Minor Terata : Extraoral Dentition, Part 2

Anomalies and Curiosities of Medicine
by George M. Gould, M.D., Walter L. Pyle, M.D.

(Page 20 of 53)

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.