."
An interesting case of pelvic phocomely was reported by J. Boubino-
vitch (Compt. rend. Soe. de hi<>/., 10 s., v. 819, 1898) : the patient, a boy
of thirteen years of age, was the child of a man who was an inveterate
absinthe drinker ; at birth a loop of the umbilical cord was noted
surrounding the left lower limb ; the morbid anatomy of the case
showed that there were only three toes in one foot, and there was,
therefore, some degree of hemimelus as well as phocomelus.

Although external examination would seem to show that many
phocomelous foetuses possess nothing more than the distal segment of
one or more limbs, dissection generally reveals the presence of other
parts than those of the hand or foot alone. Thus in Baxter Tyrie's
case (he. cit.) there was in the upper limb an aborted glenoid cavity
to which a spindle-shaped bone (humerus) was attached by fibrous
cartilage ; the radius and ulna had no upper or lower epiphysial carti-
lages ; there was no proper elbow joint ; but the carpus and manus
were normal. The lower limbs were slightly better developed, for
they showed normal hip joints, each with a ligamentum teres ; the
femora, however, were stunted ; and there was no knee joint, the
tibia and fibula being simply united by fibrous tissue to the thigh
bone of the same side. In my case of phocomelus, figured in Vol. I.
(p. 351), the bones of the thigh, leg, upper arm, and forearm were
indicated solely by tiny cartilaginous rudiments ; and in one of
Joachmisthal's cases (op. cit., p. 10) skiagrams showed no bony con-
nection between the skeleton of the hand and that of the shoulder
girdle.

The phocomelous monstrosity may exist alone or it may be
associated with other malformations. Thus, in Baxter Tyrie's speci-
men, there were various muscular anomalies — an umbilical hernia, an
inguinal hernia on the left side, undescended testicles, a penis with
complete absence of corpora cavernosa, a diaphragmatic hernia on the
left side, a cleft palate, a perforation in the interventricular septum
of the heart, and a spina bifida in the dorso-lumbar region. In my
specimen there was defective ossification of the frontal bones.

Some confusion has been introduced into the study of cases of

582 ANTENATAL PATHOLOGY AND HYGIENE

phocomelus by the employment of the term micromelus as a synonym.
It is better to allow this word to signify any dwarfing of the limbs
whatever, including the results of foetal bone disease (achondroplasia),
and to reserve the names amelus, hemimelus, and phocomelus for the
varieties which have been described above. At the same time it is
not possible, in my opinion, to separate exactly the deformities of the
limbs due to foetal diseases and the malformations due to arrested
embryonic ontogenesis ; there is a certain amount of overlapping
(vide Vol. I. p. 352).

Absence of Individual Bones of the Limbs.

Cases of congenital absence of the tibia are occasionally met with.
Sometimes the whole bone is absent ; at other times it is only a part
that is wanting, generally the lower end. The anomaly may be uni-
lateral or bilateral. It is generally associated with other defects,
such as absence of the patella, imperfect development of the lower
end of the femur, thickening of the fibula, cutaneous scars (amniotic ?),
club-foot, and digital malformations. Many valuable monographs on
absence of the tibia have been published ; I may refer specially to
Ludwig von Muralt's article in the Bibliotheca Mcdica (Abth. C,
Heft 5, 1896), to Mario Motta's (Arch, di ortoped., xiv. 65, 152,
1897), and to J. S. McLaren's (Journ. Anat. and Physiol., xxiii. 598,
1888-89).

Absence of the fibula is more frequently met with than absence of
the tibia. P. Mazzitelli (Arch, di ortoped,, xv. 310, 1898) gathered
together and analysed 120 cases, finding that the defect was bilateral
in 37'7 per cent, and unilateral in 62-3 per cent., and that it was
complete in 697 per cent., partial in 24-7 per cent., and mixed
(partial on one side, complete on the other) in 3'6 per cent. Appar-
ently the male sex is more liable to this malformation than the
female (as 69 to 23). The leg is shortened, shows a marked curve
with convexity anterior and witli a depressed scar over it, and has a
foot in the position of valgus or equino -valgus. Other malformations
may occur in association, such as absence of one or more toes, of the
foot, of the femur, or of the tibia. Fracture of the tibia may also
occur as a complication. Eecent articles on this subject are those of
H. Eheinheimer (Diss, inaug., Wiirzburg, 1898), of Cotton and Chute
(Trans Arner. Orthop. Assoc, xi. 316, 1898), of H. L. Taylor (Pedia-
trics, viii. 297, 1899), and of E. Froelich (Gaz. hcbd. de mid,, n.s., v. 49,
1900). A good account was given by E. Kirmisson in his TraiU des
maladies cfyirurgicales d'originc congenitalc (p. 551) in 1898.

Complete or partial ahsencc of the femur is very uncommon as an
isolated deformity. H. Grisson, however, reported a case in which
only the upper and lower ends of the left femur existed ; the leg and
foot were normal in length and structure (Arch.f. klin. C'hir., xlix.
252, 1891). E. A. Cleeman (Pediatrics, vi. 499, 1899) has met with
congenital absence of both femurs ; a similar observation was put on
record by W. E. Williams (Trans. Path. Soc. Pond., xxxv. 317, 1883-
84). Other cases were those of F. Lange (Deutsche Ztschr.f. Chir.,

GENU RECURVATUM 583

xliii. 528, 1896), of G. Lotheissen (Beitr. z. klin. Ghir., xxiii. 139,
1899), and J. H. Morgan (Trans. Ghir. Soc. Zona7., xvi. 259,
1882-83).

Absence of the patella on one or both sides is another malformation
of the leg bones which has of recent years been shown to be less rare
than was supposed. Lane (Lancet, i. for 1858, p. 63) noted an in-
stance of it affecting the right side in 1858 ; Labbe (Bull. Soc. de chir.
de Paris, 3 s., iii. 508, 1874) saw a bilateral example of it ; and W.
Korte reported two cases (Deutsche Ztschr. f. Chir., vii. 69, 1876).
More recent records are those of Bousquet (Bull, ct mem. Soc. de chir.
de Paris, n.s., xi. 354, 1885), of M. Salaghi (Arch, di ortoped., xi.
222, 1894), and of G. Thocas and G. Potel (Rev. d'orthop., vii. 342,
1896). In individuals suffering from this defect it is easy to feel the
condyles of the femur, the groove between them and the anterior
tuberosity of the tibia; there is also a transverse fold of skin on
the front of the knee joint during extension : and there is sometimes
dislocation forwards of the head of the tibia. The deformity known
as genu recurvation is associated with an apparent or a real absence
of the patella. I met with a case of bilateral genu recurvatum in
July 1899. It was that of a female infant, ten days old. The cranial
ossification was good and the muscles were everywhere well de-
veloped, but there was considerable lumbar cyphosis with compen-
satory lordosis higher up ; and the femora and humeri appeared
relatively short. There was hyperextension of both knees ; instead
of the normal flexion at the knee, each leg formed with the thigh an
angle open to the front. There was, therefore, no popliteal space in
the sense of a depression ; but in the position thereof was a projection
recognised to be that caused by the femoral condyles protruding
backwards behind the heads of the tibia and fibula. In front were
several folds of skin due to the relaxation of the tissues caused by
the forward flexion. The upper end of the tibia and fibula lay dis-
located forwards in front of the lower end of the femur; and no
patella could be felt, nor did the Eontgen rays reveal one. The for-
ward flexion of the knees, which was accompanied by a certain
amount of external lateral displacement, could be increased by
manipulation ; but the limbs could not be entirely straightened with-
out anaesthesia. The infant was the result of the first full-time
pregnancy of a mother who had on a previous occasion had an
abortion followed by pelvic abscess. The labour was long and the
liquor amnii was scanty. The parents were first cousins, and their
marriage was not the only one between blood relations in their
family. In this case there was some improvement under treatment :
and Bilhaut (Ann. de chir. ct d'orthoped., x. 257, 1897) in a similar
case obtained a satisfactory result after ten years. Many important
articles have appeared on the subject of genu recurvatum and its
treatment during recent years (vide the Index Catalogue, 2 s., viii.
762, 770, 1903).

Absence of the radius occurs more commonly than that of the ulna,
and it is nearly always associated with club-hand. I have met with
three cases in which these malformations occurred together. The

584 ANTENATAL PATHOLOGY AND HYGIENE

first was a stillborn premature male infant given to me for examina-
tion by Dr. T. B. Darling in 1890. Both the feet and the hands
were clubbed, and there was absence of the radii ; the thumb was
also wanting on the left side ; and there was non-descent of the
testicles and imperforate anus. This was specimen No. 21 in my
list. The other two cases were the offspring of healthy parents, but
the mother had defective formation of the short muscles of both
thumbs. She had two normal male infants, next she aborted, and
then she gave birth to a hydrocephalic female foetus with absence of
the right thumb and radius and club-hand on that side (Fig. 62,
p. 360). In subsequent pregnancies she had two normal infants, an
anencephalic female infant, and finally a premature female infant
with absence of both radii and the right thumb and with commencing
hydrocephalus. I described and figured these foetuses in an article
published in 1900 (Amer. Journ. Obstet, xli. 577, 1900).

W. Kiimmel (Bibliotheca Medica, Abth. E., Heft 3, 1896) has
collected together the literature of this malformation and has also
described four new cases ; Kirmisson also gave a good account of the
deformity in his Maladies Chirurgicales (p. 460, 1898). The radius
may be partly or entirely absent, and the latter state is the more
common ; both sides of the body may be affected, but the unilateral
variety is a little more common, although neither one side nor the
other predominates. The hand is turned to the radial side of the
arm, the thumb with its metacarpal bone and some of the outer
bones of the carpus are absent, and various muscles of the arm and
hand are wanting. When the radius is only partly absent, it is
usually the lower end that is defective ; but a few cases have been
reported in which the upper end was affected, and in them there was
no club-hand. It is necessary, of course, to remember that club-hand
does not always accompany radial defects, and also that it may occur
apart from these deformities (e.g. in absence of the ulna) and even in
individuals otherwise well formed. When there are some associated
malformations they do not necessarily affect the limb or limbs in
which the radius is absent, but may occur in distant parts (e.g. atresia
of the anus, defect of the ear, hare-lip, cleft palate, and horse-shoe
kidney). I have a skiagram of a case of absence of radius which I
got from Professor Jardine in 1897; there was a small meningocele
in the occipital region as well as absence of the thumb.

Absence of the ulna is less common : Kiimmel (loc. cit.) was able to
collect only thirteen cases from literature, while he had gathered
sixty-seven instances of defect of the radius. Among Kummel's
thirteen cases was that reported by J. H. Pringle (Journ, Anat. and
Physiol., xxvii. 239, 1892-93) in which the deformity was bilateral.
There commonly is deviation of the hand to the ulnar side, and move-
ment at the elbow joint is interfered with. In the case of a man
whom I examined in Professor Chiene's Ward in the Edinburgh
Royal Infirmary in October 1902, the head of the right radius was
enlarged and displaced upwards and outwards ; the internal condyle
of the humerus was also large ; the head of the ulna was defective ;
and the fourth and fifth digits of the right hand were absent, while

ECTRODACTYLY 585

the little finger of the left hand was double. Symmetrical ulnar
defect is not common, and associated malformations are not so often
met with as in cases of absence of the radius. In Gessner's case,
however, in which the right ulna and corresponding fingers were
absent, there was a diaphragmatic hernia and a uterus unicornis
{Ccntrlbl. f. Gyncik., xviii. 824, 1894); while in E. G. Collins' patient
there was absence of one fibula, an imperforate anus, club-foot, and a
defective penis {Chicago Clin. Rev., iv. 11, 1894-95).

Absence of the humerus, as an isolated defect, would appear to be
very rare. J. C. Gittings {Arch. PediaL, xv. 927, 1898), indeed,
reported a case in which there was apparently no humerus, but there
was a very rudimentary radius and ulna and a defective carpus, so
that it seemed that it might rather be classed as an instance of
phocomelus than of absence of the humerus.

Miscellaneous Defects of the Limbs.

I have only space to name the many minor malformations of the
extremities which may be met with. Some of them, such as club-
foot in its different varieties, may be regarded as the result of
pressure during the later months of antenatal life or as due to fcetal
diseases ; but others, such as absence of digits, must be referred to
the embryonic period of existence.

Club-foot is so fully considered in so many easily accessible text-
books that I need do no more than mention it, pointing out that
it differs essentially from club-hand ; for the latter is more than a
displacement of parts, being due to actual defects and associated
anomalies {e.g. absence of radius or ulna), while the former is merely
a distortion, and is accompanied by other anomalies only in com-
paratively rare cases.

Ectrodaetyly is the term applied to defects of the digits, such as
complete or incomplete absence of one or more fingers or toes. In
some cases all the digits of one limb may be wanting (total ectro-
daetyly), in others we find one or more digits in a normal state
(partial ectrodaetyly). Sometimes the fingers or toes that are present
are shorter than normal {brachydaetyly), and the shortness may be
due either to absence of one segment or to diminished size of all the
segments ; very often the digits that are left are joined together by
webs of skin {syndactyly). Both brachydaetyly and syndactyly may
also exist apart from ectrodaetyly. Amniotic bands have not infre-
quently been noted in connection with ectrodaetyly (Fig. 36), as I
have pointed out already in discussing the causation of malformations
(p. 184). An interesting variety of this deformity is called bidactyly
or the "lobster claw " anomaly: in it two digits alone are left, usually
the thumb (or great toe) and the little finger or toe, and thus the
appearance of the two claws is produced. I have in my possession a
good skiagram of this malformation which was given me by the late
Dr. George Elder in 1899 ; the patient was a man whose left hand
carried a thumb and a little finger with two phalanges, all the inter-
mediate digits being absent. A recent case in which all the extremi-

586 ANTENATAL PATHOLOGY AND HYGIENE

ties were affected was that of Le IJoy des Barres and Gaide (Gaz. d.
hop., lxxvii. 697, 1904).

Sometimes there is a fissure dividing a hand or a foot into two
nearly equal parts ; this constitutes the deformity called "split-hand"
(Spalthand) and " split-foot " (Spaltfuss) by Kiimmel (he. cit., p. 47).
The middle finger with its metacarpal bone or the middle toe with
its metatarsal is absent in this deformity; sometimes the remaining
pairs of digits are united by skin folds (syndactyly). All the
extremities may exhibit this anomaly. It may also be transmitted
hereditarily as in the curious case related by Kiimmel (loc. cit., p. 21):
the history given was that the great-grandfather (on the father's side) of
the little patient with the split-hand had wounded the middle finger
of his left hand to save himself from military service ; his son had no
fourth finger on the left hand; four of his children had defects of
fingers ; and the patient (his great-granddaughter) had no fourth
finger or metacarpal bone on her left hand. This is an example
of hereditary transmission, but it is doubtful if it can be alleged as
an instance of an acquired defect becoming hereditary.

Absence of thumb (radial ectrodactyly) and its metacarpal is
usually associated with absence of the radius, as has been stated
already ; but it may also occur as an isolated defect, as in P.
Ehrhardt's case (Rev. d'orthop., i. 205, 1890). Lateral deviation of the
thumb, constituting a sort of club-thumb, has been observed by G.
Joachimsthal (Ztschr.f. orthop. Chir., ii. 441, 1892-93) ; Kiimmel (loc.
cit., p. 19) saw a similar defect in a patient in whom the upper part
of the radius was absent.

Dislocations of the hip, knee, shoulder, and elbow may be met
with at birth, and have been referred to already (Vol. I. p. 49). Their
causation is obscure, but some of them at least are probably embryonic
in origin, if we may judge from the malformations of the joints and
the occurrence of associated anomalies which are undoubtedly tera-
tological. An enormous literature has of late years grown up round
congenital dislocation of the hip, more especially in connection with
operative interference.

Double Hand and Double Foot.

Under the names dichirus and dipus have been described a few
cases of apparent duplication of the terminal segment of a limb.
Such deformities ought strictly to be discussed among the poly-
somatous or double terata, but since they lie on the border line
between the two great groups of monstrosities I may refer very
briefly to them here.

In November 1892 I showed to the Edinburgh Obstetrical
Society a male infant with a bifid hand (Trans. Edinb. Obstet. Soe.,
xviii. 1, 256, 1893). It was the right hand that was affected, and
its appearances are shown in Fig. 86. The hand hangs down in a
helpless fashion, reminding one of the "wrist-drop" in lead-poisoning,
and the arm tends to be pronated and half-flexed at the elbow joint.
There are seven digits, divided into two groups, a radial and an ulnar,

DOUBLE HAND 587

by an interdigital sulcus : the ulnar group consists of four digits, each
made up of three phalanges, and these represent the little, ring,
middle, and index fingers ; the radial group consists of three digits,
probably the little, ring, and middle, each having three phalanges.
The elbow joint was abnormal ; there was pain and creaking on
manipulation, and it seemed as if the ulna were dislocated inwards.
The deformity is to be looked upon as a case of fusion of two hands
with absence of the thumbs; it is as if the right and left hands were
welded together, the index of the one coming into juxtaposition with
the middle finger of the other.

Shortly after I published this case I received a letter from Pro-
fessor Dwight drawing my attention to his article on " Fusion of
Hands " (Mem, Bost. Soc. Nat. Hist., iv., No. 10, 1892). In this article

FlG. 86.— Case of Double Hand (Dichirvs).

a case was described very similar to mine but with a dissection of the
parts after death. In it there was no radius in the forearm but two
bones which Dwight regarded as two ulnae, each with an olecranon
process ; the lower end of the humerus was also double, consisting of
an internal condyle on each side and two fused external condyles in
the middle line. On re-examining my case I came to the conclusion
that a similar state of the bones of the forearm existed in it.

In addition to Dwight's example of double hand there have been
a few others, among which I may mention J. Jardine Murray's (Med.-
Chir. Trans., xlvi. 29, 1863), C. Fumagalli's (Ann. univ. cli med,, cexvi.
313, 1871), F. Jolly's (Internat, Beitr. z. wissensch. Med. . . . Fcstschr.
B. Virchow/i. 617, 1891), and that of M. Giraldes (Maladies chirur-

588 ANTENATAL PATHOLOGY AND HYGIENE

gicales des en/ants, p. 42, I860). Double foot (dipus) is also met with,
but rarely apart from sympodia ; a probable instance was that re-
ported by B. E. Brodhurst (Lectures on Orthopedic Surgery, p. 55,
187G). I also saw a case in Professor Chiene's Ward in the Edinburgh
Royal Infirmary in 1894 that was possibly of this nature. It was
that of a little girl, four years of age, who had five fingers on each
hand, the first digit in each having three phalanges. There were
seven toes on each foot, the first and second being united by a fold
of skin in both ; the third toe in both had only two phalanges. The
heel of the right foot was drawn up by the tendo Achilles, and there
was also marked inversion ; the left foot was less deformed than the
right, but the left leg was much shorter than the right, and there was
enormous thickening of the upper part of the fibula. It was a
remarkable fact that the mother of this child had also seven toes
on each foot, that two of her sisters had six toes and five fingers,
and that the grandfather had the toe-nail of the hallux double. I
am of opinion that this little girl showed a condition of fusion of two
imperfect feet and that the so-called fibula may have been a second
tibia.

Such malformations are very difficult of explanation, whether we
regard them as instances of fusion of hands and feet or as Polydactyly.
The theory of atavism is inadequate ; and it is almost impossible to
accept the idea that the double hand or foot represents united twins
which have lost all other signs of duplicity. These cases are even
more inexplicable than double penis (diphallus) and double bladder.

Polydactyly.

In 1893 I contributed an article on six cases of Polydactyly (Arch.
Pediat., x. 573, 1893). Three of the cases occurred in the same family,
and they may be taken as typical of the common form of Polydactyly.
The mother and father had no digital deformities, but all their three
children (a boy and two girls) exhibited sexdigitism. The boy had
six toes on each foot : on the right side the supernumerary toe, with
three phalanges, was attached to the outer side of the distal end of
the fifth metatarsal, and closely resembled the fifth toe and lay
parallel with it ; on the left side the extra digit also had three
phalanges, but it articulated with the proximal phalanx of the fifth
toe instead of with its metatarsal bone and it projected outwards
from the foot. In one of the girls (next in age to the boy) there
were also six toes on each foot : her right foot clearly resembled her
brother's left, the extra toe being attached to the outer side of the
proximal phalanx of the fifth digit but without projecting outwards
so markedly ; her left foot, however, showed an accessory toe inter-
calated between the fourth and fifth and shorter than either ; her left
hand had a small nodule (rudimentary digit ?) attached to the junc-
tion of the proximal phalanx of the little finger with the correspond-
ing metacarpal bone. The third child (a girl) had a right foot
resembling her brother's left and her sister's right, the extra digit
being attached to the outer side of the proximal phalanx of the fifth

POLYDACTYLY 589

toe ; on the left foot there was also a supernumerary toe, consisting
of two phalanges, fixed by a narrow pedicle to the outer side of the
foot at the level of the proximal phalanx of the fifth toe.

These cases may be taken as characteristic instances of the com-
moner types of Polydactyly. Barer types are found in the instances of
ten, nine, eight, and seven digits which bave been from time to time
reported and which are gathered together by Taruffi (op. eit,, iii. 425,
543, 1885 ; viii. 487, 1894) and other teratologists and pathologists.
Various anomalies of the metacarpals, metatarsals, carpus, and tarsus
may be associated with Polydactyly, and the extra digits themselves may
be webbed or shortened or otherwise defective. The hands are more
often affected than the feet ; and the supernumerary fingers and toes
are generally attached to the ulnar and fibular sides of the limbs, less
often to the radial and tibial, and rarely are they intercalated between
the normal digits. Male infants are more often affected than female ;
and sometimes more than one limb in the same individual shows
Polydactyly.

Various degrees of bifidity of the thumb and great toe may be
met with, from a simple division of the nail into two (as in a little
boy whom I examined in 1892) to a complete doubling of the digit.
A teratological state which may be mentioned here is the three-
phalanged thumb : this condition of hyperphalangy or triijludangy, as
it is called, presents one of the most puzzling problems which await
solution by the antenatal pathologist. Four phalanges have been
found in the index and middle digits ; so that it is not possible to
explain away the triphalanged thumb by calling it a duplicated
index.

The teratogenesis of the anomalies of the limbs that are char-
acterised by excessive formation or development is surrounded with
great difficulty. The amniotic theory has to be forced beyond reason
to include such malformations as Polydactyly. Of course it may be
held that an amniotic band or cord may so press upon a digit or upon
the foot or hand in its undifferentiated state as to divide it into two
or more parts ; but it is difficult to fit the facts to such a view, for
amniotic defects are very rarely associated with supernumerary digits,
and the occurrence of syndactyly as a complication of Polydactyly
introduces another difficulty not easy of explanation.

One fact is widely known and must be taken into account. I
refer to the hereditary nature of these malformations. Many instances,
some historical in their importance, may be brought forward in proof
of this assertion ; and not through two generations but through many
the deformity may be traced in certain cases. For this reason one is
led to refer the cause of Polydactyly back to the germinal period of
antenatal life, and to look for an explanation in the phenomena of
atavism. Here, also, there are manifest difficulties, and in the mean-
time it is perhaps best, or at least safest, to speak of a tendency to
excessive formation existing in the germ, a sort of ill-regulated or
uncontrolled gemmation of the distal extremities of the limbs,
associated perhaps with anomalies in nutrition. The literature of
the various malformations of the limbs is enormous. The reader will

590 ANTENATAL PATHOLOGY AND HYGIENE

find many references in the Index Catalogue under such various head-
ings as Fingers and Toes (vol. iv. 979, 1883; 2 s., v. 797, 1900),
Extremities (vol. iv. 4:39, 440, 444, 1883 ; 2 s., v. 2:32, 236, 240, 1900),
Foot (vol. v. 65, 1884; 2 s., v. 926, 1900), Hand (vol. v. 816, 1884:
2 s., vi. 725, 1901), Monsters (vol. ix. 420, 1888), etc.

Malformations of the Fcetal Annexa.

It is difficult to decide where to place the malformations of the
foetal annexa in a Manual of Antenatal Pathology. Are they
foetal, embryonic, or germinal in origin ? Some of them are un-
doubtedly foetal and have been considered in the first volume of this
work (p. 400). Others must be looked upon as germinal, for it must
not be forgotten that some of the foetal annexa, e.g. the amnion and
the chorion, precede the embryo in the order of development ; for
this reason amniotic anomalies and chorionic degenerations may take
their origin at a time in antenatal life anterior to the appearance of
the embryonic rudiments in the embryonic area of the impregnated
ovum. But there are yet others which may fairly be believed to be
produced in the embryonic period ; and among these may be named
placental anomalies, persistence of the umbilical vesicle and its
vessels, and malformations of the umbilical cord. It will be remem-
bered that in dealing with teratogenesis I described at considerable
length anomalies of the amnion (narrowness, adhesions, bands, etc.)
and their effects or supposed effects upon the developing embryo, so
that I need not consider them again either here or under the path-
ology of the germ. Malformations of the early chorion, such as
hydatid mole, will be briefly referred to under germinal pathology.
For these reasons I take up here only the anomalies of the placenta,
umbilical cord, and umbilical vesicle ; these form a sort of natural
group, somewhat closely related to each other.

Placental malformations are probably less rare than is usually
stated, for many cases pass unnoticed through want of careful examina-
tion. One of the less common types is that known as the dimidiate
or duplex placenta. I received in August 1904 a specimen of this kind
from Dr. Lewis Hughes of Dowlais, Glamorganshire. The placental
mass consisted of two parts one of which was the size and shape of
an ordinary placenta, the other was a little less in size, had an irregular
shape, and was thinner than usual ; the cord was inserted along the
line of union of the two masses ; and in the neighbourhood of the
smaller part there were two smaller placental lobules (succcnturiaie
lobes). There was only one infant, and Dr. Hughes had some diffi-
culty in detaching the second and thinner part of the afterbirth during
the third stage of labour. On the foetal surface of the larger part a
persistent umbilical vesicle could be recognised. I have seen another
almost perfect specimen of placenta duplex, that shown to the Edin-
burgh Obstetrical Society by Dr. James Eitchie in 1895 (Trans., xx.
88, 1895). In both these instances the single cord had an insertion
into the membranes. Another specimen was shown by Dr. Graily
Hewitt in 1866 (Trans. Obstet. Soc, Lond., viii. 337,1867); but in

PLACENTAL MALFORMATIONS 591

that instance the cord divided into two branches, which were inserted
far from each other into the two placental masses, near their margins.

Much more common than the dimidiate placenta is the presence
of one or more small lobules of placental substance attached to the
membranes near the chief mass ; these extra lobules are called suc-
centuriate. They are supplied with small branches of the umbilical
vessels which pass to them from the placenta in the membranes.
Their clinical significance is the risk of their being left in the uterus
after the third stage of labour ; their pathological or teratological
significance is not obvious.

In some cases the placenta has the form of a horse-shoe or of a
kidney instead of being discoidal, there being a defect at one part
or another causing a sulcus between the two large segments of the
organ. Rarely the placenta has had areas in it showing simply the
chorion with no placental substance, and yet the outline has been
regular ; to this variety the name placenta fenestrata has been given.
Another anomaly is called the placenta membranacea ; in this the
organ is widely diffused and thinned out, and it may occupy as much
as four-fifths of the whole area of the chorion. I have met with one
or two instances of this, but of less extent than that indicated above.
The anomaly is apt to lead to haemorrhage in the third stage of
labour. Another anomaly is that in which the whole placental tissue
is localised to a comparatively small area of the surface of the
chorion ; the placenta then takes a peculiar conical form (placenta
eon tea).

One of the most interesting of the placental anomalies is that
in which a strip or margin of the organ all round the circumference
projects beyond the line of attachment of the membranous sac ; to
this arrangement the name placenta marginata or cireumvallata is
given. In a case of this kind which I saw in 1899 the space within
the membranes near the placenta was much diminished ; the child, a
male, was small for the age of pregnancy arrived at (eight and a half
months), and he has been a somewhat delicate child ever since.
During the last four months of pregnancy the mother gained flesh
and weight in a remarkable manner quite in contrast with her ill-
nourished state in the early months. Some interesting articles on
this placental anomaly have been contributed by Giglio (Ann. di
ostet., xvii. 1, 1895), Curatulo (ibid., xix. 505, 1897), Ferroni (ibid.,
xxi. 627, 1899), and many others. It is probably due to abnormali-
ties in the formation of the decidua refiexa.

Other placental anomalies, such as adhesion to the foetus and
abnormal insertion of the cord, are rather to be regarded as morbid
states of the amnion and cord than of the afterbirth itself. The
literature of the whole subject is large and may be found in the
Index Catalogue, vol. xi. p. 340, 1890.

Malformations of the umbilical cord include great length and
marked shortness. Any cord measuring more than 100 cms. (about
40 inches) may fairly be regarded as teratologically long; and
Taruffl (op. cit., v. 195, 1889) has collected together a considerable
number of records of such cases. The effect of a long cord in

592 ANTENATAL PATHOLOGY AND HYGIENE

causing convolutions round the foetus, and the possible effect of the
convolutions in causing amputations of foetal parts, have been already
considered (p. 150). A teratologically short cord is one measuring-
less than 8 inches (20 cm.), and several cords with measurements
of 4 or even 3 inches have been recorded (Taruffi, op. tit., v. 230,
1889). Cases also are not wanting in which no recognisable cord
at all was to be found, the vessels passing directly from the placenta
in the membranes to the margins of an opening in the abdomen of
the foetus (vide under gastroschisis, pp. 172 and 517).

In some cases the funis ceases to have a cord-like appearance
a few inches from its insertion into the placenta, and its vessels pass
outwards to be attached to the foetal surface of the afterbirth like
the spokes of an umbrella; this is called the parasol insert inn of the
cord. In other cases the whole cord enters the placenta at or near
to its margin, giving rise to the marginal insertion or battledore
'placenta. In yet other cases the cord breaks up before it reaches
the placental margin, and its vessels run in the membranes for a
small or great distance ; this is known as a velamentous insertion of
the cord. I have met with several instances of the two latter
anomalies, both in private and in Maternity Hospital practice ; they
may be associated with hydramnios and with toughness of the foetal
membranes. Various more or less ingenious suggestions have been
made as to the mode of production of the velamentous type of funic
insertion by B. S. Schultze (Jcnaischc Ztschr. f. Med. u. Naturw., iii.
198, 344, 1867), M. Stern (Diss, inaug., Marburg, 1873), F. Ahlfeld
(Ber. u. Arb. a. d. geburtsh.-gynak. Klin, zu Marburg, ii. 13, 1885), and
others ; but there are still many difficulties in the way of a complete
explanation.

I have met with at least two instances of umbilical cords con-
taining only a single artery. In some cases this anomaly is due to
fusion of the two umbilical arteries just inside the umbilical ring
before they reach the cord, and in others it is found that the one
umbilical (hypogastric) artery is much larger than the other in the
pelvis, and that consequently the smaller one never reaches the
umbilical ring. In yet another group of cases, however, there is
complete absence of one of the arteries in the cord. This occurs
specially in association with sympodia and with defective develop-
ment of the bladder. The question arises whether the single artery
that remains is really an umbilical (allantoic) vessel or a persistent
omphalo-mesenteric (vitelline) one. There is much to be said in
favour of the latter view, for the single artery is found arising from
the common iliac or from the aorta itself. On this subject I have
dwelt at considerable length elsewhere (Trans. Edinb. Obstet. Soe.,
xxiii. 54, 1898).

The arteries in the cord may unite together so that only one
arrives at the umbilicus of the foetus, or they may form an anasto-
mosis in the cord. The umbilical vein is rarely anomalous, but some
cases are on record in which it divided into two in the cord. Its
mode of termination within the abdomen is not always normal, for it
may pass into a tunnel in the liver caused by the conversion of the

ANOMALIES OF UMBILICAL CORD 593

longitudinal groove into a canal, or it may open into the vena cava
inferior, or into the iliac or splenic veins, or even into the vena
cava superior or the right auricle.

In rare cases the omphalo-mesenteric or vitelline vessels may be
found in the cord of the full-time foetus, and may be seen passing to
a persistent umbilical vesicle on the foetal surface of the placenta.
By close inspection I have several times found traces of the umbilical
vesicle in the full-time placenta, generally in the situation indicated
by the direction of Schultze's fold ; but I have only met with one
case in which the vitelline vessels were present in the cord. The
placenta is figured in Vol. I. of this Manual (p. 155). There were
four accessory vessels in addition to the two umbilical arteries and
the vein, and these four vessels (two arteries and two veins) were
apparently carrying blood up to the time of birth. Unfortunately
the state of the abdominal blood vessels in this infant was not
ascertainable. The literature of anomalies of the umbilical cord and
its vessels is to be found in the Index Catalogue, vol. xiv. 948, 1893 ;
the whole subject, also, is treated in a masterly fashion by Taruffi
(op. cit., v. pp. 188-271, 1889).

With this short sketch of the anomalies of the placenta, cord,
and vitelline vessels I bring to a close the long series of malformations
which may affect various regions and structures of the body. I have
not made a complete enumeration of them all, although I have tried
to give some facts relating to all the more typical ones, and have
generally indicated some article or book in which full details might
be found. I have all along had in mind rather the illustration of
general principles than the minute description of individual types ;
and I have specially tried to cite examples demonstrating the great
principle of arrested development in the causation of the monstrosi-
ties which occur in the single foetus. I have endeavoured to exclude
any double monstrosities, for they will be referred to under Germinal
Pathology; but it was not possible always to avoid some reference
to such intermediate types and connecting links as polymastia, Poly-
dactyly, double penis, etc. They lie on the border line between
the monosomatous and the polysomatous terata, between arrested
developments and anomalies showing apparent excessive develop-
ment ; and it is difficult to assign to them their proper place. This
remark applies also to the curious condition known as heterotaxy or
transposition of the viscera ; it differs from all other malformations
and monstrosities in that it consists of nothing more than a reversal
of the right and left sides of the body ; it is not certain whether it
is due to causes acting on the embryo as it is formed in the embry-
onic area, or to influences coming into play earlier, and affecting the
germ. At any rate, I place its consideration immediately after the
monosomatous terata, between them and the double monsters and
other morbid developments of the germinal epoch.

vol. n. — 38

CHAPTER XXX

Heterotaxy or Transposition of the Viscera : Definition, Synonyms, Historical
References, Literature, Illustrative Cases, Morbid Anatomy, Varieties
(Total, Partial, Irregular), Associated Malformations, Transposition in
Animals and in Double Terata, Clinical Aspects, Diagnosis, Prognosis,
Teratogenesis : Antenatal Diagnosis of Monstrosities : Retrospect and
Conclusions.

Heterotaxy.

Heterotaxy may be defined as that congenital anomaly in which the
relation of the parts of the body is so altered that the organs which
ought to be on the right side are found on the left, and conversely.
There is an inversion of parts as in a mirror, so that if, in such a case,
the thoracic and abdominal cavities be laid open their contents
appear as if seen in a glass. There is, therefore, not simply the
presence of right side organs on the left side and vice versd, but
organs which are single and median in position have their normal
asymmetry so changed as it would be when reflected by a mirror.

Heterotaxy has various synonyms, including " transposition,"
" inversion," and " lateral inversion of the viscera," " situs viscerum
inversus," " situs mutatus," " situs partium perversus," " dislocatio
viscerum lateralis," etc. " Transposition," " inversion," and " dis-
location " are not very exact terms, for they convey the idea of an
actual displacement of parts such as does not occur; under the
circumstances the most satisfactory term is heterotaxy, introduced by
I. (I. Saint-Hilaire in 1836 (Histoire des anomalies, ii. p. 3, 1836) ;
this name, being derived from rdfyg, arrangement, and mpoj, (liferent,
simply means an irregular disposition of parts. It has been sub-
divided into toted heterotaxy, when all the body-cavities and their
organs are transposed, and partial heterotaxy, when only the thoracic
or the abdominal viscera show the changed relations.

Historical references to cases of heterotaxy go back as far as the
time of Aristotle, who clearly observed it in animals, stating that he
had found the liver on the left side and the spleen on the right.
The references are in his History of Animals (Bk. i. c. xiv. par. 5;
Bk. ii. c. xii. par. 4), and in his treatise on Generation (Bk. iv. c. iv.
par. 3) ; and he went on to declare that this displacement might
occur in animals otherwise normal. A long silence followed Aris-
totle's statement, and then, in the seventeenth and eighteenth
centuries, cases began to be reported, but almost always as the result
of post-mortem examinations. This need cause no surprise, for the

594

HETEROTAXY 595

anomaly consists solely of the displacement of internal organs ; and it
was not till the nineteenth century, when physical diagnosis by per-
cussion, auscultation, and skiagrams began to be perfected, that the
recognition of such internal abnormalities became possible during life.
Marie de Medicis, wife of Henry iv. of France, and mother of
Louis xiii., is believed, on the authority of the younger Eiolan
(1G49), to have been the subject of transposition of the viscera, for
her heart beat on the right side ; but it is possible that the anomaly
had been acquired. Perhaps this case may have been in Moliere's
mind when he wrote the famous passage in Le Medecin malgre lui
(Act ii. sc. vi.) : Geronte says to Sganarelle, " On ne peut pas mieux
raisonner, sans doute. II n'y a qu'une seule chose qui m'a choque :
c'est l'endroit du foie et du coeur. II me semble que vous les placez
autrement qu'ils ne sont ; que le coeur est du cote gauche, et le foie
du cote droit." Sganarelle replies, " Oui, cela etait autrefois ainsi ;
mais nous avons change tout cela, et nous faisons maintenant la
nu'decine d'une methode toute nouvelle." On the other hand, it is
possible that he may have heard of the criminal with situs inversus
dissected by Bertrand in Paris in 1650. The reader will find the
whole question of the relationship of Moliere's allusion to these two
cases fully discussed by Taruffi (op. cit., vhi. 153, 1894). Morand's
observation was made upon a man, seventy-two years of age, and has
often been attributed to Mery, who communicated it to the Parisian
Academy of Sciences ; it created much interest and was celebrated
in such poetical effusions as the following :

" La nature, pen sage et sans doute en debauclie,
Plaga le foie au cote gauche,
Et de merae, vice versa,
Le cceur a la droite plaga."

An instance of " the order of all the bowels inverted " was reported
in England by H. Sampson (Pltilos. Trans., iii. Ill, 1700) towards
the close of the seventeenth century; and since then there have been
several hundreds of observations made in different parts of the world
by various medical men. Taruffi (op. cit., v. pp. 507-549, 1889 ; viii.
148-182, 1894) gathered together over two hundred references, and
the Index Catalogue (vol. xv. 775, 1894) contains many more; in
1897, I made a digest of recent literature on the subject, analysing
forty-five cases reported during the years 1894, 1895, 1890, and 1897
(Scott Med, and Surg. Journ., i. 1020, 1897).

I have seen one case, that described lately by Sir Thomas Fraser
(Scott. Med. and Surg. Journ., xv. 160, 1904); and in 1898 Dr. David
Landsborough wrote to me describing three instances of inversion
which he had seen in natives of Formosa. Dr. Landsborough's
patients were two men and a boy ; and in all of them the anomaly
was discovered in the same way. I quote from his letter. " A very
large number of my patients suffer from chronic malaria, and come
complaining of a ' hard lump in the side of their abdomen ' (that is
about the translation of what they say). On asking where exactly
the hard lump is, they usually place their hand on the left lumbar

59G ANTENATAL PATHOLOGY AND HYGIENE

region. In the three cases I have referred to, the patients indicated
the right side, and, on placing my hand there, I felt at once a hard
firm tumour, with notched horder, disappearing under the angle of
the ribs, exactly like a spleen. On passing the hand up to the
mammary region, the heart was to be very distinctly felt beating on
the right side, while not the slightest pulsation could be felt on the
left side of the chest. Auscultation and percussion confirmed the
impression that the heart was transposed. There were no signs of
disease in either lung which could have produced any change in the
position of the heart. The liver could be distinctly percussed out on
the left side in a position exactly corresponding to its normal position
on the right. In the case of the boy, a rough loud systolic murmur
was heard all over the pnecordia, most distinct over the transposed
pulmonary area, probably due to congenital stenosis of the pulmonary
orifice." I have quoted from Dr. Landsborough's letter to emphasise
the importance of watching for cases of transposition of the viscera
in malarial countries where the splenic hypertrophy is an aid in
diagnosis.

The morbid anatomy of heterotaxy is interesting chiefly on
account of the perfect way in which the transposition has been
carried out, if I may use a somewhat inexact manner of speaking,
for there is no reason to believe that any transference of parts from
one side of the body to the other occurs.

In the thorax, for instance, the oesophagus passes from the
middle line towards the right half of the diaphragm, and describes
two curves, the upper with its concavity to the left and the lowrer
with its concavitv to the right. The right lung has two lobes and
the left three ; and the bronchi also are inverted. The axis of the
heart and pericardial sac is directed obliquely downwards and to the
right ; and about two-thirds of the organ lie to the right of the
middle line and only one-third to the left. The cardiac apex lies in
the right mammillary line, corresponding to the fifth rib or the fifth
intercostal space. The pulmonary veins open into the right auricle,
and the corresponding ventricle gives origin to the aorta ; the left
auricle receives the blood from the superior and inferior cavse and
from the coronary veins, while the left ventricle sends it on to the
lungs in the pulmonary artery. On account of the functional changes
involved in this inversion, the right ventricle develops thick walls
and the left has thin ; but this character is not marked at birth. A
two-segmented valve divides the two chambers of the right heart,
while a three-segmented one is interposed between those of the left
heart. The pulmonary artery arises from the left ventricle and
passes obliquely to the right ; it divides into two branches in the
concavity of the arch of the aorta, the shorter of which goes to the
right lung and is connected with the ductus arteriosus. The same
inversion affects the aorta both in its arch and in its descending part,
the great vessels which arise from it, the great veins, the thoracic
duct, the phrenic, pneuinogastric, and sympathetic nerves, and the
smaller vessels of the thorax. The diaphragm also exhibits similar
changes, those openings in it which are normally on the left side

HETEROTAXY 597

being found on the right, and vice versd ; and its level on the left
side is higher than on the right.

In the abdomen, the stomach is found on the right side where its
greater part occupies the right hypochondriac region ; its pyloric end
is placed in the epigastrium ; and the whole organ has a direction
from above downwards, from before backwards, and from right to left.
The duodenum has its concavity facing the right ; it is directed
upwards, to the left, and backwards, and then turns vertically down-
wards and proceeds from left to right to become continuous with the
jejunum ; thus its three portions are situated in the left half of the
abdominal cavity. The large intestine, from the caecum (and
appendix) to the sigmoid flexure and first part of the rectum, is
exactly transposed, the caecum 1 icing on the left side and the sigmoid
flexure on the right. The pancreas has its head or larger end to the
left, and its tail to the right side, and lies in the concavity formed by
the inverted duodenum. The spleen is on the right side, while the
larger lobe of the liver is on the left and the smaller lobe comes into
relation with the stomach on the right side. The gall bladder and
ducts are on the left side. The right kidney is at a higher level
than the left, as is the right suprarenal capsule as compared with
the left. The right ureter is longer than the left ; the right
testicle hangs lower than the left ; and the uterus faces slightly to
the left instead of to the right. The abdominal aorta, the portal
vein, and the vena cava inferior, as well as the other vessels, are all
exactly inverted.

Even in the cranium there are signs of inversion. The right
half of the brain is larger and weighs more than the left ; and the
venous sinuses, as regards their size, also exhibit inversion (Calori,
Mem. Accad. d. sc. d. 1st. di Bologna, 4 s., ii. 597, 1880). This is also
evident when the cerebral arteries, and the fossae of the cranium, and
even the lachrymal sacs are closely examined. It is said that the
hair on the head also has a vertex directed from left to right instead
of from right to left.

Such are the appearances found on dissection in cases of complete
transposition of the viscera, I have not gone minutely into details,
but the reader who may desire more exact descriptions will find them
admirably* set forth by G. F. H. Ktichenmeister in his fine monograph
of 367 pages {Die angeborne vollstdndige seitliche Verlagerung der
Eingeweide des Menschcn, 2nd edit., Leipzig, 1888).

Transposition of the viscera has not always a morbid anatomy
so complete and exact as that described above : there are cases which
must be called incomplete, partial, or localised. For instance, the
heart alone may be affected, giving rise to the state known as
dcxiocardia, a comparatively rare occurrence, and not to be confused
with cardiac displacement due to disease. There may be simply a
transposition of the great vessels, the heart remaining on the left side
of the body, but this is hardly to be considered here; it has been
described along with the malformations of the heart, with which,
indeed, it is generally combined. Apparently the lungs are hardly
ever transposed without the other organs sharing in the change ;

598 ANTENATAL PATHOLOGY AND HYGIENE

while, on the other hand, the other viscera may be inverted without
the participation of the lungs. Some of the abdominal organs,
perhaps all of them, may be transposed without there being any
changed relations in those of the thorax.

All writers are by no means agreed as to these atypical cases of
transposition. Lochte (Beitr. z. path. Anat. u. allg. Path., xvi. 189,
1894), in a learned article, states his belief that no instance has yet
been put on record in which clearly marked situs transversus of the
organs of one body-cavity was accompanied by an entirely normal
arrangement of those in the other cavities. Apparent exceptions to
this law may exist, e.g. Auche and Bouyer's case {Joum. de me'd. de
Bordeaux, xxvii. 413, 1897) ; but these are, as a rule, founded on the
results of clinical examination only, and are, therefore, not free from
possible errors. Situs inversus of the thoracic organs is either part
of a general transposition, or is the accompaniment of anomalies in
the distribution of the great veins of the abdomen or in the position
and appearance of some of the abdominal viscera. The inverted
position of single organs {e.g. stomach, liver, and heart) is not to be
regarded as directly related to the causes of situs transversus, but
is to be ascribed to local anomalies in development, and these local
anomalies, there is good reason to believe, belong to a later stage in
embryological formation. This is the probable explanation of such
a case as Launay's {Bull. Soc. anat. de Paris, lxix. 320, 1894) : in the
body of a woman who had died from cancer of the pancreas all the
organs of the anterior and posterior mesogastrium were transposed ;
all the other abdominal and thoracic viscera were normal in position,
as were also the aorta, the vena cava inferior, etc. This occurrence
may be explained by imagining an inverse rotation of the meso-
gastrium, so that the right surface thereof, instead of the left, becomes
anterior.

Associated malformations may occur along with transposition of
the viscera. There may, for instance, be structural anomalies of the
heart; thus, in H. Jellett's case {Lancet, i. for 1897, p. 878), there
was a triccelian heart, two auricles opening into a common ventricle ;
and other instances were reported by Valleix {Bull. Soc. anat. de
Paris, ix. 253, 1834), L. Vincenzi {Arch, per le sc. med., Torino, ix.
283, 1885-86), and many more. The spleen also may be malformed
as well as misplaced, the commonest anomaly being division into
two or more lobes or separate parts (Marchand, in Ahlf eld's Bcr. u.
Arh. a. 'I. gcburtsh.-gynaeh. Klin, zu Gicssen, 1881-82, p. 254, 1883 ;
Launay, loc. eit.\ Hyrtl, Handbuch dcr topographischen Anatomic, i.
492, 1847). Bifidity of the thumb has been noted (J. P. Boyd,
Glasgow Med. Joum., xliv. 89, 205, 1895 ; Sir Thomas Eraser, loc. cit!) ;
the vena cava inferior has been seen uniting with the vena cava
superior before entering the heart (A. M. M. Whinnie, Lond. Med.
Gaz., xxvi. 31, 1840); absence of the right kidney and suprarenal
capsule has been observed (Beaufume et Caron, Bull. Soc anat. de
Paris, p. 1006, 1902): and the thymus gland was found persisting at
the age of thirty-five (A. Pic, Prov. med., Lyon, x. 535, 1895).

Although Aristotle so long ago pointed out the occurrence of

HETEROTAXY 599

visceral transposition in animals, very few modern instances have
been reported. Transposition of the heart, however, has been observed
in the embryos of birds, more especially in instances of artificial
incubation (Dareste, op. cit., 2nd edit., pp. 338-355, 1891). In other
animals, more especially in invertebrates which show marked asym-
metry, a state of affairs is found which may be grouped with
transposition of the viscera. It consists in a reversal of the usual
twisting or asymmetry of the whole body, and is seen, for instance,
in some gastropods among the mollusca, and in some pleuronectidi
among the teleostean fishes. Saint-Hilaire (Histoire des anomalies,
ii. 23, 1836) called this general inversion, to distinguish it from the
internal or splanchnic inversion seen in the human subject, and
grouped them both under heterotaxy ; but it is evident that there is
no clear distinction, for even in the human subject signs of external
inversion (difference in height of the testicles) may be seen if closely
looked for, although, of course, they are not so obvious as in a one-
sided shell or a flattened fish.

The relation of situs inversus to twins and double monsters
raises complicated questions and problems in teratogenesis. It was
stated by Forster, and some observations seem to confirm the view,
that the right twin in double monsters showed transposition of the
viscera, while the left-sided one showed the usual arrangement
(situs solitus). But many double terata have been described in which
there is no such inversion in the right hand individual ; indeed
Tarutfi (op. cit., v. 554, 1889) had difficulty in collecting together
from literature ten instances of double monsters exhibiting trans-
position. Sometimes, however, as in one of E. J. Berry's cases
(Trans. Edirib. Obstct. Soc, xxviii. 222, 1903), there is a partial
inversion of the great vessels in the right hand twin. Complete situs
inversus must, therefore, be looked upon as only an occasional com-
plication of the double terata. Similarly in separate twins it is a
rare occurrence. Baron (Rev. nu'el. /rang, et etrang., i. 323, 1826)
noted such an exceptional case, and W. B. Hadden (Lancet, ii. for
1890, p. 1156) recorded another. Neither in triplets nor in quad-
ruplets would situs inversus appear to be common (Kiichenmeister,
op. cit., p. 223, 1888).

In its clinical aspects, transposition of the viscera is full of interest,
although it must be confessed that the interest is postnatal rather
than antenatal. There is no evidence that the anomaly is transmitted
by heredity ; even cases of family prevalence are very rare, E. Eogi's
observation on a brother and sister (Sperimentale, xlv. 376, 1880)
being almost unique. It is of more common occurrence in men than
in women (twice as common in the cases I have analysed) ; but, of
course, there are accidental circumstances which may partly account
for this. It has seldom been noted at birth, save in infants stillborn
or otherwise deformed ; only about twenty-one of the cases in Taruffi's
long list of over 200 references were new-born infants. It may be
first noticed at any age, for it may give rise to no inconvenience
and to no symptoms, and it is indeed usually discovered quite by
accident, either by the individual himself or by the physician, during

600 ANTENATAL PATHOLOGY AND HYGIENE

examination for life assurance, or military service, or for a slight
ailment. It is questionable whether the anomaly reduces the prob-
ability of a long life to a point below the normal. In a few cases
the patients have been described as delicate ; in Barie's case {Bull, ct
Me'm, Soc. nu'd. d. hop. de Paris, 3 s., xi. 486, 1894) and in Hayem's
(Med. mud., vi. 397, 1895) there was cyanosis, but this may have
been due to an associated malformation. The inversion of the organs
is generally so complete that the physiology of the organs is not
interfered with ; since every organ is inverted, the functions of the
whole organism are normally performed. Procreation is unimpaired.
There are, however, two or three functional peculiarities which
have been occasionally found associated with this visceral anomaly.
One of these is left-handedness. The association is far from being
constant, but it is noted rather more frequently than among
individuals with situs solitus. Among recent instances those of R
Caton (Journ. Anat. and Physiol., xxxi. 446, 1896-97) and A. S.
Warthin (New York Med. Journ., lix. 306, 1894) may be cited ; and
A. N. Blodgett's patient was ambidextrous (Boston Med. and Surg.
Journ., cxxxiv. 213, 1896), while Galinsky's, although right-handed,
unconsciously wrote " mirror- writing " with his left hand (Jahrb. f.
Kinderh., xxxix. 91, 1894). One of Posselt's patients, although right-
handed, had greater power in his left hand, as shown by the
dynamometer (Deutsches Arch. f. klin. Med., lvi. '202, 1895).

Another function upon which transposition of the viscera may
possibly have an influence is child-birth. Cases in which women
with visceral transposition have given birth have seldom been noted
with any degree of accuracy, and K. Bodon's observation (Ccntrlbl. f.
Gyndk., xxi. 592, 1897) is therefore specially important. He noted
in his patient that during pregnancy the fundus uteri lay to the left
of the middle line, and was rotated so that the anterior surface faced
also towards the left as well as to the front, and the right upper angle
of the uterus lay in front of the left. The uterus was, therefore,
inverted like the other viscera, and Bodon looked for the cause in the
presence of the sigmoid flexure in the right instead of in the left iliac
fossa. Perhaps, however, the most important feature in the case was
the presentation of the infant with its head in the second occipital
position (Pt. 0. A.), and its birth by the mechanism peculiar to that
position.

The diagnosis of transposition of the viscera is not difficult if
attention is directed towards the chest and abdomen. At birth it
may be suspected if the cardiac impulse is seen on the right side of
the thorax ; but it must be borne in mind that this sign is common
to transposition of the viscera and to diaphragmatic hernia (left-
sided) with displacement of the heart. In a case of hernia of the
diaphragm (p. 478), which I met with some years ago, I was in doubt
as to which of these two anomalies was present until the thorax was
opened into. By means of percussion, auscultation, mensuration, and
skiagraphy the physical diagnosis can generally be carried out with
great exactness. By percussion it can be determined that the cardiac,
hepatic, and splenic areas of dulness are exactly transposed ; palpation

HETEROTAXY 601

shows the apex beat in the position on the right side of the chest that
it should occupy on the left ; and auscultation will demonstrate a
transposition also of the cardiac sounds. Further, preliminary gaseous
distension of the stomach by French's method, followed by percussion
and by auscultation during percussion will reveal that viscus lying
to the right of the middle line, with its cardiac end to the right and
its pyloric to the left, and with its greater curvature directed down-
ward and to the right. Auscultation over the spine during deglutition
will elicit oesophageal gurgling to the right instead of to the left side
of the vertebral column ; and palpation over the posterior aspect of
the thorax will show more marked vocal fremitus on the left side,
pointing to the presence of the bronchus with the greater lumen on
that side. The abdominal aorta may be felt pulsating to the right
instead of to the left of the spine, and inflation will show that the
rectum and sigmoid flexure turn to the right side instead of to the
left. Palpation and percussion may detect the caecum in the left
iliac fossa ; sometimes mensuration has shown cranial and thoracic
asymmetry of an unusual kind, the left side giving greater measure-
ments than the right.

While transposition of the viscera may have no prejudicial effect
upon the life and health of the individual, it may prove an awkward
complication in surgical procedures. It is easy, for instance, to
understand howr the success of a colotomy (for intestinal obstruction)
might be imperilled by its non-recognition by the operator. In the
performance also of an cesophagotomy, of paracentesis thoracis, and
of operations on the liver, gall-bladder, stomach, pylorus, and spleen,
the presence of situs inversus would necessitate changes in the mode
of procedure (Klichenmeister, op. cit., pp. 176-195).

The teratogenesis of situs inversus is not yet fully understood,
although some light has been thrown upon it. It is interesting to
note that there is now a tendency to carry the time of the production
of this anomaly back to the germinal period of antenatal life, or at
least to the very earliest stage of the embryonic epoch ; in this
respect modern science differs little in theory from the views held by
Winslow in the middle of the eighteenth century (Mem. de I 'Acad.,
Ann. 1733, p. 374), for according to them the transposition was due
to a primitive malformation of the germ.

At first it was thought by Serres (Heche relies cV anatomic transcen-
dante ct patholoc/iquc, art. xv. p. 108, 1832) and his followers that,
in assuming their permanent position in the body cavity, the viscera
were related to each other, and moved in some fashion like the
planets in respect to each other and to the sun. The liver was
regarded as the viscus whose position and movements determined
those of the other organs ; and, in inversion, it was supposed that
the liver was displaced and that the other viscera simply followed.
It was supposed that one of united twins always showed this
inversion, and that this was due to the relationship of the two livers.
Virchow (Arch. f. path. Anat., xxii. 432, 1861) carried the theory a
little further, and regarded the kind of torsion found in the umbilical
cord as the determining cause of the position occupied by the liver

602 ANTENATAL PATHOLOGY AND HYGIENE

and consequently by the other organs also. Facts, however, did not
fit themselves to theories, and it was not long before it was found
that the torsion of the cord might be left or right hand, and yet no
inversion be produced with either. No determining cause, therefore,
was available to explain the displacement of the liver ; further,
inversion was found to occur before the appearance of the hepatic
rudiments ; further still, displacement of the liver was noted in some
cases without inversion of the other organs, and all the other viscera
were discovered displaced without any change in the liver. Saint-
Hilaire endeavoured to relieve the difficulties of the hepatic theory
by suggesting the action of special causes in preventing some organs
following the lead of the liver, as it were, in cases of incomplete situs
inversus ; he adduced an anomaly to explain why another anomaly
did not always exist in just the same anomalous way (Histoirc des
anomalies, ii. 16, 1836). Serres' theory was further weakened by the
fact that inversion of the viscera in one twin (in cases of double
terata) is far from constant. Its author, however, aided progress by
pointing out the primitive duplicity of many embryonic organs
(e.g. the heart), and by showing that their asymmetry was clue to
more rapid growth of one half or to delayed growth or atrophy of
the other.

There is another theory of the origin of heterotaxy which has
gained a great amount of support. It proceeds from the observation
that, in the case of the chick, the embryo lies upon its left side, and
goes on to state that if it were to rest on the right side instead there
would be inversion of the viscera. It has in its favour some facts
in Comparative Embryology. Von Baer of St. Petersburg (Ueber
Eiitwickclungsgeseliichte der Thiere, i. p. 51, 1828) showed that rota-
tion of the embryo, when it is in the opposite direction to the normal,
is accompanied by inversion of the cardiac curve ; and E. d'Alton
(cited by Taruffi, op. cit., v. 559, 1889) found an embryo chick of five
days in which the anterior part of the body was rotated inversely
and the heart was inverted, while the posterior part was rotated
normally. The latter author further believed that in the case observed
1 >y him the chick would have developed situs inversus of the thoracic
and situs solitus of the abdominal organs. This idea seemed also to
agree with the supposed inverted state of the viscera in double
monsters, for one could imagine the right twin lying on its left side
and so having its heart and aorta inverted. The moment, however,
that the developmental details begin to be scrutinised, difficulties
appear in the way of a complete acceptance of the theory that
curvature of the heart-tube is due to the position of the embryo on
the embryonic area. In fact, the cardiac curve may be present
before the rotation of the embryo takes place. Further, embryos
with abnormal rotation have been found showing no inversion of the
viscera, and traces of inversion have been discovered in those showing
no abnormal rotation on the embryonic area. Evidently, therefore,
the position of the embryo is not the determining cause. Some very
ingenious suggestions have been made (by Bindtieisch and others)
regarding the effect of the circulating blood on the character of the

HETEROTAXY 603

curvature of the heart ; but it is unnecessary to linger over them, for it
has been shown, in the case of the chick at least, that the cardiac
curve is present before the circulation begins.

Dareste's theory of the cause of inversion of the viscera in
the chick demands some consideration (op. cit., p. 351, 1891). He
pointed out that the heart is constituted by the fusion of two lateral
halves ; usually the right half is the larger, and so the normal curve
of the cardiac tube is towards the right ; but if the left half be larger
then the curve will be inverted. He brought forward as proof of
this view the fact that in cases of inversion in the chick the left
half of the vascular area was larger than the right. He was able
by unequally heating the egg to produce artificially this inequality
of the two halves of the vascular area ; but, unfortunately for com-
plete proof, he did not always obtain inversion of the heart under
these circumstances. Of course this result may have been due to
defects in the apparatus used for carrying out these experiments.
The experiments of Warynski and Fol (vide p. 214 of this volume)
seemed to support the idea that unequal heating might retard or
increase the growth of one part of the vascular area and so lead to
heterotaxy; but, even more than Dareste's methods, they contained
possible fallacies, due particularly to the violence of the means
employed to influence the chick. Dareste's theory, however, to some
extent suited the facts, and it explained in a feasible fashion how
one of double twins had inverted viscera ; for the proximity of
united embryos would cause a dwarfing of one half of the vascular
area of each, and in the case of one embryo it would be the half
whose diminution led to inversion of the heart. Unfortunately, as I
have already pointed out, all arguments strengthened by references
to inversion in double monsters are really weakened thereby, for such
inversion is inconstant ; indeed it is rare.

Taruffi (op. cit., v. 579, 1889) still is of opinion that in some way
or other the size of the two parts of the vascular area, in the case of
the embryo chick, is concerned with inversion of the viscera. There
is an asymmetry of the two halves of the vascular area and of the
vessels coming from it to the embryo, and this asymmetry precedes
both the curving of the cardiac tube and the turning of the em-
bryo on to its side. In inversion there is an inverted relationship of
the vessels in the vascular area. The theory, therefore, goes back to
the cause of the inequality of the two parts of the vascular area, to
the reasons why the embryo lies on the vascular area in just the
way that it does in normal cases and in a different way in inversion.
There may be a reversal of the polarity of the ovum leading to an
inverted cleavage. But the " orientation " of the embryo, as it is
called, is still an unsolved problem both in normal and abnormal
circumstances. The important fact to bear in mind, however, is that
probabilities point to the origin of inversion of the viscera in a very
early period of antenatal life, in the pre-embryonic or germinal
epoch.

The teratogenesis of heterotaxy must, then, be left in a most
unsatisfactory position ; but the following facts or ideas are worth

604 ANTENATAL PATHOLOGY AND HYGIENE

remembering. In the first place, it is doubtful how far it is wise to
draw deductions from observations and experiments on the hen's
egg. There are important differences in the ontogenesis of the
mammalian as compared with the avian ovum and embryo, and
these complicate the question greatly. Fere (Compt. rend. Soc. de
biol., 10 s., iv. 246, 1897), it is true, succeeded in producing heterotaxy
(along with other monstrosities) in the chick by injecting hydrocy-
anic acid into the egg before incubation : but his experiments do not
reveal the cause of the inversion so much as the early date of its
occurrence ; they are of value rather in showing that heterotaxy
may apparently be determined by poisons acting on the organism in
the germinal period. In the sennit/ place, the idea that an individual
with inversion of the viscera is the right-side survivor of uniovular
twins, although a most fascinating one, is not supported by facts.
For instance, the much-talked-of visceral inversion of one individual
in double monsters has been shown to be rare, while in ordinary
uniovular twins both individuals usually show a normal arrangement
of the internal organs (situs solitus). Again, if this theory were
correct, we ought to find some trace of the twin with normal internal
organs at the birth when an infant with transposed viscera is born ;
but as a matter of fact the expulsion of a foetus papyraceus along
with an infant with heterotaxy has not been recorded (Galinsky, loe.
cit.). I do not, however, place much value on the latter objection, for,
theoretically, the death of the twin embryo with situs solitus would
be so early that no recognisable trace of it would be left. In the
third place, a theory of heterotaxy, to be satisfactory, ought to include
an explanation of the partial cases of transposition in which the
heart alone, or one or other of the abdominal organs alone, is dis-
placed. In the fourth place, it is useless to look for causes of heter-
otaxy in late embryonic life ; to do so is only to introduce endless
difficulties. To understand, for instance, how displacement of the
heart is associated with inversion of the abdominal viscera, it is
necessary to carry the mind back to the time, before the development
of the diaphragm, when the organs of the thorax and abdomen are
closely associated together. In the fifth place, there is reason to
believe that heterotaxy is determined by causes acting on the
organism in the germinal or pre-embryonic period, just as it seems
clear that the normal asymmetry of the body is also decided at this
early epoch. Possibly the cause or causes (toxic, toxinic, or other)
act by causing irregularity of growth of the embryonic annexa
(amnion, umbilical vesicle, chorion), by thus altering the usual rela-
tions which the embryo bears to its surroundings, and by influencing
the rate of growth of its two sides. In its essence heterotaxy may
be conceived as consisting of an excess of development and growth
on one side of the body and of. a deficiency on the other side, with
this peculiarity that the side, with excess is that which in normal
circumstances shows deficiency, and eice versd. Finally, the reader
will easily perceive from what lias been stated, why it is that trans-
position of the viscera is placed here at the end of the monstrosities
of the embryo, between them and the morbid states of the germ. It

DIAGNOSIS OF MONSTROSITIES 605

is also evident how it is linked on to such states as hemi-hypertrophy,
for it is essentially a unilateral inequality of growth ; in transposi-
tion of the viscera, however, there is in addition a resulting in-
equality of development or ontogenesis. On account of its complicated
relationships as well as by reason of its perplexing teratogenesis it
is obviously appropriate for heterotaxy to occupy a position on the
boundary line between the morbid states of the embryo and those of
the germ.

Diagnosis of Monstrosities.

I know of no means by which monstrosities can be diagnosed
during the first two or three months of antenatal life. Unless the
conception be expelled in the form of an abortion sac, there is no
possibility of knowing whether the embryo in it is normal or tera-
tologics! ; at least I know of none. In other words, monstrosities
cannot be diagnosed in the period of antenatal life when they are
produced, unless they are discharged from the uterine interior as
miscarriages, and then, of course, their life has ceased. It is none
the less important to examine minutely all abortion sacs. When it
is remembered that we possess so few records of carefully examined
monstrous embryos in the human subject, the importance of scrutinis-
ing every abortion sac becomes clear ; and when it is further realised
that the nearer the monstrosity is to the period of its production, the
more valuable it will be for the elucidation of the problem of its
causation, it becomes imperative that all such material should be
utilised.

It is when the monstrous embryo has grown into a monstrous
foetus, when, in other words, the later stage of pregnancy has been
reached, that the possibility of the formation of a diagnosis (without
the interruption of the gestation) may be entertained. Even then it
must be regarded as a matter of the greatest difficulty in the present
state of our knowledge. The antenatal diagnosis of monstrosities
must therefore be done, if it is to be done at all, when the unborn
infant is in the foetal period of its life. Tor a full consideration of
the possibilities of diagnosis at this time, the reader is referred to the
first volume of this Manual (pp. 430-448). I deal there mainly with
the detection of foetal diseases, but I allude also to the discovery of
monstrosities. I need not, therefore, repeat here what has been dis-
cussed at length elsewhere ; but, at the same time, I trust that the
reader will re-peruse the passage referred to.

Retrospect and Conclusions.

If the reader will now pause for a moment and cast his glance
backwards over the long series of monstrous types that have been set
forth in the preceding chapters, he cannot fail to be impressed with
the fact that underlying all the diversity there has been a singular
degree of resemblance. With some few exceptions, it has been possible
to see in the monstrous state of the full-time foetus the persistence
of an earlier and a normal arrangement of parts. What, however, is

606 ANTENATAL PATHOLOGY AND HYGIENE

normal in the third week of antenatal life is abnormal at the ninth
month ; nay, it is already abnormal at the fourth week. In the
ceaseless progression of the human organism towards the wonderful
completeness and the marvellous perfection of the new-born infant,
the slightest delay in the developmental processes and the smallest
persistence of transitory structures constitutes an anomaly, a mal-
formation, or a monstrosity, which, in a small degree or in a great
one, interferes with the life, health, happiness, and usefulness of the
individual, or with his capacity to play his part in propagating the
race. A hesitation in the forward progress of ontogenetic processes
is sufficient to endanger the successful development of the individual ;
an actual arrest produces irreparable damage. But there is a hopeful
aspect of this depressing picture of the origin of antenatal deformity :
arrested development must be due to an arresting cause ; that cause
must act at some time or another in antenatal life ; it must, there-
fore, be possible at some time or other to bring influences to bear
upon it which may check its action. If the ultimate cause is, as
there is reason to believe it is, toxinic, toxic, traumatic, or microbic
in its action, then it ought to lie possible to attack that cause in the
parents of the future offspring, for through the parents alone can it
reach the child. More than this, there is an element of hope in the
suggestion, supported as yet by a sadly small amount of evidence,
that during antenatal life Nature is not altogether quiescent in the
face of morbid states, but tries, immediately the morbid cause ceases
to act, to undo the mischief that has been done and to repair the
damage inflicted. The study of antenatal repair of injuries is an
almost unworked field, but it offers an attractive prospect. It stands
in the same relation to antenatal prevention, as, for example, the
medicinal treatment of acquired syphilis does to its prophylaxis.
But of all this I shall have to say some few words more after I have
considered the pathology of the third and earliest period of antenatal
life, the germinal ; to that I now address myself.

BOOK IV

THE PATHOLOGY OF THE GERM
CHAPTER XXXI

Pathology of Germinal Life : Physiology of Germinal Life ; Ovum and Sperm
Production, Maturation, Fertilisation, Segmentation, Blastocyst Formation :
Pathology of the Blastocyst ; Hydatid Moles, Abortion Sacs without Em-
bryos, Amniotic Defects, Heterotaxy : Pathology of the Morula Mass ;
Fractional Individuals, Neoplasms : Pathology of Fertilisation ; Poly-
spermy, Double Terata, Hybridity : Pathology of Maturation ; Partheno-
genesis, Dermoids, Teratomata or Embryomata.

The pathology of the germ is the Hinterland of Antenatal Pathology.
Like a great continent or island the subject lies facing the four seas.
We have studied and found out much regarding its shore-line with
its bays and promontories — that is Foetal Pathology. We have also
explored to some extent the difficult country lying beyond the
maritime belt, without, however, attempting anything like an
ordnance survey of it — that is the Pathology of the Embryo. But
beyond it again is the vast Hinterland, the almost unknown interior,
about which little has been definitely ascertained, although much is
rumoured — that is the Pathology of the Germ.

I had hoped, when I began the writing of this book, to have con-
sidered the pathology of germinal life with the same detail as that of
foetal and embryonic existence ; but to do so — to discuss, for instance,
double monsters in all their varieties, to deal with heredity in all its
mysterious aspects, and to investigate the antenatal origin of tumours
— would require a volume and not a chapter or two. I have determined,
therefore, to content myself with giving here a mere sketch, a synopsis
of the subject, indicating the lines along which the discussion would
have travelled had space and time permitted.

In studying the pathology of germinal life it is very necessary to
bear constantly in mind one serious limitation to which it is subject
— I refer to the absence of information regarding the human germ.
Behind all the conclusions that are drawn in reference to the physi-
ology and pathology of human germinal life, there is always the
element of uncertainty arising out of the circumstance that such
conclusions are mainly founded upon the phenomena of germinal
activity as they are met with in the lower animals. The student of
germinal pathology labours, therefore, under a very serious dis-

607

608 ANTENATAL PATHOLOGY AND HYGIENE

advantage — a disadvantage, nevertheless, which it is very easy to
exaggerate unwarrantably. No biologist and no embryologist has
ever seen the human ovum entered by the human spermatozoon, but
I am not aware that the impregnation of the ovum by the sperma-
tozoon in the human subject has, on this account, ever been denied.
It is ever necessary to remember the restriction under which the inves-
tigations into germinal pathology are being carried on, but it is surely
never necessary to suspend them altogether. The value of the conclu-
sions is undoubtedly diminished, but certainly not altogether abolished.
In the study of germinal pathology, just as in that of embryonic
and foetal pathology, it is essential to begin with the consideration of
germinal physiology.

Physiology of Germinal Life.

Germinal life ends, or may be said to end, with the first appear-
ance of the rudiments of the embryo in the blastocyst. It begins,
for the individual, when the ovum and the spermatozoon unite in
fertilisation ; but it really begins much further back, when the repro-
ductive cells of the parents become specialised from the cells of the
genital ridge. So germinal life varies in length with the age of the
parents when impregnation occurs. It begins actually in the ante-
natal existence of the parents, stretches on through their childhood
and youth till puberty, and then, when the ovum and sperm come
together, passes into the life of the new organism. But inasmuch as
the germ-cell is supposed to contain a bit of the structural elements
of the germ of the preceding generation, and that a bit of the
generation before, and so on, it becomes impossible to fix a starting-
point. Must germinal life then be regarded provisionally as without
a beginning ? If we accept the theory of the continuity of the germ-
plasm it must be conceded that it would seem so. For practical
purposes, however, I consider here that the germinal life of the in-
dividual begins in the vital activities of the two reproductive cells of
his parents, in the ovum and spermatozoon which by their union cause
him to come into being.

A striking fact with regard to germinal life is, therefore, its
primary dual character. It is at first the life of two cells, the egg-
cell or ovum and the sperm-cell, but after impregnation it becomes
the life of one cell, the fertilised ovum or oosperm. It will be well,
therefore, to consider first the dual period of germinal existence, and
the history of the ovum will be dealt with before that of the sperm,
for it is the better known of the two.

The primitive ovum has been regarded as a cell of the mesoderm,
one of the mesothelial cells of the genital ridge ; but it may be that
it is one of the original cells (germ-cells) into which the ovum divides,
and that it finds its way to the genital ridge while many of its com-
rades lose their way, so to say, in the body tissues and disappear or
perhaps form the rudiments of future tumours. In process of time
it is carried into the interior of the ovary and becomes surrounded by
an envelope of separate smaller epithelial cells, the whole constitut-

GERMINAL PHYSIOLOGY 609

ing a primordial follicle. The ovum grows in size, becomes spherical
or nearly so in shape ; its nucleus becomes larger, spherical, and more
eccentric, and the chromatin usually gathers into one nucleolus, while
the achromatic substance develops into a coarse network ; the corona
radiata is formed, and inside it the vitelline membrane, zona pellucida,
or oolemma. The primordial follicle meanwhile changes into the
Graafian follicle. Before the ovum has become full grown and ready
for maturation the individual in whose ovary it is resting has passed
through the fo3tal stage, has been born, and has reached, at any rate,
the age of puberty.

By maturation of the ovum is meant the series of phenomena
which occur in it just before or just after the rupture of the Graafian
follicle, and the consequent separation of the ovum from the ovary ;
it marks the close, therefore, of what may be called the intra-glan-
dular life of the egg-cell. There is first a centrifugal movement of
the nucleus towards what has been called the animal pole of the
ovum (in contra-distinction to the vitelline, deutoplasmic, or vegetative
pole), and a contraction of the vitellus or yolk. When near the sur-
face the nucleus as such disappears, and in its place is found the
nuclear spindle ; at both ends of the spindle are radiating threads in
the vitellus in the form of a sun, and these together constitute the
amphiaster. Then part of this chromatin spindle is extruded from
the ovum as the first polar globule ; the part that remains inside the
ovum now forms a second spindle and a second amphiaster, and again
a portion is expelled as the second and the smaller polar globule.
These polar bodies degenerate and disappear. The remnants of the
nucleus are now called the female pronucleus, and it may or may
not move towards the centre of the egg-cell again, its after-history
being determined by the early or late appearance of the male pro-
nucleus. The chromatic masses or chromosomes in the ovum, origin-
ally sixteen in number in the human subject (so it is believed), have
been reduced to eight. The ovum is now mature. It is the female
genoblast.

The life of the sperm-cell before impregnation is not so well
known as that of the egg-cell. Either from the epithelium or the
stroma of the genital ridge (mesothelium), which may, as in the
female, come from the original germ-cells of the ovum, are developed
the solid cords which become the seminiferous tubules of the testicle.
Most of the cells in the cords are small in size and epithelioid in
arrangement, but here and there are some large cells corresponding
to the primitive ova ( Ureier). In fact the testis at this stage is only
to be distinguished from the ovary by the comparative fewness of
these primitive ova. The male sexual cords remain quiescent till
puberty, when they become the seminiferous tubules in which are
developed the spermatozoa. The exact method in which first the
spermatoblasts and then the spermatozoa are produced is not known,
but it is believed by Benda that the epithelioid cells give rise to the
columns of Sertoli and the primitive ova ( Ureier) to the spermato-
cytes. In a column of spermatocytes it is possible to trace a parent
cell, mother cells, and daughter cells, the latter having relatively
vol. ii. — 39

010 ANTENATAL PATHOLOGY AND HYGIENE

large nuclei. The spermatoblasts arise from the nuclei of the
daughter cells, and from these the spermatozoa are developed by
changes in the chromatin and rupture of the nuclear membrane.
The spermatozoa are now ready to impregnate the mature ovum.
They are the male genoblasts.

The dual period of germinal life is now brought to an end by the
entrance of a spermatozoon into the ovum. Probably many sperma-
tozoa make their way into the peri-vitelline space, but so far as is
known one only normally passes through the zona pellucida and
enters the yolk to form there the male pronucleus ; the latter by a
mechanism about which there is much mystery approaches the female
pronucleus, and ultimately fuses with it to form the segmentation
nucleus. Minot points out that " since the head of the spermatozoon
is developed chiefly out of the chromatin of the nucleus of a spermato-
blast, it follows that impregnation is essentially the addition of
chromatin to the nucleus (female pronucleus) of the mature ovum."
The segmentation nucleus contains as many chromosomes as the
original ovum ; so that fertilisation has restored the chromosomes to
their previous number. It is probable that a short period of quies-
cence now ensues (it does so at any rate in invertebrates), and then
follows the first act of combined or unified germinal life, the segmen-
tation of the ovum.

It is unnecessary for me to enter into the details of the segmen-
tation of the ovum, for the production of the morula mass is surely
the best known of all the phenomena of embryology. It may, how-
ever, be well to bear in mind that, as Minot puts it, the regularity of
mammalian segmentation is an assumption which recent researches
do not warrant. Segmentation begins, to quote from Minot, when
the ovum is one-half or two-thirds of the way through the oviduct ;
the first cleavage plane passes through the axis of the ovum which
is marked by the polar globules, the two segmentation spheres are
oval and subsequently become flattened against each other; the
second cleavage plane is also meridional ; the ovum next divides into
eight, and then into twelve segments, of which four are larger than
the rest ; the succeeding cleavages have never been followed accurately,
but it would seem that they progress with great irregularity. The
segmentation being holoblastic is total, and it is believed that about
this time the segmenting ovum has reached the uterine cavity. En-
largement now takes place ; and, a cleft appearing in the middle of
the morula mass, a cavity is gradually produced. The impregnated
ovum has now reached the stage of the blastodermic vesicle, blasto-
cyst, or blastula, or diaderm.

Let me now remind the reader of the limitation referred to in the
beginning of this chapter. We have no knowledge of what happens to
and in the human ovum from the time when it is penetrated by the
spermatozoon to the time when we see it, already a complex structure, as
Peters found it, at the estimated age of five or six days. It then con-
sists {vide pp. 21-24 of this volume) of an extra-embryonic part,
blastocyst or chorionic vesicle, covered with trophoblast (probably
ectodermic in origin) and lined with a thin layer of mesoblast, which

GERMINAL PHYSIOLOGY Gil

contains an amniotic sac and a pro-embryon and other things. But
how has the ovum with its segmentation nucleus become converted
into this complicated blastocyst ? We can only hazard an opinion
from the study of mammalian ova other than those of the human
subject, and for this purpose Dr. Arthur Eobinson's three lectures
(Journ. Anat. and Physiol., xxxviii. 186, 325, 485, 1904) are invaluable
It may, for instance, be conjectured that the zona pellucida is de-
veloped from the membrana granulosa and not from the ovum itself,
that it may have the function of preventing the contact of the tropho-
blast with the mucous membrane of the tube or uterus until the
ovum has reached its proper mooring-place, and it may disappear
early or persist till the embryonic ectoderm reaches the surface of
the blastocyst. It may also be supposed that the ectoderm develops
the thick trophoblast layer during segmentation, that the trophoblast
plays a very important part in fixing and nourishing the future
embryo, and that therefore it appears before the first traces of an
embryo. How, in the human subject, the amniotic ectoderm and the
ectoderm of the pro-embryon are developed from the segmentation
sphere or morula mass, and how the entoderm forms the umbilical
vesicle, and whether any trophoblast cells remain inside the blasto-
cyst, and many other matters must remain for the present quite
obscure even to the skilled embryologist. The only supposition that
may be hazarded is that since these structures are formed in different
ways in different groups of the mammalia, they may also be con-
structed in yet another manner in the human subject.

The physiology of germinal life may be summarised as follows.
It consists of a comparatively long period (ante-conceptional) during
which it is a dual existence, an epoch in which the two parts (sperm-
cell and egg-cell) are simply specialised cells of the organism (male
or female) in which they lie, and in which it must be concluded that
they are subject to the influences (healthy or pathological) which act
upon that organism ; the most noteworthy phenomenon in this epoch
is the maturation of the egg-cell (expulsion of polar globules and
reduction of chromosomes), and a somewhat analogous process in the
sperm-cell. It consists, second, of a relatively very short period
(intra- and post-conceptional), during which the mature male cell
unites with the mature female cell to form a single cell, the impreg-
nated ovum or oosperm ; during this short epoch the cell thus pro-
duced becomes, by a process of division and specialisation, first the
morula mass, and then the blastocyst, and lastly the blastocyst with
amniotic sac and pro-embryon inside it ; the most noteworthy pheno-
mena at this time are impregnation, segmentation of the ovum, and
commencing embryo-formation, and the most noteworthy structures
are not the embryo (for of that there is only a trace) but the tropho-
blast and the amniotic sac and the umbilical vesicle.

If we ask ourselves now what is the meaning of these various
processes, we plunge at once into a labyrinth of divergent opinions.
There is, for instance, the cpuestion of the meaning of the sexual cells
and their maturation. Perhaps Minot's theory of the genoblasts
(Human Embryology, p. 77, 1892) explains it as well as any other,

612 ANTENATAL PATHOLOGY AND HYGIENE

but all writers are not agreed about this. It supposes that in the
reproductive cells both sexes are potentially present. To produce
sexual elements the cell divides into its sexual parts, the genoblasts :
in the case of the egg-cell the male polar globules are cast off, leaving
the female ovum (female genoblast) ; in the case of the sperm-cell,
the male spermatoblasts, homologous with the polar globules, multiply
considerably, and their descendants give rise by further specialisation
to the male elements (male genoblast), while the parent cell, or
homologue of the female ovum, atrophies. In both cases the sexual
cell separates into a single female element (or thelyblast), and prob-
ably two male elements (or arsenoblasts), which are capable of
multiplication by division ; but in one case it is the thelyblast, in the
other the arsenoblast, which is preserved, differentiated further and
utilised. This theory gains support from the fact that the extrusion
of two polar globules seems to be common to the whole animal king-
dom ; its purpose would appear to be to render the ovum capable of
fertilisation. It was at one time believed that in parthenogenetic ova
only one polar globule was formed, that therefore part of the male
element (arsenoblast) remained in them, and rendered them capable
of development without fertilisation. Unfortunately for the main-
tenance of the above theory, this character of parthenogenetic ova has
not been found to be constant.

Then, again, there is the question of the determination of the sex
of the future offspring. Does it take place in the germinal period,
before, during, or after impregnation ; or is it settled in the embryonic
epoch ? This is another difficult problem. The tendency of recent
research, however, seems to be to support the idea that ova are
already male or female before impregnation. I have elsewhere {Brit.
Med Journ., i. for 1903, p. 1100) reviewed the evidence brought for-
ward by various writers on this subject, and have shown that the
probability is that the sex of the ovum depends upon the mother,
and is decided before fertilisation, possibly by differences in nutrition,
well - nourished ova tending to become female, and ill -nourished,
male.

Pathology of Germinal Life.

If the same principle of study be applied to the pathology of
germinal life as was brought to bear upon embryonic pathology (vide
p. 73), that, namely, of looking for an explanation of morbid develop-
ments along the line of physiological peculiarities, it will at once
become apparent that, theoretically, the pathology of germinal life
ought to consist of anomalies in the formation of the first rudiments
of the embryo, malformation of the blastocyst and of its contained
parts (amniotic sac, umbilical vesicle, trophoblast), unusual modes of
segmentation, errors in the details of the impregnation of the ovum
by the spermatozoa, and imperfect maturation of the ovum and im-
perfect formation of spermatoblasts and spermatozoa. It will be
shown that there is some reason to believe that what have been ad-
vanced as the probable phenomena of germinal pathology are the real
phenomena. I shall discuss the pathology of the various periods in

PATHOLOGY OF THE BLASTOCYST 613

germinal life, beginning with the latest (that of the blastocyst), and
working backwards towards the early time of separate existence in
two sexual cells.

Pathology of the Blastocyst.

The segmentation of the ovum and the formation of the blasto-
cyst and first traces of the embryo in it may be said to be the
phenomena of the post-conceptional period of germinal life — the
period, in other words, which follows the impregnation of the mature
egg-cell. Now, if morbid influences act upon the new organism in
this stage (blastocyst), we have to ask ourselves what the pathological
results will be. Little that has definitely been ascertained can be
affirmed, but some interesting suggestions may be made.

It is evident that in such a blastocyst as that figured by Peters
(Figs. 3, 4, pp. 20, 21) the first traces of the embryo (pro-embryon)
have only just appeared ; and it may be asked what would happen if
they did not appear ? In answer it may be said that it has frequently
been found in teratogenic experiments on the hen's egg that, if the
time allowed to elapse between the laying of the egg and its incuba-
tion be too great, a blastoderm may develop without an embryo.
The same thing may occur in the case of mammalian vertebrates ;
and from time to time one comes across abortion sacs containing no
trace of an embryo. I have myself met with two cases of intact
abortion sac with no embryo inside, and Giacomini {Sidle anomalie
di sviluppo dell'embrione umano, Torino, 1889-92) has reported
several. We can account for the absence of the embryo in various
ways : it may die early and be entirely absorbed leaving no trace,
although I am not convinced of this ; it may, as Giaconiini has clearly
shown, be found outside its usual place in the blastoderm, and it
must, therefore, be supposed that it has emigrated there (into the
coelomic cavity from the amniotic sac, or outside the chorion), or
has been developed ab initio there ; or it may never have de-
veloped at all. Under these circumstances, the embryonic annexa,
as we may term the chorionic vesicle and the umbilical vesicle and
amnion, may go on growing for a time and be cast off as an empty
abortion sac

If, however, the enibryo fail altogether to appear, if there be an
arrest prior to the appearance of the traces of an embryo, then we
may ask what morbid result will follow. Some years ago I ventured
to suggest that the product of such a gestation might be a hydatid
mole (Edinb. Hosjj. Rep., vi. 366, 1900); and recent researches have
gone to support this view and also to explain how it may be sup-
posed to occur. If we imagine an early ovum, like Peters', implanted
in the uterine mucous membrane, but with no embryo in it and no
developing system of blood vessels, then by the continued proliferation
of its trophoblast it might soon take on an appearance resembling
that of the hvdatid mole. Sfameni (Giorn. itcd. d. sc. tried., Nos. 5, 6,
7, and 8, 1903 ; Arch. ital. de biol, xl. 219, 1903-04) believes that the
vesicles of the hydatid mole are neither due to dropsy nor to myxo-

614 ANTENATAL PATHOLOGY AND HYGIENE

matous degeneration of the chorionic villi, but are simply syncytial
buds attached to each other by filaments ; and he is able to bring
forward several facts in proof of his assertion. If then the hydatid
mole consists essentially of syncytium, its development from the
ovum covered with trophoblast (which is the forerunner of syn-
cytium) is not an unlikely event. When the embryo dies early, or
does not appear at all, it is quite conceivable that the trophoblast
may go on growing in an ill-regulated way, may bury itself in the
mother's uterine wall, and so cause the typical hydatid mole. It
may be objected that an embryo is sometimes found in a hydatid
mole, but this occurrence is very rare, and may, when it does occur,
be regarded as a twin pregnancy in which one twin is represented by
the hydatid mole. Further, if we suppose that the trophoblast con-
tinue not only to grow but also to show its capacity for burrowing
into surrounding tissues and invading blood vessels, we are led on to
the now well-known deciduoma malignum or chorion-epithelioma.
Pieces of the trophoblast or syncytium may be carried to distant parts
of the mother's body and give rise to the metastatic growths. It is
thus seen how a morbid state of the blastocyst may produce pro-
foundly important effects both on the embryo itself and on its
mother.

Another result of the action of morbid agencies upon the blasto-
cyst may be the defective development of the amniotic sac and
membrane. It will be remembered that these structures precede the
embryo in order of development, and that the latter appears in close
connection with them, as Peters' blastocyst shows. It may be that
it is now that the defective development of the amnion occurs which
is, according to the amniotic theory, so effectual in producing
monstrosities and malformations of the embryo (vide Chapter XII.) ;
or perhaps want of development of the amnion may prevent even the
first rudiments of the embryo from appearing. Here, then, is the
germinal factor in the production of monstrosities by amniotic action,
which I referred to in dealing with the pathology of the embryo
(p. 222). The state of the amnion in the germinal epoch may, there-
fore, determine the appearance of monstrosities in the embryonic
period of antenatal life.

I have already alluded to another teratological state which may
possibly be determined during the blastocyst stage of germinal life,
I mean heterotaxy or transposition of the viscera. Whether there is
anything in the human blastocyst which corresponds to the vascular
area and its elliptical deformity in the chick is not known ; but it is
quite possible that the position occupied by the pro-embryon in the
blastocyst, its orientation so to say, may have to do with the produc-
tion of heterotaxy. Again, anomalies of the umbilical vesicle and of
the mesoblastic part of the allantois may exist prior to the appear-
ance of the pro-embryon and influence its future development;
perhaps sympodia and gastroschisis may in this manner be determined
in the germinal period of existence.

Much that has been said regarding the pathology of the blastocyst
is speculative, but it is not unsupported by certain facts. Further,

PATHOLOGY OF THE MORULA 615

at a stage when the most prominent structures are not the embryo
but what may be called its annexa, it is only reasonable to suppose
that the most prominent pathological changes will be in them and
not in the embryo, although the embryo may come to suffer greatly
on account of them. In such a state as hydatid mole with its sequel
chorion-epithelioma, the fate of the embryo is lost sight of in the
danger to the mother which is produced; the embryonic annexa, by
their irregular and excessive growth, produce in the mother's body
the most malignant form of neoplasm to which she is liable. The
trophoblast, therefore, if this view be correct, is, when normal, the
great source of life to the new organism (fixing it, supplying it with
nourishment, laying down the framework of the placenta), but when
abnormal it brings with it death to both embryo and mother.

Pathology of the Morula Mass.

We may now inquire what pathological processes may occur
in the morula mass before it becomes a blastocyst. The great
phenomenon seen in the morula is segmentation ; its physiological
manifestation is cell division and probably to some extent cell differ-
entiation, so that gradually the different kinds of cells seen in the
blastocyst come into being. Of information bearing upon the be-
haviour of the morula under various morbid conditions there is no
lack ; but, unfortunately, it is gained from invertebrate ova and from
those of frogs, and is the result of experiments which are not free
from fallacy. It is difficult, therefore, to estimate how far it can be
translated into the language, so to speak, of the conditions of the
human morula.

Chabry, struck by the fact that in the case of ascidian ova
abnormal segmentation resulting in the production of fractional
embryos sometimes occurred, made the attempt to bring about the
same effect experimentally. The method employed was that of
injuring one or more of the spheres composing the segmenting ovum.
It is well known that the first segmentation groove passes through
the median plane of the future embryo, and that the second is per-
pendicular to the first ; consequently, when there are four blastomeres,
two of these represent the anterior half (made up of a right and
left quarter), and two the posterior half (also made up of a right and
left half). Chabry found that if he destroyed one of the two primary
segments of the ovum there resulted a half-individual showing one-
half of the nervous system without closure of the medullary groove.
If he destroyed one of the blastomeres in the next stage of segmenta-
tion (four segments) there arose a three-quarter individual, if the two
anterior cells were destroyed only the posterior half developed, if the
two posterior were injured only the anterior half appeared, and, most
remarkable of all, if the two diagonal cells were injured the other
diagonal pair developed.

The experiments of Chabry were repeated and extended to other
animal forms by several biologists, but the results obtained were most
contradictory. W. Eoux experimented upon the blastomeres or

G16 ANTENATAL PATHOLOGY AND HYGIENE

cleavage cells of the frog's egg. If, in the two blastomere stage, he
killed one, the other developed into a typical half-embryo, an embryo
that was either the right or the left half of a whole one. He
concluded, therefore, like Chabry, that the first cleavage plane deter-
mined already the median plane of the adult ; he thought, also, that
the basis of all differentiation was given by an unequal division of
the nuclear substance during karyokinesis. Hans Driesch, however,
repeated these experiments (which have come to be called Uastotomy
or blastomerotomy) upon the sea urchin's egg, and found that the
isolated blastomere cleaved like half the egg, but it resulted in a whole

CO '

blastula and a whole embryo which differed from a normally produced
oue only in its small size. Similar results were obtained by Wilson with
the eggs of amphioxus, and by Zoja with those of medusae. Driesch
went further and discovered that any one of the first eight or sixteen
cells of the sea urchin's egg could develop a normal embryo ; in the
case of ctenophore eggs Driesch and Morgan found an isolated
blastomere could develop a half-embryo, but so could a bit of proto-
plasm cut off from the fertilised egg before cleavage had begun.
0. Schultze and Hertwig, taking the frog's egg for experimentation,
were able to get either a whole embryo or a half one from one of the
two first blastomeres by varying the conditions. It was thought
that perhaps these different results might be due to the fact that
in some cases the destroyed segment or blastomere was removed
while in others it was allowed to remain ; in the former case its
removal may have permitted the action of post-generation, while in
the latter its retention may have prevented this phenomenon.

Since the results have been contradictory it is not permissible to
draw conclusions with much security. It may be said, however, that
in at least some forms of life the potentiality of all the blastomeres
of the segmented egg is the same ; each segment may play any or
every part in the future ontogenesis ; Driesch, therefore, calls the
whole of the segmented egg an " aequipotential system," the prospec-
tive value of each blastomere depending upon, or being a function of
its position in this segmented mass. At the same time it has to be
admitted that there is a degree of orientation in the segmentation
mass, otherwise it is impossible to imagine why organs should be
formed at one special part of it and not at another. Differentiation
depends, according to Driesch, upon a " primary, innate orientation
of the egg plasm in those forms, the segmented eggs of which repre-
sent sequipotential systems." Further, this orientation is' capal >le i >f
a sort of regulation or restoration after disturbances of any kind ;
but in the ctenophore egg such a regulation is apparently not
possible, and in that of the frog it is possible under some condition's
but not under others (facultative).

Another method of experimentation which has been used to
determine the conditions of life in the stage of segmentation lias
been by altering the environmental conditions. Light, a sufficient
temperature, oxygen, and a certain chemical constitution of the
surrounding medium appear all to be essential for the growth of
normal forms ; while the absence or alteration of these conditions

PATHOLOGY OF THE MORULA 617

lead to anomalies. It has been found, for instance, that sulphate of
quinine and chloral hydrate added to the water in which the ova
of certain marine invertebrates were segmenting, caused the segmen-
tation to begin with the tetraster stage. Gurwitsch discovered in
the case of Ranafusca and Bufo vulgaris that sodium bromide and
chloride and lithium chloride were teratogenic, and that the two first
named when acting together produced anencephaly.

Although, therefore, there is no lack of information bearing upon
the pathology of the morula stage of antenatal life, there is great
difficulty in making any use of it for the purpose of elucidating the
phenomena of human teratogenesis. There is this striking circum-
stance about human germinal life to be borne in mind that apparently
the whole activity of the morula mass is, in the first place, exercised
in constructing the annexa of the embryo, and that the embryo itself
grows upon the annexa at a later period. Thus the whole ovum
ci uiies to consist of the trophoblast and its contained amniotic and
umbilical sacs with ectoderm, entoderm, and mesoderm ; apparently
it is only then that the first traces of the embryo begin to appear
between the amniotic sac and the umbilical vesicle. We may take
it that, up to a certain stage in the formation of the blastocyst, the
cells composing it are alike in appearance, and that then they assume
the characters of ectoderm, mesoderm, and entoderm, and arrange
themselves into trophoblast, amniotic sac, umbilical vesicle, and
ccelomic cavity; but I think it would be rash to conclude, from
experimental work on the ova of invertebrates and frogs, that the
cells, even when they are similar in appearance, have not already
their destiny fixed. Possibly the fate of the cells is to some extent
determined by their position relatively to each other, but it is
unlikely (although not impossible) that this entirely decides whether
a given cell shall be ectoderm or entoderm, or, to carry it still further,
whether it become ectoderm of trophoblast, of amniotic membrane, or
of embryo. There is some probability, however, that if the cells of one
part (e.g. entoderm) be destroyed or fail to appear, there may be an
over-production of those of another part (e.g. ectoderm).

If we may hazard a guess concerning the results of the action of
morbid agencies (e.g. toxic substances, microbes, toxines) upon the
human ovum in the morula stage, it must be founded upon the
peculiarities of the physiology of this stage. If we suppose that here
again pathology takes the character of arrested development, we may
imagine the morula remaining in an undifferentiated state, or having
reached a later stage (e.g. that just before the appearance of the
amnion or of the embryo), of ceasing to advance further in onto-
genesis. In this way it is apparent that some of the morbid states
of the blastocyst (absence of embryo, hydatid mole) may be initiated
in the stage of segmentation. Further, we may perhaps suppose
that some cells are arrested in their undifferentiated state, and are
embedded in the midst of more progressive elements ; they may thus
constitute Colmheim's rests, and give origin years later to neoplasms
by a sudden resumption of activity, either of the nature of simple
multiplication or of the formation of tissues of one layer of the

618 ANTENATAL PATHOLOGY AND HYGIENE

blastoderm {e.g. dermoids) or of two (teratomata). Possibly, in this
way, some cells of the embryonic annexa (trophoblastic, amniotic, or
other) become embedded in the embryo itself, and give rise to the
curious growths in the male which histologically resemble the
hydatid mole and the chorion-epithelioma of the female. I think,
however, I have devoted quite enough space to guesses about the
pathology of the morular stage of life ; but I may add one more, in
relation to the causation of extra-uterine pregnancy. The tropho-
blast is recognised to be the means by which the ovum attaches itself
to the uterine wall ; presumably, then, if the ovum be detained in
the tube till the trophoblast is fully formed or if the trophoblast be
formed prematurely, it may effect a lodgment upon the tubal mucosa.

Pathology of Fertilisation.

The impregnation of the mature ovum by the spermatozoon is
the evident phenomenon of the intra-conceptional period of germinal
life. It is indeed the most important fact in all antenatal existence.
Like the other great events of life, such as the birth of the infant
into its extra-uterine environment and that of the embryo into the
foetal period, it is marked by an appalling mortality. It is at any
rate well known that not every ovum becomes fertilised, and Balfour
has stated that the whole of the spermatozoa derived from a sper-
matocyst are together equal to one ovum, — that is to say, eight
hundred and forty-eight millions of spermatozoa. Surely I am not
exaggerating when I describe such a sperm and germ mortality
as appalling. Nature in this, as in her other operations where the
survival of the race is at stake, provides lavishly for all emergencies ;
apparently many cells must die that one human being may be
produced.

The physiological event of fertilisation is the entrance of one
spermatozoon into an ovum ; so it may be supposed that the patho-
logical event may be the entrance of two or more spermatozoa.
The physiological purpose of the act would seem to be the production
of one individual from the two elements (male and female genoblasts) ;
so the chief result of its pathology may be the production of two
individuals, united directly (structurally continuous, as in the double
terata) or indirectly (through the vessels of the umbilical cords,
as in the placental parasites). Binovular twins arise doubtless in
another way (two ova) ; but uniovular or monochorionic ones may
be imagined as also produced by the penetration of one ovum by
two spermatozoa.

The earliest theory of the origin of double terata regarded them
as due to the fusion of two originally separate ova. In the case of
the hen's egg, double yolks are often found, and according to this
theory these ought to give rise when incubated to double monsters.
This result has not been obtained by the numerous experimenters
who have essayed it. A curious exception to this general experience
was brought under my notice by A. H. Loeb of Montreal in 1897.
Mr. Loeb sent me a photograph of a partly double chick, which I

PATHOLOGY OF FERTILISATION 619

showed at a meeting of the Edinburgh Obstetrical Society (Trans.
Edinb. Obstet. Soc, xxiii. 53, 1898) ; it had a pair of supplementary
legs, which seemed to have a distinct articulation slightly posterior
to the normal set ; and the pelvis was longer than usual. The chick,
which lived two weeks, was the product of the incubation of a large
egg with, presumably, a double yolk, which had been placed under
a sitting hen. The circumstances of the case did not quite exclude
fallacy ; but the occurrence is worthy of record in face of the
accumulated mass of evidence against the theory that double-yolked
eggs produce double monsters.

Another theory of diplogenesis looks to fissure or splitting of an
originally single embryo as the determining cause : but it is very
noteworthy that experimental teratogeny has not supported this
view. The production of double embryos by means of teratogenic
influences acting upon an already formed embryo has not been
accomplished, and it is now reasonable to suppose that the double
nature of the organism is determined before the appearance of the
rudiments of the embryo in the germinal area. The determining
cause may be the entrance of more than one spermatozoon into the
ovum in impregnation, namely, polyspermy. At the same time it is
possible— theoretically possible — that it may be the existence of an
ovum with two germinal vesicles ; but such a phenomenon is much
rarer than polyspermy, and requires after all two spermatozoa for
the fertilisation of the two female nuclei. The theory of polyspermy
is that accepted by Duval as most satisfactorily meeting all the
requirements of the case, and it certainly affords a very good working
hypothesis to account for the various kinds of double terata. At the
same time it has to be borne in mind that it is largely founded upon
the experiments of Fol upon echinoderm ova, and that the same
results have not been obtained by other authors. Fol found that by
submitting the ova to the influence of water containing much car-
bonic acid, two, three, or more spermatozoa entered and a complex
karyokinetic figure with three or four poles in place of two resulted,
and the segmentation spheres were double in number to what they
were in other ova of the same age. Duval concluded that two (or
more) spermatozoa united with the single female pronucleus and so
produced the double development. It is now known that in some
animal forms there is a physiological polyspermy which does not
result in the changes described by F^ol. Further, 0. Schultze (Arch,
f. Eritwelingsmeehan, cl. Organ., i. 268, 1894) has in the case of frogs'
eggs succeeded in getting double monsters by mechanical means
(gravity) quite apart from polyspermy. It is significant and note-
worthy, however, that he agrees with Duval in believing that the
formation of double monsters is determined before or during fecun-
dation. He does not think it is due either to polyspermy or to
anomalies in the structure of the spermatozoa. He is therefore
forced to admit that it must be caused by a condition of the ovarian
ovum before impregnation, and he believes this to be an incomplete
cellular division. He thinks that it is of the same nature as the
formation of twins, and that in both instances we have to do not

620 ANTENATAL PATHOLOGY AND HYGIENE

with an over-production of embryonic material, but with an arrested
development.

In the presence of such contradictory results it is impossible to do
more than express the opinion that the formation of double monsters
is decided either before or during impregnation and not after the
appearance of the embryo. It is due, therefore, to a germinal cause.
I shall have something to say of the different varieties of double
terata in the following chapter, as well as of the mode in which
polyspermy is believed to produce them.

I have already referred (on p. 10:5) to another possible patho-
logical phenomenon of the period of fertilisation, I mean the entrance
of the ovum by a spermatozoon of a different species, or hybridity.
It is doubtful whether this question need to be considered from the
standpoint of human teratology, but it is noteworthy that Duval
regarded it as a possible cause of grave monstrosities in animals.
He thought that the sterility which attends the union of animals of
different species may not really be due to absence of the production
of an embryo, but to the production of one so grossly and intensely
teratological as to be incapable of any development beyond the
earliest stages. If this be so (I think it is open to doubt), there is
here another teratological process taking its origin during the act of
fertilisation.

Pathology of Maturation.

The terminal event of the preconceptional period of germinal life
is maturation of the ovum and a somewhat similar phenomenon in
the sperm-cell. There is a very considerable difference of opinion
among embryologists as to the meaning to be given to maturation,
but its purpose seems to be the introduction of sexuality and sexual
reproduction into the life of the species. Further, sexuality may
have as its object rejuvenation, the production of variability, or the
prevention of variability. Now, there may exist very different views
about these matters, but there can be no doubt that maturation is
the preliminary condition of normal impregnation and subsequent
development. It is, therefore, quite permissible to inquire what may
lie the results of abnormal maturation. Theoretically it would appear
that the results must be teratological, and very markedly teratological ;
and there is good reason to believe that this is really the case.

Parthenogenesis or development without impregnation is a
phenomenon met with not infrequently among the lower animal
types. Several generations may be thus produced, but sexual repro-
duction must sooner or later intervene, and it has been noted that
the individuals produced parthenogenetically become feeble and
monstrous as the limit of the parthenogenetic period is bein.u
approached and the necessity for sexual reproduction becomes
clamant. Now it is quite in keeping with what is known of the
animal world to expect that in the higher vertebrates, and even in
the highest vertebrate, an occurrence which is common among the
lower forms may lie met with exceptionally. As a matter of fact
there is evidence that an imperfect parthenogenetic reproduction

PATHOLOGY OF MATURATION 621

consisting in the segmentation of the imimpregnated ovum does
sometimes take place in the ovary of the human subject ; the result
is a dermoid cyst of the ovary. Kecent researches have revealed the
existence of a long series of types of dermoid cysts showing all the
gradations from a growth containing some hairs and skin to one con-
taining a rudimentary hut quite recognisable embryo. It is note-
worthy that structures known to be derived from the ectoderm are
those most commonly met with in such cysts, that parts arising
in the mesoderm are less common, and that endodermic productions
are extraordinarily scarce. In many cases only the parts arising
from the ectoderm are present. It is clear, therefore, that here we
have to do with teratological productions so monstrous that one or
two of the three layers of the blastoderm are quite undeveloped ; in
them teratology has reached its limit. It is permissible also to
conclude that the predominance of structures derived from the
ectoderm is due to the fact that the ectoderm is the earliest of the
layers to be formed.

It is believed that the dermoid cysts of the ovary are due to
imperfect segmentation of ova that have not been expelled from their
Graafian follicles. Such imperfectly segmented ova have been found
in various mammals, including the Cheiroptera, and even in the
human subject, especially in the case of puerperal peritonitis, where
it is fair to suppose that an irritant may have excited the ovum to an
abortive segmentation. The development is always very rudimentary,
and may have the character of two or even three or more centres of
segmentation, so to speak. In the case of the dermoid cyst described
by Eepin, for instance, there was found an embryo with a spinal
column, a head, and four limbs, but with no intestinal canal ; lying-
near to it, however, but not attached to it, was a cylindrical, con-
voluted, cord-like structure, containing a substance similar to
meconium: there had been two centres of embryonic formation.
This peculiarity Duval calls defect of individualisation, or apo-
larity of the blastoderm. Other theories have, of course, been
advanced to explain ovarian dermoids (such as extra-uterine preg-
nancy, foetal inclusion, and enclavement) ; but parthenogenesis meets
the difficulties of the case best of all, for it explains how dermoids
may appear before puberty, and can therefore not be due to extra-
uterine pregnancy, and how they may contain teeth of the second
dentition, and very long tresses of hair, for they may have lived a
long time in the ovary.

A similar explanation may be advanced to account for some of
the teratomata or embryomata of the testicle, for it is known that
primordial ova exist in the testis as well as in the ovary, but in
smaller numbers, and one of these may have taken on a partheno-
genetic development. It may also be that the sperm-cell is capable
of similar changes. According to this theory of ovarian and testicular
dermoids, they are to be regarded as very rudimentary embryos
produced parthenogenetically : they are therefore, as Duval points
out, to be looked upon not as the sisters or brothers of the individual
in whose genital gland they are found, but as his or her children.

%

G22 ANTENATAL PATHOLOGY AND HYGIENE

Finally, it is, I think, quite in keeping with what is known of the
life of the egg-cell and sperm-cell, that defects in maturation, arising
perhaps from the presence of microbic or toxic substances, may lie
the exciting causes of the parthenogenetic segmentation. Possibly
the expulsion of one polar globule instead of two, or the expulsion of
none at all, may 1 ie the error in maturation ; but we are dealing with
matters so debatable that speculation becomes futile, perhaps also
dangerous.

Before I proceed to the study of the pathology of the reproductive
cells before maturation, I must consider very briefly, synoptically in
fact, the varieties of double terata and included foetuses.

CHAPTER XXXII

Polysomatous Tcrata : Uniovular ov Monochorionic Twins : Allantoido-
angiopagous Twins ; Definition, .Synonyms, Varieties (Paracephalus,
Acephalus, Amorphus), Teratogenesia : United Twins or Double Terata ;
Symmetrically United (Syncephalus, Dicephalus, Thoracopagus), Asym-
metrically United, Teratogenesis : Triplets, Normal, Malformed, United :
Quadruplets : Quintuplets : Sextuplets : Septuplets.

In this chapter I shall endeavour to give a short sketch of the
subject of double terata. It will be nothing more than a sketch,
for reasons which I have already stated ; but I may add that since
Taruffi gives 1650 pages out of a total of 4500 in his great Storia
del/a teratologia to the consideration of double monsters, it is plain
that if I were to devote a proportionate amount of space it ought to
be two or three hundred pages rather than twenty or thirty. At the
same time it is to be borne in mind that Taruffi deals with the double
monsters in an exceptionally thorough fashion.

Polysomatous Terata.

If the reader will turn back to page 235 he will find there the
various monstrosities which are usually grouped under the term
" polysomatous terata." The name, roughly speaking, corresponds
to the " monstrosities by excess or abundance " of other writers ; and
it includes uniovular (or monochorionic) twins, allantoido-angiopagous
twins or placental parasites, united twins or double monsters proper,
and triplets, quadruplets, and other plural births. On pages 237 and
238 I have given the leading features of these four groups ; my reason
for placing them here under the pathology of the germ is that I
believe them all to be determined during the germinal period before
the first rudiments of the embryo have appeared in the blastocyst.

Taruffi (op. cit., ii. 103, 1882) grouped the monochorionic twins,
the placental parasites, and the united twins together under the
heading disomata; then he divided them rather appositely into
dicerctic and synceretic disomata. The diaeretic are the monochorionic
twins and the placental parasites, in both of which the union is not
direct but indirect, a matter of diaeresis, through the placenta or vessels
of the umbilical cord ; the synseretic are the united twins, in which
the union is intimate and direct between the bodies of the twins,
a matter of symeresis. Although the names are appropriate and
the distinction is an important one, I hesitate to use them here on
account of their unfamiliarity to the British reader.

623

624 ANTENATAL PATHOLOGY AND HYGIENE

Uniovular Twins.

Under the names of " uniovular twins," " monochorionic disomata,"
and " homologous twins " are described those cases in which there are
twins of the same sex, which show no gross malformations, enclosed
within a single chorion and provided with a single placenta.
Binovular (or dichorionic) twins may be either of the same sex or
of different sexes, but uniovular twins are always of the same sex.
Exceptions to this rule are very rare and are capable of being
explained away : the twins may be really of the same sex but one
may be a pseudo-hermaphrodite. Possibly this is the reason why in
veterinary practice the " free martin " is sterile, the " free martin "
being the supposed cow-calf born co-twin with a bull-calf but being
really a pseudo-hermaphroditic male. In the absence of exact details
regarding the annexa (placenta, chorion) it is impossible to be certain
about this matter. It is a striking fact that in the human subject
cases in which both twins were the subject of a genital mal-
formation have been put on record ; errors in the declaration of sex
were thus caused. There is no foundation for the belief that the
female born co-twin with a male is sterile in the case of the human
subject.

As a general rule, uniovular twins show a strong resemblance to
each other. A good example of this occurred in connection with a
patient who was being treated for hydramnios in the " Hamilton
Bed" in the Royal Maternity Hospital, Edinburgh, in November
1901. The patient was a X-para, thirty-seven years of age, and was
admitted suffering from vomiting ; her abdomen was much distended,
and many foetal parts could be felt ; labour came on, and when the
membranes ruptured there was a great gush of liquor amnii ; one
twin presented by the head and the other by the breech ; both twins
were females and closely resembled each other in appearance ; and
the placenta and the chorion were single. Some cases are on record
where twins had identical malformations (e.g. Polydactyly), but it is
not always possible to ascertain whether they were uniovular or
binovular (Windle, Journ. Anat. ami Physiol., xxvi. 295, 1891-92 ;
Proc. Birmingham Philos. Soc, viii. 139, 1892).

Uniovular twins are not always of equal size, and when one dies
in utero it may be expelled as a small flattened fcetus (foetus cvm-
prcssus) along with a living full-sized infant (vide p. 145). It has
been found, however, that the foetus compressus may be the result
of the death of one of binovular twins, so that it is not peculiar to the
uniovular type. Both twins, however, may be born alive and yet
differ much in size ; this occurrence is probably to be ascribed to
asymmetry of the circulatory relations of the twins in the placenta,
and that in its turn may be due to one embryo being originally
weaker than the other. This vascular asymmetry reaches its maxi-
mum in the next group of monstrosities, the allantoido-angiopagous
twins.

In uniovular twins the placenta and chorion are single ; but there
may be, and there usually are, two amnions. In the very rare cases

UNIOVULAR TWINS 625

in which there is only one amnion, it is generally believed that the
amniotic membrane between the two originally separate sacs has been
destroyed. There may be two umbilical vesicles, widely separate or
near to each other, or there may be only one (F. Ahlfeld, Arch. f.
Gynaek., xi. 160, 1877). One cord may have a central and the other
a marginal insertion into the single placenta ; occasionally the two
cords get knotted and twisted together.

Uniovular twins are rarer than binovular, the former constituting
about 12-25 per cent, and the latter about 87"75 per cent, of all twin
births; about one uniovular twin pregnancy occurs in 711 single
gestations. Different results, however, are obtained in different
countries. The following general characters have been fairly well
established for uniovular twins : there are more males than females ;
the mothers are apt to be young or relatively old, and multipara are
not commoner than primiparse ; the difference in weight between the
twins is often considerable; foetal death and malformations and
hydramnios are commoner in uniovular than in binovular twins ; and
heredity is not usually present (liumpe, Ztschr. f. Geburtsh. u. Gyndk.,
xxii. 344, 1891).

While binovular twins are doubtless due in most cases to the
impregnation of more than one ovum, and this, again, is predisposed
to by an excess of ovisacs in the ovaries (persistence of a foetal
condition) ; uniovular twins may be ascribed, perhaps, to polyspermy,
to the penetration of one ovum by two spermatozoa, or, perhaps, to
the presence of two germinal vesicles in one ovum. Polyspermy
may lie due to an arrest in the train of phenomena which ensue
when one spermatozoon gains entrance to an ovum ; it may be sup-
posed that normally some mechanical or chemical condition prevents
the entrance of a second spermatozoon, and that in abnormal circum-
stances this is interfered with, and so another impregnating element
finds its way in. Two embryos may then appear in the blastocyst.
Both in the binovular and in the uniovular twins, therefore, the
ultimate cause may be an arrested development, the persistence of a
state which ought to be temporary ; but this is much more evident
in the former than in the latter. This being so, it is permissible,
I think, to call both kinds of twins teratological ; but it is customary
to separate the uniovular off from the others as being more clearly
anomalous, and the separation may be retained although it is probably
artificial.

Allantoido-Ang-iopagous Twins.

Allantoido-angiopagous twins are uniovular or monochorionic
foetuses which communicate wTith each other by means of the vessels
of their umbilical cords near to or in the single placenta; one of
them is usually fairly normal in structure, while the other shows
an amount of deformity which is always considerable and may be
extreme. They are always of the same sex. They have been called
placental parasites ; but the name is hardly suitable, for one of them
(the deformed one) is really a parasite upon the other (the normal
VOL. ii. — 40

626 ANTENATAL PATHOLOGY AND HYGIENE

one) by means of the vessels in the placenta ; they are not, there-
fore, parasites upon the placenta. They have been called omphalo-
angiopagous disomata by Tarufri (op. cit., ii. 133, 1882), but I prefer
the term " allantoido-angiopagous " as etymologically more correct,
for it is through the allantoic vessels that the communication between
the twins is established. The most striking feature about those
foetuses is the degree of deformity which is reached by one of them,
the parasitic one ; it is in such monstrosities as the acephalic, acormic,
anidean, and acardiac, that Nature attains to the extreme limit of
teratological expression. Apparently, the presence in utero of two
foetuses makes it possible for one to assume such grotesque, bizarre,
and extraordinarily aberrant forms, in which the appearance of the
human embryo is nearly, if not quite, lost. Doubtless, it is through
its parasitic relation to its normal brother or sister (the autosite)
that the deformed twin (the parasite) is able to reach such a degree
of monstrosity and yet live on in the uterus.

It has been my fortune to meet with several specimens of the
allantoido-angiopagous twin, and some years ago I published a series
of articles upon them (Trans. Edinb. Obstet. Soc, xviii. 38, 94, 201,
257, 1892-93, xix. 41, 61, 73, 1893-94; Teratologics, i. 1, 25, 158,
1894, ii. 182, 1895), to which I refer my readers for details and
bibliographies. Fine articles on the same subject, with literature
lists, are contained in Tarum's work (Storia delta teratolor/ia, ii. 1 ■">■">,
1882 ; hi. 480, 1885 ; iv. 137, 1886 ; viii. 444, 1894), and in that by
F. Schatz which bears the somewhat inappropriate title of KliniscJie
Beitrage zur Physiologic dcs Fetus} I may give here the leading
characters of the three chief groups into which allantoido-
angiopagous deformed twins have been divided — the paraomphalic,
the acephalic, and the amorphous monstrosities.

The paraceplialic twin is distinguished by the possession of a head ;
the other parts of the body and the limbs show different degrees of
imperfection, indeed the lower limbs or the lower limbs and trunk
may be absent, and the upper limbs are commonly very defective.
The type which approaches most nearly to the normal is the foetus
with a more or less deformed head, a trunk, and lower limbs, with
malformed or absent upper limbs, and with a heart ; to this foetus
the name parac&flialus dijms cardiacus has been given, and I have
described a case in Tcratologia (vol. i. 158, 1894). The heart,
although present, is nearly always greatly malformed; it is some-
times not in direct communication with the umbilical vessels, so
that for physiological purposes the foetus might almost be without a
heart. Most of the internal organs may be absent or grossly
deformed. The vessels of the umbilical cord are usually defective
and communicate with those of the cord of the normal twin. There
must, then, be two circulations in the monster, one in connection
with its own heart, and the other kept going by the heart of the
co-twin through the allantoidal communicating vessels ; both these
vascular arrangements are doubtless imperfect, an imperfection which

1 This work, published in Berlin in 1900, consists mainly of papers reprinted from
the Arehivfur Gynoekologie,

ALLANTOIDO-ANGIOPAGOUS TWINS 627

is the cause of the dropsical state of the subcutaneous tissue which
is so commonly noticed. The two distinguishing features are the
presence of a heart and the existence of two feet (dipus), although
some toes may be wanting. The more common type of paracephalus
dipus has no trace of a heart, hence its name, paracephalus dipus
acardiacus. A striking example of this monstrosity I described in
1892 (Trans. Edirib. Obstet. Soc, xviii. 44, 201, 1892-93): it had a
fairly well formed head, but there was no foramen magnum ; there
was no trace whatever of a vertebral column ; and the shoulder and
pelvic girdles (with their attached limbs) were simply held together
by a narrow bridge of muscular and adipose tissue. In foetuses of
this kind the only circulation is that (an inverted one) which is
forced into them by the heart of the normal co-twin. At the same
time it is very remarkable that in their morbid anatomy they so
closely resemble the paracephalics with a heart.

Another variety of the paracephalic twin is that in which there
is only one lower limb ; the single lower limb may represent two
fused together (paracephalus sympus), or it may really consist of the
right or left lower extremity (paracephalus monopus). In all the
known specimens of this type (there are not many) there is no record
of a heart ; they are all, therefore, acardiac. There is usually only
one artery in the umbilical cord. Another rare variety is paracephalus
apus, in which there are no traces of lower limits at all : the upper
limbs, likewise, may be wanting. I have found records of two speci-
mens with a heart, and of four without that organ (Trans. Edirib.
Obstet. Soc,, xviii. 263, 1893).

Finally, there is the paracephalus acormus or pscudo-acormus, the
foetus which consists solely of a more or less perfect head and an
absolutely rudimentary trunk. This type marks the extreme limit
of imperfection which can be reached by the paracephalic monstrosity ;
the individual is reduced to a head without a body. It is no matter
for wonder that for some time teratologists refused to believe in the
existence of such " asomatic omphalosites " or " cephalides." The
cases reported by Nicholson (Diss, inaug., Berlin, 1837), Eumpholz
(Diss, inaufj., Halis, 1848), H. C. L. Barkow (Ueber Pseudo-akormus,
Breslau, 1854), and others are, however, so clear that doubt is no
longer possible. I have met with a case which was either an acormic
paracephalus or a dermoid cyst expelled per vaginam in labour ; it
was an osseous box, like a cranium, with a pedicle and vessels running
in it. Unfortunately the placenta of the normal child born at the
same time was not kept (Trans. Edinb. Obstet. Soc, xvii. 49, 1891-92).
The head in such cases is generally deformed ; thus, in Eumpholz'
specimen there was a single eye with a nasal projection above it as in
cyclopia, and in Nicholson's there was pseudencephaly. In Barkow's
case the only traces of a body were some rudimentary vertebrse
and ribs.

The acephalic twin is sharply marked off from the paracephalic
by the absence of a head ; a trunk is usually present, but the foetus
may in some cases be reduced to a pelvis and lower limbs. This is
the best known type of the allantoido-angiopagous twins ; and it is

628 ANTENATAL PATHOLOGY AND HYGIENE

relatively common, for Taruffi (op. tit, ii. 174, 1882) was able to
collect about 120 instances from literature, and there have been other
instances described during the past twenty years. In the great
majority of the records the heart is described as absent, so that the
term " acardiac " has been used as synonymous with " acephalic " ;
but this is not an invariable rule, and a group has been constructed
under the designation acephalus thorns cardiacus to include the rare
exceptions. In these (e.g. in YYulfshein's specimen, Diss, inaug., p. 13,
Berolini, IS.'!.")) there is a trace of a structure which may be regarded
as a heart. The more common variety, acephalus thorns acardiacus,
has a thorax and occasionally some traces of upper limbs, but no
heart; the arms, however, are often wanting as well as the heart.
Again, the thorax may be absent and the foetus reduced to an
abdomen, a pelvis, and lower limbs : this type is known as acephalus
athorus. Finally, just as the extreme limit of deformity in the
paracephalians was reached in the presence of little more than a
head, so in the acephalians it is attained in the existence of little
more than the lower limbs (acephalus pscudo-acormus). A specimen
somewhat difficult to classify was that reported by A. E. Simpson
(Trans. Edinb. Obstef. Soc, iii. 26, 1872); it showed three legs, and
resembled the impression on an Isle of Man penny.

The amorphous twin is one which lacks the external appearances
proper to a foetus ; it consists of little more than an ovate or rounded
skin-covered mass with the indications of limbs (foetus amorphus
mylacephalus) or without these (fcetus amorphus anideus). The co-
twin here, as in nearly all these varieties, is almost if not quite
normally formed. I have seen two specimens of the mylacephalous
twin ; one was that reported to the Edinburgh Obstetrical Society by
Sir Halliday Croom in 189.°> (Trans. Edinb. Obstef. Soc, xviii. 69,
189:5), the other I had an opportunity of examining in the Obstetrical
Museum in the University of Aberdeen in 1897. I have met with
two cases of the aniclean variety, one of which was described by
J. C. Webster (Teratologic/,, ii. 178, 1895), and the other by myself
(Teratologia, i. 1, 1894); the report of a third specimen, peculiar on
account of the small size of the anidean fcetus (little over an inch in
length), was sent to me by Drs. Eidley Mackenzie and John M'Crae
of Montreal.

My specimen of anideus (Fig. 87) consisted of an ovate body
about the size of the closed fist, completely enclosed in transparent
membranes (chorion and amnion). Near one end, regarded pro-
visionally as the upper, a few hairs were visible, and on one surface,
regarded as the anterior, were certain elevations and grooves. An
examination of the membranes showed that the foetus was enclosed
in an amnion of its own, and was also attached to the common chorion
and to the amnion of the normal twin. In order to reveal the internal
appearances a section was made through the mass (Fig. 88). There
was an external covering of skin and a markedly oedematous sub-
cutaneous substratum ; below that was a dense layer of adipose tissue
divided up into lobules by bands of connective tissue ; under that
again was an irregular layer of striped muscular fibres, showing a

FCETUS ANIDEUS

629

^

*

\

V

^N^

sss

^

Fig. 87. — External Appearances of Fcctus Anideus and its Investing
Membranes (reduced by about one-third).

a and b, amnion and chorion ; c, tuft of hairs near upper end ; d, blood
vessel entering fretus ; e, second blood vessel passing in tag of
membrane ; /, projection on anterior aspect, containing coils of
intestines.

630

ANTENATAL PATHOLOGY AND HYGIENE

kind of fatty degeneration, and lying in close contact with the osseous
kernel. Here and there were sebaceous glands and blood vessels.
On the anterior aspect, near the upper end of the foetus anideus, was
a furrow or fissure leading into a somewhat irregular cavity, lined by
a smooth serous membrane and containing some loops of intestine
(12 to 15 cms. in length). The osseous kernel consisted of a confused
mass of pieces of bone and cartilage, which probably represented
rudimentary sacral and coccygeal vertebras. The vascular supply, an

Fie. 88. — Sectional Appearances of Fietus Anideus (natural size).

a, amnion passing into furrow ; b, the furrow on anterior surface ; c, coil of
intestine ; d, surface of large projection ; e, slight furrow ; /, blood
vessels ; y, long bone in transverse section ; h, sebaceous gland ; l\
muscular fibres ; I, adipose tissue ; m, central osseous mass (vertebrse).

artery and a vein, passed to the foetus in the membranes and entered
it in the neighbourhood of the intestine-containing cavity.

The teratogenesis of the allantoido-angiopagous twin has been the
fruitful source of much speculation and of many ingenious theories.
These strange twins have always excited interest and roused curiosity.
At first they were used in arguments as to the method in which the
foetus was nourished, until it was discovered that the unborn infant
receives its food supply through its umbilicus and not through its

ALLANTOIDO-ANGIOPAGOUS TWINS 631

mouth. Then, again, the circulation of blood in them has been the
subject of much discussion, for obviously the absence of a heart made
it difficult to understand how that function could be carried on ; at
first the contractile force of the arteries was invoked, and then the
heart of the normal twin was recognised as the propelling agent,
and gradually the placental anastomoses were recognised and their
significance realised. It came to be seen that the deformed twin was
dependent upon the placental circulation of the normal co-twin for
his life, such as it was, for, of course, there is no possibility of any
other than an antenatal life for such grossly malformed foetuses as
the acephalic and the acormic.

Then a new series of difficult problems arose for solution. Why
was one twin normal and the other so gravely malformed ? How did
the heart of one twin come to carry on the circulation of both ?
Many explanations were suggested, but they were unsatisfactory.
Light began to be thrown on the subject when M. Claudius (Die
Entvricklung der herzlosen Missgt burten, Kiel, 1859), F. Ahlfeld (op. cit.,
p. 39, 1880), Dareste (op. cit., 482, 1891), and F. Schatz (op. cit, 1900)
commenced to study it. The idea that one twin obtains a larger
share of or an earlier attachment to the vessels of the chorion forms
a good working hypothesis, and the details, although complicated, can
then be elaborated and individual peculiarities explained. It may be
that the allantois of one twin grows out earlier than that of the other ;
it may be that one twin's placental connection is through the omphalo-
mesenteric vessels and not through the allantoic at all ; in one way
or another, one twin, as it were, becomes the predominant partner,
and the other is so imperfectly nourished that it grows malformed
and monstrous. If it were not for the twinship existing in the uterus
no such malformed fcetus could be imagined to be capable of even an
antenatal existence. One twin, then, according to this view, first
starves the other, and, having reduced it to a markedly imperfect
state, next keeps it alive by sending some of its blood to it through
the placental circulation which is common to both. Another ex-
planation, however, is possible : one twin may, to begin with, be
imperfect, so imperfect as to be incapable of more than the most
ephemeral antenatal life ; but, through the presence of a twin embryo,
it becomes attached to a new source of nourishment, and is, as it were,
preserved in existence for a little longer time. According to the
second explanation, the normal twin plays an entirely beneficent part ;
while, according to the first, it has a maleficent effect, and then, later,
a beneficent one. It may be that the defective twin is one of those
fractional individuals which experimental teratogeny has succeeded in
producing, and that, but for the fact of the presence of a twin in utero,
it would not have lived long enough to be recognised. The terato-
genesis of the allantoido-angiopagous fcetus, therefore, resolves itself
into two separate conditions, the twinship and the deformity ; and it
is not clear whether the former is the cause of the deformity or is the
means by which the life of the deformed embryo is continued although
but for a brief space of time. The whole question is one of the most
interesting which can be well imagined.

G32 ANTENATAL PATHOLOGY AND HYGIENE

United Twins or Double Terata.

If we imagine an allantoido-angiopagous twin situated so near to
its normal co-twin as to be structurally continuous with it, we have
one of the types of double monsters that we are now about to
describe. For instance, a foetus consisting of a trunk and limbs may
be fixed to the epigastric region of a normal foetus ; this is just as if
the umbilical cord attaching an acephalus to its co-twin had been
shortened till it came to be unrepresented outside the body at all.
Cases of this kind are called asymmetrical or parasitic double terata,
and concerning them something more will be said immediately ; but
the more typical united twins are the symmetrical ones, those in which
two individuals of practically the same size are more or less com-
pletely fused together.

To the first great type of the symmetrically united twins or
disomata the name syncephalus has been given: in it the two foetuses
are joined together more or less closely by the heads, the trunks
being either separate or fused. A synonymous expression is
prozyyosis. The chief sub-types, with their leading features, may be
indicated in tabular form, Tarum's arrangement being followed.

I. Craniopagus : Twins united solely by the heads,

a. Acrocephalus pagus. The union is by the sinciputs.

b. Iniopagus. The union is by the occiputs.

c. Metopagus. The union is by the frontal regions.

II. Syncephalus thoracopagus : Twins united by the heads and thoraces.

a. Diprosopus monopedius or Hemipagus. There are two faces

more or less incomplete which are united laterally and are
turned more or less from the abdominal aspect. The trunks
are united as low as the umbilicus.

1. Head with two faces and four eyes (diprosopus tetra-

ophthalmus).

2. Head with two faces and three eyes (diprosopus tri-

ophthalmus).

3. Head with rather wide face (really two) but only two

eyes (diprosopus diophthalmus).

b. Janiceps teleus. There are two more or less perfect faces,

placed opposite to each other and symmetrical.

c. Janiceps ateleus. One of the two opposite faces is imperfect,

e.g. has a median eye (Janiceps cycl< >pus) or two rudimentary
approximated ears (Janiceps synotus).
'I. Syncephalus monoprosopus. There is a single head and face
with two thoraces and the trunks are separate below the
umbilicus.

III. Syncephalus dilecanus: Twins united so that there is a single head

and thorax but two more or less complete pelves. Synonyms,"
s. ileopagus or ileadelphus.
a. Dilecanus dipleurus. The two pelves are inserted laterally
upon a vertebral column which shows signs of duplicity
posteriorly.

1. There are four lower limbs (Dilecanus dipleurus
tetrapus).

DICEPHALUS

633

2. There are two lower limbs but two penes or vulvse, and

signs of duplicity in sacrum (Dilecanus dipleurus

dipus).

Dilecanus ibipagus. The ilia of one pelvis are fused with

those of the other by means of the pubic bones, so that there

is a single pelvic cavity.

1. Each pelvis has its ilia complete with their acetabula

(Dilecanus ibipagus tetrapus).

2. Each pelvis has only the external ilium complete, the

internal ilia being incomplete and having only one
acetabulum for both (Dilecanus ibipagus tripus).

Fig. 89. — Dr. Pallares' Diceplialic Twin Foetus, Variety
Dipus DibracMus.

These, then, are the various sub-types of the syn cephalic double
monsters. Perhaps the best known and most striking of them are
the Janus or double-faced twins, some good examples of which are in
the Museum of the Eoyal College of Surgeons of England (Thompson
Lowne's Descriptive Catalogue, p. 29, 1893). The curious cases of
twins united only by the vertices (craniopagus) are very rare, and
seem to have been more often reported in Russia than any other
country. It will be noted that some of these sub-types, e.g. the cases

634

ANTENATAL PATHOLOGY AND HYGIENE

of double penis (Dilecanus dipus diphallus), can hardly be separated
from the inonosomatous terata, among which, indeed, I have described
them (p. 554).

To the second great type of the symmetrically united twins the names
of dic&phalus and lecanopagus have been given. These monstrosities
show a more or less intimate union of the pelves and the parts below,
along with a separateness of the heads and usually also of the necks
and of part of the thoraces. I give illustrations of two of the chief
sub-types (to be enumerated below) : one (Fig. 89) represents a
specimen in the possession of Dr. Pallarcs of Lorca in Spain (to whom
I am indebted for the photograph), which showed two separate heads
and necks, a common trunk, containing (as was found on dissection) a
double respiratory apparatus and a single heart and digestive system,

two arms and two legs, and
a single penis and scrotum.
Its birth was complicated with
placenta prsevia, and it was
extracted dead (Teratologic!,
ii. 210, 1895). It belonged to
the group of dicephalus dipus
dibrachius. The other illus-
tration (Fig. 90) is also taken
from a photograph, and it
formed one of a series kindly
sent to me by Dr. Michin of
Charkow in Eussia. It be-
longed to the variety diceph-
alus tripus tribrachius. Each
foetus had a heart and lungs ;
the two hearts lay in one
pericardium ; there was one
sternum for the two ; there
was only one clavicle but
two scapulas for each. In
the fused upper limb there
was one humerus, but two
ulnar and two radial bones were present. The vertebral columns
were separate above but united below in a single sacrum. There was
a single liver, spleen, and intestinal canal for the two foetuses. The
pelvis was split anteriorly (pelvis fissa), there was ectopia vesica-
urinaria?, and the penis and scrotum were double, there being one
testicle in each scrotum. On the posterior aspect of the sacral
region was a third imperfectly developed lower limb, which had
no femur, but only the bones of the leg and one large digit. The
chief varieties of dicephalic united twins are as follow : —

Fig.

90. —Dr. Miclnn's Dicephalic Twin Fcetus,
Variety Tripus Tribrachius.

I. Lecanopagus tetrabrach ius et tetrapus: Twins united by the pelves,
each having its own limbs.
a. Ischiopagus. The union is by the innominate bone.s, a single
pelvic cavity resulting.

"THE SCOTTISH BROTHERS" 635

b. Pygopagus. The union is by the region of the nates, each
twin having its own pelvis, directed outwards.
1 1. Lecano-Somatopagus : Twins united by the pelves and thoraces or
through the spinal columns directly.

a. Somatopagus parallelus. The twins have their axes parallel

and are united by the pelves and thoraces ; there are four
legs and arms.

b. Somato-catagoniodes tripus. The union is by the pelves and

thoraces, and there are three legs ; there may be four arms
(tetrabrachius) or three (tribrachius).

c. Somato-catagoniodes dipus. The union is by the pelves and

thoraces, and there are two legs ; there may be four (tetra-
brachius), three (tribrachius), or two (dibrachius) arms.
(L Somato-mesopagus. The union is by the middle parts of the
axes • there is separation above and below.
III. Lecanopagus Diprosopus : Twins united by the pelves and thoraces,
and having also the heads joined together laterally.

a. Diprosopus tetraophthalmus or Iniodymus. There is a single

body with two heads joined laterally and converging so as to
describe an angle with its apex inferior ; each face has two
orbits.

b. 1 Uprosopus triophthalmus. There is a single body with two

heads fused together laterally and converging so as to form
an angle above, leaving space for only three eyes.

c. Diprosopus diophthalmus. There is a single body with a head

larger than usual, with two eyes, two noses, and one or two
mouths.

In the dicephalic twin foetuses, just as in the syncephalic,the terminal
members of the series tend to approximate closely to the monosoma-
tous terata, and to become difficult of separation. A good example
of this is seen in Diprosopus diophthalmus, in which the size of the
head and the presence of two noses may be almost the only signs of
duplicity. I must find space here for a few words of description of a
well-known historical instance of dicephalus, the " Scottish Brothers,"
who lived in the reign of James IV. of Scotland, and about whom
Lindesay of Pitscottie wrote in his -inimitable fashion. " In this mean
Time there was a great Marvel seen in Scotland. A Bairn was born
reckoned to be a Man-Child ; but, from the Waste up, was two fair
Persons, with all Members and Portraitures pertaining to two Bodies,
to wit, Two Heads, well-eyed, well-eared, and well-handed. The two
Bodies, the One's Back was fast to the Other's ; but, from the "Waste
down, they were but one Personage, and could not know by the
Ingyne of Man, from which of the two Bodies the Legs and Privy
Members proceeded. Notwithstanding the King's Majesty caused
take great Care and Diligence upon the Upbringing of their two
Bodies in one Personage, caused nourish them, and learn them to sing
and play upon Instruments of Musick ; who, within short Time,
became very ingenious and cunning in the Art of Musick ; whereby
they could sing and play two Parts ; the one the Treble, and the other
the Tenor ; which was very dulce and melodious to hear. The
common People who treated them also, wondered that they could

636 ANTENATAL PATHOLOGY AND HYGIENE

speak diverse and sundry Languages ; that is to say, Latin, French,
Italian, Spanish, Dutch, Danish, English, and Irish. Their two Bodies
long continued, to the age of twenty-eight years ; and the one
departed long before the other, which was dolorous and heavy to the
other ; for which many required of the other to be merry. He
answered, How can I be merry, that have my true Marrow as a dead
Carrion about my Back, which was wont to sing and play with me.
When I was sad he would give me Comfort, and I would do the like
to him : But now I have nothing but Dolour of the Bearing so heavy
a Burden, dead, cold, and unsavoury, on my Back, which taketh all
earthly Pleasure from me in this present Life : Therefore I pray to
Almighty God, to deliver me out of this present Life, that we may be
laid and dissolved in the Earth, wherefrom we came."

The third and last of the great subdivisions of the symmetrically
united disomata contains the well-known and comparatively common
thoracopagous monsters (emprosthozygosis, Thompson Lowne). They
are twins joined together by the epigastric regions and by more or
less extensive parts of the thoraces. To them belong such historical
instances as the Siamese twin-brothers, Maria-Posalina, operated on
by Chapot-Prevost of Kio de Janeiro (Chirurgie <ks teratopages, Paris,
1901), and the Eadica-Doodica sisters, separated by Doyen. I have
met with a specimen of this type (Fig. 91), and, recently, Pi. J. A.
Berry described very fully two other cases (Trails. Edirib. Obstct. Soc,
xxviii. 220, 1903). There are altogether many instances of thoraco-
pagous twins on record, and the literature is to be found in Taruffi's
work (op. tit, ii. 515, 1882 ; iii. 507, 1885 ; iv. 224, 1886 ; viii. 458,
1894) and the Index Catalogue (vol. ix. 394, 1888; xiii. 1, 1892).
The sub-types are as follow : —

I. Xiphopagus : Twins united by the epigastric regions and by the

ensiform cartilages of the sterna.
II. Sternopagus : Twins united by the sterna and by the epigastric
regions, and separate in all the other parts.

a. Sternopagus tetrabrachius. Each twin has two upper limbs

(Fig. 91) ; the internal organs, and more particularly the
heart, show various degrees of fusion.

b. Sternopagus tribrachius or ectopagus. The twins are rotated

somewhat outwards ; there are three arms, one being median
and posterior.
'•. Sternopagus dibrachius. The twins are rotated markedly out-
wards and each has one upper limb.

If sternopagous twins can be extracted alive from the uterus, they
not uncommonly survive their birth, but most frequently one or both of
the foetuses has to be broken up before delivery can be accomplished.
With modern surgical asepsis renewed efforts have, of recent years,
been made to separate such united twins, more especially when one of
them became the subject of such a disease as tuberculosis. Thus far,
the results have been encouraging, but not brilliantly so. Skiagraphy
will doubtless enable the surgeons of the future more carefully to
choose the proper cases for operation, and with better means of

PARASITIC UNITED FOETUS

037

checking haemorrhage {e.g. from the liver) lie will almost certainly
be able to separate twins of the xiphopagous type at any rate.

The asymmetrically united twins or parasitic double terata are not
so easily classified as are the symmetrical ones. At the one end of a
long series of types is the foetus (or autosite) with an attached foetus
(or parasite) defective in some part, but not much smaller than the
autosite ; at the other end of this series are such conditions as the
foetus in fcetu (or included twin) and dermoid cysts, teratomata, and,

.*•**

Fig. 91. — Thoracopagous Twins.

perhaps, Polydactyly and polymastia. This, like so many other parts
of the classification of teratological states, shades off in an indefinite
manner into neighbouring or allied types: foetus in fcetu passes by
degrees into tumours of various kinds, and Polydactyly is not far
removed from the single (monosomatous) terata if, indeed, it be not
included in them. Perhaps the best arrangement that we can adopt
for these defective, attached, or included foetuses is the regional ; we
discuss, therefore, the parasites of the head, face, neck, thorax.

638 ANTENATAL PATHOLOGY AND HYGIENE

abdomen, pelvis, and limbs. A few words must suffice about each in
turn.

I. Gephalo-Parasitus. — This group includes the epicoma or cephalo-

paracephalus, the foetus with limbs and sometimes with a defective
body which is attached by its sinciput to that of the perfect twin;
there are sub-varieties (dipus, apus, and pseudo-aeormus) according
as the parasite is provided with limbs or not. It also includes the
cephalo-diprosopus, in which the autosite carries on the side or front
of its head a second more or less incomplete head ; the cephalomeles,
in which an accessory limb is inserted on the head; the encephalo-
amorphus, in which a tumour, with foetal parts, is inclosed within
the cranium ; the teratoid intra- or extra-cranial growth ; and the
dermoid cysts, situated inside or outside the cranium.

II. Trachclo-Parasihts. — This group contains a number of parasitic
growths of the neck and lower part of the face. It includes such
cases as that of accessory superior maxilla attached to the superior
maxilla of the autosite, of which the specimen shown in Figs. 70 and
71 was probably an example. It includes also the accessory parts
attached to the lower jaw ; and here may be placed the instances of
a supernumerary limb arising from the neck, which have as yet only
been met with (and that rarely) in animals (aucheno-melus or dero-
melus) ; here also the extraordinary state known as epignathus, in
which a tumour containing foetal parts springs from the palate and
protrudes through the mouth, may find its proper position in a system
of classification. Some of the cervical cysts, teratoid or dermoid in
character, must likewise be included here, and perhaps also the
instances of cervical auricles (pleonotus).

III. Thoraco-Parasitus. — Individuals like the Chinaman Ake and
the boy Laloo, in which a nearly complete but small foetus is attached
to the anterior aspect of the thorax, belong to this group. Sometimes
the parasite may have a rudimentary head (thoraco-paracephalus or
heteropagus), sometimes it has little more than a head (thoraco-
paracephalus pseudo-aeormus or heterodymus), but most often it has
no head at all (thoraco-acephalus or heteradelphus). In another
group of cases accessory limbs spring from the thorax (thoraco-melus),
either from the posterior aspect (notomelus) or from the side (pleuro-
melus). Teratoid and dermoid growths may also be found attached
to or lying inside the thorax ; and, finally, the rigid laws of classifica-
tion (which are not represented in Nature) would place polymastia or
accessory mammary glands here.

IV. Gastro-Parasitus. — It is comparatively rare to find an
acephalic foetus (gastro-acephalus) or a supernumerary limb (gastro-
melus) attached to the abdomen of the autosite ; but the curious
included embryos (gastro-amorphus), the cases of foetus in foetu or
congenital pregnancy, are not so very rare. I gave an account
(Brit. Med. Journ., ii. for 1900, p. 1428) of the dissection of one of
these remarkable amorphous foetuses which was removed by Mr.
AYright of Manchester from the cavity of the lesser peritoneum of a
female child aged two months. Its appearances are shown in Figs.
92 and 93 ; and its structure closely resembled that of the twin foetus

FOETUS IN FOETU

639

amorphus anideus already described (p. 628). In this group, also,
must be placed the teratomata of the scrotum, and the teratoids and
dermoid tumours of the abdomen, the testicle, and the ovary.

V. Lecano-Parasitus. — Parasitic attachments to the pelvis and
neighbouring parts are very difficult to classify or to understand.
They include the sacral and perineal teratomata, an example of
which I saw in December 1903 under Mr. Stiles' care in the
Edinburgh Sick Children's Hospital. Perhaps the best idea of the
various parasitic growths found in this region of the body may be

Fig. 92. — Appearances of Dermal Aspect of Foetus in Fcetu.

obtained from the consideration of Tarufh's classification {op. cit., iii.
316 et seq., 1885).

a. Ischio-parasitus. — The parasite is attached to the ramus of the pubes

or to the perineum of the autosite ; and it may consist of an
acephalus or a paracephalus (heteromorphus and heterotypus of
Saint-Hilaire's nomenclature), or of an amorphus, or simply of an
accessory limb (ischio-melus) as in the well-known case of the man
Dos Santos (the "human tripod").

b. Tlio-parasitus. — The parasite consists of one or two accessory limbs

040

ANTENATAL PATHOLOGY AND HYGIENE

c.

VI

attached to one or both iliac bones; it is a very rare state in the.
human subject, but less rare in animals.

Tschio-sacro-parasitus. — This group contains the parasitic foetuses
(generally of the nature of accessory limbs) which are attached to
the ischia and sacrum and spring from the sciatic notch.

Pijtjo-parasitu*. — Here are included the accessory limbs adhering to
the nates (pygomelus) ; the sacral teratomata (pygo-amorphus), in
which the attachment is not so much to the sacrum as to the parts
covering it; the teratoid growth of the nates (pygo-teratoides) ; and
the dermoid cysts of the same region.

Melomelus— This group includes the cases in which an

accessory limb or part of one is inserted upon another normal limb.

Fig. 93. — Section of Fcetus inFcetu, showing much adipose
tissue, pieces of cartilage, and cavities lined with pig-
mented membrane.

It is the most artificial of the groups into which the asymmetrically
united double terata have been divided. Cases in which a super-
numerary limb is attached to the normal femur or humerus are rare,
but authentic instances have been observed ; the insertion of an
accessory member upon the bones of the leg or of the forearm has
not been observed in the human subject ; but there have been a few
examples of double hand (dichirus) and double foot (dipus), and to
these reference has already been made under the monosomatous
terata. Perhaps the most artificial and strained part of the whole of
this system of teratological classification is the inclusion of Poly-
dactyly here among the double terata. Although, theoretically, it

" BIDDENDEN MAIDS "

641

may be correct, I have not hesitated for once to abandon the
classificational system for common sense, and accordingly I dealt
with Polydactyly along with the malformations of the limbs. This
concludes the long list of the types of the polysomatous terata,
diseretic as well as symeretic.

I have endeavoured, in the preceding pages, to give an idea of the
scope of the subject of double monsters (" diplo-teratology ") and to
refer, shortly at least, to most of the leading types. It will have
been noticed that almost every imaginable combination of characters
has been enumerated : the twins have been attached by the heads, by
the backs, by the chests, by the pelves, and so on ; and, generally
speaking, the rule of " like
parts being attached to like "
has been observed. Among
all the wealth of observation
and the multiplicity of forms
there are still some lacunar
in our knowledge which time
may perhaps fill up. Every
double monster is well worth
dissecting.

I may refer, here, in pass-
ing to one of the most in-
teresting of the double terata,
the historical instance of the
" Biddenden Maids." These
were maidens, supposed to
have been born in the year
1100 in the village of Bid-
denden in Kent, who lived
joined together for thirty-four
years, and at their death left
money to be employed in the
baking of cakes or rolls with
their impression on them
which were to be distributed
to the poor every Easter
Sunday. I collected some-
time ago all the details of
their history that I could find (Tcratologia, ii. 268, 1895), and the
representation of them, as it appears on the cakes and broadside,
is reproduced in Figs. 94 and 95. The reason why I refer to them
at all is twofold : first, because they are the earliest known English
double terata, and, second, because their mode of union, if correctly
represented, is unique. It would appear from Fig. 95, but more
especially from Fig. 94, as if the maids were joined by the arms
at the elbows. Now it is unwise to say that any particular mode
of union of double terata is impossible ; but it seems to me prob-
able that in this respect the artist took a liberty with the facts.
Probably the maids were pygopagous twins like the well-known

Fig. 94.— The Biddenden Maids.

VOL. II.

-4 1

G42 ANTENATAL PATHOLOGY AND HYGIENE

Hungarian sisters (Helen and Judith), the North Carolina twins
(Millie and Christine), and the Bohemian twins (Rosalie and Josepha

g^^^^agffqffiB^g^S^^

A SHORT AND CONCISE ACCOUNT OF

ELIZA AND MARY CHULKHURST.

Who mete born joined together bj fce Ilins :»'<• Shoulders.
IN THK YK.U; <>r OUB M)BD liOft

At BIDDENDEN, in the County of KENT,

COMMONLY CALLED

THE BIDDENDEN MAIDS.

THE READER will observe by the Plate of them, that they
lived together in the above -tate Thirty-four years, at the expiration
of which titne onenf th«?m was taken ill and in a short, time died;
the surviving one was advised to be separated from the body of her
deceased Sister by dissection, but she absolutely refused the separa-
tion by saying these words.— "A* wo came together we will also go
together*" and in the space of about Six Hours after her Sister's
decease slit' was taken ill and died also.

By their Will they betpieath to the Churchwardens of the Parish
ot Biddenden and their successors Churchwardens for ever, certain
Pieces or Parcels of Land in the Parish of Biddenden, containing
Twenty Acres, more or less, which now let at -40 Guineas per
annum. There are usually made, in commemoration of these
wonderful Phenomena of Nature, about 1,000 Rolls with their
Impression printed on them, and given away to all strangers on
Kaster Sunday after Divine Servica in the afternoon ; also about
aOQ Quartern Loaves and Cheese in proportion, to all the poor
Inhabitants <,\ th,- said Parish.

Cv _._._._.._

Fig. 95.

-"Broadside" of the Biddenden Maids.

Blazek). They were, doubtless, united by the nates in a postero-
lateral manner, and the idea of union by the arms may have

TRIPLETS 643

arisen from their going about with their arms round each other's
necks.

The teratogenesis of the united twins has, as might well be
imagined, excited much interest and discussion. I have no space
to enter into the long controversy, not yet finished, concerning the
origin of the various types of double terata ; but I may say that I am
inclined to accept the theory of polyspermy as the determining cause
of these monstrosities, although possibly in some cases, more especially
in relation to the parasitic or included fetuses, the notion of partheno-
genetic development in an ovum or in the testicle may be enter-
tained. AYe may suppose that the entrance of one spermatozoon into
the ovum determines the appearance at a later date of one embryonic
rudiment with a definite orientation in the blastocyst ; if, now, two
fertilising spermatozoa gain entrance, two primitive streaks, each with
its own orientation, may be looked for ; according to the proximity
or separation of the primitive streaks will be the relation of the
future twins. The streaks may be in opposition to each other, when
the craniopagous type of double monster may result ; they may lie
side by side, when thoracopagous twins will be produced ; or they
may be inclined towards each other either at their anterior or
posterior ends, when syncephalic or dicephalic terata will be formed.
A still closer union of the primitive streaks may suffice to explain
the parasitic types. It must always be borne in mind, also, that in
considering included foetuses there is a time in development when
the body cavity of the embryo is not closed and when it is quite
possible for one rudimentary embryo to become surrounded by the
other and the larger twin (" enclavement "). In all these monstrosities
the twinship is, to my mind, the essential and original pathological
fact. The reader, who desires it, will find a suggestive account of
the origin of double terata in Duval's article, in Bouchard's Traite
tie pathologie gtnirale, i. pp. 211-233, 1895.

Triplets and Quadruplets, etc.

Pregnancies with more than two children, whether they be mono-
chorionic or dichorionic, must be regarded as teratological. A few
sentences must be devoted to triplets and a few words to the rarer
quadruplets and quintuplets.

Triplets occur about once in seven thousand pregnancies. They
are, however, much more likely to occur in the case of women who
have given birth to twins or are the mothers of large families ; a
woman, herself a twin, has been known to give birth to triplets.
Uniovular triplets do not appear to be hereditary, while binovular
and triovular ones often are. All the morbid states of pregnancy
{e.g. albuminuria, oedema, haemorrhage) are more frequent in triplet
gestations ; vertex presentations diminish in frequency and abnormal
ones (breech and shoulder) increase. About 50 per cent, of the
infants are to be regarded as viable, but only about 33 per cent,
survive the first year of life. They are most often of different sexes
(two boys and one girl, or two girls and one boy), but it is not un-

644 ANTENATAL PATHOLOGY AND HYGIENE

common to find them all of one sex (three boys or three girls). When
we come to study the membranes, we find that triplets may be
triovular (three chorions and three placentas or more often two
placentas), or binovular (two chorions and two or rarely one placenta),
or uniovular (one chorion and one placenta). The binovular are the
commonest and the uniovular the rarest types; in 1901, E. Saniter
(Ztschr.f. Geburtsh. u. Gyndh, xlvi. 347, 1901) was only able to collect
together records of eight well-established cases of uniovular triplets,
and although Walla (Ccntrlbl.f. Gyndh, xxvi. 403, 1902) has reported
another, they are undoubtedly very rare. The amniotic sacs seem
always to have been separate.

One of the triplets is not uncommonly an acardiac ; curiously
enough, the recorded instances have so far been all but one in the
binovular variety ; in other words, they have been cases of allantoido-
angiopagous twins born along with a third foetus. The single
exception is that reported by G. Giglio (Arch, di ostet., iii. 235, 1896);
in it there was a single chorion and three distinct amnions, one of
which showed hydramnios and contained an acephalic embryo. An
instance of uniovular triplets, one of which was a fcetus papyraceus,
was reported by von Erlach (Ccntrlbl. f. Gyndh, xviii. 439, 1894) ;
a case of triplets, two of which were foetuses papyracei and one was
a living child, was described by C. Gutmann (Diss, inaug., Strassburg,
1885). United triplets or triple terata are so rare as to be almost
unknown. A case of a tricephalic infant was reported in the year
1834; it consisted of three heads set upon two united trunks,
supported by two lower limbs ; there were three upper limbs and a
single set of male genitals ; all the heads had to be perforated during
labour, and two of them amputated. The occurrence was reported
by Eeina and Galvagni (Saint-Hilaire, op. cit, iii. 341, 1836). Perhaps
the most extraordinary monstrosity ever put upon record was that
described by J. Baart de la Faille in 1874 (lets over den Epignathus,
Groningen) : it consisted of a female fcetus, from whose mouth pro-
truded a large tumour (epignathus), and to this tumour were attached
by their umbilical cords two small acephalic foetuses, each consisting
of only the pelvis and two lower limbs. If we regard the epignathus
as part of the autosite, this was a case of two parasitic foetuses
inserted upon it by their cords ; but if we regard the epignathus as
itself a parasite attached to the autosite, then we are in view of a
teratological complex which could hardly have been imagined had
the specimen not existed.

I must bring these notes on triplets to a conclusion with a
reference to the curious case reported by G. W. Thompson (Indiana
Med. Journ., p. 373, April 1899) : united twins of the female sex with
two heads, four arms, four legs, and two bodies united by the thoraces,
were expelled at the seventh month of pregnancy and breathed
for twelve minutes ; they were preceded, however, by the birth of a
single male infant, which had a separate placenta and membranes.

The bibliography of triplets and triple monsters will be found in
the Index Catalogue (vols. ix. 405, 1888; xiv. 775, 1893), and in E.
Saniter's article (Ztschr.f. Geburtsh. u. Gyndh, xlvi. 347, 1901).

QUADRUPLETS 645

Quadruplets are still rarer than triplets, occurring perhaps about
once in 370,000 labours. At the same time, over seventy cases have
been recorded in medical literature {Index Catalogue, xi. 919, 1890).
Theoretically they may be derived from one, two, three, or four ova,
and may be normal, deformed, or united together by their cords
or structurally; but they are not numerous enough to allow of
generalisations being made. A similar statement may be made
regarding the state of their placentae and membranes.

With regard to the occurrence of an allantoido-angiopagous foetus
as one of quadruplets, I may refer to Dr. (Tunsburg's extraordinary
observation, communicated by me to the Edinburgh Obstetrical
Society in 1896 {Trans. Edinb. Obstct. Soc, xxi. 250, 1895-96). The
mother, a Russian peasant woman, aborted at the fourth month of
her first pregnancy of four separate foetuses, one of which was
monstrous (paracephalic). Three were males (including the para-
cephalus) and one was a female ; there were two separate placental
masses, one of which, was made up of three placentas united into
one, and the other was single. It was then a case of uniovular
triplets with a single foetus ; it was a binovular pregnancy in which
one of the ovules produced three male foetuses and the other a single
female foetus.

Charbonnier {Etude des grossesscs triples et plus que triples, p. 65,
1895) refers to one or two cases of double terata born with two
other foetuses, and J. M. Rodriguez {Gac. mkl. de Mexico, v. 17, 33,
1870) described a quadruple human monster ; but exact records are
very rare, and I have not been able to consult either the case of
Rodriguez or those mentioned by Charbonnier in the original.

Recent instances of quadruplets have been recorded by Stahl
{Trans. Chicago Gyn. Soc, p. 6, 1896), C. C. Henry {Brooklyn Med.
Journ., x. 618, 1896), Bousquet {Ann. de gyndc, xlii. p. 55, 1894),
Schatz {Kor.-Bl. d. ally, mecJclenb. Aerztcvcr., No. 190, p. 568, 1898),
E. T. Goode {Lancet, i. for 1897, p. 590), and O. Ausch {Miinchcn. med.
Wehmehr., xxii. 123, 137, 1897).

Quintuplets are among the curiosities of obstetrics, occurring as
they do not oftener than once in many millions of labours (1 in
13,400,000, Charbonnier). In the Index Catalogue (xi. 968, 1890)
there are references to twenty-one cases ; recently a few more cases
have been added, including those of R. A. Hibbs {Arncr. Med. Surg.
Bull, x. 207, 1896), of Stoker {Lancet, ii. for 1895, p. 1164), and of
Sato {Sei-i-ku:ai Med. Journ., xxi. 40, 1902). In Sato's case there
were three placental masses, two with two cords each, and one with
one ; the infants were three males and two females ; in Stoker's, the
placenta consisted of two parts united by an isthmus, and one part
had two and the other three cords attached to it ; while in Hibbs'
case there were five cords attached to one placenta. G. C. Nijhoff's
quintuplets {Nederl, Tijdsehr. v. Geneesk, ii. for 1903, p. 1305 ; Journ.
Obst. Gynmc. Brit. Empire, vi. 32, 1904) were four females and one
male, and were born alive, but, being immature, survived only one
hour. The placenta consisted of one continuous cake, to which five
separate cords were attached ; each foetus had its own amnion, but

646 ANTENATAL PATHOLOGY AND HYGIENE

three had a common chorion and the two others a separate chorion ;
the case was, therefore, a triovular one. On the mother's side there
were several instances of multiple pregnancy : her mother once had
twins ; two maternal uncles became fathers of twins ; and a maternal
aunt was delivered of triplets. Little is known concerning monstrosi-
ties in quintuplets ; but in F. Poljakoff's case (Med. Obozr., xxiii. 1015,
1885) one of the foetuses was a microcephalus.

Sextuplcis have been reported on a few occasions, but the records
have usually been received with doubt. At any rate the reporters
of the cases have seldom been actually present at the confinement.
In this respect the recent instance of Drs. Kerr and Cookman (Med.
Press, lxxv. 537, 1903) does not differ from the usual rule. The
mother, a woman of the Gold Coast, was found lying exhausted in a
hut, and six foetuses were found beside her. The infants were five
boys and one girl ; there were four placentas, of which one belonged
to the girl, one to one of the five boys, and the other two to two boys
each. It was said that labour had lasted only four hours, and that
each placenta was born after the children to whom it belonged. This
was the woman's fourth pregnancy ; in her first she had given birth
to quadruplets, and in her second and third ones to triplets in each
instance. There had thus been the extraordinary record of sixteen
children in four pregnancies.

Other instances of sextuplets have been referred to by Guzzoni
degli Ancarani (Rassen<j7ia di sc. mcd., Modena, iv. 437, 489, 569, 617,
1889) and F. Vassalli (Gazz. med. ital. lomb., xlviii. 216, 1888 ; Ann,
diostct.,x\i. 343, 1894).

Sextuplets in the human subject are of doubtful authenticity, but
cases of scphqilets are almost mythical. There is a story (Charbonnier,
up. cit., p. 83) of a man so alarmed by the rapid growth of his family
that he left his home for seven years ; a year after his return his
wife gave birth to seven infants ! Charbonnier also refers to two or
three other reported cases; Roy (R6v. med. f rang, et ttrang., i. 255,
1877) also dealt with the subject ; and I may conclude with the
following quotation from J. B. Bianchi (Be Naturali in Humano
Corpore, Vitiosa Morbosaque Gencratione, p. 249, 1741) : " Sic nostra
hac ajtate, nostraque in hac Urbe, tres eodem partu, semel iterumque
sunt editi : In quibusdam Pedemontanis Regionibus interdum quinque :
et in Insubriae Pago non diu ab hinc, septem simul in lucem prodiere.
Nanique non ita pridem testatus est mihi, II. P. David, Clericus
Regularis Tbeatinus piissimus, nostrique Academic! Sacelli Pra'fectus ;
ante hos annos xiv progredientem se Mediolano Robeccum, ad
navigabilem Canalem habuisse obviam Mulierem, sibi jam famfi
notam, qua? uno partu septem filios, masculos feminasque, vivos et
vi tales omnes, cito quidem, at non sine magnis cruciatibus, pepererat ;
turn vero ex iis primum aut alterum, masculmnque, obvia' ipsius
Matris vidisse comitem; cujus turn setas xi aut xii annorum esse
potuisset."

CHAPTER XXXIII

Pathology of Germinal Life (cord.) : Physiology of the Egg- and Sperm-Cell ;
Normal or Ancestral Heredity ; Pathology of the Egg- and Sperm-Cell ;
Popular and Scientific Ideas regarding Morbid Heredity and its Various
Phenomena ; Degeneration ; Effect of Impregnation upon Heredity and
Degeneration ; Arrest of Growth, Development, and the Performance of
Functions ; Etiological Uniformity in the Various Periods of Life ; Treat-
ment of Antenatal Morbid States, and especially of those arising in the
Germinal Epoch.

I enter with reluctance upon the discussion of the effect of morbid
influences upon antenatal life still in the ovular and sperminal stage ;
I hesitate to attempt even to outline my views, or rather my view
of others' views upon it. But having gone thus far in my search
after the causes of antenatal disease, deformity, and malformation,
I can hardly refuse to pass on a step or two further, even although
it be in almost complete darkness that the advance is made. At the
same time the reader must not imagine that I am now for the first
time referring to the influence of morbid agents upon the egg-cell
and sperm-cell before their fusion in fertilisation ; both in this
volume and in the one which preceded it I have already dealt
incidentally with morbid heredity, for that is but another name for
the pathology of the ovum and spermatozoon. I have referred to it
more particularly when considering the transmission of syphilis from
parent to child (Vol. I. p. 251), and have alluded to it when writing
of the hereditary nature of certain malformations (polydactyly, hare-
lip, etc.). Morbid heredity has really been with me in the writing
of each chapter, and upon it I have not infrequently fallen back
when faced by exceedingly difficult problems in teratogenesis. But
it is a subject about which so very little is known and concerning
which so very, very much has been spoken and written, that the
seeker after truth is bewildered and rendered well-nigh hopeless. It
is being crushed beneath a ponderous load of theories, many of them
contradictory, and all of them built up upon scanty data. Is it
matter for surprise if I approach with great reluctance the study of
a subject upon which I can hardly hope to throw much light, while
I may justly fear that I may very easily increase the existing dark-
ness ? The matter, however, must be dealt with if this Manual of
Antenatal Pathology is not to be left with one very important factor
in antenatal morbid processes omitted. I can at least endeavour to earn
for my presentation of the subject the merit of brevity ; and if any-
thing that appears here lead the reader to the study of such masters of
thought as Darwin, Weismann, Galton, Delage, Fere, Xaegeli, Mehnert,
and Karl rearson, I shall be happy in that I have placed him on the

647

648 ANTENATAL PATHOLOGY AND HYGIENE

right road to gain a knowledge of the best that is known regarding
the deep mysteries of the deeply mysterious matter of heredity.1

Physiology of the Egg- and Sperm-Cell.

The earliest part of antenatal life is the long period during which
the egg-cell and the sperm-cell are lying in the bodies of the parents
of the unborn and as yet "unconceived" infant. Regarding this life's
phenomena we are almost ignorant; but we know that their sum
and effect are best represented by the word " heredity." We know
something, it is true, of the ante-conceptional existence of the ova, —
that there are thousands of them in the ovary at birth, that the
number has decreased markedly at puberty, that thereafter one or
two come to maturity and are expelled from their ovi-sacs once a
month or thereby, that certain changes occur in the empty ovi-sac
after rupture, and that a structure is formed there, called the corpus
luteum, which possibly has a far-reaching effect on the nutrition and
health of the whole body, — but of the early life of the sperm-cell and
of the spermatozoa before they become prominent in fertilisation we
know almost nothing. We may guess further that the ova as they lie
in the ovary are influenced by the health or disease of the individual
in whose ovaries they lie, that they in their turn may affect her life
and the life and fate of the ova lying beside them, and that the
corpora lutea also are not without their effect upon the physiology
of the mother and of the impregnated ovum in her uterus, — we may
guess these and other things about the ovum, but we have hardly
begun even to guess about the spermatozoon. Yet, if we are to
accept the evidences of after events, there is already in the ovum
and the spermatozoon the subtle and complex machinery of heredity ;
in the reproductive cell, in its nucleus, in its chromosomes, or in
parts of it not yet differentiated or denominated by science, there is
the mysterious something which determines that the child shall
resemble his parents, his grandparents, and his remoter ancestors.
The individual is, as it were, the meeting-place of two long lines of
spectral forms, stretching away back into the past and melting into
an army of shadows, each of which in its time was a living being
with habits, good or bad, with health or disease, with structures
stoutly normal or defectively abnormal ; the infant at birth is the
living ganglion towards which many lines of influence converge and
in which they meet, with effects which we cannot foretell, but about
which, when we see them, we say " heredity determined them." In
every human face we can read, if we have eyes and understanding,
the " sequence of posterity."

" Look here upon thy hrother Geffrey's face ;
These eves, these brows, were moulded out of Lis ;
This little abstract doth contain that large
Which died in Geffrey, and the hand of time
Shall draw this brief into as huge a volume."

1 The literature of Heredity, which is vast, will he found in the Index Catalogue.
(vol. vi. 74, 1885 ; 2 s., vol. vi. 1032, 1901) under Heredity and cross references.

MORBID HEREDITY 649

It is necessary, therefore, to believe that all hereditary tendencies
are already in the ovum and spermatozoon before fertilisation, for it
is hardly possible to conceive of them being suddenly implanted in
or conferred upon the substance of the reproductive cells at any set
time, such as that of maturation. Whatever possibilities of growth,
of division, of elaboration, and of differentiation, therefore, the
original reproductive cells have, they also have the power in them
of producing an individual closely resembling the individuals from
whom they come. How the various strains of tendency are combined
or antagonised or modified by such phenomena as maturation and
impregnation it is hardly possible as yet to do more than surmise ;
but that these events do influence the future of the individual
cannot, I think, be doubted. Further, the environment of the germ-
cells (as we may conveniently term both the male and female repro-
ductive elements) is not, I believe, without influence upon their
future development. Thus far I have been considering solely what
may be called normal or ancestral heredity, the tendency every
living thing has to reproduce its like, its like but with variations,
the variations constituting individuality ; but it is necessary also to
deal with morbid heredity, and that leads me to the discussion of the
pathology of the ovum and the spermatozoon.

Pathology of the Egg- and Sperm-Cell.

Kuxov %6pazo; %u.vJ>\ uov is a Greek proverb ; it may be rendered " a
bad crow lays a bad egg " ; and it represents the popular idea of
morbid heredity. That an apparently good crow occasionally lays
a bad egg, and that an undoubtedly bad crow sometimes lays a good
one, are facts which are kept in the background, or explained away
by a reference to an earlier ancestor of the crow tribe if they become
too prominent. That is the popular view of the subject ; let us,
however, look at the subject from the scientific standpoint.

Heredity in relation to morbid processes is believed to show
itself in various ways. Let us consider some of these.

In the first place, there exists in some families a special tendency
for the members to be affected with certain maladies which manifest
themselves late in postnatal life. " It may be supposed," wrote
Montaigne, " that I am indebted to my father for this stonie quality,
for he died exceedingly tormented with a great stone in his bladder.
... I was borne five and twenty yeares before his sickness, and
during the course of his healthy state, his third child. Where was all
this while the propension or inclination to this defect, hatched ?
And when he was so farre from such a disease, that light part of his
substance wherewith he composed me, how could it for her part
beare so great an impression of it ? And how so closely covered, that
after fortie five yeares after, I have begun to have a feeling of it ?
and hitherto alone, among so many brethren and sisters, and all of
one mother." The hereditary nature of renal lithiasis so graphically
enunciated by Montaigne, has been affirmed for it by modern
science, which has gone on to postulate a similar character for the

650 ANTENATAL PATHOLOGY AND HYGIENE

biliary calculus, for obesity, for rheumatism, for gout, for asthma, for
eczema, for migraine, and for diabetes. It is maintained, also, that
what is transmitted is not so much the disease itself as the tendency
to develop it ; it is thought that a certain type of constitution with slow
nutrition causes one or other of the above-named maladies ; and there
is abundant clinical evidence to prove this, as well as to show that in
a certain family some of the ascendants may exhibit one of this group
of diseases while the descendants may suffer from others. There
may be similar heredity, or it may be of the heteromorphic kind :
the gouty man has a son who becomes gouty, or he has one who
suffers from hepatic calculus, from asthma, from eczema. It is
even thought that after death the chemical processes of dissolution
have their hereditary type, and that the tissues of the body break
up in a way that has been determined for that family by remote
ancestors.

In the second place, there are various nervous maladies for which
a hereditary character has been claimed ; from them the members of
the " neuropathic family " suffer. They may be unaccompanied by
structural alterations, such as various forms of insanity and epilepsy
and hysteria, or they may be so accompanied, as in general paralysis,
locomotor ataxia, hereditary ataxia, cerebellar ataxia, chorea, pseudo-
hypertrophic paralysis, etc. There may be similar (or homologous)
heredity or dissimilar (or heterologous) heredity ; the maladies may
show themselves at a special age and always at that age (homo-
chronous heredity) ; twins may be affected with the same disease ; and
many minor malformations (e.g. hare-lip, hypospadias, microcephaly)
may be associated with these nervous maladies, or may exist in the
relatives of the individuals suffering from them. It is maintained
that it is not always the disease itself that is transmitted, but
simply a predisposition to it, a weakness of the nervous system
which makes the individual prone to develop it ; further, a pre-
disposition to suffer from such maladies as gout, rheumatism,
migraine, and eczema which indicate slowness of nutrition may
coexist with it.

In the third place, there is the heredity of cancer and other
tumours, such as uterine myomata. This also must be looked upon
as a heredity of predisposition. It is not admitted by all as a real
thing, and it is true that it would seem sometimes to be dependent on
other factors than heredity ; but I believe in the tendency to a
" habit of growth " in cells, so well described and elaborated by
Adami (Brit. Mai. Journ., i. for 1901, p. 021). What makes the
tumour is the assumption by its primary cells of the habit of growth
instead of the habit of work, and according to the extent of this re-
placement arise the various grades of tumour formation, from the
most benign to the most malignant. I have elsewhere (Brit. Med.
'foam., i. for 1901, p. 057) pointed out that the foetus itself is at one
time in its life (about the fourth month) a most remarkable tumour,
quadrupling its weight in a month ; yet this rapidity of growth is
checked, and differentiation of structure and functional activities
take its place. If we could discover the cause which checks its

MORBID HEREDITY 651

growth, might we not be near the discovery of how the growth of a
malignant tumour might also be arrested ? At any rate I think there
is abundant evidence that the tendency to a " habit of growth " may be
hereditarily transmitted from ascendants to descendants.

In the fourth place, there are several diseases a predisposition to
which is occasionally transmitted hereditarily, among which may be
named heart disease, bronchitis, nephritis, and certain eye diseases,
such as strabismus and cataract.

Now, before I proceed further in this review of morbid heredity
and its manifestations, let me summarise what has been said, and
draw from it a deduction or two. In all the transmitted conditions
to which reference has been made (arthritic troubles, nervous diseases,
neoplasms, etc.), we have been dealing with the transference from
ascendants to descendants of predispositions or tendencies ; we have not
stated a belief in the transmission of any structural characters under-
lying these, and have left it to be imagined that none such existed.
But it is a very open question whether such structural causes for the
predispositions do not, after all, exist. If we take one or two other
hereditarily transmitted diseases, such as hemophilia, chlorosis, some
forms of cataract, and the like, we know that in them there is gener-
ally believed to exist a structural substratum, such as fragility of the
walls of the blood vessels, and narrowness of the arteries, which
explains how this predisposition is brought about. There are, in
other words, states intermediate between those in which no struc-
tural alteration is recognisable (gout, cancer) and those in which
definite and specific changes can be seen (syphilis) ; these connecting
links lead us to ask the question whether in all hereditary maladies
there may not be a subtle substratum of tissue-defect which acts as
the vehicle for the manifestation of the functional disorders which
constitute the diseases. May there not, for instance, be a congenital
narrowness of the renal arteries which predisposes in certain families
to Blight's disease 1 Do not, therefore, these two groups of hereditary
states merge into each other by an intermediate series of types'? Do
not the same rules apply to hereditary maladies in which there are
underlying structural alterations which are transmitted and to those
in which none have as yet been seen ? But I must return to the
main argument.

In the fifth place, there are the microbic disorders. Tuberculosis
is constantly spoken of as transmitted hereditarily, but it may be
meant in two different senses. In the ordinary sense it is assumed
that the children of the phthisical are born with tissues which do not
easily resist the onslaught of the tubercle bacillus on the attainment
of a certain age and under certain conditions. There is the transmis-
sion of a predisposition. But, as I have shown in the first volume of
this Manual (p. 207), there are some undoubted instances in which a
tubercular mother transmitted tuberculosis directly to her foetus ;
the cases are rare, and there are reasons for their rarity, but they are
real. In tuberculosis, therefore, there is the possibility of the trans-
mission of a predisposition to the disease or of the disease itself.
Does this not prove that it is impossible to shut off one group of

652 ANTENATAL PATHOLOGY AND HYGIENE

transmitted morbid states from another, or to say " these are foetal
infections, the others are hereditary transmissions " ? Is there, after
all, any fundamental difference between the transmission of the germ
of disease and the transmission of the liability to be attacked by the
germ ? Smallpox, scarlet fever, measles, malaria, and many other
maladies may be transmitted directly from mother to foetus ; may
there not also be a transmission of the liability to be attacked by
them in postnatal life ? In some families there is a special proneness
to be affected by one of the exanthemata rather than by another ; in
other families there is a certain immunity against certain diseases. I
do not think we can shut off the diseases which are transmitted
through the placenta to the foetus from the maladies which are
hereditarily predisposed to in antenatal life and which develop (under
favourable circumstances) after birth.

In support of the preceding statement, it may be noted that many
foetal diseases agree with recognised hereditary postnatal disorders in
their obedience to the laws of morbid heredity. They may show
similar or dissimilar heredity ; they may exhibit family prevalence ;
they may affect only one sex ; they may be present in the children of
a woman by her first but not in those by her second husband ; and
so on. Many references to and illustrations of these characters will
be found in the first volume of this work under foetal ichthyosis, foetal
dropsy, foetal rickets, and the like.

In the sixth place, there is heredity among the teratological
formations. The malformations and monstrosities of the embryo
may, like the diseases of the foetus, be transmitted hereditarily.
Direct heredity is often prevented by the fact that so many mon-
strosities do not permit of postnatal life ; but there are sufficient
instances of it among the minor malformations, such as Polydactyly,
hare-lip, ocular anomalies, aural deformities, and hernia?, to prove that
it undoubtedly exists. Family prevalence is comparatively frequent,
and indirect heredity is common. A most striking instance of the
combination of direct and indirect heredity was reported by me some
years ago (Amcr. Journ. Ubxtct., xli. 577, 1900) : in three generation*
there were defects of the thumbs ; but in the third generation these
suddenly became grave in character and were associated with hydro-
cephalus, anencephaly, absence of the radius, and spina bifida.
Further, experimental teratogenesis has shown that malformations
and monstrosities may be produced by acting on the embryo with the
agents (toxic, microbic) which produce the diseases of the adult, the
child, and the foetus. There is not, therefore, as great a difference as
might at first be imagined between the heredity of diseases and of
malformations. Each group has its limitations in certain directions,
but there are many characters possessed in common.

In the seventh place, and finally, there is evidence of heredity in
regard to morbid conditions which are generally supposed to arise in
the germinal period of life. Germinal pathology falls under the laws
of morbid heredity. Twin pregnancies and large families (prolificacy)
are often distinctly hereditary ; the hydatid mole may occur several
times in the same patient ; sex anomalies may show family prevalence.

TELEGONY 653

Further, I believe, it is precisely in the early part of the germinal
period that predispositions and tendencies to disease and malforma-
tion are developed, and, as we have seen, they are often hereditarily
transmitted. Possibly, also, it is during this early epoch that
tumours are originated. I have accepted Adami's suggestion that
they are due to a " habit of growth," but that does not prevent me
believing also that this habit of growth was acquired in the germinal
period of life. Indeed, if we imagine an arrest of the evolution of the
germ at the stage of rapid growth we have a temporary condition be-
coming permanent ; the habit of growth, instead of being checked,
may continue in a few cells and so lead to the formation of a
neoplasm. It is, of course, difficult to realise how it may remain
latent for years, as in cancer, but I believe that in some way it
does so lie quiescent until the checking force is removed and there is,
again, an outburst of growth-energy. Possibly some gland, like the
thymus, may check exuberant cellular growth at first ; possibly the
commencement of reproductive life may check it later, or turn its
energies in another direction ; but after active reproduction has ceased,
cellular growth of an irregular uncontrolled kind may return, and a
malignant neoplasm result.

I may here refer very briefly to telegony. This phenomenon may
be defined as " the alleged influence of a previous sire on the
progeny produced by a subsequent one from the same mother."
That it occurs has been fairly well established both for plants (when
it is termed xenia) and for animals and perhaps also for the human
subject. The reader will find a good description of its phenomena in
Brunton Blaikie's article in Teratologia, vol. ii. p. 157, 1895. It is
possibly to be explained by the passage of small pieces of the foetal
membranes and placenta {e.g. syncytial fragments) into the circulation
of the mother, from whom again they find their way to the ova in her
ovaries and impress upon these certain characters reminiscent of the
male parent. Even this theory does not explain away all the diffi-
culties in connection with it ; and I may call it again as I named
it before (Vol. I. p. 185), " the wireless telegraphy of antenatal
life."

Such is a brief sketch of the phenomena of morbid heredity as
they are usually understood. It is to be observed that they differ in
many respects from those of normal heredity. For instance, direct
heredity is rare and indirect is very common ; examples of family
prevalence abound ; the individual inherits tendencies and predis-
positions to various abnormal states, and he may, for instance, have
one morbid state, his father another, and a brother a third. Groups
of diseases are transmitted rather than single maladies, and these
may apparently make an interchange with or occur along with other
hereditary morbid states of another kind, such as malformations,
foetal diseases, tumours, twin-bearing, and insanity. In all these
particulars morbid heredity differs from normal heredity.

I do not intend to discuss in detail the mechanism of morbid
heredity ; but I may indicate very simply, and without the adoption
of any of the scientific nomenclatures now in vogue, how it may be

654 ANTENATAL PATHOLOGY AND HYGIENE

supposed to arise and what it really is. I think it cannot be
doubted that, during the life of the egg-cell and the sperm -cell as
individual cellular elements in the body, corporeal conditions are
influential in impressing upon them characters which manifest
themselves later in disturbances of development, of growth, and of
health. In this way two specialised cells are produced (the ovum
and the spermatozoon), each of which has, in addition to its normal
heredity or the hereditary tendencies of its germ-plasm, the ten-
dencies which have been impressed upon it by morbid conditions
existing in the body of the organism of which it has formed a part.
In each cell there are two streams of tendency which are to influence
the future fate of the new being which is to be formed from the
coalescence of two of these cells. In each there is normal heredity
(or simple heredity) which consists in the tendency to develop
an organism resembling that of the typical ancestor, and morbid
heredity (or, better, non-heredity or degenercdion) which consists in the
tendency to lose some of the energy of development present in the
typical ancestor. The former tendency is always inherited, and it
may be regarded as constantly associated with the germ-plasm,
although it is unnecessary for a simple conception of it to introduce
this idea ; whereas the latter is commonly acquired in the germinal
period of life, although it may also be taken on to a small extent
in the embryonic and foetal epochs, and may be handed down from
one generation to another for a number of times, many or few,
as surrounding circumstances determine. It is generally spoken of
as morbid heredity ; but it is more correct to regard it as a dissolu-
tion or breaking of heredity, as a process in which inheritance is an
incident not a requisite, as an arrest in the processes of the impressing
of normal tendencies upon the germ, of the ontogenesis of the
embryo, of the physiological life of the foetus, and of the health of
the organism after birth. It is as if the fingers of the clock cease
for some minutes to advance ; the works go on but in a laboured
fashion ; then perhaps the cause of arrest is removed and the fingers
move forward again and the mechanism once again works freely :
but at the end of the hour or the day the clock is found to be slow,
it has lost something which it does not regain, it has fallen behind
other clocks.

But, now, there is another factor in the germinal life of each
individual which requires to be referred to : there is the occurrence
of impregnation of the ovum with its heredity and its non-heredity
or degeneration, by the spermatozoon with its heredity and its non-
heredity or degeneration. If it were not for this phenomenon the new
being would be simply the old being influenced in his characters by
the conditions acting upon the old during his lifetime, he would repre-
sent the sum-total of his heredity with the modifications (or arrests)
imposed upon it by his environment. But sexual reproduction occurs,
there is a mixing of the heredities and the acquired degenerations of
two cells, and so the new being obtains his own individuality and is
something different from each of his parents although resembling
them both. This occurrence profoundly complicates the results of

THE PRINCIPLE OF ARREST 655

the combined action of heredity and acquired tendencies : it is not at
the present time possible to predict the effect of the union of two
reproductive cells, each of which has a tendency to health or to
disease, and it is doubtful whether it will ever be possible to do more
than make the most provisional of prognoses on the matter. It is
possible that in some cases the healthy sperm-cell may check the
degeneration going on in the morbid ovum, and vice versd ; but it is
also possible that this may not result ; and I doubt if higher mathe-
matics can even state the problem for us, of such extraordinary
complexity is it. One thing, however, seems to me to be clear — the
question whether normal heredity or the abnormal breaking of
heredity (degeneracy) is to predominate in any case, is largely
determined during the years when the sperm and ovum are parts of
the bodies of the parents.

Let us now consider whether pathological processes, causes, and
effects in the various periods of life can be brought under the same
laws. In the first part of germinal life, morbid causes are chiefly
effective in producing tendencies which may afterwards become
monstrous developments or non-developments or diseases. In the
later part of germinal life, these tendencies are doubtless still being
produced, but in addition there are other occurrences taking place,
such as the production or determination of twins and of double
terata, of teratomata, and perhaps also of some other neoplasms. In
the embryonic period the action of morbid causes is producing
monstrosities, malformations, and anomalies of growth ; while in the
fcetal period the results are diseases, but diseases with special
characters, due to the peculiarities of foetal life. After birth, as we
know, morbid causes produce well-known diseases with their well-
known characters. In all these periods of life, I think we can
recognise that morbid causes act in much the same way, and can
conclude that that way is by arresting normal processes of growth,
development, and functional activity. It is easy to understand how
the arrest of part of the architectural elaboration of the embryonic
body produces a monstrosity ; in the chapters that form the main
bulk of this volume such arrested developments . have been over and
over again referred to and their mechanism described. Similarly,
in the fcetal epoch of antenatal life, the arrested performance of
physiological functions will weaken the antenatal defences (placental
and other) of the unborn infant, and allow the invasion of his body
by microbes or their toxines or by toxic products ; the fcetal diseases
result. In the germinal period the principle of arrest is not so easily
grasped, but it is, to my mind, equally real. If the function of the
reproductive cell before impregnation be to carry forward to the
next generation the normal heredity of structure and function of the
past, then it can be seen that arrest at this time means the possibility
of surrounding influences interfering with this transmission ; there is
a break in the handing-on process, some links are wanting from the
chain of health, and the future individual has points of weakness in
his heredity. Again, there comes a time when maturation takes
place or ought to take place ; if arrestment occur here it will take

650 ANTENATAL PATHOLOGY AND HYGIENE

the form of incomplete maturation ; one polar globule alone may be
expelled, not two ; and so in some way, which we do not now know,
the future life of the cell is interfered with. Imperfect maturation
may take from the reproductive cell the power of normal fertilisation,
or it may alter in some way the phenomena which follow it. Again,
when a spermatozoon penetrates an ovum, some subtle mechanical or
chemical change takes place whereby no other spermatozoon gains
entrance ; but if this mechanical or chemical change be arrested or
delayed, another sperm may make its way in and monochorionic
twins or a double monster may result. Similarly in the other forms
of excessive development, an arrest, paradoxical as it may sound,
may be the cause. Again, in the stage of the morula mass there
comes a time when continuous cell -division gives place to cell-
differentiation, when there is no longer simple multiplication of cells,
but when one cell begins to be recognisably unlike its neighbour;
if, now, an arrest occurs, the tendency will be for the cells to go on
multiplying without their forming the blastocyst with all its different
parts and more especially the embryo ; the continuous cell division
will be the result of the arrest of the specialisation of function in the
massed cells. Perhaps, in this way, the hydatid mole takes origin ;
perhaps in this way the rudiment of the future cancer is implanted
in the tissues, a cell possessing the power of indefinitely multiplying
its like, but bereft of the faculty of varying the appearances and
functions of its daughter cells. Other examples might be given, but
enough has been said to show how this principle of partial arrest
may run through the whole series of the morbid phenomena of life.
Complete arrest will, of course, cause death at any of the stages of
antenatal existence.

Let us now see whether we can make any generalisations regard-
ing the causes or cause of this process of arrested growth, develop-
ment, and functional activity. In the etiology of the morbid states
which supervene after birth we recognise mechanical and physical
traumatism, toxic agencies, microbes and their toxines, and parasites
as the chief factors, along with the predispositions to contain maladies
or the malformations and maladies which have existed before birth.
These causes account for the pathological developments of postnatal
life. When we studied the maladies of fcetal life (vide Vol. I. of this
Manual) we found the same causes in action with certain limitations,
and with variations in the results due to the intrauterine environ-
ment of the foetus and some other circumstances. The causes of
fcetal diseases were not dissimilar to these of postnatal maladies.
The pathological manifestations of embryonic life have been found
to be monstrosities and malformations. Now, the results of experi-
mental teratogenesis have shown that the toxic and pathogenic
agencies of postnatal life (alcohol, microbes, their toxines, etc.)
produce monstrosities and defective developments when acting on
the embryo ; further, evidence is being gradually accumulated that
malformations and monstrosities occur very frequently in the de-
scendants of the men and women who suffer from such maladies as
syphilis and tuberculosis, or who indulge to excess in alcohol, cocaine,

ANTENATAL TREATMENT 657

morphia, and the like. It is more than likely, therefore, that once
more the causes of the morhid states are not dissimilar but similar,
and that the monstrosities and malformations of the embryo are due
to no special causes but to the same toxic, toxinic, microbic, and
traumatic agencies as exist in foetal and postnatal life. The results
are widely different, for the causes act upon an organism forming, not
upon one already formed, and so malformations rather than diseases are
the consequence. Finally, we may carry the same argument back to
the germinal period of life. I believe that in this, just as in the
fcetal and embryonic epochs, there are in action the same kinds of
morbid causes, microbes, poisons, toxines, traumatisms: but again
the results are peculiar, being alterations, by arrest, of the physio-
logical conditions of the period. In this way twin-bearing, sterility,
hydatid moles, double terata, anomalies of the fcetal annexa, tumours,
and degenerative tendencies are linked on to monstrosities, mal-
formations, and fcetal diseases as results of similar and not of
dissimilar causes. It seems to me that there is abundant support
for the view, that the ultimate causes of all morbid processes,
antenatal and postnatal, are essentially the same in their nature,
while their results differ widely because of differences in the
structure and physiology of the organism at the time when they
come into action. There is an etiological similarity or uniformity,
therefore, in the various stages of life. The wall of division which
has separated antenatal from postnatal pathology is thus broken
clown ; can it be doubted that the result will be a greater and a
clearer knowledge of the peculiarities and characters of the morbid
processes of both periods of life ?

Treatment of Antenatal Morbid States.

I have already, in the closing pages of the first volume of this
work (pp. 451-487), indicated how antenatal morbid states, and more
especially fcetal diseases, may be treated with a view to their pre-
vention or cure, and I shall not repeat here what was written
there ; 1 but I may add a few conclusions which seem to me to follow
from the consideration of the special matters dealt with in this
volume.

It has been shown, for instance, that embryonic monstrosities
and malformations sometimes show a tendency towards repair during
the foetal period of antenatal life (vide spina bifida, hare-lip, etc.) ;
it has also been demonstrated that, even in the family with most
malformations and monstrosities, some members come into the world
free from morbid states (e.g. the offspring of the monstriparous
woman described in the American Journal of Obstetrics, xli. 577,
1900); and it has been suggested that morbid heredity is rather a
break in the heredity of health and normal formation which may be
mended again if not perhaps in one generation, at any rate in two
or three. These phenomena show, at least so it seems to me, that
there is a constant tendency towards recovery and the return to a

1 But it will greatly help the reader if he will re-read these pages now.
VOL. II. — 42

658 ANTENATAL PATHOLOGY AND HYGIENE

healthy stock. The practical conclusion as to therapeutics is, there-
fore, to try to secure that the specialised reproductive cells which
are to become the infants of the next and of succeeding genera-
tions be preserved from the impact of morbid agencies (toxic,
microbic, toxinic, traumatic) so long as they are in the bodies of
the men and women of this generation. Let me expand this idea
a little.

It is obvious that if a man or a woman has a body poisoned
with the septic products of smallpox, infected with the toxines of
tuberculosis or syphilis, or irrigated with fluids containing morphia,
lead, cocaine, or (most of all) alcohol, his and her reproductive cells
cannot escape the morbid influence thus brought to bear upon all the
other tissues. We know that certain poisons specially affect certain
tissues {e.g. lead, the nervous system), and possibly others specially
affect the reproductive cells ; but these peculiarities do not influence
the argument, they only serve to explain the diversities of the
results. There is reason to believe that reproductive cells so
surrounded by malign influences grow into malformed embryos,
diseased foetuses, and infants predisposed to various maladies; in
other words, the chain of normal heredity is broken in them, and
morbid heredity or degeneration takes its place. But the individual
in whose body these reproductive cells lie may recover from the
illness from which he or she is suffering (e.g. typhoid fever, tuber-
culosis, syphilis), or may cease to supply his other tissues with noxious
drugs, narcotic or alcoholic ; under these circumstances there is
evidence to show that the reproductive cells may join in the general
tissue recovery and convalescence which sets in. A drunkard's nerve
cells may seem to be hopelessly enfeebled, and yet total abstinence
may wonderfully recover to him a mental vigour which had seemed
as if lost for ever; is it not possible that it may do as much for
the reproductive cells ? But, it is said, his reproductive cells
have already been weakened by the excesses of his father before
him ; they are predestined to be easy victims to morbid influences.
There is the predestination of morbid heredity. True, but there is
also the initiative of individual effort to be taken into account. " I
am I, howe'er I was begot." In one generation much may be done
to restore the germ-plasm or the substance of the reproductive cell
to a healthy state, for I conceive that its true heredity ever impels
it in that direction. It is a family matter, is this kind of treatment.
The cleanness of life and the control of the indulgence of morbid
cravings for narcotics and stimulants of the parents does much, but
it may be insufficient to effect a cure ; the children may, so to say,
have to continue the treatment for the benefit of their children, and
so on, possibly, for one or two generations. Family diseases require
family treatment. Further, during the process, it will be well that
marriages of consanguinity be avoided, and that each individual shall
not postpone matrimony till late in life (" opsigamy ") or embark
upon it at too early an age (e.g. before nubility). It need hardly be
said that it will likewise be well for the individual, against whom
" from his cradle fate and his fathers fight," to make no matrimonial

ANTENATAL TREATMENT 659

alliance with one endowed with the sad legacy of family ill-health,
unless indeed she also is making a good struggle to redeem for her
germ-cells some measure of health and strength. Eugenesis or well-
begetting is one of the world's most pressing problems — it is far
from being a hopeless one, but it must be attempted before it can
be solved.

APPENDIX

List of Names of Medical Men who have supplied me with Specimens or
Records (generally with Photographs) of Cases of Antenatal Disease or
Malformation, or who have enabled me to examine Patients suffering from
Congenital Anomalies. Of Specimens there have heen 206, of Patients
54, and of Reports 170, making a total (including my own observations
in Private and Hospital Practice) of 500. The numbers within brackets
simply refer to my List in my Case Books.

Amner, Mr. F. H., Edinburgh. Specimen of Stillborn Twin (16).
Andrews, Dr. II. R., London. Report on case of Congenital Prolapsus

Uteri (441).
Annan dale, Prof., Edinburgh. Patient with Congenital Defect of

Perineum (241).
Anonymous, Grimsby. Specimen of Iniencephaly (277).
Anonymous. Report on Pygopagous Twins (471).
Ashby, Dr. H., Manchester. Report on case of Foetal Nephritis (447).
Baildon, Dr., Southport. Specimen of Meningocele (34).
Ballantyne, Dr. A., Eskbank. Specimens of Encephalocele (22), Gastro-

schisis and Anencephalus (24, 178).
Ballantyne, Dr. H. S., Eskbank. specimen of Hydrocephalus (235).
Barbour, Dr. A. H. F., Edinburgh. Sp>ecimen of Foetal Anasarca and

Obesity (11). Patient with Anomaly of Hairy Scalp (305).
Barnardo, Dr. G. F., London. Report on cases of Ectopia Cordis (390)

and Hypospadias and Uterine Malformation (426).
Bell, Dr. Joseph, Edinburgh. Patient with Imperforate Anus and Double

Angina (201).
Beveridge, Dr., Leith. Specimen of Fcetal Dropsy (218).
Bidone, Dr. E., Florence. Report of Foetal Endocarditis (474).
Blackwell, Dr. E., Hastings. Report on case of Spina Bifida (433).
Bluck, Dr. Cardy, London. Specimen of Congenital Hydronephrosis

(140). Report on two cases of Anencephalus (364, 365), two of Foetal

Dropsy (366, 367), one of Foetal Ascites (368), one of Duodenal

Atresia (369), one of Absence of Radius (370), and one of Anchyloses

(371).
Booth, Dr. M., Edinburgh. Specimen of Twin Abortion Sac (236).
Boxer, Dr. E. A., Edinburgh. Specimen of Foetal Death (244).
Brayton, Dr. A. W., Indiana, U.S.A. Report on Thoracopagous Twins

Sj>ecimen of Deformed Extrauterine

Report on two cases of Congenital

601

(384).
Brewis, Dr

Foetus
Brook, Dr.

N".

(35).

W.

T.,
H.

Edinburgh.
B., Lincoln

Haemophilia (444, 445).

662 ANTENATAL PATHOLOGY AND HYGIENE

Brooke, Dr. W. F., Swansea. Report on Congenital Obstruction of

Intestine (363).
Brown, Dr., Hanley. Specimen of Gastroschisis (23).
Brown, Dr. E. E., Inverness. Report on Meningocele (468).
Buist, Dr. R. C, Dundee. Reports of specimen of Foetus Papyraccus (374),

Congenital Teeth (387), Hydrocephalus and Cervical Spina Bifida

(397).
Bunting, Dr., Edinburgh. Patient with Unilateral Anophthalmias (240).

Report on Congenital Amputation of Digits (436).
Butchart, Dr. C. A., Leith. Specimen of Fcetus with Multiple De-
formities, including Spina Bifida (113).
Caird, Mr., Edinburgh" Patient with Polydactyly (138).
Cairns, Dr. Murray, Liverpool. Specimen of Iniencephaly (252).
Calder, Dr., Leith. Specimen of Cystic Kidney and Hypospadias (327).
Callender, Dr. T. M., Edinburgh. Report on Foetal Dropsy (428).
Calwell, Dr., Wellington. Sp>ecimen of Anencephalus (172).
Cappie, Dr. James, Edinburgh. Specimen of Anencephalus (194).
Carmichael, Dr. E., Edinburgh. Specimen of Foetal Ascites and Distended

Bladder (197).
Carmichael, Dr. James, Edinburgh. Specimens of Deadborn Foetus (107,

125).
Charles, Dr. E., Indiana, U.S.A. Report on cases of Encephalocele (425),

of Anencephalus (449).
Chiarleone, Dr., Catania. Report on specimen of Foetus Amorphus

Anideus (360).
Chiene, Prof. J., Edinburgh. Patient with Ectrodactyly (292).
Clarkson, Dr. R. D., Falkirk. Specimen of Anencephalus with Placenta

(278).
Cordes, Dr. L., New York. Report on case of Foetal Ichthyosis (498).
Cormack, Dr. W. P., Dudley. Report on cases of Polydactyly (453) and

Epispadias (459). *

Cotterill, Mr. J. M., Edinburgh. Patient with Double Labial Fistula

(221).
Croom, Sir Halliday, Edinburgh. Specimen of Foetal Dropsy (102).
Darling, Dr. T. B., Edinburgh. Specimens of Atresia Ani and Club-
Hands (21), Foetal Measles (52), Stillborn Infant (80), Deadborn

Infant (123, 131, 171). Patients with Congenital Amputation (41),

Occipital Encephalocele (304). Report on Congenital Singultus

(389).
Davidson, Dr. S., Penrith. Specimen of Foetal Chondrodystrophia (101).

Report of case of Polydactyly (77).
Dendle, Dr. F., Lochgelly. Specimen of Anencephalus (169). .
Dickson, Dr. D. E., Lochgelly. Specimen of Foetal Death (301). Report

on Facial Paralysis in Newborn Infant (429).
Dickson, Dr. G. A., South Queensferry. Specimen of Pseudencepbalus

(309).
Doherty, Dr., Chicago. Report on case of Intrauterine Fracture (435).
Doran, Dr. A., London. Report on Cystic Ovary in Foetus (349).
Drysdale, Dr., Edinburgh. Specimen of Kitten with Absence of Fore

Limb (195).
Dumesnil, Dr. Ohmann, St. Louis, U.S.A. Reports on Foetus Amorphus

Mylacephalus (383), Hairy Ntevus (412).
Dwight, Dr. T., Boston, U.S.A. Report on case of Double Hand (353).

LIST OF SPECIMENS, ETC 663

Easby, Dr., Peterborough. Report on Antenatal Fracture of Clavicle
(396).

Edmondson, Dr. J., St. Helens. Specimen of Paracephalic Twin (30).

Elder, Dr. G., Leith. Patients with Congenital Tylosis Palmse et Plantse
(164), Hemiatrophy of Face and Malformed Ear (181). Reports on
Vulvar Anus (399), Congenital Fragility of Bones (430), and Oligo-
dactyly (431).

Elder, Dr. W., Edinburgh. Specimen of Iniencephaly (44).

Evans, Dr. J. H., London. Report on case of Cervical Auricle (466).

Falconer, Dr. J. F., Edinburgh. Report on Twins with Erysipelas
Neonatorum (4LS).

Farncombe, Dr. W. T., Ilarborne, Birmingham. Report on case of Con-
genital Heart Disease (439).

Farquharson, Dr. J. M., Edinburgh. Specimen of Stillborn Foetus and
Placenta (132).

Farquharson, Dr., Newcastle. Specimen of Anencephalus, etc. (155).

Felkin, Dr. R., London. Patient with Vascular Naevus (75). Report on
various cases of Maternal Impression (339-345), Congenital Contraction
of Fingers (357).

Ferguson, Dr. R. T., Anstruther. Specimen of Anencephalus (222).
Report on Anencephalus (470).

Fleming, Dr. R. A., Edinburgh. Specimen of Imperforate Anus (248).

Fordyce, Dr. W., Edinburgh. Specimens of Hernia of Umbilical Cord
(39), Iniencephaly (251). Report of case of Foetal Ascites (473).

Fowler, Dr. J. 8., Edinburgh. Patient with Heini-Hypertrophy (224).
Specimen of Placenta and Cord (291). Report on Antenatal Con-
vulsions (440).

Fkeelaxd, Dr., Broxburn. Specimens of Foetal Dropsy (9, 10) and
Anencephalus (226).

Geddes, Dr. A. M. C., London. Report of Placenta with Tumour (417).

Giles, Dr. A., London. Report on Foetus with Distended Bladder and
Absence of Urethra (386).

Glegg, Dr., Edinburgh. Specimen of Anencephalus (279).

Goudie, Dr., South Shields. Specimen of Anencephalus (135).

Govan, Dr. G.j Cockermouth. Specimen of Iniencephaly (320).

Gradwohl, Dr. R. B. H, St Louis, LT.S.A. Report on case of Foetal
Cerebro-Spinal Meningitis (434).

Graham, Dr. F. M., Edinburgh. Specimens of Placenta from Hydramnios
(116), Lobulation of Kidney (128), and Congenital Diaphragmatic
Hernia (166). Patients with Arestibular Cyst (127), Encephalocele
(143).

Grant, Dr. Taylor, Edinburgh. Specimen of Battledore Placenta (303).

Green, Dr. J. L., Edinburgh. Specimen of Hydrocephalus (286).

Gulland, Dr. G. L., Edinburgh. Specimen of Abortion Sac (106).

('. txsburg, Dr. M., Charkow, Russia. Report of Foetal Ascites (402),
Quadruplets (499), and Spina Bifida (500).

Guthrie, Dr. Cowan. Specimen of Dermoid Cyst or Pseudo-acormus (33).

Harris, Dr. R. P., Philadelphia. Report on two cases of Absence of
Penis (413, 414).

Hart, Dr. D. Berry, Edinburgh. Specimens of Embryonic Gastroschisis
(65, 66), Anencephalus (122), Hydrocephalus (151), Anencephalus
(206). Patient with Polymastia (149).

Harvey, Dr., Edinburgh. Specimen of Anencephalus (168).

664 ANTENATAL PATHOLOGY AND HYGIENE

Haultain, Dr. F. W. N., Edinburgh. Specimen of Achondroplasia
(273).

Hellier, Dr. G. B., Leeds. Report on two cases of Elephantiasis Con-
genita Cystica (455, 456) and on Malformed Cranium (493).

Helm, Dr. J., Edinburgh. Specimens of Abortion Sacs (42, 64, 70).

Helme, Dr. A. T., Manchester. Specimen of Phocomelus (15).

Henderson, Dr. E., Kirkcaldy. Gases of Premature Infant (307), Post-
mature Infant (308).

Henderson, Dr. E. R., Hull. Report on case of Hernia of Umbilical Cord

(461)-
Hinsdale, Dr., Philadelphia. Report on case of Pseudohermaphroditism

(362).
Hughes, Dr. H. L., Dowlais, Wales. Specimen of Dimidiate Placenta

(332).
Hutchinson, Dr. J., London. Report on cases of Absence of Pectoral

Muscles (437), Double Penis (438).
Hutchison, Dr. R., London. Specimen of Fcetal Dropsy (104).
Hutton, Dr., Edinburgh. Specimen of Constriction of Duodenum (190).
James, Dr. Alexander, Edinburgh. Patients with Hereditary Syndactyly

(202), Yaginal Atresia and Cervical Ribs (227).
Jamieson, Dr. W. Allan, Edinburgh. Patient with Tylosis Palmae (217).
Jennings, Dr. D. D., New York. Report on case of Congenital Cranial

Depressions (356).
Jones, Dr. 15., Leigh, Lancashire. Report of case of Fcetal Rigor Mortis

(375).
Jones, Dr. Howard, Newport, Wales. Specimen of Sympodia (250).
Kennedy, Dr. C, Edinburgh. Specimen of Postmature Anencephalic

Foetus (258).
Ker, Dr. C. B., Edinburgh. Specimen of Gastroschisis (124).
Kynoch, Prof. J. A. C, Dundee. Repjort on cases of Coiling of Cord (361),

Dwarf Embryo (379), Stenosis of Bile Duct (380).
Lackie, Dr. J. Lamond, Edinburgh. Specimen of Double Chicken (242).
Landsborough, Dr. D., Changhoa, Formosa. Report on three cases of

Transposition of Viscera (419, 420, 421).
Lang will, Dr. G., Leith. Specimen of Encephalocele (193).
Lawson, Dr. T., Edinburgh. Specimen of Hernia of Umbilical Cord (27).

Patient with Deformity of Penis (79).
Loeb, Dr. A. H., Montreal, Canada. Report on Chick with Four Legs

(416).
Lundie, Dr. R. A., Edinburgh. Specimens of Hydrocephalus and Absence

of Thumb (71), Anencephalus and Deformed Thumbs (148), Dead

Foetus and Placental Haemorrhage (165), Foetus Papyraceus (209),

1 )ead Foetus and Placenta (238), Foetus with Absence of Radii, etc.

(245), Knot on Cord (253), Dead Foetus and Placenta (257), Diseased

Placenta (275). Patient with Desquamation of Cuticle (213). Report

on two cases of Defective Thumb Muscles (354, 355).
Macgregor, Dr., Jedburgh. Specimens of Anencephalus (232), Abortion

Sac (262).
Mackenzie, Dr. R., Montreal, Canada. Report on case of Foetus Amorphus

Anideus (454).
Mackness, Dr. G. O. C, Broughty-Ferry. Report on case of Macrodactyly

(442).
Maclagan, Dr., Sleaford, Lincolnshire. Specimen of Gastroschisis (268).

LIST OF SPECIMENS, ETC. 665

MacVicar, Dr., Dundee. Report of Congenital Prolapsus Uteri (398).

Macvie, Dr. S., Chirnside. Report on Foetal Death (448).

M'Bridk, Dr. P., Edinburgh. Patient with Dermoid on Nose (263).

M'Elnev, Dr. E., Birmingham. Report on case of Hypoagnathus (443).

M'Gregor, Dr. R., Hull. Report on case of Foetal Death (464).

Male, Dr. H. C, Essex. Specimen of the Foetus Papyraceus (103).

Report of Congenital Intestinal (Duodenal) Atresia (338).
Mann, Dr. F. W., Ashton-under-Lyne. Specimen of Foetal Dropsy (54).
Martin, Dr. A. A., North Shields. Specimen of Encephalocele (26).
Martin, Dr. Charles, Birmingham. Report on Anencephalic Postmature

Foetus (382).
Martin, Dr. J. W., Leith. Specimen of Hydatid Mole (46).
Martine, Dr. W. R., Haddington. Report on Congenital Teeth (394).
Menzies, Dr. D., Edinburgh. Specimen of Anencephalus (6).
Mi chin, Dr., Charkow, Russia. Report of cases of Thoracopagous Twins

(403, 404), of Dicephalus (405), of Ectopia Vesica; (406), of Anen-
cephalus (407, 408, 409), of Micromelus (410), and of Amelus (411).
Miles, Mr. A., Edinburgh. Specimen of Anencephalus (105).
Millard, Dr. W. W., Edinburgh. Specimens of Foetus Amorphus (56),

Anencephalus (204, 281).
Miller, Dr. W. H, Edinburgh. Specimens of Foetal Death (247), Di-

chorionic Twins (256).
Milligan, Dr. D., Edinburgh. Patients with Polydactyly (61, 62, 63),

Congenital Scarlet Fever (72), Spina Bifida, etc. (199). Specimen of

Paracephalic Twin (126).
Moncorvo, Dr., Brazil. Report on four cases of Congenital Elephantiasis

(385, 479, 480, 481).
Montgomery, Dr. D. W., San Francisco. Report on case of Naevus

Linearis (446).
Moore, Dr. E. C, Edinburgh. Specimens of Anencephalus (108), Abortion

Sac (112, 136), Foetal Goitre (129). Patient with Preauricular

Appendage (134). Report on Webbing of Penis (359).
Morgan, Mr. J. H, London. Reports on Double Penis (376), Haredip

(377), Preauricular Appendages (378).
Morison, Dr. A. E., Hartlepool. Specimens of Foetal Dropsy (141),

Foetus Papyraceus (152).
Morison, Mr. Rutherford, Newcastle. Specimen of Teratoma on Infant's

Face (175).
Mowat, Dr. J., Edinburgh. Patient with Congenital Hypertrophy of

Pylorus (282).
Mowat, Dr. R. S., Edinburgh. Specimen of Achondroplasia (310).
Murray, Dr. P., Leith. Specimen of Anencephalus (267).
Murray, Dr. R. Milne, Edinburgh. Specimen of FCxencephalus (203).
Nannetti, Dr. M., Kirkmichael. Patient with Infantile Uterus, etc.

(211).
Neugebauer, Dr. F., Warsaw. Report on case of Congenital Prolapsus

Uteri and Hypertrichosis (395), Amniotic Band (484), Polymastia

(485), and various cases of Pseudodiermaphroditism (186-490).
Nicholson, Dr. H. O., Edinburgh. Specimen of Cyst on Placenta (311).

Patients with Aniridia and Microphthalmus (158), Vulvar Anus

(283).
Nightingale, Dr. P. H., Bangkok. Reports on case of Macrodactyly

(491) and on one of Facial Teratoma (496).

666 ANTENATAL PATHOLOGY AND HYGIENE

Oldright, Dr., Canada. Specimen of Congenital Teeth (274). Report on
case of Congenital Tooth (415).

Osler, Prof. W., Baltimore. Report of Cerebral Haemorrhage in Foetus
(478).

Padilla, Dr., Buenos Aires. Report on specimen of Cyclopia (372).

Pallares, Dr. J. F. G, Spain. Report of case of Dicephalus (483).

Palmer, Dr. S., Ludhiana, India. Report on case of Spina Bifida (462).

Paterson, Dr. W., Edinburgh. Specimen of Gastroschisis, etc. (28).

Peddie, Dr. H., Edinburgh. Specimen of Missed Abortion (179).

Pendred, Dr. V., Hammersmith. Report on Tylosis Palma? (427).

Pernet, Dr. G., London. Report on case of Multiple Sarcoma Cutis of
Newborn (452).

Pirie, Dr., Dundee. Specimen of Iniencephaly (162).

Playfair, Dr., Edinburgh. Specimen of Abortion Sac with Dwarf Embryo
(184).

Price, Dr., Edinburgh. Specimen of Anencephalus (58).

Richards, Dr. S. H., Middlesbrough. Specimens of Amniotic Adhesions
and Multiple Malformations (300), Early Abortion Sac (328).

Robertson, Dr. W, I., London. Reports of Ectopia Vesicae (236), Con-
genital Idiocy (337).

Robinson, Dr. E. S., Stourport. Report on case of Epignathus (465).

Rodgers, Dr. J. W., Clifton. Report of case of Cyclopia (40).

Rogers, Dr. B., Clifton. Report on cases of Hypoagnathus (400) and
Anencephalus (401).

Rome, Dr., Cardenden. Specimens of Gastroschisis (331), Anencephalus
(207). Reports on Foetus with Hypospadias, Spina Bifida, etc. (424),
and of Anencephalic Foetus, third by same woman (460).

Routii, Dr. A., London. Report on Diprosopous Anencephalic Foetus (388).

Russell, Dr. J. Lawson, Todmorden. Specimen of Anencephalus (188).

Salt, Dr., Edinburgh. Specimen of Dead Foetus (142).

Shaw, Dr., Cockburnspath. Specimens of Foetal Ascites and Hare-lip (229),
Early Abortion Sac (264).

Shipway, Dr. F. E., London. Report on Foetus Amorphus Anideus (467).

Simpson, Prof. A. R., Edinburgh. Specimens of Intestinal Anomaly (1),
Enlargement of Spleen (3), Fcetal Ascites (4), Anencephalus (5, 7, 8),
Foetal Death (12), Vulvar Anomaly (13), Cephalhematoma (17), Fcetal
Peritonitis (18), Vascular Chorion (20), Iniencephaly (25), Sclerema
Neonatorum (31), (Ldema Neonatorum (32), Foetal Syphilis (36),
Vitelline Vessels in Cord (37), Anencephalus (51), Rupture of Spleen
(69), Knot on Cord (86), Single Artery in Cord (87), Gastroschisis (99),
Anencephalus (133, 150), Fcetal Dropsy (215), Pattledore Placenta
(216). Patients with Polydactyly (59, 76), Congenital Pemphigus
(89), Polythelia (98).

Skirving, Dr. Scot, Edinburgh. Patient with Diphallus (137).

Sloan, Dr. A. T., Edinburgh. Specimens of Stillborn Twin (2), Abortion
Sac (57), Stillborn Infant (78, 82), Pseudencephalus (111), Stillborn
Infant (120), Deadbom Infant (130), Stillborn Foetus (145), Ante-
natal Rigor Mortis (177), Habitual Foetal Death (182), Gastroschisis
( 1 96). Patient with Deformity of Penis (96).

Smith, Dr. James, Edinburgh. Specimens of Aprosopus (19), Anencephalus
(47).

Smith, Dr. James, Falkirk. Specimen of Anencephalus (167).

Smith, Dr. G. T, New Brunswick. Report on case of Cebocephalus (432).

LIST OF SPECIMENS, ETC. 667

Stephenson, Prof. W., Aberdeen. Specimen of Gastroschisis and Double

Genital Tubercle (200).
Stevens, Dr. John, Edinburgh. Specimens of Foetus Papyraceus (173),
Abortion Sac (183), Abortion Sac with no Embryo (186), Abortion
Sac (189, 208), Hemorrhagic Mole (212, 237), Abortion Sac (287),
Abortion Sac with Dwarf Embryo (290), Abortion Sac (293), Anen-
cephalus (298), Abortion Sac (323), Knot on Cord (330). Patient
with Congenital Mobility of Knee Joint (219), Naevus on Eyelids (220).

Stevenson, Dr., London, Canada. Specimen of Congenital Amputation in
Kitten (272).

Stewart, Dr. K., Edinburgh. Specimens of Thoracopagous Double Mon-
ster (50), Foetus from Eclampsia (83).

Stewart, Dr. W., Leith. Patient with Congenital Cystic Kidney (249).

Stiles, Mr. H. J., Edinburgh. Patient with Congenital Enlargement of
Cutaneous Veins (284). Report on case of Perineal Teratoma (318).

Still, Dr., London. Report on case of Foetal Dropsy (463).

Stractian, Dr., Dollar. Report on two specimens of Aneneephaly
(347, 348).

Stumbles, Dr. H. M., Amble, Northumberland. Specimen of Congenital
Dislocations and Anchyloses (325).

Symington, Prof. Johnson, Belfast. Specimen of Foetal Rickets (53).

Thatcher, Dr. C. H., Edinburgh. Specimen of Hydrocephalus, Spina
Bifida, etc. (246).

Thomson, Dr. G., Glasgow. Report on case of Amelus (492).

Thomson, Dr. John, Edinburgh. Patient with Cervical Auricle (161),
Congenital Prolapsus Uteri (163), with Eyes set wide apart (326).
Report of three cases of Absence of Pectoral Muscles (475, 476, 477),
on one of Cervical Fistula (494), on one of Imperforate (Esophagus
(495), and on one of Horse-shoe Kidney (497).

Thomson, Dr. T. J., Edinburgh. Specimen of Amniotic Adhesions and
Multiple Malformations (299).

Thyne, Dr. T. J., Edinburgh. Specimen of Calcareous Placenta (85).
Patient with Caudal Appendage (271).

Tod, Dr. John, Leith. Specimen of Sympodia (121).

Tuke, Dr. A. L. S., Dunfermline. Specimen of Dead Twin Foetus (269).

Turner, Sir William, Edinburgh. Specimen of Foetal Chondrodystrophia
(14).

Tyrie, Dr. C. C. Baxter, Leeds. Report of case of Phocomelus (472).

Underhill, Dr. C. E., Edinburgh. Specimens of Anencephalus and Gastro-
schisis (29), Abortion Sac (214), Spina Bifida (233), Diseased Placenta
(280). Patients with Double Genu Recurvatum (231), Cervical
Hygroma (254).

Van Someren, Dr. G. A., Sydney, N.S.W. Report on case of Foetal
Sepsis (451).

Vargas, Dr., Barcelona, Spain. Report on cases of Congenital Teeth (358),
Haredip (457), and Ectrodactyly (458).

Vigurs, Dr., Newquay, Cornwall. S}>ecimen of Abortion Sac (265).

Waddell, Dr., Slamannan. Patient with Hernia of Umbilical Cord (185).

Wade, Dr., Melrose. Specimen of Pseudencephalus (316).

Watson, Dr. A. M., Aberdeen. Report on case of Melsena Neonatorum
(450).

Watson, Dr. J. 11., Hamilton. Patient with Imperfect Cranial Ossifica-
tion (230).

668 ANTENATAL PATHOLOGY AND HYGIENE

Webb, Dr. M., Queensland, Report on Foetal Poisoning with Opium (469).

Webster, Dr. A. D., Edinburgh. Report on Hairy Naevus (350).

Webster, Prof. J. C, Chicago. Report on case of Foetus Anideus (482).

White, Dr. P. J., Bangor. Specimen of Anencephalus (159). Report on
Foetus in Foetu in Rabbit (381).

Williams, Dr. J. D., Cardiff. Specimens of Abortion Sac (49), Foetal
Dropsy (55). Report on specimen of Syncephalus (373).

Wilson, Dr. A., Edinburgh. Specimens of Exencephalus (110), Placenta
from case of Heart Disease (210), Twin Placenta (317, 333). Patient
with Hypospadias (67), Spina Bifida (228). Report on Maternal
Impressions (351, 352).

Wilson, Dr. James, Edinburgh. Specimen of Iniencephaly (225).

Wise, Dr. B., Kendal. Record of Cephalhematoma (90). Report on
Placental Cysts (346).

Wooo, Dr. W. A., Melbourne. Report on cases of Congenital Teeth (391),
of Fistula Auris in Father and Son (392, 393), of Hereditary Syn-
dactyly in Mother and Five Children (422), and of Sacral Depression
in Mother and Child (423).

Wright, Dr. Fraser, Leith. Specimen of Hydatid Cyst (45).

Wylie, Dr. D. S., Manchester. Specimen of Foetus in Fcetu (234).

Addendum.

In my own Practice (Private and Hospital) I have met with the follow-
ing Specimens : — Velamentous Insertion of Umbilical Cord (38), Knot on
Cord (43), Placental Cysts (48), Abortion Sac without Embryo (60), Twins
(73, 74), Embryonic Gastroschisis (84), Stillborn Infant (88), Imperforate
Anus (109), Knot on Cord (147), Placenta with Umbilical Vesicle (160),
Acanthoma of Scalp (180), Abortion Sac (191), Marginate Placenta (239),
Dimidiate Placenta (243), Abortion Sac with Dwarf Embryo (255),
Placenta with Umbilical Vesicle (260), Cyclopia (270), Battledore Placenta
(285), Postmature Foetus (294), Early Abortion Sac (302), Foetus with
Hypospadias and Hermaphroditism (306), Anencephalus (312), Dichorionic
Twins (313, 314), Foetal Dropsy (315), Spina Bifida (319), Cystic Kidney
(334), Hydrocephalus (335). Patients with Double Hand (68), Double
Hallux (81), Microcephalus (91), Spina Bifida and Club-Feet (92), Con-
genital Hydrocele (93), Tongue Tie (94), Congenital Dislocation of Elbow
(95), Deformity of Penis (97), Cephalhsematoma (100), Congenital Influenza
(114), Congenital Teeth (115), Congenital Strabismus (117), Tongue Tie
(118), Congenital Acne (119), Preauricular Appendage (139), Hypospadias
(144), Epispadias (146), Preauricular Appendage (153), Contracted Fingers
(154), Congenital Inguinal Hernia (156), Congenital Hydrocele (157), Pre-
auricular Appendage (170), Vascular Nsevus (174), Fistula Auris Congenita
(176), Melaena Neonatorum (187), Ectopia of Testicle (192), Hernia of
Umbilical Cord (198), Congenital Inguinal Hernia (205), Bifenestrated
Hymen (223), Syndactyly (259), Cyst on Occiput (261), Absence of Areas
of Skin of Head (276), Congenital Hydrocele (288), Double Hallux (289),
Talipes Calcaneo- Valgus (295, 296), Defects in Parietal Bones (297), Con-
genital Ptosis (321), Absence of Nails on Halluces (322), Vaginal Septum
(324), Congenital Heart Disease (329).

INDEX OF SUBJECTS

Ablepharon

Abortion Sac with no Embryo
Abortion Sacs, examination of
at'ardiac fcetus ix triplets
Acephalic Twin .

ACEPHALUs AtHORUS
in triplets
pseudo-acornras
thorus acardiacus
thorus cardiacus

ACHELIA

ACOCONO-LECANUS .
ACRANIA .
AcKOCEPHALY

Adhesion of Loweb Limbs to each otheb
Adhesions, amniotic, on placenta

amniotic, to back

amniotic, to limbs

amniotic, to umbilical cord

amniotic, with abdomen

amniotic, with cranium
Adrenals, anomalies of .
Ageniocephalus .
Agenosomus
Agnathocephalus
Agnathus .

associated malformations

comparative teratology

morbid anatomy of

teratogenesis .
Ainhum, congenital
Ake

Albinism .

Alcohol, as a teratogenic agent
Allantoido-angiopagous Twins
Allantois, anomalies of .

arteries of

formation of .
Amazia
Amelus
Amnion, a cause of multiple malformations

adhesions of

arrested development of

defective development of

diseases of, in teratogenesis

early appearance of

fluid of

formation of .

609

PAGE

406, 412
613
77
644
627
628
644
628
628
628
389
317
333
371
182
183
.75-180
184
180
171-175
167-171
. 536
425
514, 515
. 425
424, 428
. 430
. 428
430
430
154, 194, 195
638
415, 417
. 21S
623, 625
614
. 32, 49
. 26, 48
. 471
186, 577, 578
209
76
193, 210
614
158, 166
. 197
29
24, 28, 192, 197

670

ANTENATAL PATHOLOGY AND HYGIENE

Amnion — continued.

human, development .

inflammation of

influence in teratology

in teratogenesis

pressure of

structure of
Amniotic Bands, cases of
Amniotic Sao

appearance of .

formation of
Amniotic Theories in Teratogenesis

literature of
Amorphous Twin .
Amoephus Anideus

mylacephalus .
Amphioxus, experiments on
Amputations, congenital .

congenital, causes of .
Anchyloglossia .
Androgynus
Anencephalus

alleged causes of

amniotic adhesions in .

anatomy, sectional of .

an arrested development

anurus .

associated malformations

basis cranii in

cases of

caused by hydrocephalus

cerebrum in

clinical details of

definition of

ears in .

Egyptian mummy

etiology of

eyes in .

face in .

family prevalence in .

frequency in man

historical notes on

hydramnios in .

literature of

morbid anatomy of

musculus sternalis in .

neck in

nomenclature of

obstetrics of

oligohydramnion in

origin of

physiology of .

rarity in animals

sex of .

spina bifida

spinal cord in .

suprarenal capsules in .

sympathetic nerves in .

synonyms of .

teratogenesis of

varieties of

vitality, antenatal of .
Animals, absence of tail in

amniotic adhesions in .
Aniridia .
Anisotropy of the Ovum

168

186

159

PAGE

76

193

74

. 209

75, 193

29

169, 177, 179

63

39

26

167-202

. 198

628

628

194,

131

86,
332-

137,

342,

629

629

628

616

579

150

389

565

351

140

349

346

. 166

318

347

341, 343

172, 176, 178

160, 164, 165

. 344

334

. 332

. 342

. 101

. 348

341, 344

. 341

335, 350

. 332

. 333

335

. 350

. 338

. 347

. 341

. 332

. 336

. 335

76

. 337

350

. 336

340

345

346

346

333

348

339

336

318

189

416

15

300,

338,

18S,

INDEX OF SUBJECTS

671

Ankyloblepharon

Annkxa, fcetal, malformations of

Anophthalmia

Anopsia

Anorchidy

Antihelix, defects in

A \ riTKAGUS, anomalies of.

Antjrtjs Endemicus

Anus, imperforate, case of.

stenosis of

vulvar .
AORTA, anomalies of the .

formation of

transposition of
Aphakia

Apolarity of Blastoderm
Appendage, caudal
Appendages, preauricular .

associated malformations

clinical history of

definition

family prevalence

historical notes

literature of

morbid anatomy

synonyms of

teratogenesis .
Appendix Vermifqrmis, anomalies of
Aprosoptjs .

frequency

morbid anatomy

synonyms

teratogenesis .
Archenteeon, formation of
Arches, aortic

branchial
Area Vasculosa, anomalies of
Arhynchtjs
Aristotle's Definition of Monsi

theory of monstrosities
Arm, defect of, in pleurosomus
Arrest of Development .

causes of

principle of

teratological phenomena

varieties of
Arsenoblast
Artery, hyaloid, persistent

pulmonary, anomalies of

umbilical, single
Ascidian Ova, experiments on

ASPALASOMUS

Assimilation, pelvis of
Astomus, specimen of
Astrology and Teratology
Asymmetry, facial

of pinnae
Atavism and Absence of Clavicle

and hour-glass stomach

and polymastia
Ateleiosis
Athelia .

Atlas Vertebra, anomalies
Atresia ani Urethralis

ani vaginalis .

ani vesicalis

30,

34, 35,

204

PAGE

.

406

.

590

,

407

407

,

561

,

445

,

446

,

318

174, 175

564

,

563

564

,

500

32, 49, 59

t

501

,

418

,

621

,

320

437

-442

440

438

,

437

.

438

.

438

.

438

(

439

.

438

,

441

.

534

424

433

(

434

,

435

t

433

,

435

,

26

35, 49, 59

41, 50, 68

423

.

603

,

401

. 80, 99

,

98

,

493

, 205, 207,

216

,

656

,

655

.

10

.

207

,

612

,

419

#

499

,

592

,

615

514,

515

315,

316

85, 388,

429

91, 92, 99

m

388

,

445

,

468

.

533

,

475

,

248

472

,

329

,

564

563,

564

,

564

672

ANTENATAL PATHOLOGY AND HYGIENE

Atresia — continued,

intestinal

of nares

vulva' superficialis
ATRETOCORMUS APROCTUS

Atrochius
Atrophy, evolution by

AUCHENOMELUS

Auricles, cardiac, anomalies

cervical

supernumerary
Atjtosite .

Bands, amnio-amniotic

amniotic, as cause of amputation
foeto-fcetal
Basi-Occiput, canal in
Basiotic Bone
Battledore Placenta
Bauchstiel

formation of .
Bibliography, teratological
Bidactyly.
" 1)Iddenden Maids"
Bile Ducts, obliteration of
Biogenesis, great law of .
Birth-Mark, racial
Bladder, diverticula
double .

exstrophy, associated malformations
cause of
clinical history
cloacal
definition .
feminine .
frequency .
historical notes
literature of
masculine .
morbid anatomy
synonyms .
teratogenesis
varieties .
extroversion of
formation of .
inversion of
septate
Blastocyst

embedment of .
embryonic part
extra-embryonic part .
formation of .
pathology of .
without embryo
Blastoderm, apolarity of .
Blastodeemic Vesicle
Blastoderms without Embryos
Blastomeres, potentiality of
Blastomerotomt .
Blastotomy
Blastula .
Blephaeocoloboma
Blepharophimosis
Bodies, Wolffian .
" Bohemian Twins "
Brachycephaly .

112, 4

TAOE

532
421
566
318
401
13
638
496
56, 638
438
637

180,

26, 32,

641

".43,

180
154
183
370
370
592
53
33, 39
241
585
642
535
12
314
550
550
546
162
540
545
539
544
541
540
548
542
542
540
546
542
539
48, 57
539
550
610, 611
19
24
22
19
613
613
621
610
216
616
616
61 ti
610
411
412
67
642
371

INDEX OF SUBJECTS

673

Brachychelia
Brachydactyly .
Brachyrhynchus
Brain, formation of

in cebocephalus

in cyclopia

vesicles of
Bkanchial Arches

anomalies of .

cysts .

fibro-chondromata

grooves
Bronchi, anomalies of
" Brothers, Scottish "

BlJPHTHALMUS

Cecum, displacement of
Calf, retroflexion of spine in
Campylorrhachis Set ilk is
Campylorrhinus .
Canal, Cloquet's, persistent

cranio-pharyngeal

neurenteric, formation of
Canals, anomalous penile

multiple in spinal cord
Cancer, heredity of
Cataract, congenital
Cats, tailless
Cebocephalus
Celosomus
Cei'HALucele, associated malformations

basis cranii in .

clinical history

comparative teratology of

contents of

cranial opening in

cranial vault in

definition of .

diagnosis of

frequency of .

historical notes on

hydrocephalus and

morbid anatomy of

sac of .

teratogenesis of

varieties
Cephalo-diprosopus
Cephalomeles
Cephalo-pauasitlts
Cephalo-terata .
Cerebellum, absence of

ectopia of

state of, in spina bifid
Chaldea, teratology of
Cheiloschisis

ClI EI ROMEO ALY
ClIELONISOMUS

Chick, double

used in experimental teratoge
Chirapsy .

Circulation, vitelline, signs of
Circulatory System, relation of,
Chondrification .
Chondrodystrophy Fostalis
Choroid, absence of pigment of

coloboma of

y

to malformations

TAQF.

. 390
. 585
388, 405
31, 34, 55, 65
. 405
399
69
30, 34, 35, 41, 50, 68
423
. 457
438, 450
31, 34, 35, 41, 50, 53, 68
511
635
410

534
312
311
420
420
370
26
558
329
650
419
318
377, 404
492, 514, 515
358
357
353
358
356
356
357
352
355
352
353
358
355
356
358
355
638
638
638
332-372
370
339
300

87, 89
379
266
313, 515
618
211
109
26
206

59, 67
322
417
417

VOL. II.

674

ANTENATAL PATHOLOGY AND HYGIENE

Choroid — contin ued.

TAOF.

malformations of ... . .117

Choriox, formation of

25, 28, 63

parts of ...

28

Chorionic Vesicle

52

ClIORIONEPITHELIOMA

614

Chromosomes ....

609, 610

Classification, teratological

225-239

Clavicle, absence of

467

bifurcation of .

468

malformations of

467

Cleft Palate ....

382

Clinocephaly ....

. 371

Cloaca, bncco-nasal

. 382

ectodermal ....

66

entodermal ....

48, 51, 57

formation of .

. 547

persistence of .

74

Cloquet, canal of, persistence

. 420

Clouding of Cornea

414

Club-Foot .....

. 585

in spina bifida ....

. 301

mechanical cause of

129, 142, 143

Club-Hand ....

583, 584

Coccyx, absence of

. 317

CffiLOM .....

24, 39, 52, 63

Cohnheim's Theory of Tumour-Formation

. 617

Coloboma, choroid.

. 417

eyelid .....

411

iris .....

. 416

lentis .....

. 418

lobule of ear ....

. 445

Colour Anomalies

415

Comparative Teratology

. 230

Complexus Muscle, absence of .

. 466

Concha, anomalies of

. 446

Conical Cornea ....

414

Consanguinity ....

658

Constrictions, congenital, due to amniotic band

186

due to funis

. 151

C'ONTRECOUP AS CAUSE OF MONSTROSITIES .

138, 140

Cord, spinal ....

64

spinal, duplication of .

. 330

spinal, in anencephalus

345

spinal, in microcephalia

. 367

spinal, malformations of

329-331

umbilical ....

64

umbilical, as cause of monstrosities

. 150

umbilical, malformations of .

. 591

CORDENTERY ....

. 372

CORECTOPIA ....

415

Cornea, malformations of .

414

Corpus Callosum, absence of

369

Corsets, pressure of, in teratogenesis

. 139

Crania Bifida ....

333

Cranial Nerves ....

56

ganglia of

45

Craniopagus ....

632, 633, 643

Cranio-pharyngeal Canal

. 370

Cranioschisis ....

333

Cranium, in cyclopia

. 399

perforations of.

. 369

Cranium Progenium

388, 426

Crypto-mero-rhachischisis

. 288

Cryptophthalmus

406

CltYPTORCHIDY ....

. 561

INDEX OF SUBJECTS

675

TAGE

Ctenophoee Eggs, experiments on . . 616

Curvature, spinal, antenatal

. 310

Cuvier, duct of

. 48, 49

Cyclocephalus ....

397

Cyclopia, associated malformations

399

brain in ... .

399

clinical history of

394

comparative teratology

400

cranium in

399

definition of

. 393

dropsical causation of .

162

euhemeristic theory of

84

eye in .

. 395

face, bones of, in

. 398

family prevalence in .

394

frequency

401

histology of

. 396

historical notes on

. 393

literature of

394, 403

mode of origin ....

207, 216

morbid anatomy of

. 395

nose in ...

. 397

proboscis in

. 397

specimen of

. 85, 86

synonyms of .

393

teratogenesis ....

401

twins with ....

395

varieties of

. 397

Cyclops Hypoaonathus .

. 431

Cyclotia .....

400

, 424, 426, 431

brain in ... .

. 433

ears in .

. 432

teratogenesis ....

. 433

Cyllosomus ....

514, 515, 521

Cyphosis, congenital

. 311

sternal ....

. 487

Cysts, branchial ....

457

cervical

457, 638

diverticular

. 538

urachal ....

. 550

Dactylolysis, epithelial .

154, 194

Decidual Membranes, formation of

20

25, 38, 52, 62

Deciduoma Malignum

614

Degeneration, stigmata of

444

Deltoid, absence of

. 477

Depressions, circular, on limbs .

. 153

Derencephalus .

340

Dermoid Cysts, cervical .

458

cranial

638

nasal ....

. 421

origin of

618, 621

sacro-coecygeal

328

thoracic

638

umbilical

. 538

Dermoid Tumours, corneal

. 415

Deromelus

638

Development, centripetal or centrifugal

206

Dexiocardia

. 597

Diaderm ....

610

Diaphragm, formation of .

35

hernia of

175, 477-487

Diastematomyelia

293, 299

Dicephalic Double Monsters, cases of

132, 133

Dicephalus

1 •

. 633, 634, 643

DllHEILUS ....

.

. 390

670

ANTENATAL PATHOLOGY AND HYGIENE

Dichibxts .

DlENCEPHALON

Digits, amniotic malformations of

formation of .

malformations of
DlLECANUS

Dimidiate Placenta
Dimple, post-anal

DlPIIALLUS

clinical history
definition
literature of
morbid anatomy
specimens
teratogenesis .

DlPLOGENESIS

origin of

DlPLOTERATOLOGY
DlPROSOPUS

Dipus

DlRRHINUS

Diseases and Malformations
Diseases as Causes of Monstrosities
Dislocations, congenital causes of
Disomata, dneretic

omplialo-angiopagous .

syncretic
Distichiasis
Distomus .
Diverticula, intestinal .

associated malformations

Meckel ian

teratogenesis .

vesical .
Dolichocephaly .
Double Monsters
Dracontisomus
Ductus Arteriosus, persistence
Ductus Cuvieri .
Ductus Thoracicus, anomalies of
Duct, omphalo-mesenteric, persistent

pancreatic, anomalies of

thyroglossal

vitelline

vitelline, persistent
Ducts, Miillerian .

Wolffian
Duodenum, artesia of

diverticula of .

loss of lumen .
Duplex Placenta
Dwarfism .
Dysostosis, cleido-cranial

Ear, external, absence of .
Ears in Agnathus
Ears in Aprosopus
Ears in Cyclotia
Ears, malformations of
Echinodermata, experiments on
Ectodermal Cloaca
Ecto-otus-aplania

EfTOrAGUS

Ectopia Cordis, mode of origin of
Ectopia Lentis

Ectopia Testis

34,

323

l'AOK

586,

587, 640

43, I

.5, 65, 70

184, 185

64, 194

76

632, 633

. 590

324,

327, 329

566, 634

557

. 554

. 555

. 555

. 554

557

224

618, 619

224, 641

632, 635

586,

588, 640

. 397

196

158

. 129

623

626

. 623

. 412

389

, 446-449

529

. 531

. 530

. 531

. 550

. 371

130

132, 325

. 515

. 502

. 32, 35

503

. 530

. 537

56, 455

39, 48, 57

. 530

66

66

532

529

57

590

242-252

. 468

436

429

435.

. 432

423

616, 619

66

. 437

636

204,

4S9, 493

418

. 561

INDEX OF SUBJECTS

677

Ectopia Vesice ....

l'AGK

. 539

Ectopic Gestation, malformations in

144

EcTRODACTYLY ....

. 585

EcTROMELUS ....

577, 578

Ectropion, congenital

. 412

Edocephalus ....

100, 42L

, 424, 425, 431

Eggs, double-yolked, incubation of

212, 618

Elbow, dislocation of

. 586

Electricity, as a teratogenic agent

. 217

Embrya Papyracea

. 146

Embryo, age of

' 25, 27

, 30, 34, 39, 52

amniotic environment

75

anatomy of

. 8, 9

cephalic and caudal ends

30

dwarf .....

. 243

early death of .

. 245

formation of .

25

life of .

. 1, 18, 73, 196

microsomia of the

. 243

pathology of .

2, 5, 72, 196, 607

pathology of, germinal factor .

77

physiology of .

4, S, 73

size of .

28, 30, 34, 39, 53, 64

Em BKYOGRAPHY, meaning of

9

Embryological Theory of Teratouenesis

. 203

Embryology ....

• 4,7

comparative ....

17

complexity of .

15

definition of .

8

disordered .

72

general principles

. 9, 15

laws of .

. 10, 15

the explanation of teratology .

. 203

Embryomata .

. 621

Embryos, fractional . . . .

616

Emprosthozygosis . . . .

636

Encephalo-amorphus . . . .

638

Encephalocele . . . . .

356

alleged cause of

140

" Enclavement " . . . . .

. 621

" Enclavement " in Teuatogexesis

. 208

Endocarditis, foetal .

495, 507

Exentery ......

. 372

Ensiform Cartilage, anomalies of

. 488

Entropion, congenital .

. 412

Epactal Bone . . . . .

. 370

Epiagnathus .

. 428

Epicanthus .

412

Epigastroschisis . . . .

516

Epigenesis, theory of

134

Epiglottis, anomalies of .

. 465

Epignathus . . . . .

■149, 638

in triplets . . . . .

644

Epispadias .

5

43, 545

547, 559, 563

Epi-sympus Dipus .

573

Epitrichium, characters of

54

Ethmocephalus . . . . .

404

Etiology, uniformity of .

657

ElTGENESIS ......

659

Euhemerism, in teratology

83

Evolution by Atrophy .

13

Excessive Development, cause of

. 656

EXENCEPIIALUS . . . . .

340

Exocardia .

490, 493

Exomphalos, definition of

. 514

Exstrophy of Bladder .

. 539

678

ANTENATAL PATHOLOGY AND HYGIENE

Exstrophy of Bladder — continued

alleged cause of

cause of
Extra-uterine Fcetus, malformations of

EXTRA-UTBRINB PREGNANCY, origin of

Extremities, macrosomia of
Extremity, macrosomia of
Eye, formation of .

in cyclopia
Eyelid, coloboma of
Eyelids, formation of
Eyes in Anencephalus

malformations of

Face, asymmetry of

fissures of

in anencephalus

in cyclopia

macrosomia of .

malformations of

teratoma pf

unilateral hypertrophy
Family Diseases .
Family, neuropathic
Femur, absence of
Fertilisation, pathology of

physiology of .
Fibro-chondromata, branchial
Fibula, absence of
Figures on the Iris
Filament, caudal
Fish, ova of, in teratogenesis
Fission, partial, as cause of spina bifida
Fissure, abdominal

anomalous facial

cervical median

commissural facial

facial, cases of

fronto-maxillary

genal .

glossal

intermaxillary

labial .

median facial .

nasal .

oblique facial .
Fistula Auris Congenita
Fistula, coccygeal

labial .

lachrymal

nasal .

umbilical urinary
Fistula Colli

associated mal formations

definition of

historical notes on

infra-hyoidean

lateral .

literature of

median

supra-hyoidean

teratogenesis .

varieties
Flexion, attitude of, in en
Flexure, cephalic

cervical

ibryo

168, 1

70, 171

341

PAGE
140

162
144
618
'264
266

66
395
411

64
344

373

438,
120,

393, 406-420

388
374
341
398
264
-449
449
388
658
650
582
618
610
456
582
417
326
214
305
515
376
460
376
374
375
374
387
376
379
377
421
375
-444
328
386
414
421
549
450
454
450
451
452
451
455
454
451
454
451
50
45
45

175, 176,

377,
378,

442-

327,
378,

INDEX OF SUBJECTS

679

Flextj re — coni in tied.

cerebral

dorsal, in embryo

pontine
FffiTUS Amorphtjs .
FtETUS Anukus
Farms Compressus
F(ETU3, diseases of, as causes of monstrosities

extra-amniotic

extra-chorionic

FtETUS IN FcETU

Fostus, life of

macrosomia of

microsomia of .
Fcstus Papyracetjs
FffiTUS, pathology of

physiology of
Foot, club . .

double
Foramen Cecum, formation of
Foramen Ovale, formation of
Foramina of Monro
Fossa Rhomboidalis
Fossa, scaphoid, anomalies of
FOVEOLA CoCCYGEA

Foveola Pharyngea

Fracture as Cause of Spontaneous Amputati

Fractures, congenital, causes of

" Free Martin," meaning of

Frog, ova of, experiments on

in teratogenesis
Funic Pressure in Teratogenesis, literature
Funis as Teratogenic Cause

Gall Bladder, absence of

displacement of

double .

formation of
Gangrene, fetal, as cause
Gastro-Acethalus
( Iastro-Amorphus

(iASTROMELUS

( rASTRO-PARASITUS

Gastroschisis, cases of . 76, 168,

cause of

classification .

clinical history

comparative teratology

definition of

diagnosis

historical notes on

literature of

morbid anatomy

nomenclature .

specimens of .

synonyms

teratogenesis .

varieties of
Gastro-Terata

Genitals, female, malformations of
Genoblasts, theory of
Cenu Recurvatum
Germ, human, absence of knowledge of

life of .

pathology of .

trophic equation of

of amputations

iN

ro, i;

, 173,

74, 175

PAGE

35

. 32, 36

45

628, 629

. 317

, 145,

146, 624

158

187, 188

1S8

. 638

. 1, 196

253

. 247

78,

145, 146

5, 72,

196, 607

4

. 585

586, 588

47

48

. 65, 71

41

446

323,

324, 327

370

153

. 141

624

616, 617

213

. 156

150

. 535

536

. 536

57

. 154

638

638

. 638

638

, 176,

177, 181

169,

172, 173

. 514

516

. 521

. 513

. 517

513

523

. 517

. 514

. 518

. 514

. 522

519

513

. 561

. 612

143, 583

. 607

1, 2, 18

2

, 77, 607

253, 256

680

ANTENATAL PATHOLOGY AND HYGIENE

Germinal Factor, in etiology of spina bifida

in teratogenesis
Germs, pathogenic, as teratogenic agents
Giantism .
Glabella Coccygea
Glaucoma, congenital
Glottis, narrowness of
Godlyman, Maria Tofts of
GODS, as teratogenic causes
Gonio-Cheiloschisis
Graf Spee's Early Ovum
Groux, E. A., case of
Grooves, branchial
Growth, disturbances of
Guiociardi's Early Ovum
Gynecomastia
Gynandry

Hand, club

double .
Hands, fusion of .
Half-Vertebre .
Hare-Lip .

antenatal cure of

associated malformation

bilateral

comparative teratology

complicated

family prevalence in

frequency of .

heredity in

mode of origin of

morbid anatomy of

teratogenesis of

treatment of .

varieties of

Harvey's Theory of Arrested Formation
Head, macrosomia of
Heart, bilocular .

displacement of

J isorderly development

formation of

inversion of
Heart, malformations of

associations

associated anomalies

compensation .

diagnosis of

family prevalence

symptomatology

teratogenesis .

varieties of
Heart, pathology of

physiology of .

"reptilian"

valvular anomalies of
Helix, defects in .
Hemi -Atrophy, facial
Hemicephalus
Hemignathus
Hemi-Hypertrophy

alternate

etiology of

literature of

Hemimeltts
Hemipagus

31

■^l\

34, 35

31, 3s

11

TAGE

305
222
220
252-270
328
410
465
114
83
376
27
488
50, 53, 68
241-271
29
561, 565
565, 566

583,
586,

579-

189,

48, 51,

;,s

495-

584
587
587
311
386
382
382
381
384
381
380
380
380
204
380
384
385
381
163
264
505
493
73
,67
602
509
504
506
509
509
508
509
507
496
73
73
505
503
445-
388
333
427
257
265
259
260
579
632

INDEX OF SUBJECTS

681

Hemisphere-Brain
Hemiterata, definition of
Hepata Succenturiata .
Hepatomphalocele
Heredity, ancestral

cancerous
heterologous

homoelironous .

homologous

in teratogenesis

in teratology .

morbid

teratological

theories of

tuberculous
Hermaphrobixism
He p.n i A, cerebral .
Hernia, diaphragmatic

associated malformations

clinical history

definition of

frequency of .

literature

morbid anatomy of

sectional anatomy of .

specimens

teratogenesis .

varieties of
Hernia, femoral .
Hernia Funiculi Umbilicalis
Hernia, inguinal .
Hernia of Umbilical Cord, cause of
Hernia, spinal
Hernia, umbilical

associated malformations

clinical history

definition of

frequency

morbid anatomy

teratogenesis .
Heteradelphus .
Heteropymus

Heterogeneity in Ontogenesis
Heteromorphus .
Heteropagu.s
Heterotaxy, associated malformations

atypical varieties

cases of

clinical aspects of

comparative teratology of

definition of

diagnosis of

double monsters and .

family prevalence in

historical notes

left-handed ness in

literature of

mode of origin of

morbid anatomy of

parturition and

prognosis of

synonyms of .

teratogenesis of

varieties

Heterotopia of Gp.ey Matter of Spinal Cord
Heterotypic .

PAGE

43, 55, 65, 70

. 215
534
526
647
650
650

. 650
650

. 222

79

647, 658

. 652
654

. 651

208, 564, 566

145, 171

477-487
484
481
477
477
486
484
478
477
485
479
534
523
534
207
286
523-528
527
524
523
524
525
527
638
638
11
639
638
598
598

595, 596
599
598
594
600
599
599
594
600
595
208

596, 597
600
601
594

601, 614
594
330
639

682

ANTENATAL PATHOLOGY AND HYGIENE

relation to teratogenesis

Hip, dislocation of.
HoLO-AcRANIA

holooastrosohisis
Holo-Rhachischisis

Homo Cauj» ati'.s .

Horoscopes, teratological .

Human Germ, absence of knowledge ol'

Humerus, absence of

" Hungarian Sisters "

Hyaloid Artery, persistence of

Hybridity,

teratogenic
Hydatid Mole and Early Pregnancy
Hydatid Mole, origin of .

" HyDRAMNIOS " OF THE OyUM .

Hydrencephalocele

Hydrocele, congenital
Hydrocephalus and Anencephalus
Hydrocephalus and Cephalocele
Hydrocephalus and Spina Bifida

associated malformations

cerebellum in .

cyclopia and

family prevalence in .

foetal .

morbid anatomy of

syphilis in

treatment of .

varieties of
Hydro-Meningocele
Hydro-Meningo-Encephalocele
Hydromyelia

Hy'drophthalmus Congenitus
Hydrorrhachis .
Hygroma of the Neck, cystic
Hymen, anomalies of
Hyperencephalus
Hypeknephromata
Hyperphalangy .
Hypoacnathus
Hypogastroschisis
Hypogastro-Exro-Sohisis
Hypomicrognathus
Hypophysis
Hypospadias
Hypostomus

Idols, origin in monstrous births

Ileum, atresia of .

Ilio-Parasitus

Imagination, maternal, in teratogenesis

Immunity, heredity of

Impregnation, date of

Impressions, maternal, among Hebrews

among Romans

at present time

conclusions regarding .

in America

in eighteenth century .

in Greece

in Middle Ages

in nineteenth century .

in seventeenth century

in sixteenth eentury .

in spina bifida .

in Talmud

95, 97,

169,

PAGE

. 586

338, 340
516
287

319, 320
91
607
585
642
419
620

101, 103
254
613
245
357
534
348
358

300, 303
362
362
363
361

160, 161
361
361
363
360
356
357

299, 330
410
286
458
563

340, 492
537
589

424, 428, 429
516
540
424
56, 71

"»63, 565
429

83
533
639
105
652
17, 18
106
107
125
125
122
114
106
108
119
110
109
302
10S

559,

INDEX OF SUBJECTS

683

Impressions — continued.

in teratogenesis

mechanism of .
Inclusion, foetal .
Incubation, artificial, of eggs
Index

Inoiyidualisation, defect of
Infra-Anomalies .
Infundibulum, coccygeal .
Iniencephaly

associated malformations

definition of .

formation of

literature of

morbid anatomy of

specimens of .

teratogenesis .

varieties of
Iniodymus.
Inkipagus .
Insemination, date of
Inter-Parietal Bone
Intestine, formation of .

malformations of
Intestines, anomalies in position of
Inversion of the Viscera
Irideremia
Iris, absence of

coloboma of

figures on the .

malformations of
Ischiomelus

IsCHIOPAGUS

Ischio-Parasitus .
isotropy of the ovum
Isthmus Rhombencephali

Janiceps .

Jaw, lower, malformations of

Jejunum, atresia of

Keratitis, fcetal .
Kidney, absence of

cystic .

floating

fused .

horse-shoe

lobulation

malformations of

rudimentary .

single .

solitary
Knee, dislocation of

Labium Leporinum

Lachrymal Apparatus, malformations of

Lagophthalmus .

Laloo

Lamina Terminalis

Larval Stage in Ontogenesis

Larynx, formation of

imperforation of

malformations of
Lecanopagus
Lecano-Parasitus
Lecanosomatopagus

PAGE

10".

11:3

210,

327,

273-
273-

32.

127
621
211
669
621
219
328
340
282
272

33
283
281
-281
283
-282
635
632

18

370

48, 57, 66

529-534

534

594

416

416

416

417

415-417

639

634

639, 640

15

45

632, 633
423
532

414
551
553
553
552
551
552, 553
551-554
. 552
. 551
551, 552
583, 586

. 379

. 413

412

638

44

245

57, 67

. 465

. 465

634, 635

639

635

684

ANTENATAL PATHOLOGY AND HYGIENE

Lkcanu-Tekata .

Lens, crystalline, formation of

malformations of
Lenticonus

Leopold's Early Ovum .
Leptocephaly
Leptomicrognathus

LlENCULI .

Life, antenatal, divisions of

embryonic, length of .

fcetal, length of

germinal, characters of

germinal, length of

germinal, pathology of

germinal, physiology of
Limb-Buds, formation of .
Limbs, circular grooves on

malformations of
Lip, fissures of

fistula of
Lipomata, sacral .
Literature of Absence of Pectoral Muscles

of allantoido-angiopagous twins

of amniotic theory

of anencephalus

of anophthalmus

of cardiac anomalies . . . 496, 498

of cervical fistulse

of cervical ribs

of clavicular anomalies

of coloboma of eyelid .

of cyclopia

of diaphragmatic hernia

of diphallus

of ectopia cordis

of extroversion of bladder

of fistula auris

of funic pressure

of gastroschisis

of hemi-hypertrophy .

of heterotaxy .

of hydrophthalmus

of imperforate oesophagus

of limb malformations

of macrosomia

of microphthalmus

of microsomia .

of partial macrosomia .

of persistent urachus .

of polymastia .

of preauricular appendages

of ptosis congenita

of quadruplets

of quintuplets .

of sternal cyphosis

of sympodia

of teratological types .

of triplets
Lithiasis, renal, heredity of
Liver, accessory lobes of .

cystic disease of

formation of .

malformations of

transposition of
" Lipbstkr Claw " Anomaly
Lobule of Ear, anomalies of

, 499, 5

00, 501

32

l'AGE

539

41, 54

418

418

21, 22

371

426

536

1, 3, 18

1, 16

1

608

1, 608

607, 612

608

40, 50, 53

169

568-590

377, 378, 379

378, 386

321

476

626

198-202

. 350

■ 408

, 502, 503, 504

. 455

462

468

. 412

394, 403

. 486

. 555

490-491

548

444

156

. 523

260

595

. 411

464

590

256

. 410

. 251

. 268

. 550

. 472

438

. 413

. 645

. 645

487

. 570

241

644

649

535

. 535

35, 47, 54, 57

. 534

535, 595, 598

. 5S5

445

INDEX OF SUBJECTS

685

Lordosis, congenital
Lordosis of Sternum
Lungs, formation of
malformations of
Lymph, threads or bands of

Macrocheilia
Macrochelia

microcornea
Microdactyly
Macroglossia
Macropodia
microsomia
bilateral
cephalic
ftctal .
etiology of
infantile
literature of
partial

partial, etiology
partial, literature of .
serotina
unilateral
MICROSTOMIA

Magnetism, as a teratogenic agent
" Maids, Biddenden "
Malformations, heredity of
Mall's Early Ovum
Mammje, hypoplasia of

malformations of
Mandible, malformations of
Manx Cats

Marginal Insertion of Cord
Marginate Placenta
Maria Tofts of Godlyman
Maturation, pathology of

physiology of .
Maxilla, accessory
Mechanical Causes of Monstrosities
Meckel's Cartilage
Meckel's Diverticulum .
Medullary Groove, closure of
Medusae, experiments on .
Megaloglossia
Megalophthalmus
Melomelus
Membrana Nictitans
Membrane, cloacal

pupillary, persistent .
Meningocele, cranial
sacral, case of .
spinal .
Menstrual Blood, teratogenic cause
Mental Causes of Monstrosities
" Mermaid " Fcstus
Mero-Acrania
Mero-Microsomia
Mero-Rhachischisis
Mesencephalon .
Mesoblast, formation of .
Mesocheilorhinoschisis .
Mesoepicheiloschisis
Mesonephros, appearance of
Mesorhinoschisis
Metamerism

266,

PAGE
312

487

57

. 510

153, 191

378

. 390

414

267

390

. 266

252-270

265, 266

264

. 253

. 255

. 255

. 256

263

. 269

268

265

257-260

389

214,

641,

79,

43, 55

217
642
652
26
. 471
471-475
423
. 318
. 592
. 591
. 114
. 620
609
447
. 129
59
. 530
. 31, 34
616
391
410
640
412
48
416
. 356
. 174
295
97, 98
105
569
338, 340
270
288
65, 70
23
378
377
36
378
269

95

686

ANTENATAL PATHOLOGY AND HYGIENE

MetANEPHROS, formation of
Metencephalon .
Metopagus

Metopism .

MlCROBLEPHARON .

MlCROCEPHALTTS, associated malformations

brain in

clinical history of

cranium in

definition of

frequency of .

histology of

historical notes of

morbid anatomy of

spinal cord in .

teratogenesis of

treatment of .

MlCROOHELIA

Microcoria

Microcornea

Microglossia

Micrognathias

mlcrogyry in microcephalics

MlCROMASTIA
MlCROMELUR

mlcromyelia in mlcrocephaltts
Microphthalmias .
Microsomia

embryonic

etiology of

foetal .

literature of .

malformations and

partial .

postnatal

unilateral
Microstomia
Microstomia
Moles, origin of .
moliere and transposition of
Mono-Anileus Apus
Monophthalmus .
Monopodia
Monorchidy
Monro, foramina of

foramina of, in hydrocephalus
Monsters, double .

MONSTRIPARITY

Monstrosities, Aristotelian theory
bestial origin .
by excess
Chaldean
classification of
destruction of .
diabolic origin
diagnosis of
due to comets .
expiatory nature
heredity of
hybridity and .
mental theories
predictions of future .
pressure as cause of
punitive nature
triple .
warnini's of calamities

he Viscera

in

PAGE

. 48, 58
43, 65, 69
632
371
412
367
365
364
365
363
364
367
364
365
367
366, 368
369
390
415
414
389

424, 426, 427
366
471
582
367
409
242-252
243

246, 248, 250
247
251
246
270
249
261
389
429
93, 100
595
21, 576
407
521, 569, 576
561

44
362

77

657

97, 98, 99

102, 103

623

88, 89

' 96

86

90
605

92

88
652
101
105

88
129

90
644

86

92.

INDEX OF SUBJECTS

687

Monstrosity, definition of

synonyms of .
Moon, teratogenic cause .
" Moon-Calf," etymology of
Morton's Treatment of Spina Bifida
Morula Mass, pathology of

physiology of .
Mouth, formation of

malformations of
MuLLERlAN Ducts, formation of .
Muscles, anomalies of cervical

anomalies of thoracic .
Mi siulus Sternalis
Myelencephalon .
Myelitis, foetal
Myelo-Cystocele .
Myelo-Meninoocele, spinal
Myotomes, formation of .

Nackengsvbe
NjEcele, pelvis of .
N.evus, lumbo-sacral

origin of
Nanocephalus Brachygnathus .

NANUCEPHALUS MlCIlOMMATUS

Natural Caitses of Monstrositiks
Neck, disorderly development of .

fissure of, median

malformations of

muscular anomalies of .
Neofoztus, life of the
Neoplasms, congenital, in spina bifida

heredity of

origin of

sacro-coccygeal
Nerves, cranial, formation of
Nervous Diseases as Causes of Monstrosities
Nervous System, disease of, as cause of spina bifi

relation of, to malformations .
Neurenteric Canal, formation of
Neuroblasts in Spinal Cord
Neuropathic Family
Nipples, absence of
Nomenclature, teratological
" North Carolina Twins"
Nose, double

fissure of, cases of

fissure of, lateral
median

formation of .

malformations of

NOSOGRAPHY INCLUDES FcSTAL DISEASES

Nosological Theory of Teratogenesis

NoTENCEPHALUS .

Notochord, formation of .

malformations of

persistence of .
Notomelus

Occiput, third condyle of .
(Esophagus, absence of

diverticula

duplicity

fistula of

formation of

im perforation, congenital

hi

34,

92

47, 56

43.

PAGE

80, 215

84

92

93, 94

306

615

610

64, 66
388

58, 66
466
476
477

65, 69
330
297
297

49

53, 64

316

314

92

424

409

94

74

460

450-466

466

16, 18

300

653

617

321

45, 56

164

302

205

26

42

650

472

234

642

233, 379

171

379

378

41, 50, 53, 64
393, 420
6
158
340
32, 49
372
329
638

26,

370
462
462
462
462, 464
47
462

688

ANTENATAL PATHOLOGY AND HYGIENE

(Esophagus — continued.

imperforation, associated malformations

literature of .

teratogenesis
malformations of
narrowness

Oligohydramnios influence of

OMPHALOCELE
OmPHALOCEPHALY .

Omphalo-Mesenteric Vessels, persistence of
Ontogenesis and Growth

defective

definition of

" detours" in .

heterogeneity in

larval stage in .

not epitomised phylogeuy

organogenesis in

rearrangement in

specificness in .
Oocephalus
Opacity of Cornea
Ophthalmocephalus
Opocephalus
Opsigamv .

Optic Vesicles, formation of
Organism, transition
Organogenesis, a function of the embryo

methods in

rearrangements

secrets of
Organs, formation of

functions of
Orientation of the Embryo
Ossicles, suprasternal
Ossification, beginning of
Otocephalus
Otocyst
ourentery
Ovo-Testis
Ovum, " hydramnios " of .

maturation of .

pathology of .

physiology of .
Oxycephaly

Pachycephaly
Palate, cleft
Palatoschisis
Pancreas, anomalies of

formation of .
Paracephalic Twin
Paracephalus Acormus .
Paracephalus and Quadruplets
Parackphalus Apus
Parackphalus Dipcs Acardiacus
Paracephalus Dipus Cahdiacus
Paracephalus, dropsical causation of
Paracephalus Monopus .
Paracephalus Pseudo- Acormus
Paracephalus Sympus
Paraphimosis, antenatal .
Parasites, placental
"Parasol" Insertion of Cord
Parthenogenesis .
Pat ag i um .

400, 423

102, 1

62, 400

462-

214,

11

425,
45

11

i'AOE

463

464

464

465

464

76

523

371

593

243

74

11

12

11

245

14

11

13

, 12

405

414

401

431

658

,54

til

4

13

68

36

4

4

616

488

, 67

425

,41

372

567

245

609

649

648

371

371

382

382

537

48

626

627

645

627

627

626-

162

627

627

627

[49, 560

523, 625

592

612, 620, 621

603,

59

423, 424,
30. 34

216, 6

183

>*\.

INDEX OF SUBJECTS

689

Patagitjm — continued.

PAGE

cervical ... . . . 460

thoracic ....

. 493

Patella, absence of

. 583

Pathogenesis, nature of .

6

Pathological Theory of Teratogenesis

158

Pectoral Muscles, absence of

148, 476

Pelvis, assimilation

315, 316

fissa

.

541

Nsgele's

.

316

obteeta

. . .

. 313

pseudo-pithecoid

317

Robert's

316

" Pendeloques " .

. 456

Ten is, absence of .

540, 554

duplication of .

,

. 554

fissure of

.

. 559

hypertrophy of

.

149

malformations of

. . ,

554-561

perineal

. 561

supernumerary glans

.

560

webbing of

.

. 560

Perfi i rath ins, cranial

369

Pericardium, formation of

.31, 35

Perineum, congenital defect of

. 563

formation of .

66

Peritonitis, foetal, as cause of monstrosities

. 163

Perocephalus Anommatus

408

Perocephalus Aprosopus

434

Perocormus Ecaudatus .

318

Peromely

323

Perosomus Elumbis

. 317

Peters' Early Ovum

. 20, 21

Pharyngeal Pouches

68

Pharynx, fistuhe .

452

formation of .

56

primitive, appearance

if

. 30, 34

Phimosis, congenital

559

Phocomelus

580, 581

Phylogenesis, in relation

to ontogenesis .

14

in teratology .

.

205

Pineal Body

, ,

55

Pinna, absence of .

. 436

formation of

53, 64, 66

malformations of

444-446

rudimentary

. 436

Pituitary Body' .

.

. 56, 71

Placenta Circimvallat.

L

. 591

Placenta Conica .

.

. 591

Placenta Fenestrata

• . .

. 591

Placenta, formation of

• • *

67

in quadruplets

645

in quintuplets

645

in triplets

,

644

malformations of

590, 591

Placenta Marginata

. 591

Placenta Memrranacea

. 591

Placenta, primary, formation of

. 23, 27

Placentitis as a Cause of Monstrosities

166

Plagiocephaly' ....

. 371

Plasmodium, characters of

63

formation of .

25

Plate, olfactory, appearance of

41

Platycephaly

. 371

Pleonotus ....

. 638

Pleuromelus

.

. 638

vol. ii. — 44

690

ANTENATAL PATHOLOGY AND HYGIENE

Pleuroprosoposchisis

Pleurosomatoschisis
Pleurosomus

PoDENCEPHALUS .

Polak Bodies
polyblepharon .
polycoria .
Polydactyly
Polymastia

associated malformations

comparative teratology

definition

heredity

historical notes

literature of

teratogencsis .

varieties
polyorchidy
polysomatous terata
Polyspermy
Polythelia
Porencephaly

Post-Generation, phenomena of .
Postmature Infant
Pouches, pharyngeal
Preauricular Appendages
"Predestination" in Heredity
Predispositions, heredity of
Pregnancy, congenital

extra-uterine, origin of
Prepuce, absence of
Pressure as Cause of Spina Bifida
Pressure, funic, in teratogenesis .

geminous, in teratogenesis
Pressure-Theories in Teratogenesis,
Pressure, uterine, in teratogenesis
Pro-embryon
Proencephalus
Prognathism, congenital .
Projection, coccygeal
Prolapsus Uteri, congenital
Prolapsus Uteri in Spina Bifida
Prolificacy, heredity of
Prosencephalon .
Prosoposchisis
Prosopo-Terata .
Prosopus Varus .
Protovertebr^e, formation of
Prozygosis
Pseudencephalus

PSEUD0-Ml( ' U< K EPHALUS

Pseudo-Hermaphroditism

Ptosis Congenita

Puberty, precocious

Pupil, malformations of

Pupillary Membrane, persistence of

Pulmonary Artery, anomalies of

transposition of
Pulmonary Veins, anomalies of
Pygo-Amorphus .
Pygomelus
Pygopagus
Pygo-Parasitus
Pygo-Teratoides .

varieties

223, 618

471

TAGE

. 374

516, 521

492, 514, 515

339

609, 612

412

415

588, 640

472, 638

474

474

472

475

472

472

474

473

561

623

25, 643

619, 6

43;

1

26

472

369

616

253

68

-442

658

651

638

618

559

302

150

145, 147

136

31, 142

24, 611

339

388

326

541

301

652

34, 43

374

173-449

388

32, 36

632

340, 344

367

560, 565, 624
413
254
415
416
499
501
502
640
640
635, 641
640
640

Quadruplets

. 236, 238, 645

INDEX OF SUBJECTS

691

Quadruplets — continued.
allantoido-angiopagous
Quintuplets

Rachicele .
Rachi-Tekata
Radius, absence of
Rathke's Pouch, formation of
Rearrangements, organogenetic
Recapitulation, law of .
Recesses, mandibular
Rectum, anomalous terminations

formation of .

stenosis of
Regeneration of Lost Parts
Repair, antenatal .
Reproduction of Amputated Parts
Retina, malformations of.
Retroflexion of the Spine
Rhachischisis
Rhinocephalus .
Rhinodyme
Rhombencephalon
Ribs, bicipital

cervical

defects of, causes of .

imperfect

malformations of

supernumerary
Robert, pelvis of .

Sacralisation of the Spine
Sacrum, absence of
Scaphocephaly .
Scai'Ula, elevation of, congenital

foramina in

malformations of
schistocephalus blfidus
Schistocephalus Fissilabrus
Schistocormus Exomphalus
schistocormus flssicollis
Schistocormus Fissisternalis
Schistocormus Fissiventralis
schistoglossia
Schistosomus
st'histosomus reflexus .
sciapody .
scillocephaly
Scoliosis, congenital
" Scottish Brothers " .
Segmentation, abnormal .
Segmentation of Ovum .
Semen, male, teratogenic cause
Septum, interauricular, anomalies of

interauricular, formation of

interventricular, anomalies of

interventricular, formation of

nasal, anomalies of
Septum Transvf.rsum
Septuplets
Sex, determination of

histological evidence of

origin of
Sexuality, meaning of
Sextuplets
Shoulder, dislocation of .

TAQE

645

236, 238, 645

286, 292, 294

272, 285-331

583, 584

47

68

13

. 387

. 564

57

. 563

195, 196

. 657

153, 195

. 417

312

285-309

397

233, 422

43, 55, 68, 70

470

461

14S

470

470

470

. 316

315

. 317

371

468

468

. 468

78, 379

384

. 523

460

491

515

387

515

313

266

371

311

635

615

610

95, 96

496

48, 58

498

48

420

35

646

2

23, 612

60

95, 97

620

238

,

586

692

ANTENATAL PATHOLOGY AND HYGIENE

Sinus, coccygeal
Sinus PkjECERVicalis

Sinus Venosus

SlRENOMELIA

Situs Inversus Viscerum
Situs Mutatus
somatopagus
"Spaltfuss"
"Spalthand"
Spek's Early Ovum
Sperm Cell, maturation of
Sperm, pathology of

physiology of .
Spermatogenesis .
Sphenocephaly .
Spherocephalus .
Spina Bifida, alleged cause of

antenatal cure of

anterior

avian .

cases of

complications of

definition of .

fluid in

literature of

localised

masked

morbid anatomy of

occulta

rarity in animals

resembling a tail

spinal cord in .

spine in

synonyms of .

teratogenesis of

treatment of .

varieties of
Spinal Cord

duplication of .

formation of .

malformations of
Spinal Nerves, formation of
Spine, anomalies .

retroflexion of .

sacralisation of
Spirits, evil, teratogenic causes
Spleen, absence of

formation of .

supernumerary

transposition of

Sl'LENUNCULI

Spondylolisthesis

Spondylolysis

Spondyloschizis Congenita

Spongioblasts in Spinal Cord

Stars, as teratogenic causes

Steatopyga

Stenosis, intestinal

Stenosis of Nares

Sterility of " Free Martins"

Sternalis, musculus, in ancnceplialus

Stkrno-Mastoid Muscle, absence <il

StERNOI'ACCS

Stbrnoschisis

Sternum, absence of

cyphosis of, cause of .

371

423

424,

425,

172, 1

76,

PAGE

832
41, 53
48, 58
569
594
594
635
586
586
27
609
649
648
609
425
433
140
290
293
301
179
299
285
298
307
290
288
292
288-290
301
322
298
292
285
302
305
286
64
330
42, 50, 55

329-331

43

329

173, 312, 519

. 315
.90

. 536
67

. 536
536, 595, 598

. 536

313

314

313

42

. 91, 99
313
532
421

. 624

. 347
466
636
48S
488
148

INDEX OF SUBJECTS

693

ephalus

Sternum — continued.

fissure of

foramina in

malformations of
Stomach, formation of

hour-glass constriction
Stomocephalus
Stkeptosomus
Strophocephalus
"Struggle foe Life," antenatal
Steuma Congenita
succenturiate placent
supernatural causes of
Supba- Anomalies
Sui'EAEenal Capsules, anomalies

formation of .
SUPRASTERNAL Ossicles
Sylvius, aqueduct of

aqueduct of, in hydroc

fossa of
svmblephaeon
Symelia

Sympathetic System
Sympodia, classification

clinical history of

definition of .

early stage of .

frequency of .

historical notes

literature of

mode of origin.

morbid anatomy of

specimen of

synonyms of .

tails in

teratogenesis of

uromelic

varieties
Syncephalus
Synchelia .
Syncytium, formation of

characters of .
Syndactyly
Synechia, buccal .
Synopsia .
Synokchidy
Synotus
Syringomyelia
Syeingo-Myelocele

Tail, absence of

embryonic
"Tails" .

adherent

false

free

literature of .

origin of

osseous

soft

teratogenesis of

true
Tapeisocephaly .
Taxonomy, teratological
Teeth, congenital .
Telegony .

Monstrosities

PAGE

488, 521

. 488

487-492

47

. 533

400

515

425

78

. 459

5

90, 591

82

219

. 536

58

488

44, 70

362

65

406

569

55

573

572

568

182

568

569

570

75, 216

572

87

569

325

575

383

573

0

32, 643

388

23, 25

39, 63

585

389

393

561

85, 429

, 430, 431, 432

■

. 330

. 297

. 317

53

. 319

322

321, 322

322, 324

. 326

. 208

323

322, 323, 324

. 325

. 322

. 371

225-239

391, 398

t

653

694

ANTENATAL PATHOLOGY AND HYGIENE

Telencephalon
Teleology and Teratology
Temperatube, change in, in teratogenesis
Tendencies, heredity of .
Terata, double

double, parasitic

double, and quadruplets

heterotaxic

inerosomatous .

inonosomatous .

pantosomatous

polysomatous .

quadruple

triple .
Teratogenesis of Absence of Clavicli

of absence of pectoral muscles

ofagnathus

of allantoido-angiopagous twins

amniotic disease in

of anencephalus

of aprosopus

of binovular twins

of cardiac anomalies .

of cebocephalus

of cephalocele .

of cervical auricles

of cervical cysts

of cervical fistuhe

clinical aspect of

of congenital elevation of scapula

of cyclopia

of cyclotia

of diaphragmatic hernia

of diphallus

of double-hand

of ectopia cordis

embryological theory .

of extroversion of bladder

experimental .

of fissure of sternum .

of fistula auris .

of gastroschisis

germinal factor in

of heterotaxy .

of imperforate oesophagus

of intestinal atresia

of intestinal diverticula

ofmicrocephalus

modern theories

nature of

nosological theory of .

of omphalocephaly

of pleurosomus

of Polydactyly

of polymastia .

of preauricular appendages

of sternal cyphosis

of sympodia

theories of

of umbilical hernia

i if uniovular twins

of united twins
Teratology, clasification of

comparative

general principles

hybridity and .

SI, 1

34, 43, 55, 56, 65
134,

623,

05, 129

235,
235,
235,
235,
235, 237,

630,

l'AGK

, 70
228
215
651
632
637
645
237
237
236
237
623
645
644
468
476
430
631
167
348
435
625
507
405
358
456
458
454
220
469
401
433
485
557
588
491
203
546
-222
489
443
522
222

601-605, 614
464
533
531
368
129
6
191
371
493
589
474
441
488-
575

158, 20:;, 606
527
625
643
-239
230
72
103

193,

10

66,

58,

INDEX OF SUBJECTS

695

T e ratology — continued.
nomenclature of
the pathology of the embryo
types of
Teratoma of Face
Teratomata

in spina bifida .

origin of

sacral .

umbilical
Teratoscopy, art of
Testicles, malformations of
Thalamencephalon
Thelyblast

Theories, teratogen etic, past
Thlipsencephalus
Thoracic Defects, causes of
Thoracic Duct, anomalies of
Thoraco-Acephaltjs
Thoracomeltjs
Thoraco-Omphalo-Schisis
Thoracopagus
Thoraco-Paracephalus .
Thoraco-Parasitus
Thorax, malformations of.

muscular anomalies of.
Thumb, absence of .

deformity of
Thymus Gland, formation of

malformations of
Thyroglossal Duct
Thyroid, cysts of the
Thyroid Gland, formation of
Tim a, absence of .

double . ...

Tight-Lacing in Teratogen esis
Tofts, Maria, of Godlyman
Tongue, bifid

ectopia of, tracheal

fissure of

formation of .
Tongue-Tie
Tongue, trifid

unilateral hypertrophy of
Toxic Substances, as teratogenic agents
Toxines, as teratogenic agents
Trachea, anomalies of
Trachelo-Parasitus
Tracheloschisis .
Trachelo-Terata
Tragus, anomalies of

supernumerary
Transition-Organism
Transposition of Aorta .
Transposition of Pulmonary Artery
Transposition of the Viscera
Trapezius, absence of
Traumatism, as a teratogenic agent

as cause of spina bifida

as a cause of monstrosities
Treatment, antenatal, general principles
Tricephalus
Trichterbrust

cause of
Triencephalus
Trigonocephalus .

l'AGE

234

5

240, 241

449

638, 639

300

217, 618, 621

321

538

88

561

34, 43, 70

612

82

340

148

503

638

638

516

636, 637, 643

638

638

467-512

476

586

178

66

511

56, 455

459

47, 57

582

588

132, 135, 136, 139
114
387
460
387
34, 47, 56
389
387
269
218
217
511
638
460
450
446
438
61
501
501
594
477
211, 213
302
129, 136
657
644
487
148
433
371, 405

606

ANTENATAL PATHOLOGY AND HYGIENE

Triocephalus
Triorchidy
Triphalangy
Triple Monsters .
Teiplets .

allantoido-angiopagous

iniited .
Teipod Fgetus

TrOCHOCEPHALY
Teophoblast, formation of

functions of

pathology of .
Trophoneurosis, autocopic
Tubercle, Darwinian

genital .

genital, formation of .
Tuberculosis, heredity of
Tumours, as causes of monstrositie

congenital, classification of

in spina bifida .

umbilical

vitelline
Twix, amorphous .
Twin Foetus with Sympodia
Twins, acephalic .

allantoido-angiopagous
definition of.
literature of .
teratogenesis of
varieties of .

dichorionic

giantism and .

hereditary

homologous

identical malformations in

monochorionic

origin of

paracephalic .

pathology of .

pressure of, in teratogenesis

uniovular
Twins, united

definition

specimens

teratogenesis of

varieties
Twins, varieties of
Types, teratological

Ulna, absence of .

double .
Umbilical Artery, single
Umbilical Cord, anomalies of
Umbilical Vesicle

anomalies of .
Umbilicus, posterior
Uraujus, cyst of .

fistula .

persistence of .
Ureter, supernumerary
Ureters, low insertion of.

malformations of
Urethra, anomalies of

atresia of

double .

phimosis

l'Ai.K.

216, 424, 426, 433
. 561
. 589

644
236, 238, 643, 644

644

644
88, 639
. 371
. 21, 23
. 611

614

154

445
64
58

651
. 145

238
. 300

538

. 538

628, 629

182
. 627

623, 624
625

. 626
630
626

624, 625
. 254

652

. 624

624

623, 624, 625

77

626

78

145, 147

623, 624, 625

. 623

632

633, 634, 637, 639

643

632

235, 236, 237

240, 241

. 584
. 587
. 592
591, 592
24, 26, 30, 33, 39, 63
614
. 327
550-
. 550
207, 549
551, 553
. 553
553
558-561
559
551, 55S
559

INDEX OF SUBJECTS

697

Urinary Umbilical Fistula

LTro-Anthropos

Uromelus ....

Uterus, anomalies of

VAGINA, anomalies of

Valvular Anomalies of Heart

Varnishing, as a teratogenic agent

Vascular Area, anomalies of

Vascular Changes, as cause of spina bifid

Vein, umbilical, anomalies of

Veins, early embryonic

cardinal, anomalies of .

omphalo-mesenteric

pulmonary, anomalies of

vitelline
Velamentous Insertion of Cord
Vena Cava, anomalies of .
Vena Cava Inferior, anomalies of
Ventricle, third, formation of .
Ventricles, cardiac, anomalies of

lateral, formation of .
. Verteurje, decrease in number

fusion of neighbouring

half ....

increase in number
Vertex Coccygeus
Vertex, cranial
Vesicle, blastodermic

chorionic

umbilical

umbilical, anomalies of
Vesicles, cerebral, appearance of .

optic, appearance of .
Vessels, great, anomalies of
Villi, chorionic, formation of
Viscera, inversion of

transposition of
Vitelline Duct .

persistent
Vitelline Prolap.se
Vitelline Tumours
Vitelline Vessels, persistence of
Vitreous, malformations of
Vulva, double
Vulvar Anus

"Wildermuth's Ear
Wolffian Bodies, formation of .
"Wolffian Ducts .
Wormian Bones .

Xenia ....
Xiphopagus

Yolk-Sac ....
Yolk-Stalk

30.

PAGE

. 549

319

573

562, 563

. 563

. 503

211, 214

603, 614

305
. 592
. 32, 49
. 538
. 32, 49
. 502
. 32, 49

592
502, 503

538
44

498

43

. 314

315

. 311

. 314

. 328

328, 329

610
52
63

614

35, 43, 69

34, 41, 45, 54

. 502

23, 25, 28, 63

. 594

. 594

. 39, 57

. 530

. 531

. 538

. 593

. 419

. 566

564

. 445

48, 51, 58, 67

. 48, 66

370

653
636

63
33, 39, 48, 57

Printed by

Morrison & Gibb Limited

Edinburgh

W 0 R K S

BY

J. W. BALLANTYNE, M.D., F.R.C.P.E., F.R.S.E.,

LECTURER ON MIDWIFERY AND DISEASES OF WOMEN, SCHOOL OF THE ROYAL COLLEGES

(SURGEONS' HALL), EDINBURGH, AND MEDICAL COLLEGE FOR WOMEN, EDINBURGH ;

EXAMINER IN MIDWIFERY IN THE UNIVERSITY OF EDINBURGH ;

PHYSICIAN TO THE ROYAL MATERNITY AND SIMPSON MEMORIAL HOSPITAL ;

HONORARY FELLOW OF THE AMERICAN ASSOCIATION OF OBSTETRICIANS AND GYNECOLOGISTS,

AND OF THE GLASGOW OBSTETRICAL AND GYNECOLOGICAL SOCIETY ;

FORMERLY LECTURER ON ANTENATAL PATHOLOGY AND TERATOLOGY IN THE

UNIVERSITY OF EDINBURGH.

MANUAL OP

ANTENATAL PATHOLOGY & HYGIENE

In Two Volumes. Price 21s. each. Profusely Illustrated with
23 Coloured Lithographs and 150 Engravings.

REVIEWS (OF VOLUME I.).

" The author's reputation as an original worker and authority upon diseases of the
foetus is an international one, and it is a matter for congratulation that such an im-
portant work upon this subject should be from the pen of a British writer. . . . The
present volume is a work of the most fascinating interest. . . . There are few of us who
really recognise the possibilities that lie in a further study of this subject." — Lancet
(October 1902).

" Rarely can it be said of a prolific writer that he has never penned one single note
that was needless or of little worth, yet we fancy that such may truly be said of Dr.
Ballantyne, in respect of his publications relating to foetal disease. . . . The first
volume, on 'The Fietus,' is published in an attractive form, and has the merit, rare in
medico-scientific publications, of being practically original." — British Medical Journal
(March 1902).

" Ballantyne's book is perhaps the most important medical work which has ap-
peared during the past year ... an original work of great merit and of extreme
practical importance." — Medical Press (July 1902).

" No one who begins reading the volume before us will stop doing so until he ter-
minates the work, and then he will sigh for the companion volume which the author
promises us will be forthcoming." — St. Louis Medical and Surgical Journal (July 1902).

"Antenatal pathology is preventive medicine in its most hopeful aspect, yet it is
practically unexplored. . . . The work evidently represents years of research. In
many particulars it is a most original work. There is much of practical value, much to
stimulate thought, and much toward the solution of the riddle of antenatal death,
disease, and deformity." — American Journal of Obstetrics (July 1902).

" The volume teems with facts and problems of the deepest significance and widest
interest." — Pediatrics (September 1902).

"In a book, which of its kind is without parallel in medical literature, Ballantyne,
the eminent English scientist, has compiled all that is known about the normal and
abnormal development of the ovum, and the physiology and pathology of the fo;tus.
His classical work culminates in the idea of recognising and, if possible, preventing
diseases of the child in utero — a high aim well in accord with the dominating trend of
preventive hygiene of modern medicine." — Interstate Medical Journal (January 1903).

"The author of this book has already become widely and justly known by the
large amount of original investigation performed by him and by his numerous writings,
chiefly on the subjects connected with foetal pathology. . . . He is also the originator
of the idea of a prematernity hospital, which is fast coming into favour, and is un-
doubtedly bound to have a place in connection with lying-in hospitals in the mar
future. . . . Much of value might be quoted from this most interesting and instructive
book, and it has been a pleasure to read it for review. No equally satisfactory book on
the subject has ever been produced, and it is a cause for congratulation that the writer
is English, and that the book is published in the English language." — Boston Medical
and Surgical Journal (April 1903).

W. GREEN &, SONS, EDINBURGH AND LONDON.

TERATOLOGIA:

A QUARTERLY JOURNAL OF ANTENATAL

PATHOLOGY.

Two Volumes (1894-1895) bound in one, with all the Indexes.
Nearly 600 pages. Thirty Plates. Contains Articles on
Monstrosities and Foetal Diseases, by Professors Osler and
J. C. Webster, Drs. Fere (Paris), Bidone (Florence),
Moncorvo (Brazil), Oiimann-Dumesnil (St. Louis), John
Thomson, W. Fokdyce, and Baxter Tyuie, and by the
Editor.

Price 21s. nett.

REVIEWS.

" As scholarly a production as anyone need wish to see." — Provincial Medical
Journal.

" The importance of the subject with which it is concerned is manifest.
Much may be learned from failures in every sphere of nature, physical and
mental. The debris of a workshop may be instructive. This periodical, how-
ever, devotes its attention also to congenital diseases, and thus has opened up a
large field for practical usefulness. Not the least valuable portion of the volume
is that which gives an abstract of current literature. The editor's work in this
sphere of research is well known, and it augurs well for the success of the work
that so capable a man has undertaken its supervision." — Lancet.

" The abstracts form an important and most valuable portion of the journal .-
We must reserve a special word of praise for the admirable ' Analytical Index.'"
— British Gynaecological Journal.

W. GREEN & SONS, EDINBURGH.

RECENTLY PUBLISHED.

ESSENTIALS OF OBSTETRICS.

Tins work contains the essentials of Obstetrics, so far as these can be
set forth in print, without the aid of demonstration by specimens,
models, diagrams, and photographs, such as is given in and belongs
to the Lecture Room.

251 pages. Price 5s.

W. GREEN & SONS, EDINBURGH.

AN INTRODUCTION TO THE
DISEASES OF INFANCY:

THE ANATOMY, PHYSIOLOGY, AND HYGIENE OF
THE NEW-BORN INFANT.

Demy 8uo, price 10s. 6d., with Coloured and other Illustrations.

REVIEWS.

" This is a work of very high order of merit, and is, as far as we are aware,
the only serious attempt in this country to deal solely with the normal anatomy
and physiology of the human infant. The author has largely availed himself of
the method of frozen sections in the anatomical portion, and supports his
arguments with numerous coloured illustrations." — The Practitioner.

" This is a most interesting and well-written book, which gives a rational
conception of the differences which exist between disease as it occurs in the
infant and is met with in the adult. Disease, aside from a certain moiety which
is truly peculiar to infancy, is in children modified by the anatomical and
physiological conditions then in existence, and it is to a close and in many
places an original study of these conditions that the author has devoted himself."
— American Journal of Obstetrics.

" It will be seen that the scope of the book is large. It treats of most
important and essential subjects, which are far too often overlooked. One is
too tempted to think of infants as merely little men and women, and to forget
that the differences are not only in quantity but also in kind. We can warmly
recommend Dr. Ballantyne's work." — Dublin Journal of Medical Science.

" The idea of the book is, to some extent, novel and original. We have
books on applied anatomy of the adult, but we have not many books, and these
not very accessible, on the anatomy of the child, and this is the first systematic
attempt, of which we are aware, to apply the facts of anatomy and physiology,
peculiar to infancy, ' to the elucidation of the phenomena of disease in early life,
and to found on them the rules which ought to guide us in the feeding and
rearing of infants.'" — Australian Medical Journal.

OLIVER & BOYD, Tweeddale Court, High Street, EDINBURGH.

DISEASES AND DEFORMITIES
"OF THE FCETUS:

AN ATTEMPT TOWARDS A SYSTEM OF
ANTENATAL PATHOLOGY.

In Two Volumes. Price 12s. 6d. each. Pages 265 and 275.

20 Plates.

These Volumes contain a historical sketch of the Subject of Foetal
Pathology, and an account of many interesting Congenital Dis-
eases of the Skin and Subcutaneous Tissue.

REVIEWS.

" This is a most timely publication. . . . The author has avoided all strictly
theoretical deductions, stating and grouping his facts most carefully, and draw-
ing conclusions solely from data established or confirmed by himself." — Prac-
titioner.

" We cordially recommend Dr. Ballantyne's work as one which will prove of
real value as well as interest to future workers in various branches of pathology
and medicine, and one which is a credit not only to the author, but also to the
Medical School to which he belongs." — Edinburgh Medical Journal.

" Altogether this work is to be welcomed as a masterly one, destined to in-
fluence profoundly and advance the study of antenatal disease and death."—
Medical Chronicle.

OLIVER & BOYD, Tweeddale Court, High Street, EDINBURGH.

BY THE SAME AUTHOR, in association with the late Dr. J. D.WILLIAMS.

THE STRUCTURES IN THE
MESOSALPINX:

THEIR NORMAL AND PATHOLOGICAL ANATOMY.
Demy 8uo. 51 pages. 12 Illustrations. Price 2s. 6d.

REVIEWS.

" Drs. Ballantyne and Williams are to be congratulated on having produced
this little book, which is a mine of information on the subjects of which it
treats." — Lancet.

" In glancing over a work like this, what strikes us is the patient and careful
method of examining each specimen ; that nothing is guessed at or copied from
other works to fill in and form padding ; and that only things dissected out, or"
made evident microscopically, are described. Hence the great intrinsic value of
such work." — Bvrmingha/ni Medical Rerun-.

" Contains the results of the examination of the mesosalpinx and the struc-
tures lying in it from two hundred and twenty broad ligaments. ... A valuable
contribution to our literature of the subject." — Boston Medical <</<<l Surgical
Journal.

OLIVER & BOYD, Tweeddale Court, High Street, EDINBURGH.

UNIVERSITY OF TORONTO
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