Correcting Scoliosis in Rett Syndrome

Abstract

Objectives Rett syndrome is a rare disorder characterised by severe scoliosis in 80% of cases. In this retrospective case series, we analysed the radiographic, clinical, and functional outcomes of consecutive patients treated for scoliosis associated with Rett syndrome. We sought to understand the results of the treatment of scoliosis in Rett syndrome and evaluate the need to fuse to the pelvis. Methods A retrospective case series was used to analyse the radiographic, clinical, and functional outcomes of consecutive patients treated for Rett syndrome scoliosis between the ages of 10 and 8 years in a single tertiary paediatric spinal unit. Cases were identified through departmental and neurophysiological records, and patients were excluded if the diagnosis of Rett syndrome was not confirmed. Results Seven eligible cases were identified. At presentation, the mean coronal Cobb angle was 90.9°, mean sagittal Cobb 72.0°, and pelvic obliquity 24.5°. The mean post-operative improvement in coronal Cobb was 53.2° and pelvic obliquity reduced to 5.8°. These did not change during a mean follow up of 3.5 years. None showed any post-operative complications. Three patients with a mean 16.1° pelvic obliquity underwent a fusion to L5. The postoperative result in those cases remained stable at 3.5 years mean follow-up and full skeletal maturity. Conclusion Our data suggests that with modern technology, severe curves can be safely treated. Fusion to the pelvis is not necessary in patients with mild, flexible pelvic obliquity.