Jm. Gonzalez-darder et Jv. Pesudo-martinez, Surgical treatment of multiple neurofibromas of the ulnar nerve in segmental neurofibromatosis - Case report, J NEUROSURG, 90(6), 1999, pp. 1137-1140
The case of an 18-year-old man with numerous neurofibromas along his left u
lnar 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 dis
tal portion of the retroepitroclear groove and another near the Guyon tunne
l 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. Duri
ng surgery many neurofibromas were found; the three painful neurofibromas a
nd some of the other larger lesions were microsurgically excised. The patie
nt'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 bo
dy.