BACKGROUND: Scoliosis is a common orthopaedic complication of Rett syndrome, and surgery is commonly used to reduce asymmetry in cases with severe scoliosis. METHODS: Data 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. RESULTS: Surgery 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. CONCLUSIONS: Improvements 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 EVIDENCE: Therapeutic study: level III.
BACKGROUND:Scoliosis is a common orthopaedic complication of Rett syndrome, and surgery is commonly used to reduce asymmetry in cases with severe scoliosis. METHODS: Data 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. RESULTS: Surgery 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. CONCLUSIONS: Improvements 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 EVIDENCE: Therapeutic study: level III.
Authors: Alberto Romano; Elena Ippolito; Camilla Risoli; Edoardo Malerba; Martina Favetta; Andrea Sancesario; Meir Lotan; Daniel Sender Moran Journal: J Clin Med Date: 2022-01-22 Impact factor: 4.241
Authors: Sandra Louise; Sue Fyfe; Ami Bebbington; Nadia Bahi-Buisson; Alison Anderson; Mercé Pineda; Alan Percy; Bruria Ben Zeev; Xi Ru Wu; Xinhua Bao; Patrick Mac Leod; Judith Armstrong; Helen Leonard Journal: Res Autism Spectr Disord Date: 2009-07
Authors: Bushra Kamal; David Russell; Anthony Payne; Diogo Constante; K Elizabeth Tanner; Hanna Isaksson; Neashan Mathavan; Stuart R Cobb Journal: Bone Date: 2014-10-24 Impact factor: 4.398