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.

Supernumerary ribs are not at all uncommon in man, nearly every medical

museum having some examples. Cervical ribs are not rare. Gordon

describes a young man of seventeen in whom there was a pair of

supernumerary ribs attached to the cervical vertebrae. Bernhardt

mentions an instance in which cervical ribs caused motor and sensory

disturbances. Dumerin of Lyons showed an infant of eight days which had

an arrested development of the 2d, 3d, 4th, and 5th ribs. Cases of

deficient ribs are occasionally met. Wistar in 1818 gives an account

of a person in whom one side of the thorax was at rest while the other

performed the movements of breathing in the usual manner.

In some cases we see fissure of the sternum, caused either by deficient

union or absence of one of its constituent parts. In the most

exaggerated cases these fissures permit the exit of the heart, and as a

general rule ectopies of the heart are thus caused. Pavy has given a

most remarkable case of sternal fissure in a young man of twenty-five,

a native of Hamburg. He exhibited himself in one medical clinic after

another all over Europe, and was always viewed with the greatest

interest. In the median line, corresponding to the absence of sternum,

was a longitudinal groove bounded on either side by a continuous hard

ridge which articulated with the costal cartilages. The skin passed

naturally over the chest from one side to another, but was raised at

one part of the groove by a pulsatile swelling which occupied the

position of the right auricle. The clavicle and the two margins of the

sternum had no connections whatever, and below the groove was a hard

substance corresponding to the ensiform cartilage, which, however, was

very elastic, and allowed the patient, under the influence of the

pectoral muscles, when the upper extremity was fixed, to open the

groove to nearly the extent of three inches, which was more than twice

its natural width. By approximating his arms he made the ends of his

clavicles overlap. When he coughed, the right lung suddenly protruded

from the chest through the groove and ascended a considerable distance

above the clavicle into the neck. Between the clavicles another

pulsatile swelling was easily felt but hardly seen, which was doubtless

the arch of the aorta, as by putting the fingers on it one could feel a

double shock, synchronous with distention and recoil of a vessel or

opening and closing of the semilunar valves.

Madden pictures (Figs. 134 and 135) a Swede of forty with congenital

absence of osseous structure in the middle line of the sternum, leaving

a fissure 5 3/8 X 1 3/16 X 2 inches, the longest diameter being

vertical. Madden also mentions several analogous instances on record.

Groux's case was in a person of forty-five, and the fissure had the

vertical length of four inches. Hodgen of St. Louis reports a case in

which there was exstrophy of the heart through the fissure. Slocum

reports the occurrence of a sternal fissure 3 X 1 1/2 inches in an

Irishman of twenty-five. Madden also cites the case of Abbott in an

adult negress and a mother. Obermeier mentions several cases. Gibson

and Malet describe a presternal fissure uncovering the base of the

heart. Ziemssen, Wrany, and Williams also record congenital fissures

of the sternum.

Thomson has collected 86 cases of thoracic defects and summarizes his

paper by saying that the structures deficient are generally the hair in

the mammary and axillary regions, the subcutaneous fat over the

muscles, nipples, and breasts, the pectorals and adjacent muscles, the

costal cartilages and anterior ends of ribs, the hand and forearm; he

also adds that there may be a hernia of the lung, not hereditary, but

probably due to the pressure of the arm against the chest. De Marque

gives a curious instance in which the chin and chest were congenitally

fastened together. Muirhead cites an instance in which a firm, broad

strip of cartilage resembling sternomastoid extended from below the

left ear to the left upper corner of the sternum, being entirely

separate from the jaw.

Some preliminary knowledge of embryology is essential to understand the

formation of branchial fissures, and we refer the reader to any of the

standard works on embryology for this information. Dzondi was one of

the first to recognize and classify congenital fistulas of the neck.

The proper classification is into lateral and median fissures. In a

case studied by Fevrier the exploration of a lateral pharyngeal fistula

produced by the introduction of the sound violent reflex phenomena,

such as pallor of the face and irregular, violent beating of the heart.

The rarest of the lateral class is the preauricular fissure, which has

been observed by Fevrier, Le Dentu, Marchand, Peyrot, and Routier.

The median congenital fissures of the neck are probably caused by

defective union of the branchial arches, although Arndt thinks that he

sees in these median fistulas a persistence of the hypobranchial furrow

which exists normally in the amphioxus. They are less frequent than the

preceding variety.

The most typical form of malformation of the esophagus is imperforation

or obliteration. Van Cuyck of Brussels in 1824 delivered a child which

died on the third day from malnutrition. Postmortem it was found that

the inferior extremity of the esophagus to the extent of about two

inches was converted into a ligamentous cord. Porro describes a case of

congenital obliteration of the esophagus which ended in a cecal pouch

about one inch below the inferior portion of the glottidean aperture

and from this point to the stomach only measured an inch; there was

also tracheal communication. The child was noticed to take to the

breast with avidity, but after a little suckling it would cough, become

livid, and reject most of the milk through the nose, in this way almost

suffocating at each paroxysm; it died on the third day.

In some cases the esophagus is divided, one portion opening into the

bronchial or other thoracic organs. Brentano describes an infant dying

ten days after birth whose esophagus was divided into two portions, one

terminating in a culdesac, the other opening into the bronchi; the left

kidney was also displaced downward. Blasius describes an anomalous case

of duplication of the esophagus. Grashuys, and subsequently Vicq

d'Azir, saw a dilatation of the esophagus resembling the crop of a bird.

Anomalies of the Lungs.--Carper describes a fetus of thirty-seven weeks

in whose thorax he found a very voluminous thymus gland but no lungs.

These organs were simply represented by two little oval bodies having

no lobes, with the color of the tissue of the liver. The heart had only

one cavity but all the other organs were perfectly formed. This case

seems to be unique. Tichomiroff records the case of a woman of

twenty-four who died of pneumonia in whom the left lung was entirely

missing. No traces of a left bronchus existed. The subject was very

poorly developed physically. Tichomiroff finds four other cases in

literature, in all of which the left lung was absent. Theremin and

Tyson record cases of the absence of the left lung.

Supplementary pulmonary lobes are occasionally seen in man and are

taken by some authorities to be examples of retrogressive anomalies

tending to prove that the derivation of the human race is from the

quadrupeds which show analogous pulmonary malformation. Eckley reports

an instance of supernumerary lobe of the right lung in close connection

with the vena azygos major. Collins mentions a similar case. Bonnet

and Edwards speak of instances of four lobes in the right lung. Testut

and Marcondes report a description of a lung with six lobes.

Anomalies of the Diaphragm.--Diemerbroeck is said to have dissected a

human subject in whom the diaphragm and mediastinum were apparently

missing, but such cases must be very rare, although we frequently find

marked deficiency of this organ. Bouchand reports an instance of

absence of the right half of the diaphragm in an infant born at term.

Lawrence mentions congenital deficiency of the muscular fibers of the

left half of the diaphragm with displacement of the stomach. The

patient died of double pneumonia. Carruthers, McClintock, Polaillon,

and van Geison also record instances of congenital deficiency of part

of the diaphragm. Recently Dittel reported unilateral defect in the

diaphragm of an infant that died soon after birth. The stomach, small

intestines, and part of the large omentum lay in the left pleural

cavity; both the phrenic nerves were normal. Many similar cases of

diaphragmatic hernia have been observed. In such cases the opening may

be large enough to allow a great part of the visceral constituents to

pass into the thorax, sometimes seriously interfering with respiration

and circulation by the pressure which ensues. Alderson reports a fatal

case of diaphragmatic hernia with symptoms of pneumothorax. The

stomach, spleen, omentum, and transverse colon were found lying in the

left pleura. Berchon mentions double perforation of the diaphragm with

hernia of the epiploon. The most extensive paper on this subject was

contributed by Bodwitch, who, besides reporting an instance in the

Massachusetts General Hospital, gives a numerical analysis of all the

cases of this affection found recorded in the writings of medical

authors between the years 1610 and 1846. Hillier speaks of an instance

of congenital diaphragmatic hernia in which nearly all the small

intestines and two-thirds of the large passed into the right side of

the thorax. Macnab reports an instance in which three years after the

cure of empyema the whole stomach constituted the hernia. Recently Joly

described congenital hernia of the stomach in a man of thirty-seven,

who died from collapse following lymphangitis, persistent vomiting, and

diarrhea. At the postmortem there was found a defect in the diaphragm

on the left side, permitting herniation of the stomach and first part

of the duodenum into the left pleural cavity. There was no history of

traumatism to account for strangulation. Longworth cites an instance

of inversion of the diaphragm in a human subject. Bartholinus mentions

coalition of the diaphragm and liver; and similar cases are spoken of

by Morgagni and the Ephemerides. Hoffman describes diaphragmatic

junction with the lung.

Anomalies of the Stomach.--The Ephemerides contains the account of a

dissection in which the stomach was found wanting, and also speaks of

two instances of duplex stomach. Bartholinus, Heister, Hufeland,

Morgagni, Riolan, and Sandifort cite examples of duplex stomach. Bonet

speaks of a case of vomiting which was caused by a double stomach.

Struthers reports two cases in which there were two cavities to the

stomach. Struthers also mentions that Morgagni, Home, Monro, Palmer,

Larry, Blasius, Hufeland, and Walther also record instances in which

there was contraction in the middle of the stomach, accounting for

their instances of duplex stomach. Musser reports an instance of

hour-glass contraction of the stomach. Hart dissected the stomach of a

woman of thirty which resembled the stomach of a predaceous bird, with

patches of tendon on its surface. The right extremity instead of

continuously contracting ended in a culdesac one-half as large as the

greater end of the stomach. The duodenum proceeded from the depression

marking the lesser arch of the organ midway between the cardiac orifice

and the right extremity. Crooks speaks of a case in which the stomach

of an infant terminated in a culdesac.

Hernia of the stomach is not uncommon, especially in diaphragmatic or

umbilical deficiency. There are many cases on record, some terminating

fatally from strangulation or exposure to traumatism. Paterson reports

a case of congenital hernia of the stomach into the left portion of the

thoracic cavity. It was covered with fat and occupied the whole left

half of the thoracic cavity. The spleen, pancreas, and transverse colon

were also superior to the diaphragm. Death was caused by a well-defined

round perforation at the cardiac curvature the size of a sixpence.

Anomalies of the Intestines.--The Ephemerides contains the account of

an example of double cecum, and Alexander speaks of a double colon, and

there are other cases of duplication of the bowel recorded. There is an

instance of coalition of the jejunum with the liver, and Treuner

parallels this case. Aubery, Charrier Poelman, and others speak of

congenital division of the intestinal canal. Congenital occlusion is

quite frequently reported.