PEDIATRIC PERIPHERAL NEUROPATHY IN PROTEUS-SYNDROME

Proteus syndrome is a rare congenital disorder comprised of subcutaneo us and internal hamartomas, pigmented skin nevi, skull exostoses, hemi hypertrophy, and macrodactyly of the hands and feet. A 5-year-old girl diagnosed with Proteus syndrome presented with distal median compress ion neuropathy with the primary complaint of severe pain involving the left hand. Surgical exploration of the hand revealed a lipofibromatou s hamartoma of the median nerve. The transverse carpal ligament was re leased and epineurectomy of the median nerve was performed. The patien t remains symptom free at the 9-month follow-up. This report is the fi rst description of a hamartoma directly involving a peripheral nerve i n Proteus syndrome. Decompression of the nerve with the removal of the fibrofatty neural sheath resulted in the resolution of the symptoms i n this patient. The surgeon should consider this approach as a potenti al first line of treatment before a more radical resection of the nerv e is contemplated.