The orthopedic management of Rett syndrome.

Musculoskeletal deformity sufficiently severe to require orthopedic surgery is a significant problem in Rett syndrome. Preliminary results from the study of 16 patients suggest deformity in nearly all patients. Eight patients in stage III and seven patients in stage IV showed clinical evidence of scoliosis. Radiographic studies confirmed a structural curve in nine of ten patients studied. Heel cord tightening was seen in nine of 16 patients. Hip instability was identified as an area of potential concern in the Rett patient.