Surgical treatment of multiple neurofibromas of the ulnar nerve in segmental neurofibromatosis. Case report.

Abstract

The case of an 18-year-old man with numerous neurofibromas along his left ulnar nerve is described. The patient had a painful mass in the medial third of the internal aspect of his left forearm, and two additional symptomatic painful masses were identified during clinical examination: one in the distal portion of the retroepitroclear groove and another near the Guyon tunnel in the wrist. The main symptom was neurogenic pain; however, sensory and motor disturbances were also present. No other stigma of neurofibromatosis (NF) was found, and no cases of NF were known in the patient's family. During surgery many neurofibromas were found; the three painful neurofibromas and some of the other larger lesions were microsurgically excised. The patient's symptoms fit the criteria for segmental NF or NF5. This is a very rare form of NF characterized by lesions located in a particular area of the body.