These resources include the best available evidence about the course of Rett syndrome and its management as well as practical information about family associations, specialist clinical centres and links to relevant websites.
Downs, J., Young, D., de Klerk, N., Bebbington, A., Baikie, G., & Leonard, H. (2009). Impact of scoliosis surgery on activities of daily living in females with Rett syndrome.Journal of Pediatric Orthopedics, 29, 369-374. Download snapshot, see journal abstract.
Scoliosis occurs commonly in girls and women with Rett syndrome and severe scoliosis can be treated with spinal fusion. We wanted to examine the effects of this surgery on the functional abilities of girls and women with Rett syndrome.
What we did
Families whose daughter had been diagnosed with scoliosis provided information about functional abilities to the Australian Rett Syndrome Database in family surveys conducted approximately every 2 years. We then compared changes in functional abilities such as mobility, eye contact and communication skills, of those who had had spinal fusion with those who had not had spinal fusion.
What we found
Those who had surgery had, on average, developed scoliosis at a younger age. Overall, the functional abilities of all girls who had surgery improved slightly approximately 18 months after surgery, with greater improvement in those who were wheelchair-bound. Six girls could walk before they had surgery and mobility was improved in 2, maintained in 3, and decreased slightly in 1 girl. Abilities to make eye contact, communicate and interact socially, as well as the frequency of daytime napping were similar for those who had surgery and those who did not.
What does it mean
Families reported an improvement or maintenance of skills after spinal fusion. In particular, those who were wheelchair-bound may be able to make gains in many aspects of their life after scoliosis surgery. Overall, surgery for scoliosis for those with Rett syndrome is unlikely to result in worse functional abilities and some of these skills may improve.
You may be interested to read a booklet "Scoliosis in Rett Syndrome" which is a guide for the management of scoliosis for families and clinicians. This is available in English and Spanish.
For further reports and findings, see our publications list.