Abstract

Proteus syndrome is a rare congenital condition with various clinical features such as hemihypertrophy, macrodactyly, subcutaneous masses, brain-like hyperplasia of the soles and/or palms, epidermal naevi and scoliosis with other mesodermal malformations. Multifocal overgrowth can affect various tissues causing severe functional and cosmetic disability, but intellectual and language development are mostly normal. Orthopaedic problems include macrodactyly, hindfoot deformity, limb length inequality, genu valgum and scoliosis. Usually, scoliosis does not respond to bracing, and surgical intervention may be required. Despite surgical correction, instrumentation and fusion, progression of deformity can occur. The authors describe a case with a Th7-L2 scoliosis, which completely relapsed, 20 months after posterior instrumented fusion. Surgery should not be undertaken lightly, given the abnormal growth potency typical for Proteus syndrome.