• Jenny Downs, Deidra Young, Nick de Klerk, Ami Bebbington, Gordon Baikie, and Helen Leonard.
• Telethon Institute for Child Health Research, Centre for Child Health Research, The University of Western Australia, Perth, Western Australia.
• J Pediatr Orthop. 2009 Jun 1; 29 (4): 369-74.

BackgroundScoliosis is a common orthopaedic complication of Rett syndrome, and surgery is commonly used to reduce asymmetry in cases with severe scoliosis.MethodsData from questionnaires administered to caregivers biennially from 2000 to 2006 were used to describe functional skill levels in subjects with Rett syndrome, and within-subject change in 16 subjects with scoliosis surgery were compared with within-subject change in 186 pairs of data from 86 subjects with conservatively managed scoliosis. Postsurgical assessment was conducted after a mean of 17.8 months.ResultsSurgery was associated with improved activities of daily living as measured by the WeeFIM for subjects who were wheelchair bound (P = 0.05). Mobility levels, social interaction, communication skills, and the frequency of daytime napping remained similar for the group as a whole.ConclusionsImprovements in activities of daily living are likely to represent an increase in the quality of life for subjects and caregivers and were mainly found in subjects who were wheelchair bound, indicating that those who were more severely affected were able to benefit from this intervention.Level Of EvidenceTherapeutic study: level III.

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