[Fibrolipomatous hamartoma of the nerve--a rare etiology of macrodactyly. A case report].

Fibrolipomatous hamartoma of nerve is a rare, tumor-like fibro-fatty growth arising from the epi- and perineurium. It mainly affects peripheral nerves and the median nerve in particular. An association with Klippel-Trenaunay syndrome and macrodactyly has been described. We present a 30-year-old patient, in whom the diagnosis of Klippel-Trenaunay syndrome had been made at the age of two, based on macrodactyly of the right hand. Because of increasing functional limitation and pain, the patient was referred to our department, after angiographic exclusion of Klippel-Trenaunay syndrome, for operative reduction of the tumor. Histologic examination revealed a fibrolipomatous hamartoma of the nerve. Further treatment consisted of surgical tumor reduction, which resulted in satisfactory recovery of function in the right hand. After eight months follow-up, the patient was able to perform all normal daily activities, and there was no evidence of recurrence of the lesion. Diagnostic evaluation of macrodactyly is necessary, since there may be an underlying cause such as fibrolipomatous hamartoma of a nerve, which if corrected early by surgical excision may prevent long-term disability.