Musculoskeletal manifestations of Proteus syndrome: report of two cases with literature review.

Abstract

Proteus syndrome is a recently described hamartomatous condition characterized by macrodactyly, hemihypertrophy, subcutaneous (s.c.) tumors, epidermal nevi, and skull anomalies. Two new cases are described to illustrate the diagnostic features and the orthopedic problems associated with this rare syndrome. Review of available literature shows that 61 patients with proteus syndrome commonly develop macrodactyly, limb overgrowth, spinal deformity, hip dysplasia, genu valgum, exostoses, joint contractures, and hindfoot deformities.