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MINOR TERATA. 5 pagerecords 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: 786
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