Rare_Congenital_Affections

Court Transcript

KREW. The abbey of Belloc was crammed with deformities of all kinds, and the many days I spent within its walls proved vital to my studies in teratology. However, the cause of these monstrosities remained a mystery; at first I thought it might be some impurity in the water of the mountains, or some other teratological agent. But my theory proved to be false for the animals drank the same water and remained quite unaffected. Yet I could not accept that all these human monsters were simply caused by a long history of inbreeding. No, there was another cause – an imbalance in the telluric currents of earth. These black lines of force poisoned the land with a dark and noxious energy, for their pulsating currents were harmful to the proper organisation and formation of cells. This was the cause of much human misery. I need not tell you that the study of teratology is still in its infancy, but there are four main classes of deformity: Hemiteratic, Heterotaxic, Hermaphroditic, and Monstrous.(i)

The first class, Hemiterata, is principally concerned with anomalies of volume, whether it be of stature, for example, giantism and dwarfism, or diminutions of specific regions, id est the limbs, head and organs. There are of course other anomalies of shape and colour that come into this category, for example, a deformed pelvis or albinism. Anomalies of structure also include deficiencies in in consistency, such as the cartilaginous condition of the bones, or an excess of consistency, such as anomalous ossification. Then there are those hemiteratic deformities that are caused by anomalies of disposition and displacement, such as misplaced teeth, club feet, curvature of the spine, a misplaced heart, hernias and exstrophy of the bladder, etcetera. And let us not forget anomalous articulations, implantations, attachments and openings; these include branches of the vessels and nerves; imperforations of the rectum, vulva, vagina, mouth and oesophagus; anomalous unions of the organs, such as the kidneys, testicles, digits, teeth and ribs; adhesion of the tongue to the soft pallet; transverse septum of the vagina and anus; anomalous perforations and divisions, such as splits, fissures, hare-lips, fissured tongue, cleft palette, fissured cheeks, etcetera. Finally there are those anomalies of number and existence – either by numerical defect, such as the absence of limbs, eyes and ears; or by numerical excess, such as supernumerary digits, ribs, teeth, breasts and double uteri, etcetera. Joseph the ectromelus, who was born without limbs, was an autositic hemiteratic monster.

The second class, Heterotaxis, concerns inversions during foetal development, such as splanchnic inversions. But since these monsters rarely survive beyond birth, they are irrelevant to the case.

And so we come to the third class, the Hermaphrodites. There are true hermaphrodites and pseudo-hermaphrodites. True hermaphrodites may be bilateral, unilateral or lateral. Whilst pseudo-hermaphrodites have double sexual function of the external genitals, but with unisexual development of the reproductive glands. A male pseudo-hermaphrodite with testicles may be an internal pseudo-hermaphrodite, (with the development of a uterus), or an external pseudo-hermaphrodite, where the genitals are more female with clitoris and vulva. A female pseudo-hermaphrodite with ovaries has a persistence of male sexual parts. Again, the condition may be internal with formation of a vas deferens and tubes, or external, with an enlarged clitoris that more resembles a fully formed penis. Finally there are complete hermaphrodites both internal and external.

The fourth class of Monsters is split into single, composite and triple. Single monsters may be autositic or omphalositic. Composite monsters may be double autositic or double parasitic, and within these various subclasses are countless subdivisions, to numerous to list here. Suffice to say that the Janus abbot was a double autositic known as a diprosopus – which means double faced.

After considering the many teratologies within the abbey, I became interested with three monks in particular: Bernard, Fabien and Poufille.

As well as a tumorous head, Bernard suffered a chronic deformity of the legs and spine. I am referring of course to osteitis deformans: an insidious malady of the bones that is usually met in middle age. The first symptoms are vague pains in the back and limbs. As the disease progresses, the legs become grossly deformed and enlarged by bending of the tibia and femur. There is also a gradual increase in the size of the cranium, which necessities the wearing of larger hats and cowls. When the condition is fully developed, the posture is severely affected. The curved spine and legs diminish the height, and the arms appear unnaturally long. The head and upper spine are bent forwards whilst the curved legs are fused permanently apart, rotated outwards, and slightly flexed at the knees. The sufferer has the tragic appearance of an overgrown ape. The leg muscles waste away to such an extent that the misshapen bones are covered only by skin. And whilst the capacity of walking is clumsily maintained, the disease is further complicated by fractures that take place over many years. To cure such a man is no easy task, although nothing is beyond the powers of the Light Stream. It is to his great credit that when referring to his terrible condition, Bernard only mentioned his facial warts which seemed to disturb him more than anything else. I should like Jacques to lay hands on Bernard, but I have my sights set on an altogether more impossible task: Fabien.

Fabien and Poufille were brothers, and both suffered with the same chronic condition which was in fact an amalgamation of several conflicting diseases. Fabien had a peculiar elongation of the skull, with orbits set a span apart. His fleshy lips were pocked with cysts and his cranium was cloven down the occipital. With his bulging eyes and pouting lips, he resembled a monkfish, or tadpole in the early stages of metamorphosis.

The condition, as far as I could ascertain, arose from a central arrest of development in the course of ossification. The pathological changes in his bones started in early life, and consisted in decalcification of existing bone, whilst the formation of new bone remained imperfectly calcified. This change in bone consistency and structure lay principally within the cranium, so that the bones were pliable as wax. How his head became so elongated, I could not easily determine, though I imagine the pressure of lying in bed deformed his skull over a period of years. His vertebral column was also twisted like a length of old rope. The clavicle was steeply pitched, so that he his neck remained cocked to the left, whilst his pelvis thrust in the opposing direction. The rest of his skeletal structure was riddled with horny tuberosities. His feet had shrunk to such a degree that he had the appearance of walking on pins. This regressive metamorphosis was pitiful to behold, and whilst Poufille did not suffer decalcification to the same degree as his brother, the weight of his giant hand put such strain on his spine such that his whole torso was bent to the right.

Fabien also suffered another rare congenital affection: ichthyosis, commonly known as “fishy skin” – a disease characterized by an overgrowth of the epidermis and cornification of its superficial layers. The greater part of Fabien’s body was covered in large, flattened plaques or scales of a rough horny texture, so that his skin resembled that of a fish. On his cheeks and forehead the epidermis was comparatively smooth, but with a number of ragged edges, which were caused by constant exfoliation; yet the dermis around his mouth and eyelids was rough and flaking, with the appearance of bran or oatmeal. The pores of his scalp were prominent with the accumulation of a hard wax within the ducts, and what little hair he had was brittle and dry. The skin of his neck was so parched and shrivelled that he looked like a person of extreme age and decrepitude.

Around his joints, the scales turned up at the edges, and were lined with fissures, furrows and sores, which constantly oozed blood and pus. His elbows and knees were crossed with prominent wrinkles, traversed with deep clefts and chaps. His hands were remarkably course and furrowed, like those of a ploughman, with great welts across the knuckles, whilst his forearms were marked by a reticulated tracery of white lines. Around his ankles the skin had gathered into prominent folds, and across the insteps were pale bands, marked by a number of longitudinal grooves which broke into stiff ridges running parallel with the feet. The flesh of his thighs, buttocks and torso had little connective tissue between the under surface of the corium and the structures beneath; and instead of falling into folds during movement, it remained stiff as old leather. Indeed, this part of the dermis had the appearance of an ill fitting suit made from the hide of a water horse. [Hippopotamus].

After conferring with my aerial cohorts, it was decided that we would cure Fabien, as testament to the power of prayer and the healing powers of the Light Stream. Jacques would perform the miracle before the whole monastery, although the timing of this event was not yet determined…

Copyright © Nicholas Shea 2010

i. I have based Krew’s classification of human monsters on that given in the classic work ‘Human Monstrosities’ Volumes 1-4, by Hirst Barton Cooke, published in 1891. Of course, Krew himself is a Cyclops.

Image credit: Eventration with partial retroflexion and shortening of the trunk. (Potthoff.) Human Monstrosities Vol.2 p. 96.