Scoliosis in Rett syndrome

Scoliosis is the most common orthopaedic problem encountered in Rett syndrome. It is characterized by a long C-shaped thoracolumbar curve of neurologic origin. The occurrence of scoliosis in Rett syndrome is age- dependent, with a reported incidence of 36% to 100%. The onset of scoliosis is usually before age 8 years, and rapid curve progression is usually detected early in the second decade. In Rett syndrome, sagittal deformity with excessive kyphosis can progress and necessitates close observation. Orthotic treatment does not alter the natural history of scoliosis or kyphosis. Indications for surgery are curve progression exceeding a 40° or 45° Cobb angle or curves that cause pain or loss of function. Anterior discectomy, interbody fusion, and posterior fusion with instrumentation can achieve improved correction in young adolescents with significant curves. Surgical intervention should include fusing the scoliotic and the excessively kyphotic segments.