Scoliosis in the Rett syndrome: natural history and treatment

The Rett syndrome (RS) is associated with a neurological form of scoliosis. From 1985 we have instituted a postal survey of families with Rett girls. The prevalence of scoliosis in the survey population is 64%. The age at onset of scoliosis has a normal distribution about a peak age of 8 years, with 72% of cases occurring before age 8. The scoliosis in RS is typically a long thoracolumbar curvature that progresses rapidly in girls over the age of 10 years. Operative treatment is successful in reducing the curvature, preventing curve progression and improving spinal balance for sitting and walking. The 5 girls who walked pre-operatively are still able to do so.