Local gigantism of the hand associated with a plexiform neurofibroma of the ulnar nerve. Report of a case

Report of a case of soft tissue hypertrophy of the ulnar area of the hand and of the fifth finger with hyperpathic anesthesia in the same region. This hypertrophy existed at birth, as well as a large nevus verrucosus of the axilla. Many years later, a plexiform neurofibroma of the ulnar nerve developed at the elbow level. The resection of the neurofibroma, of the hypertrophied finger and partly of the ulnar region of the palm was followed by an excellent functional result. The causal relationship between a lesion of the nervous system and the local gigantism is debated. The embryologic or teratologic theory which implies a disturbance of induction seems most probable.SCOPUS: ar.j