MINOR TERATA. 5 page

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

Date: 2014-12-29; view: 696