MACRODACTYLY IN PROTEUS SYNDROME

A case of Proteus syndrome in a 13-year-old boy with macrodactyly, hemihypertrophy, exostosis of the skull, epidermal naevi, palmar and plantar masses, and scoliosis is reported. Macrodactyly involving the left thumb was treated surgically. The usual findings in macrodactyly, hypertrophy of the digital nerves and proliferation of subcutaneous fat, were not observed in this case. Macrodactyly associated with Proteus syndrome should be distinguished from other forms of macrodactyly because of its poor prognosis and high rate of recurrence. Journal of Hand Surgery (British and European Volume, 1993) 18B: 308-309 Proteus syndrome is a rare congenital hamartomatous disorder first described by Wiedemann et al (1983). Although more than 50 cases have been reported (Viljoen et al, 1988), treatment is rarely discussed in the orthopaedic literature. A case of Proteus syndrome with macrodactyly is described and the relevant literature is reviewed. CASE REPOR