Surgical fusion of early onset severe scoliosis increases survival in Rett syndrome: A cohort study
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Aim: Scoliosis is a common comorbidity in Rett syndrome and spinal fusion may be recommended if severe. We investigated the impact of spinal fusion on survival and risk of severe lower respiratory tract infection in Rett syndrome. Method: Data were ascertained from hospital medical records, the Australian Rett Syndrome Database, a longitudinal and population-based registry, and from the Australian Institute of Health and Welfare National Death Index database. Cox regression and generalized estimating equation models were used to estimate the effects of spinal surgery on survival and severe respiratory infection respectively in 140 females who developed severe scoliosis (Cobb angle =45°) before adulthood. Results: After adjusting for mutation type and age of scoliosis onset, the rate of death was lower in the surgery group (hazard ratio [HR] 0.30, 95% confidence interval [CI] 0.12-0.74; p=0.009) compared to those without surgery. Rate of death was particularly reduced for those with early onset scoliosis (HR 0.17, 95% CI 0.06-0.52; p=0.002). There was some evidence to suggest that spinal fusion was associated with a reduction in risk of severe respiratory infection among those with early onset scoliosis (risk ratio 0.41, 95% CI 0.16-1.03; p=0.06). Interpretation: With appropriate cautions, spinal fusion confers an advantage to life expectancy in Rett syndrome.
This is the peer reviewed version of the following article: Downs, J. and Torode, I. and Wong, K. and Ellaway, C. and Elliott, E. and Izatt, M. and Askin, G. et al. 2016. Surgical fusion of early onset severe scoliosis increases survival in Rett syndrome: A cohort study. Developmental Medicine and Child Neurology. 58 (6): pp. 632-638, which has been published in final form at http://doi.org/10.1111/dmcn.12984. This article may be used for non-commercial purposes in accordance with Wiley Terms and Conditions for Self-Archiving at http://olabout.wiley.com/WileyCDA/Section/id-820227.html#terms
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Marr, C.; Leonard, H.; Torode, I.; Downs, Jennepher (2015)Background: Rett syndrome is a severe neurodevelopmental disorder mainly affecting females and scoliosis is a common co-morbidity. Spinal fusion may be recommended if the scoliosis is progressive. This qualitative study ...
Downs, Jennepher; Torode, I.; Wong, K.; Ellaway, C.; Elliott, E.; Christodoulou, J.; Jacoby, P.; Thomson, M.; Izatt, M.; Askin, G.; McPhee, B.; Bridge, C.; Cundy, P.; Leonard, H. (2015)STUDY DESIGN.: Population-based longitudinal observational study. OBJECTIVES.: To describe the prevalence of scoliosis in Rett syndrome, structural characteristics and progression, taking into account the influences of ...
Downs, Jennepher; Forbes, D.; Johnson, M.; Leonard, H. (2016)Rett syndrome is a rare disorder caused by a mutation in the MECP2 gene. Those affected generally have severe functional impairments, and medical comorbidities such as scoliosis and poor growth are common. There is a ...