OBJECTIVE: This study investigates outcome of scoliosis treatment for 11 @entity1 with Angelman syndrome (AS), with particular focus on activity, participation and the musculoskeletal factors that may affect these outcomes. METHODS: Retrospective review of medical records, radiographs and questionnaires administered to caregivers of 11 @entity1 (8M:3F) with AS and scoliosis. Six underwent observational treatment during childhood and five underwent spinal fusion. The Activities Scale for Kids ( @entity30868 ) questionnaire was used to measure activity and participation. Questionnaire and radiographic data were recorded over a 2 year period. RESULTS: In the observational group, scoliosis increased from 31   to 46  . Mean @entity30868 decreased from 13.8 to 11.9 (p   =   0.06). In the operative group, scoliosis decreased from 68   to 29  . Mean @entity30868 increased from 11.4 to 15.9 (p   <   0.01). There was also a reduction in spinal-related pain and mean number of hospital admissions for chest infection. However, there was a 60% major complication rate. There was no difference in mobility, GMFCS level, feeding or communication in either group before or after treatment. CONCLUSION: In @entity1 with significant scoliosis and AS, spinal fusion was associated with a small improvement in activity and participation, reduction in pain and a decrease in frequency of severe chest infections. Non-operative treatment resulted in progression of scoliosis during childhood and decrease in activity.

A retrospective review to assess whether spinal fusion and scoliosis correction improved activity and participation for XXXX with Angelman syndrome: Brief report.

@entity1 :: ('9606', 'Species') :: ['children'] @entity30868 :: ('153642', 'Gene') :: ['ASKp']
@entity1 :: (9606,Species) :: ['children']
