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
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