to have seen the union of all the teeth, making a continuous mass. In the "Musee de l'ecole dentaire de Paris" there are several milk-teeth, both of the superior and inferior maxilla, which are fused together. Bloch cites a case in which there were two rows of teeth in the superior maxilla. Hellwig has observed three rows of teeth, and the Ephemerides contain an account of a similar anomaly.
Extraoral Dentition.--Probably the most curious anomaly of teeth is that in which they are found in other than normal positions. Albinus speaks of teeth in the nose and orbit; Borellus, in the palate; Fabricius Hildanus, under the tongue; Schenck, from the palate; and there are many similar modern records. Heister in 1743 wrote a dissertation on extraoral teeth. The following is a recent quotation: --
"In the Norsk Magazin fur Laegevidenskaben, January, 1895, it is reported that Dr. Dave, at a meeting of the Medical Society in Christiania, showed a tooth removed from the nose of a woman aged fifty-three. The patient had consulted him for ear-trouble, and the tooth was found accidentally during the routine examination. It was easily removed, having been situated in a small depression at the junction of the floor and external wall of the nasal cavity, 22 mm. from the external nares. This patient had all her teeth; they were placed somewhat far from each other. The tooth resembled a milk canine; the end of the imperfect root was covered with a fold of mucous membrane, with stratified epithelium. The speaker suggested that part of the mucous membrane of the mouth with its tooth-germ had become impacted between the superior and premaxillary bones and thus cut off from the cavity of the mouth. Another speaker criticised this fetal dislocation and believed it to be due to an inversion--a development in the wrong direction--by which the tooth had grown upward into the nose. The same speaker also pointed out that the stratified epithelium of the mucous membrane did not prove a connection with the cavity of the mouth, as it is known that cylindric epithelium-cells after irritative processes are replaced by flat ones."
Delpech saw a young man in 1829 who had an opening in the palatine vault occasioned by the extraction of a tooth. This opening communicated with the nasal fossa by a fracture of the palatine and maxillary bones; the employment of an obturator was necessary. It is not rare to see teeth, generally canine, make their eruption from the vault of the palate; and these teeth are not generally supernumerary, but examples of vice and deviation of position. Fanton-Touvet, however, gives an example of a supernumerary tooth implanted in the palatine arch. Branch a describes a little negro boy who had two large teeth in the nose; his dentition was otherwise normal, but a portion of the nose was destroyed by ulceration. Roy describes a Hindoo lad of fourteen who had a tooth in the nose, supposed to have been a tumor. It was of the canine type, and was covered with enamel to the junction with the root, which was deeply imbedded in the side and upper part of the antrum. The boy had a perfect set of permanent teeth and no deformity, swelling, or cystic formation of the jaw. This was clearly a case of extrafollicular development and eruption of the tooth in an anomalous position, the peculiarity being that while in other similar cases the crown of the tooth shows itself at the floor of the nasal cavity from below upward, in this instance the dental follicle was transposed, the eruption being from above downward. Hall cites an instance in which the right upper canine of a girl erupted in the nose. The subject showed marked evidence of hereditary syphilis. Carver describes a child who had a tooth growing from the lower right eyelid. The number of deciduous teeth was perfect; although this tooth was canine it had a somewhat bulbulous fang.
Of anomalies of the head the first to be considered will be the anencephalous monsters who, strange to say, have been known to survive birth. Clericus cites an example of life for five days in a child without a cerebrum. Heysham records the birth of a child without a cerebrum and remarks that it was kept alive for six days. There was a child born alive in Italy in 1831 without a brain or a cerebellum--in fact, no cranial cavity--and yet it lived eleven hours. A somewhat similar case is recorded in the last century. In the Philosophical Transactions there is mentioned a child virtually born without a head who lived four days; and Le Duc records a case of a child born without brain, cerebellum, or medulla oblongata, and who lived half an hour. Brunet describes an anencephalous boy born at term who survived his birth. Saviard delivered an anencephalous child at term which died in thirty-six hours. Lawrence mentions a child with brain and cranium deficient that lived five days. Putnam speaks of a female nosencephalous monster that lived twenty-nine hours. Angell and Elsner in March, 1895, reported a case of anencephaly, or rather pseudencephaly, associated with double divergent strabismus and limbs in a state of constant spastic contraction. The infant lived eight days. Geoffroy-Saint-Hilaire cites an example of anencephaly which lived a quarter of an hour. Fauvel mentioned one that lived two hours, and Sue describes a similar instance in which life persisted for seven hours and distinct motions were noticed. Malacarne saw life in one for twelve hours, and Mery has given a description of a child born without brain that lived almost a full day and took nourishment. In the Hotel-Dieu in Paris in 1812 Serres saw a monster of this type which lived three days, and was fed on milk and sugared water, as no nurse could be found who was willing to suckle it.
Fraser mentions a brother and sister, aged twenty and thirty, respectively, who from birth had exhibited signs of defective development of the cerebellum. They lacked power of coordination and walked with a drunken, staggering gait; they could not touch the nose with the finger when their eyes were shut, etc. The parents of these unfortunate persons were perfectly healthy, as were the rest of their family. Cruveilhier cites a case of a girl of eleven who had absolutely no cerebellum, with the same symptoms which are characteristic in such cases. There is also recorded the history of a man who was deficient in the corpus callosum; at the age of sixty-two, though of feeble intelligence, he presented no signs of nervous disorder. Claude Bernard made an autopsy on a woman who had no trace of olfactory lobes, and after a minute inquiry into her life he found that her sense of smell had been good despite her deficiency.
Buhring relates the history of a case somewhat analogous to viability of anencephalous monsters. It was a bicephalous child that lived thirty-two hours after he had ligated one of its heads.
{footnote} The argument that the brain is not the sole organ of the mind is in a measure substantiated by a wonderful case of a decapitated rooster, reported from Michigan. A stroke of the knife bad severed the larynx and removed the whole mass of the cerebrum, leaving the inner aspect and base of the skull exposed. The cerebrum was partly removed; the external auditory meatus was preserved. Immediately after the decapitation the rooster was left to its supposed death struggles, but it ran headless to the barn, where it was secured and subsequently fed by pushing corn down its esophagus, and allowing water to trickle into this tube from the spout of an oil-can. The phenomena exhibited by the rooster were quite interesting. It made all the motions of pecking, strutted about, flapped its wings, attempted to crow, but, of course, without making any sound. It exhibited no signs of incoordination, but did not seem to hear. A ludicrous exhibition was the absurd, sidelong pas seul made toward the hens.
Ward mentions an instance of congenital absence of the corpora callosum. Paget and Henry mention cases in which the corpora callosum, the fornix, and septum lucidum were imperfectly formed. Maunoir reports congenital malformation of the brain, consisting of almost complete absence of the occipital lobe. The patient died at the twenty-eighth month. Combettes reports the case of a girl who died at the age of eleven who had complete absence of the cerebellum in addition to other minor structural defects; this was probably the case mentioned by Cruveilhier.
Diminution in volume of the head is called microcephaly. Probably the most remarkable case on record is that mentioned by Lombroso. The individual was called "l'homme-oiseau," or the human bird, and his cranial capacity was only 390 c.c. Lombroso speaks of another individual called "l'homme-lapin," or man-rabbit, whose cranium was only slightly larger than that of the other, measuring 490 mm. in circumference. Castelli alludes to endemic microcephaly among some of the peoples of Asia. We also find it in the Caribbean Islands, and from the skulls and portraits of the ancient Aztecs we are led to believe that they were also microcephalic.
Two creatures of celebrity were Maximo and Bartola, who for twenty-five years have been shown in America and in Europe under the name of the "Aztecs" or the "Aztec children". They were male and female and very short, with heads resembling closely the bas-reliefs on the ancient Aztec temples of Mexico. Their facial angle was about 45 degrees, and they had jutting lips and little or no chin. They wore their hair in an enormous bunch to magnify the deformity. These curiosities were born in Central America and were possibly half Indian and Negro. They were little better than idiots in point of intelligence.
Figure 92 represents a microcephalic youth known as the "Mexican wild boy," who was shown with the Wallace circus.
Virchow exhibited a girl of fourteen whose face was no larger than that of a new-born child, and whose head was scarcely as large as a man's fist. Magitot reported a case of a microcephalic woman of thirty who weighed 70 pounds.
Hippocrates and Strabonius both speak of head-binding as a custom inducing artificial microcephaly, and some tribes of North American Indians still retain this custom.
As a rule, microcephaly is attended with associate idiocy and arrested development of the rest of the body. Ossification of the fontanelles in a mature infant would necessarily prevent full development of the brain. Osiander and others have noticed this anomaly. There are cases on record in which the fontanelles have remained open until adulthood.
Augmentation of the volume of the head is called macrocephaly, and there are a number of curious examples related. Benvenuti describes an individual, otherwise well formed, whose head began to enlarge at seven. At twenty-seven it measured over 37 inches in circumference and the man's face was 15 inches in height; no other portion of his body increased abnormally; his voice was normal and he was very intelligent. He died of apoplexy at the age of thirty.
Fournier speaks of a cranium in the cabinet of the Natural History Museum of Marseilles of a man by the name of Borghini, who died in 1616. At the time he was described he was fifty years old, four feet in height; his head measured three feet in circumference and one foot in height. There was a proverb in Marseilles, "Apas mai de sen que Borghini," meaning in the local dialect, "Thou hast no more wit than Borghini." This man, whose fame became known all over France, was not able, as he grew older, to maintain the weight of his head, but carried a cushion on each shoulder to prop it up. Fournier also quotes the history of a man who died in the same city in 1807 at the age of sixty-seven. His head was enormous, and he never lay on a bed for thirty years, passing his nights in a chair, generally reading or writing. He only ate once in twenty-four or thirty hours, never warmed himself, and never used warm water. His knowledge was said to have been great and encyclopedic, and he pretended never to have heard the proverb of Borghini. There is related the account of a Moor, who was seen in Tunis early in this century, thirty-one years of age, of middle height, with a head so prodigious in dimensions that crowds flocked after him in the streets. His nose was quite long, and his mouth so large that he could eat a melon as others would an apple. He was an imbecile. William Thomas Andrews was a dwarf seventeen years old, whose head measured in circumference 35 inches; from one external auditory meatus to another, 27 1/4 inches; from the chin over the cranial summit to the suboccipital protuberance, 37 1/2 inches; the distance from the chin to the pubes was 20 inches; and from the pubes to the soles of the feet, 16; he was a monorchid. James Cardinal, who died in Guy's Hospital in 1825, and who was so celebrated for the size of his head, only measured 32 1/2 inches in head-circumference.
The largest healthy brains on record, that is, of men of prominence, are those of Cuvier, weighing 64 1/3 ounces; of Daniel Webster, weighing 63 3/4 ounces (the circumference of whose head was 23 3/4 inches); of Abercrombie, weighing 63 ounces, and of Spurzheim, weighing 55 1/16 ounces. Byron and Cromwell had abnormally heavy brains, showing marked evidence of disease.
A curious instance in this connection is that quoted by Pigne, who gives an account of a double brain found in an infant. Keen reports finding a fornix which, instead of being solid from side to side, consisted of two lateral halves with a triangular space between them.
When the augmentation of the volume of the cranium is caused by an abundant quantity of serous fluid the anomaly is known as hydrocephaly. In this condition there is usually no change in the size of the brain-structure itself, but often the cranial bones are rent far asunder. Minot speaks of a hydrocephalic infant whose head measured 27 1/2 inches in circumference; Bright describes one whose head measured 32 inches; and Klein, one 43 inches. Figure 93 represents a child of six whose head circumference was 36 inches. Figure 94 shows a hydrocephalic adult who was exhibited through this country.
There is a record of a curious monster born of healthy half-caste African parents. The deformity was caused by a deficiency of osseous material of the bones of the head. There was considerable arrest of development of the parietal, temporal, and superior maxillary bones, in consequence of which a very small amount of the cerebral substance could be protected by the membranous expansion of the cranial centers. The inferior maxilla and the frontal bone were both perfect; the ears were well developed and the tongue strong and active; the nostrils were imperforate and there was no roof to the mouth nor floor to the nares. The eyes were curiously free from eyelashes, eyelids, or brows. The cornea threatened to slough. There was double harelip on the left side; the second and third fingers of both hands were webbed for their whole length; the right foot wanted the distal phalanx of the great toe and the left foot was clubbed and drawn inward. The child swallowed when fed from a spoon, appeared to hear, but exhibited no sense of light. It died shortly after the accompanying sketch was made.
Occasionally a deficiency in the osseous material of the cranium or an abnormal dilatation of the fontanelles gives rise to a hernia of the meninges, which, if accompanied by cerebrospinal fluid in any quantity, causes a large and peculiarly shaped tumor called meningocele. If there is a protrusion of brain-substance itself, a condition known as hernia cerebri results.
Complete absence of the inferior maxilla is much rarer in man than in animals. Nicolas and Prenant have described a curious case of this anomaly in a sheep. Gurlt has named subjects presenting the total or partial absence of the inferior maxilla, agnathes or hemiagnathes. Simple atrophy of the inferior maxilla has been seen in man as well as in the lower animals, but is much less frequent than atrophy of the superior maxilla. Langenbeck reports the case of a young man who had the inferior maxilla so atrophied that in infancy it was impossible for him to take milk from the breast. He had also almost complete immobility of the jaws. Boullard reports a deformity of the visage, resulting in a deficiency of the condyles of the lower jaw. Maurice made an observation on a vice of conformation of the lower jaw which rendered lactation impossible, probably causing the death of the infant on this account. Tomes gives a description of a lower jaw the development of the left ramus of which had been arrested. Canton mentions arrest of development of the left perpendicular ramus of the lower jaw combined with malformation of the external ear.
Exaggerated prominence of the maxillaries is called prognathism; that of the superior maxilla is seen in the North American Indians. Inferior prognathism is observed in man as well as in animals. The bull-dog, for example, displays this, but in this instance the deformity is really superior brachygnathism, the superior maxilla being arrested in development.
Congenital absence of the nose is a very rare anomaly. Maisonneuve has seen an example in an individual in which, in place of the nasal appendix, there was a plane surface perforated by two small openings a little less than one mm. in diameter and three mm. apart.
Exaggeration in volume of the nose is quite frequent. Ballonius speaks of a nose six times larger than ordinary. Viewing the Roman celebrities, we find that Numa, to whom was given the surname Pompilius, had a nose which measured six inches. Plutarch, Lyourgus, and Solon had a similar enlargement, as had all the kings of Italy except Tarquin the Superb.
Early in the last century a man, Thomas Wedders (or Wadhouse), with a nose 7 1/2 inches long, was exhibited throughout Yorkshire. This man expired as he had lived, in a condition of mind best described as the most abject idiocy. The accompanying illustration is taken from a reproduction of an old print and is supposed to be a true likeness of this unfortunate individual.
There are curious pathologic formations about the nose which increase its volume so enormously as to interfere with respiration and even with alimentation; but these will be spoken of in another chapter.
There have been some celebrities whose noses were undersized. The Duc de Guise, the Dauphin d'Auvergne, and William of Orange, celebrated in the romances of chivalry, had extremely short noses.
There are a few recorded cases of congenital division of the nose. Bartholinus, Borellus, and the Ephemerides speak of duplex noses. Thomas of Tours has observed congenital fissure of the nose. Rikere reports the case of an infant of three weeks who possessed a supernumerary nose on the right nasal bone near the inner canthus of the eye. It was pear-shaped, with its base down, and was the size of the natural nose of an infant of that age, and air passed through it. Hubbell, Ronaldson, and Luscha speak of congenital occlusion of the posterior nares. Smith and Jarvis record cases of congenital occlusion of the anterior nares.
Anomalies in size of the mouth are not uncommon. Fournier quotes the history of a man who had a mouth so large that when he opened it all his back teeth could be seen. There is a history of a boy of seventeen who had a preternaturally-sized mouth, the transverse diameter being 6 1/2 inches. The mother claimed that the boy was born with his foot in his mouth and to this fact attributed his deformity. The negro races are noted for their large mouths and thick lips. A negro called "Black Diamond," recently exhibited in Philadelphia, could put both his fists in his mouth.
Morgan reports two cases of congenital macrostoma accompanied by malformation of the auricles and by auricular appendages. Van Duyse mentions congenital macrostoma with preauricular tumors and a dermoid of the eye. Macrostoma is sometimes produced by lateral fissures. In other cases this malformation is unilateral and the fissure ascends, in which instance the fissure may be accompanied by a fistula of the duct of Stensen. Sometimes there is associated with these anomalies curious terminations of the salivary ducts, either through the cheek by means of a fistula or on the anterior part of the neck.
Microstoma.--There are a few cases on record in which the mouth has been so small or ill-defined as not to admit of alimentation. Molliere knew an individual of forty whose mouth was the exact size of a ten-centime piece.
Buchnerus records a case of congenital atresia of the mouth. Cayley, Smith, Sourrouille, and Stankiewiez of Warsaw discuss atresia of the mouth. Cancrum oris, scarlet fever, burns, scurvy, etc., are occasional causes that have been mentioned, the atresia in these instances taking place at any time of life.
Anomalies of the Lips.--The aboriginal tribes are particularly noted for their large and thick lips, some of which people consider enormous lips signs of adornment. Elephantiasis or other pathologic hypertrophy of the labial tissues can produce revolting deformity, such as is seen in Figure 100, representing an individual who was exhibited several years ago in Philadelphia. We have in English the expression, "pulling a long lip." Its origin is said to date back to a semimythical hero of King Arthur's time, who, "when sad at heart and melancholic," would let one of his lips drop below his waist, while he turned the other up like a cap on his head.
Blot records a case of monstrous congenital hypertrophy of the superior lip in an infant of eight months. Buck successfully treated by surgical operations a case of congenital hypertrophy of the under lip, and Detmold mentions a similar result in a young lady with hypertrophy of the lip and lower part of the nose. Murray reports an undescribed malformation of the lower lip occurring in one family.
Hare-lip may be unilateral or double, and may or may not include the palatine arch. In the worst cases it extends in fissures on both sides to the orbit. In other cases the minimum degree of this deformity is seen.
Congenital absence of the tongue does not necessarily make speech, taste, or deglutition impossible. Jussieu cites the case of a girl who was born without a tongue but who spoke very distinctly. Berdot describes a case in which the tongue was deficient, without apparent disturbance of any of the functions. Riolan mentions speech after loss of the tongue from small-pox.
Boddington gives an account of Margaret Cutting, who spoke readily and intelligibly, although she had lost her tongue. Saulquin has an observation of a girl without a tongue who spoke, sang, and swallowed normally. Aurran, Bartholinus, Louis, Parsons, Tulpius, and others mention speech without the presence of a tongue.
Philib reports a case in which mutism, almost simulating that of one congenitally deaf, was due to congenital adhesions of the tongue to the floor of the buccal cavity. Speech was established after removal of the abnormal adhesion. Routier speaks of ankylosis of the tongue of seventeen years' duration.
Jurist records such abnormal mobility of the tongue that the patient was able to project the tongue into the nasopharynx. Wherry and Winslow record similar instances.
There have been individuals with bifid tongues, after the normal type of serpents and saurians, and others who possessed a supernumerary tongue. Rev. Henry Wharton, Chaplain to Archbishop Sancroft, in his journal, written in the seventeenth century, says that he was born with two tongues and passed through life so, one, however, gradually atrophying. In the polyclinic of Schnitzer in Vienna in 1892 Hajek observed in a lad of twelve an accessory tongue 2.4 cm. in length and eight mm. in breadth, forming a tumor at the base of the normal tongue. It was removed by scissors, and on histologic examination proved to be a true tongue with the typical tissues and constituents. Borellus, Ephemerides, Eschenbach, Mortimer, Penada, and Schenck speak of double tongues, and Avicenna and Schenck have seen fissured tongues. Dolaeus records an instance of double tongue in a paper entitled "De puella bilingui," and Beaudry and Brothers speak of cleft tongue. Braine records a case in which there was a large hypertrophied fold of membrane coming from each side of the upper lip.
In some cases there is marked augmentation of the volume of the tongue. Fournier has seen a juggler with a tongue so long that he could extrude it six inches from his mouth. He also refers to a woman in Berlin with a long tongue, but it was thinner than that of a cat. When she laughed it hung over her teeth like a curtain, and was always extremely cold to the touch. In the same article there is a description of a man with a very long neck who could touch his tongue to his chest without reclining his head. Congenital and acquired hypertrophy of the tongue will be discussed later.
Amatus Lusitanus and Portal refer to the presence of hair on the tongue, and later there was an account of a medical student who complained of dyspepsia and a sticky sensation in the mouth. On examination a considerable growth of hair was found on the surface of the tongue. The hairs would be detached in vomiting but would grow again, and when he was last seen they were one inch long. Such are possibly nevoid in formation.
The ordinary anomalies of the palate are the fissures, unilateral, bilateral, median, etc.: they are generally associated with hare-lip. The median fissure commencing between the middle incisors is quite rare.
Many curious forms of obturator or artificial palate are employed to remedy congenital defects. Sercombe mentions a case in which destruction of the entire palate was successfully relieved by mechanical means. In some instances among the lower classes these obturators are simple pieces of wood, so fashioned as to fit into the palatine cleft, and not infrequently the obturator has been swallowed, causing obstruction of the air-passages or occluding the esophagus.
Abnormalism of the Uvula.--Examples of double uvula are found in the older writers, and Hagendorn speaks of a man who was born without a uvula. The Ephemerides and Salmuth describe uvulae so defective as to be hardly noticeable. Bolster, Delius, Hodges, Mackenzie of Baltimore, Orr, Riedel, Schufeldt, and Tidyman are among observers reporting bifurcated and double uvula, and they are quite common. Ogle records instances of congenital absence of the uvula.
Anomalies of the Epiglottis.--Morgagni mentions a man without an epiglottis who ate and spoke without difficulty. He thought the arytenoids were so strongly developed that they replaced the functions of the missing organ. Enos of Brooklyn in 1854 reported absence of the epiglottis without interference with deglutition. Manifold speaks of a case of bifurcated epiglottis. Debloisi records an instance of congenital web of the vocal bands. Mackenzie removed a congenital papillomatous web which had united the vocal cords until the age of twenty-three, thus establishing the voice. Poore also recorded a case of congenital web in the larynx. Elsberg and Scheff mention occlusion of the rima glottidis by a membrane.
Instances of duplication of the epiglottis attended with a species of double voice possess great interest. French described a man of thirty, by occupation a singer and contortionist, who became possessed of an extra voice when he was sixteen. In high and falsetto tones he could run the scale from A to F in an upper and lower range. The compass of the low voice was so small that he could not reach the high notes of any song with it, and in singing he only used it to break in on the falsetto and produce a sensation. He was supposed to possess a double epiglottis.
Roe describes a young lady who could whistle at will with the lower part of her throat and without the aid of her lips. Laryngeal examination showed that the fundamental tones were produced by vibrations of the edges of the vocal cords, and the modifications were effected by a minute adjustment of the ventricular bands, which regulated the laryngeal opening above the cord, and pressing firmly down closed the ventricle and acted as a damper preventing the vibrations of the cords except in their middle third. Morgan in the same journal mentions the case of a boy of nineteen, who seemed to be affected with laryngeal catarrh, and who exhibited distinct diphthongia. He was seen to have two glottic orifices with associate bands. The treatment was directed to the catarrh and consequent paresis of the posterior bands, and he soon lost his evidences of double voice.
{footnote} The following is a description of the laryngeal formation of a singer who has recently acquired considerable notice by her ability to sing notes of the highest tones and to display the greatest compass of voice. It is extracted from a Cleveland, Ohio, newspaper: "She has unusual development of the larynx, which enables her to throw into vibration and with different degrees of rapidity the entire length of the vocal cords or only a part thereof. But of greatest interest is her remarkable control over the muscles which regulate the division and modification of the resonant cavities, the laryngeal, pharyngeal, oral, and nasal, and upon this depends the quality of her voice. The uvula is bifurcated, and the two divisions sometimes act independently. The epiglottis during the production of the highest notes rises upward and backward against the posterior pharyngeal wall in such a way as almost entirely to separate the pharyngeal cavities, at the same time that it gives an unusual conformation to those resonant chambers."
Complete absence of the eyes is a very rare anomaly. Wordsworth describes a baby of seven weeks, otherwise well formed and healthy, which had congenital absence of both eyes. The parents of this child were in every respect healthy. There are some cases of monstrosities with closed, adherent eyelids and absence of eyes. Holmes reports a case of congenital absence of both eyes, the child otherwise being strong and perfect. The child died of cholera infantum. He also reports a case very similar in a female child of American parents. In a girl of eight, of German parents, he reports deficiency of the external walls of each orbit, in addition to great deformity of the side of the head. He also gives an instance of congenital paralysis of the levator palpebrae muscles in a child whose vision was perfect and who was otherwise perfect. Holmes also reports a case of enormous congenital exophthalmos, in which the right eye protruded from the orbit and was no longer covered by the cornea. Kinney has an account of a child born without eyeballs. The delivery was normal, and there was no history of any maternal impression; the child was otherwise healthy and well formed.
Landes reports the case of an infant in which both eyes were absent. There were six fingers on each hand and six toes on each foot. The child lived a few weeks. In some instances of supposed absence of the eyeball the eye is present but diminutive and in the posterior portion of the orbit. There are instances of a single orbit with no eyes and also a single orbit containing two eyes. Again we may have two orbits with an absence of eyes but the presence of the lacrimal glands, or the eyes may be present or very imperfectly developed. Mackenzie mentions cases in which the orbit was more or less completely wanting and a mass of cellular tissue in each eye.
Cases of living cyclopia, or individuals with one eye in the center of the forehead after the manner of the mythical Cyclops, are quite rare. Vallentini in 1884 reports a case of a male cyclopic infant which lived for seventy-three hours. There were median fissures of the upper lip, preauricular appendages, oral deformity, and absence of the olfactory proboscis The fetus was therefore a cyclops arrhynchus, or cyclocephalus. Blok describes a new-born infant which lived for six or seven hours, having but one eye and an extremely small mouth.
The "Four-eyed Man of Cricklade" was a celebrated English monstrosity of whom little reliable information is obtainable. He was visited by W. Drury, who is accredited with reporting the following--
" 'So wondrous a thing, such a lusus naturae, such a scorn and spite of nature I have never seen. It was a dreadful and shocking sight.' This unfortunate had four eyes placed in pairs, 'one eye above the other and all four of a dull brown, encircled with red, the pupils enormously large.' The vision in each organ appeared to be perfect. 'He could shut any particular eye, the other three remaining open, or, indeed, as many as he chose, each several eye seeming to be controlled by his will and acting independently of the remainder. He could also revolve each eye separately in its orbit, looking backward with one and forward with another, upward with one and downward with another simultaneously.' He was of a savage, malignant disposition, delighting in ugly tricks, teasing children, torturing helpless animals, uttering profane and blasphemous words, and acting altogether like the monster, mental and physical, that he was. 'He could play the fiddle, though in a silly sort, having his notes on the left side, while closing the right pair of eyes. He also sang, but in a rough, screeching voice not to be listened to without disgust.'"
There is a recent report of a child born in Paris with its eyes in the top of its head. The infant seemed to be doing well and crowds of people have flocked to see it. Recent reports speak of a child born in Portland, Oregon, which had a median rudimentary eye between two normal eyes. Fournier describes an infant born with perfectly formed eyes, but with adherent eyelids and closed ocular aperture. Forlenze has seen the pupils adherent to the conjunctiva, and by dissection has given sight to the subject.
Dubois cites an instance of supernumerary eyelid. At the external angle of the eyelid was a fold of conjunctiva which extended 0.5 cm. in front of the conjunctiva, to which it did not adhere, therefore constituting a fourth eyelid. Fano presents a similar case in a child of four months, in whom no other anomaly, either of organs or of vision, was observed. On the right side, in front of the external half of the sclerotic, was observed a semilunar fold with the concavity inward, and which projected much more when the lower lid was depressed. When the eyelid rolled inward the fold rolled with the globe, but never reached so far as the circumference of the cornea and did not interfere with vision.
Total absence of both irides has been seen in a man of eighteen. Dixon reports a case of total aniridia with excellent sight in a woman of thirty-seven. In Guy's Hospital there was seen a case of complete congenital absence of the iris. Hentzschel speaks of a man with congenital absence of the iris who had five children, three of whom exhibited the same anomaly while the others were normal. Benson, Burnett, Demaux, Lawson, Morison, Reuling, Samelson, and others also report congenital deficiency of the irides in both eyes.
Jeaffreson describes a female of thirty, living in India, who was affected with complete ossification of the iris. It was immovable and quite beautiful when seen through the transparent cornea; the sight was only slightly impaired. No cause was traceable.
Multiple Pupils.--More than one pupil in the eye has often been noticed, and as many as six have been seen. They may be congenital or due to some pathologic disturbance after birth. Marcellus Donatus speaks of two pupils in one eye. Beer, Fritsche, and Heuermann are among the older writers who have noticed supernumerary pupils. Higgens in 1885 described a boy whose right iris was perforated by four pupils,--one above, one to the inner side, one below, and a fourth to the outer side. The first three were slit-shaped; the fourth was the largest and had the appearance as of the separation of the iris from its insertion. There were two pupils in the left eye, both to the outer side of the iris, one being slit-like and the other resembling the fourth pupil in the right eye. All six pupils commenced at the periphery, extended inward, and were of different sizes. The fundus could be clearly seen through all of the pupils, and there was no posterior staphyloma nor any choroidal changes. There was a rather high degree of myopia. This peculiarity was evidently congenital, and no traces of a central pupil nor marks of a past iritis could be found. Clinical Sketches a contains quite an extensive article on and several illustrations of congenital anomalies of the iris.
Double crystalline lenses are sometimes seen. Fritsch and Valisneri have seen this anomaly and there are modern references to it. Wordsworth presented to the Medical Society of London six members of one family, all of whom had congenital displacement of the crystalline lens outward and upward. The family consisted of a woman of fifty, two sons, thirty-five and thirty-seven, and three grandchildren--a girl of ten and boys of five and seven. The irides were tremulous.
Clark reports a case of congenital dislocation of both crystalline lenses. The lenses moved freely through the pupil into the anterior chambers. The condition remained unchanged for four years, when glaucoma supervened.
Differences in Color of the Two Eyes.--It is not uncommon to see people with different colored eyes. Anastasius I had one black eye and the other blue, from whence he derived his name "Dicore," by which this Emperor of the Orient was generally known. Two distinct colors have been seen in an iris. Berry gives a colored illustration of such a case.
The varieties of strabismus are so common that they will be passed without mention. Kuhn presents an exhaustive analysis of 73 cases of congenital defects of the movements of the eyes, considered clinically and didactically. Some or all of the muscles may be absent or two or more may be amalgamated, with anomalies of insertion, false, double, or degenerated, etc.
The influence of heredity in the causation of congenital defects of the eye is strikingly illustrated by De Beck. In three generations twelve members of one family had either coloboma iridis or irideremia. He performed two operations for the cure of cataract in two brothers. The operations were attended with difficulty in all four eyes and followed by cyclitis. The result was good in one eye of each patient, the eye most recently blind. Posey had a case of coloboma in the macular region in a patient who had a supernumerary tooth. He believes the defects were inherited, as the patient's mother also had a supernumerary tooth.
Nunnely reports cases of congenital malformation in three children of one family. The globes of two of them (a boy and a girl) were smaller than natural, and in the boy in addition were flattened by the action of the recti muscles and were soft; the sclera were very vascular and the cornea, conical, the irides dull, thin, and tremulous; the pupils were not in the axis of vision, but were to the nasal side. The elder sister had the same congenital condition, but to a lesser degree. The other boy in the family had a total absence of irides, but he could see fairly well with the left eye.
Anomalies of the Ears.--Bilateral absence of the external ears is quite rare, although there is a species of sheep, native of China, called the "Yungti," in which this anomaly is constant. Bartholinus, Lycosthenes, Pare, Schenck, and Oberteuffer have remarked on deficient external ears. Guys, the celebrated Marseilles litterateur of the eighteenth century, was born with only one ear. Chantreuil mentions obliteration of the external auditory canal in the new-born. Bannofont reports a case of congenital imperforation of the left auditory canal existing near the tympanic membrane with total deafness in that ear. Lloyd described a fetus showing absence of the external auditory meatus on both sides. Munro reports a case of congenital absence of the external auditory meatus of the right ear; and Richardson speaks of congenital malformation of the external auditory apparatus of the right side. There is an instance of absence of the auditory canal with but partial loss of hearing. Mussey reports several cases of congenitally deficient or absent aural appendages. One case was that in which there was congenital absence of the external auditory meatus of both ears without much impairment of hearing. In neither ear of N. W. Goddard, aged twenty-seven, of Vermont, reported in 1834, was there a vestige of an opening or passage in the external ear, and not even an indentation. The Eustachian tube was closed. The integuments of the face and scalp were capable of receiving acoustic impressions and of transmitting them to the organs of hearing. The authors know of a student of a prominent New York University who is congenitally deficient in external ears, yet his hearing is acute. He hides his deformity by wearing his hair long and combed over his ears.
The knowledge of anomalous auricles is lost in antiquity. Figure 103 represents the head of an aegipan in the British Museum showing a supernumerary auricle. As a rule, supernumerary auricles are preauricular appendages. Warner, in a report of the examination of 50,000 children, quoted by Ballantyne, describes 33 with supernumerary auricles, represented by sessile or pedunculated outgrowths in front of the tragus. They are more commonly unilateral, always congenital, and can be easily removed, giving rise to no unpleasant symptoms. They have a soft and elastic consistency, and are usually composed of a hyaline or reticular cartilaginous axis covered with connective or adipose tissue and skin bearing fine hairs; sometimes both cartilage and fat are absent. They are often associated with some form of defective audition--harelip, ocular disturbance, club-feet, congenital hernia, etc. These supernumerary members vary from one to five in number and are sometimes hereditary. Reverdin describes a man having a supernumerary nipple on the right side of his chest, of whose five children three had preauricular appendages. Figure 104 represents a girl with a supernumerary auricle in the neck, described in the Lancet, 1888. A little girl under Birkett's care in Guy's Hospital more than answered to Macbeth's requisition, "Had I three ears I'd hear thee!" since she possessed two superfluous ones at the sides of the neck, somewhat lower than the angle of the jaw, which were well developed as to their external contour and made up of fibrocartilage. There is mentioned the case of a boy of six months on the left side of whose neck, over the middle anterior border of the sternocleidomastoid muscle, was a nipple-like projection 1/2 inch in length; a rod of cartilage was prolonged into it from a thin plate, which was freely movable in the subcutaneous tissue, forming a striking analogue to an auricle. Moxhay cites the instance of a mother who was frightened by the sight of a boy with hideous contractions in the neck, and who gave birth to a child with two perfect ears and three rudimentary auricles on the right side, and on the left side two rudimentary auricles.
In some people there is an excessive development of the auricular muscles, enabling them to move their ears in a manner similar to that of the lower animals. Of the celebrated instances the Abbe de Marolles, says Vigneul-Marville, bears witness in his "Memoires" that the Regent Crassot could easily move his ears. Saint Augustine mentions this anomaly.
Double tympanitic membrane is spoken of by Loeseke. There is sometimes natural perforation of the tympanum in an otherwise perfect ear, which explains how some people can blow tobacco-smoke from the ear. Fournier has seen several Spaniards and Germans who could perform this feat, and knew one man who could smoke a whole cigar without losing any smoke, since he made it leave either by his mouth, his ears, or in both ways. Fournier in the same article mentions that he has seen a woman with ears over four inches long.
Strange to say, there have been reports of cases in which the ossicles were deficient without causing any imperfection of hearing. Caldani mentions a case with the incus and malleus deficient, and Scarpa and Torreau quote instances of deficient ossicles. Thomka in 1895 reported a case of supernumerary tympanic ossicle, the nature of which was unknown, although it was neither an inflammatory product nor a remnant of Meckel's cartilage.
Absence of the Limbs.--Those persons born without limbs are either the subjects of intrauterine amputation or of embryonic malformation. Probably the most celebrated of this class was Marc Cazotte, otherwise known as "Pepin," who died in Paris in the last century at the age of sixty-two of a chronic intestinal disorder. He had no arms, legs, or scrotum, but from very jutting shoulders on each side were well-formed hands. His abdomen ended in a flattened buttock with badly-formed feet attached. He was exhibited before the public and was celebrated for his dexterity. He performed nearly all the necessary actions, exhibited skilfulness in all his movements, and was credited with the ability of coitus. He was quite intellectual, being able to write in several languages. His skeleton is preserved in the Musee Dupuytren. Flachsland speaks of a woman who three times had borne children without arms and legs. Hastings describes a living child born without any traces of arms or legs. Garlick has seen a child with neither upper nor lower extremities. In place of them were short stumps three or four inches long, closely resembling the ordinary stumps after amputation. The head, chest, body, and male genitals were well formed, and the child survived. Hutchinson reports the history of a child born without extremities, probably the result of intrauterine amputation. The flaps were healed at the deltoid insertion and just below the groin. Pare says he saw in Paris a man without arms, who by means of his head and neck could crack a whip or hold an axe. He ate by means of his feet, dealt and played cards, and threw dice with the same members, exhibiting such dexterity that finally his companions refused to play with him. He was proved to be a thief and a murderer and was finally hanged at Gueldres. Pare also relates having seen a woman in Paris who sewed, embroidered, and did other things with her feet. Jansen speaks of a man in Spain, born without arms, who could use his feet as well as most people use their arms. Schenck and Lotichius give descriptions of armless people.
Hulke describes a child of four whose upper limbs were absent, a small dimple only being in their place. He had free movement of the shoulders in every direction. and could grasp objects between his cheeks and his acromian process; the prehensile power of the toes was well developed, as he could pick up a coin thrown to him. A monster of the same conformation was the celebrated painter, Ducornet, who was born at Lille on the 10th of January, 1806. He was completely deprived of arms, but the rest of the body was well formed with the exception of the feet, of which the second toe was faulty. The deformity of the feet, however, had the happiest result, as the space between the great toe and its neighbor was much larger than ordinary and the toes much more mobile. He became so skilful in his adopted profession that he finally painted a picture eleven feet in height (representing Mary Magdalene at the feet of Christ after the resurrection), which was purchased by the Government and given to the city of Lille. Broca describes James Leedgwood, who was deprived of his arms and had only one leg. He exhibited great dexterity with his single foot, wrote, discharged a pistol, etc.; he was said to have been able to pick up a sewing-needle on a slippery surface with his eyes blindfolded. Capitan described to the Societe d'anthropologie de Paris a young man without arms, who was said to play a violin and cornet with his feet. He was able to take a kerchief from his pocket and to blow his nose; he could make a cigarette, light it, and put it in his mouth, play cards, drink from a glass, and eat with a fork by the aid of his dexterous toes. There was a creature exhibited some time since in the principal cities of France, who was called the "l'homme tronc." He was totally deprived of all his members. Curran describes a Hindoo, a prostitute of forty, with congenital absence of both upper extremities. A slight fleshy protuberance depended from the cicatrix of the humerus and shoulder-joint of the left side, and until the age of ten there was one on the right side. She performed many tricks with her toes. Caldani speaks of a monster without arms, Davis mentions one, and Smith describes a boy of four with his upper limbs entirely absent. Breschet has seen a child of nine with only portions of the upper arms and deformity of lower extremities and pelvis. Pare says that he saw in Paris in 1573, at the gate of St. Andrew des Arts, a boy of nine, a native of a small village near Guise, who had no legs and whose left foot was represented by a fleshy body hanging from the trunk; he had but two fingers hanging on his right hand, and had between his legs what resembled a virile penis. Pare attributes this anomaly to a default in the quantity of semen.
The figure and skeleton of Harvey Leach, called "Hervio Nono," is in the museum of the University College in London. The pelvis was comparatively weak, the femurs hardly to be recognized, and the right tibia and foot defective; the left foot was better developed, although far from being in due proportion to the trunk above. He was one of the most remarkable gymnasts of his day, and notwithstanding the distortion of his lower limbs had marvelous power and agility in them. As an arena-horseman, either standing or sitting, he was scarcely excelled. He walked and even ran quite well, and his power of leaping, partly with his feet and partly with his hands, was unusual. His lower limbs were so short that, erect, he touched the floor with his fingers, but he earned his livelihood as much with his lower as with his upper limbs. In his skeleton his left lower limb, between the hip and heel, measured 16 inches, while the right, between the same points, measured nine inches. Hare mentions a boy of five and a half whose head and trunk were the same as in any other child of like age. He was 22 1/2 inches high, had no spinal curvature, but was absolutely devoid of lower extremities. The right arm was two inches long and the left 2 1/4. Each contained the head and a small adjoining portion of the humerus. The legs were represented by masses of cellular tissue and fat covered by skin which projected about an inch. He was intelligent, had a good memory, and exhibited considerable activity. He seemed to have had more than usual mobility and power of flexion of the lower lumbar region. When on his back he was unable to rise up, but resting on the lower part of the pelvis he was able to maintain himself erect. He usually picked up objects with his teeth, and could hold a coin in the axilla as he rolled from place to place. His rolling was accomplished by a peculiar twisting of the thorax and bending of the pelvis. There was no history of maternal impression during pregnancy, no injury, and no hereditary disposition to anomalous members. Figure 112 represents a boy with congenital deficiency of the lower extremities. who was exhibited a few years ago in Philadelphia. In Figure 113, which represents a similar case in a girl whose photograph is deposited in the Mutter Museum of the College of Physicians, Philadelphia, we see how cleverly the congenital defect may be remedied by mechanical contrivance. With her crutches and artificial legs this girl was said to have moved about easily.
Parvin describes a "turtle-man" as an ectromelian, almost entering the class of phocomelians or seal-like monsters; the former term signifies abortive or imperfect formation of the members. The hands and feet were normally developed, but the arms, forearms, and legs are much shortened.
The "turtle-woman" of Demerara was so called because her mother when pregnant was frightened by a turtle, and also from the child's fancied resemblance to a turtle. The femur was six inches long, the woman had a foot of six bones, four being toes, viz., the first and second phalanges of the first and second toes. She had an acetabulum, capsule, and ligamentum teres, but no tibia or fibula; she also had a defective right forearm. She was never the victim of rachitis or like disease, but died of syphilis in the Colonial Hospital. In her twenty-second year she was delivered of a full-grown child free of deformity.
There was a woman living in Bavaria, under the observation of Buhl, who had congenital absence of both femurs and both fibulas. Almost all the muscles of the thigh existed, and the main attachment to the pelvis was by a large capsular articulation. Charpentier gives the portrait of a woman in whom there was a uniform diminution in the size of the limbs. Debout portrays a young man with almost complete absence of the thigh and leg, from whose right hip there depended a foot. Accrell describes a peasant of twenty-six, born without a hip, thigh, or leg on the right side. The external genital organs were in their usual place, but there was only one testicle in the scrotum. The man was virile. The rectum instead of opening outward and underneath was deflected to the right.
Supernumerary Limbs.--Haller reports several cases of supernumerary extremities. Plancus speaks of an infant with a complete third leg, and Dumeril cites a similar instance. Geoffroy-Saint-Hilaire presented to the Academie des Sciences in 1830 a child with four legs and feet who was in good health. Amman saw a girl with a large thigh attached to her nates. Below the thigh was a single leg made by the fusion of two legs. No patella was found and the knee was anchylosed. One of the feet of the supernumerary limb had six toes, while the other, which was merely an outgrowth, had two toes on it.
According to Jules Guerin, the child named Gustav Evrard was born with a thigh ending in two legs and two imperfect feet depending from the left nates.
Tucker describes a baby born in the Sloane Maternity in New York, October 1, 1894, who had a third leg hanging from a bony and fleshy union attached to the dorsal spine. The supernumerary leg was well formed and had a left foot attached to it. Larkin and Jones mention the removal of a meningocele and a supernumerary limb from an infant of four months. This limb contained three fingers only, one of which did not have a bony skeleton.
Pare says that on the day the Venetians and the Genevois made peace a monster was born in Italy which had four legs of equal proportions, and besides had two supernumerary arms from the elbows of the normal limbs. This creature lived and was baptized.
Anomalies of the Feet.--Hatte has seen a woman who bore a child that had three feet. Bull gives a description of a female infant with the left foot double or cloven. There was only one heel, but the anterior portion consisted of an anterior and a posterior part. The anterior foot presented a great toe and four smaller ones, but deformed like an example of talipes equinovarus. Continuous with the outer edge of the anterior part and curving beneath it was a posterior part, looking not unlike a second foot, containing six well-formed toes situated directly beneath the other five. The eleven toes were all perfect and none of them were webbed.
There is a class of monsters called "Sirens" on account of their resemblance to the fabulous creatures of mythology of that name. Under the influence of compression exercised in the uterus during the early period of gestation fusion of the inferior extremities is effected. The accompanying illustration shows the appearance of these monsters, which are thought to resemble the enchantresses celebrated by Homer.
Anomalies of the Hand.--Blumenbach speaks of an officer who, having lost his right hand, was subsequently presented by his wife with infants of both sexes showing the same deformity. Murray cites the instance of a woman of thirty-eight, well developed, healthy, and the mother of normal children, who had a double hand. The left arm was abnormal, the flexion of the elbow imperfect, and the forearm terminated in a double hand with only rudimentary thumbs. In working as a charwoman she leaned on the back of the flexed carpus. The double hand could grasp firmly, though the maximum power was not so great as that of the right hand. Sensation was equally acute in all three of the hands. The middle and ring fingers of the supernumerary hand were webbed as far as the proximal joints, and the movements of this hand were stiff and imperfect. No single finger of the two hands could be extended while the other seven were flexed. Giraldes saw an infant in 1864 with somewhat the same deformity, but in which the disposition of the muscles and tendons permitted the ordinary movements.
Absence of Digits.--Maygrier describes a woman of twenty-four who instead of having a hand on each arm had only one finger, and each foot had but two toes. She was delivered of two female children in 1827 and one in 1829, each having exactly the same deformities. Her mother was perfectly formed, but the father had but one toe on his foot and one finger on his left hand.
Kohler gives photographs of quite a remarkable case of suppression and deformity of the digits of both the fingers and toes.
Figure 123 shows a man who was recently exhibited in Philadelphia. He had but two fingers on each hand and two toes on each foot, and resembles Kohler's case in the anomalous digital conformation.
Figure 124 represents an exhibitionist with congenital suppression of four digits on each hand.
Tubby has seen a boy of three in whom the first, second, and third toes of each foot were suppressed, the great toe and the little toe being so overgrown that they could be opposed. In this family for four generations 15 individuals out of 22 presented this defect of the lower extremity. The patient's brothers and a sister had exactly the same deformity, which has been called "lobster-claw foot."
Falla of Jedburgh speaks of an infant who was born without forearms or hands; at the elbow there was a single finger attached by a thin string of tissue. This was the sixth child, and it presented no other deformity. Falla also says that instances of intrauterine digital amputation are occasionally seen.
According to Annandale, supernumerary digits may be classified as follows:--
(1) A deficient organ, loosely attached by a narrow pedicle to the hand or foot (or to another digit).
(2) A more or less developed organ, free at its extremity, and articulating with the head or sides of a metacarpal, metatarsal, or phalangeal bone.
(3) A fully developed separate digit.
(4) A digit intimately united along its whole length with another digit, and having either an additional metacarpal or metatarsal bone of its own, or articulating with the head of one which is common to it and another digit.
Superstitions relative to supernumerary fingers have long been prevalent. In the days of the ancient Chaldeans it was for those of royal birth especially that divinations relative to extra digits were cast. Among the ancients we also occasionally see illustrations emblematic of wisdom in an individual with many fingers, or rather double hands, on each arm.
Hutchinson, in his comments on a short-limbed, polydactylous dwarf which was dissected by Ruysch, the celebrated Amsterdam anatomist, writes as follows.--
"This quaint figure is copied from Theodore Kerckring's 'Spicilegium Anatomicum,' published in Amsterdam in 1670. The description states that the body was that of an infant found drowned in the river on October 16, 1668. It was dissected by the renowned Ruysch. A detailed description of the skeleton is given. My reason for now reproducing the plate is that it offers an important item of evidence in reference to the development of short-limbed dwarfs. Although we must not place too much reliance on the accuracy of the draughtsman, since he has figured some superfluous lumbar vertebrae, yet there can be no doubt that the limbs are much too short for the trunk and head. This remark especially applies to the lower limbs and pelvis. These are exactly like those of the Norwich dwarf and of the skeleton in the Heidelberg Museum which I described in a recent number of the 'Archives.' The point of extreme interest in the present case is that this dwarfing of the limbs is associated with polydactylism. Both the hands have seven digits. The right foot has eight and the left nine. The conditions are not exactly symmetrical, since in some instances a metacarpal or metatarsal bone is wanting; or, to put it otherwise, two are welded together. It will be seen that the upper extremities are so short that the tips of the digits will only just touch the iliac crests.
"This occurrence of short limbs with polydactylism seems to prove conclusively that the condition may be due to a modification of development of a totally different nature from rickets. It is probable that the infant was not at full term. Among the points which the author has noticed in his description are that the fontanelle was double its usual size; that the orbits were somewhat deformed; that the two halves of the lower jaw were already united; and that the ribs were short and badly formed. He also, of course, draws attention to the shortness of the limbs, the stoutness of the long bones, and the supernumerary digits. I find no statement that the skeleton was deposited in any museum, but it is very possible that it is still in existence in Amsterdam, and if so it is very desirable that it should be more exactly described,"
In Figure 126, A represents division of thumb after Guyot-Daubes, shows a typical case of supernumerary fingers, and C pictures Morand's case of duplication of several toes.
Forster gives a sketch of a hand with nine fingers and a foot with nine toes. Voight records an instance of 13 fingers on each hand and 12 toes on each foot. Saviard saw an infant at the Hotel-Dieu in Paris in 1687 which had 40 digits, ten on each member. Annandale relates the history of a woman who had six fingers and two thumbs on each hand, and another who had eight toes on one foot.
Meckel tells of a case in which a man had 12 fingers and 12 toes, all well formed, and whose children and grandchildren inherited the deformity. Mason has seen nine toes on the left foot. There is recorded the account of a child who had 12 toes and six fingers on each hand, one fractured. Braid describes talipes varus in a child of a few months who had ten toes. There is also on record a collection of cases of from seven to ten fingers on each hand and from seven to ten toes on each foot. Scherer gives an illustration of a female infant, otherwise normally formed, with seven fingers on each hand, all united and bearing claw-like nails. On each foot there was a double halux and five other digits, some of which were webbed.
The influence of heredity on this anomaly is well demonstrated. Reaumur was one of the first to prove this, as shown by the Kelleia family of Malta, and there have been many corroboratory instances reported; it is shown to last for three, four, and even five generations; intermarriage with normal persons finally eradicates it.
It is particularly in places where consanguineous marriages are prevalent that supernumerary digits persist in a family. The family of Foldi in the tribe of Hyabites living in Arabia are very numerous and confine their marriages to their tribe. They all have 24 digits, and infants born with the normal number are sacrificed as being the offspring of adultery. The inhabitants of the village of Eycaux in France, at the end of the last century, had nearly all supernumerary digits either on the hands or feet. Being isolated in an inaccessible and mountainous region, they had for many years intermarried and thus perpetuated the anomaly. Communication being opened, they emigrated or married strangers and the sexdigitism vanished. Maupertuis recalls the history of a family living in Berlin whose members had 24 digits for many generations. One of them being presented with a normal infant refused to acknowledge it. There is an instance in the Western United States in which supernumerary digits have lasted through five generations. Cameron speaks of two children in the same family who were polydactylic, though not having the same number of supernumerary fingers.
Smith and Norwell report the case of a boy of fifteen both of whose hands showed webbing of the middle and ring fingers and accessory nodules of bone between the metacarpals, and six toes on each foot. The boy's father showed similar malformations, and in five generations 21 out of 28 individuals were thus malformed, ten females and 11 males. The deformity was especially transmitted in the female line.
Instances of supernumerary thumbs are cited by Panaroli, Ephemerides, Munconys, as well as in numerous journals since. This anomaly is not confined to man alone; apes, dogs, and other lower animals possess it. Bucephalus, the celebrated horse of Alexander, and the horse of Caesar were said to have been cloven-hoofed.
Hypertrophy of the digits is the result of many different processes, and true hypertrophy or gigantism must be differentiated from acromegaly, elephantiasis, leontiasis, and arthritis deformans, for which distinction the reader is referred to an article by Park. Park also calls attention to the difference between acquired gigantism, particularly of the finger and toes, and another condition of congenital gigantism, in which either after or before birth there is a relatively disproportionate, sometimes enormous, overgrowth of perhaps one finger or two, perhaps of a limited portion of a hand or foot, or possibly of a part of one of the limbs. The best collection of this kind of specimens is in the College of Surgeons in London.
Curling quotes a most peculiar instance of hypertrophy of the fingers in a sickly girl. The middle and ring fingers of the right hand were of unusual size, the middle finger measuring 5 1/2 inches in length four inches in circumference. On the left hand the thumb and middle fingers were hypertrophied and the index finger was as long as the middle one of the right hand. The middle finger had a lateral curvature outward, due to a displacement of the extensor tendon. This affection resembled acromegaly. Curling cites similar cases, one in a Spanish gentleman, Governor of Luzon, in the Philippine Islands, in 1850, who had an extraordinary middle finger, which he concealed by carrying it in the breast of his coat.
Hutchinson exhibited a photograph showing the absence of the radius and thumb, with shortening of the forearm. Conditions more or less approaching this had occurred in several members of the same family. In some they were associated with defects of development in the lower extremities also.
The varieties of club-foot--talipes varus, valgus, equinus, equino-varus, etc.--are so well known that they will be passed with mention only of a few persons who have been noted for their activity despite their deformity. Tyrtee, Parini, Byron, and Scott are among the poets who were club-footed; some writers say that Shakespeare suffered in a slight degree from this deformity. Agesilas, Genserie, Robert II, Duke of Normandy, Henry II, Emperor of the West, Otto II, Duke of Brunswick, Charles II, King of Naples, and Tamerlane were victims of deformed feet. Mlle. Valliere, the mistress of Louis XIV, was supposed to have both club-foot and hip-disease. Genu valgum and genu varum are ordinary deformities and quite common in all classes.
Transpositions of the character of the vertebrae are sometimes seen. In man the lumbar vertebrae have sometimes assumed the character of the sacral vertebrae, the sacral vertebrae presenting the aspect of lumbar vertebrae, etc. It is quite common to see the first lumbar vertebra presenting certain characteristics of the dorsal.
Numerical anomalies of the vertebrae are quite common, generally in the lumbar and dorsal regions, being quite rare in the cervical, although there have been instances of six or eight cervical vertebrae. In the lower animals the vertebrae are prolonged into a tail, which, however, is sometimes absent, particularly when hereditary influence exists. It has been noticed in the class of dogs whose tails are habitually amputated to improve their appearance that the tail gradually decreases in length. Some breeders deny this fact.
Human Tails.--The prolongation of the coccyx sometimes takes the shape of a caudal extremity in man. Broca and others claim that the sacrum and the coccyx represent the normal tail of man, but examples are not infrequent in which there has been a fleshy or bony tail appended to the coccygeal region. Traditions of tailed men are old and widespread, and tailed races were supposed to reside in almost every country. There was at one time an ancient belief that all Cornishmen had tails, and certain men of Kent were said to have been afflicted with tails in retribution for their insults to Thomas a Becket. Struys, a Dutch traveler in Formosa in the seventeenth century, describes a wild man caught and tied for execution who had a tail more than a foot long, which was covered with red hair like that of a cow.
The Niam Niams of Central Africa are reported to have tails smooth and hairy and from two to ten inches long. Hubsch of Constantinople remarks that both men and women of this tribe have tails. Carpus, or Berengarius Carpensis, as he is called, in one of his Commentaries said that there were some people in Hibernia with long tails, but whether they were fleshy or cartilaginous could not be known, as the people could not be approached. Certain supposed tailed races which have been described by sea-captains and voyagers are really only examples of people who wear artificial appendages about the waists, such as palm-leaves and hair. A certain Wesleyan missionary, George Brown, in 1876 spoke of a formal breeding of a tailed race in Kali, off the coast of New Britain. Tailless children were slain at once, as they would be exposed to public ridicule. The tailed men of Borneo are people afflicted with hereditary malformation analogous to sexdigitism. A tailed race of princes have ruled Rajoopootana, and are fond of their ancestral mark. There are fabulous stories told of canoes in the East Indies which have holes in their benches made for the tails of the rowers. At one time in the East the presence of tails was taken as a sign of brute force.
There was reported from Caracas the discovery of a tribe of Indians in Paraguay who were provided with tails. The narrative reads somewhat after this manner: One day a number of workmen belonging to Tacura Tuyn while engaged in cutting grass had their mules attacked by some Guayacuyan Indians. The workmen pursued the Indians but only succeeded in capturing a boy of eight. He was taken to the house of Senor Francisco Galeochoa at Posedas, and was there discovered to have a tail ten inches long. On interrogation the boy stated that he had a brother who had a tail as long as his own, and that all the tribe had tails.
Aetius, Bartholinus, Falk, Harvey, Kolping, Hesse, Paulinus, Strauss, and Wolff give descriptions of tails. Blanchard says he saw a tail fully a span in length: and there is a description in 1690 of a man by the name of Emanuel Konig, a son of a doctor of laws who had a tail half a span long, which grew directly downward from the coccyx and was coiled on the perineum, causing much discomfort. Jacob describes a pouch of skin resembling a tail which hung from the tip of the coccyx to the length of six inches. It was removed and was found to be thicker than the thumb, consisted of distinctly jointed portions with synovial capsules. Gosselin saw at his clinic a caudal appendix in an infant which measured about ten cm. Lissner says that in 1872 he assisted in the delivery of a young girl who had a tail consisting of a coccyx prolonged and covered with skin, and in 1884 he saw the same girl, at this time the tail measuring nearly 13 cm.
Virchow received for examination a tail three inches long amputated from a boy of eight weeks. Ornstein, chief physician of the Greek army, describes a Greek of twenty-six who had a hairless, conical tail, free only at the tip, two inches long and containing three vertebrae. He also remarks that other instances have been observed in recruits. Thirk of Broussa in 1820 described the tail of a Kurd of twenty-two which contained four vertebrae. Belinovski gives an account of a hip-joint amputation and extirpation of a fatty caudal extremity, the only one he had ever observed.
Before the Berlin Anthropological Society there were presented two adult male Papuans, in good health and spirits, who had been brought from New Guinea; their coccygeal bones projected 1 1/2 inches. Oliver Wendell Holmes in the Atlantic Monthly, June, 1890, says that he saw in London a photograph of a boy with a considerable tail. The "Moi Boy" was a lad of twelve, who was found in Cochin China, with a tail a foot long which was simply a mass of flesh. Miller tells of a West Point student who had an elongation of the coccyx, forming a protuberance which bulged very visibly under the skin. Exercise at the riding school always gave him great distress, and the protuberance would often chafe until the skin was broken, the blood trickling into his boots.
Bartels presents a very complete article in which he describes 21 persons born with tails, most of the tails being merely fleshy protuberances. Darwin speaks of a person with a fleshy tail and refers to a French article on human tails.
Science contains a description of a negro child born near Louisville, eight weeks old, with a pedunculated tail 2 1/2 inches long, with a base 1 1/4 inches in circumference. The tail resembled in shape a pig's tail and had grown 1/4 inch since birth. It showed no signs of cartilage or bone, and had its origin from a point slightly to the left of the median line and about an inch above the end of the spinal column.
Dickinson recently reported the birth of a child with a tail. It was a well-developed female between 5 1/2 and six pounds in weight. The coccyx was covered with the skin on both the anterior and posterior surfaces. It thus formed a tail of the size of the nail of the little finger, with a length of nearly 3/16 inch on the inner surface and 3/8 inch on the rear surface. This little tip could be raised from the body and it slowly sank back.
In addition to the familiar caudal projection of the human fetus, Dickinson mentions a group of other vestigial remains of a former state of things. Briefly these are:--
(1) The plica semilunaris as a vestige of the nictitating membrane of certain birds.
(2) The pointed ear, or the turned-down tip of the ears of many men.
(3) The atrophied muscles, such as those that move the ear, that are well developed in certain people, or that shift the scalp, resembling the action of a horse in ridding itself of flies.
(4) The supracondyloid foremen of the humerus.
(5) The vermiform appendix.
(6) The location and direction of the hair on the trunk and limbs.
(7) The dwindling wisdom-teeth.
(8) The feet of the fetus strongly deflected inward, as in the apes, and persisting in the early months of life, together with great mobility and a distinct projection of the great toe at an angle from the side of the foot.
(9) The remarkable grasping power of the hand at birth and for a few weeks thereafter, that permits young babies to suspend their whole weight on a cane for a period varying from half a minute to two minutes.
Horrocks ascribes to these anal tags a pathologic importance. He claims that they may be productive of fistula in ano, superficial ulcerations, fecal concretions, fissure in ano, and that they may hypertrophy and set up tenesmus and other troubles. The presence of human tails has given rise to discussion between friends and opponents of the Darwinian theory. By some it is considered a reversion to the lower species, while others deny this and claim it to be simply a pathologic appendix.
Anomalies of the Spinal Canal and Contents.--When there is a default in the spinal column, the vice of conformation is called spina bifida. This is of two classes: first, a simple opening in the vertebral canal, and, second, a large cleft sufficient to allow the egress of spinal membranes and substance. Figure 130 represents a large congenital sacral tumor.
Achard speaks of partial duplication of the central canal of the spinal cord. De Cecco reports a singular case of duplication of the lumbar segment of the spinal cord. Wagner speaks of duplication of a portion of the spinal cord.
Foot records a case of amyelia, or absence of the spinal cord, in a fetus with hernia cerebri and complete fissure of the spinal column. Nicoll and Arnold describe an anencephalous fetus with absence of spinal marrow; and Smith also records the birth of an amyelitic fetus.
In some persons there are exaggerated curvatures of the spine. The first of these curvatures is called kyphosis, in which the curvature is posterior; second, lordosis, in which the curvature is anterior; third, scoliosis, in which it is lateral, to the right or left.
Kyphosis is the most common of the deviations in man and is most often found in the dorsal region, although it may be in the lumbar region. Congenital kyphosis is very rare in man, is generally seen in monsters, and when it does exist is usually accompanied by lordosis or spine bifida. We sometimes observe a condition of anterior curvature of the lumbar and sacral regions, which might be taken for a congenital lordosis, but this is really a deformity produced after birth by the physiologic weight of the body. Figure 131 represents a case of lordosis caused by paralysis of the spinal muscles.
Analogous to this is what the accoucheurs call spondylolisthesis. Scoliosis may be a cervicodorsal, dorsolumbar, or lumbosacral curve, and the inclination of the vertebral column may be to the right or left. The pathologists divide scoliosis into a myopathic variety, in which the trouble is a physiologic antagonism of the muscles; or osteopathic, ordinarily associated with rachitis, which latter variety is generally accountable for congenital scoliosis. In some cases the diameter of the chest is shortened to an almost incredible degree, but may yet be compatible with life. Glover speaks of an extraordinary deformity of the chest with lateral curvature of the spine, in which the diameter from the pit of the stomach to the spinal integument was only 5 1/2 inches.
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