Surgical fusion of early onset severe scoliosis increases survival in Rett syndrome: A cohort study

Downs, Jenny, Torode, Ian, Wong, Kingsley, Ellaway, Carolyn, Elliott, Elizabeth J., Izatt, Maree T., Askin, Geoffrey N., McPhee, Bruce I., Cundy, Peter, & Leonard, Helen J. (2016) Surgical fusion of early onset severe scoliosis increases survival in Rett syndrome: A cohort study. Developmental Medicine & Child Neurology, 58(6), pp. 632-638.

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  • Scoliosis is a common co-morbidity 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.


  • 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.


  • 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).


  • With appropriate cautions, spinal fusion confers an advantage to life expectancy in Rett syndrome.

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ID Code: 92333
Item Type: Journal Article
Refereed: Yes
Keywords: Rett Syndrome, Survival, surgical fusion, Scoliosis surgery, spinal fusion, early onset scoliosis, life expectancy
DOI: 10.1111/dmcn.12984
ISSN: 0012-1622
Subjects: Australian and New Zealand Standard Research Classification > ENGINEERING (090000) > BIOMEDICAL ENGINEERING (090300) > Biomechanical Engineering (090302)
Australian and New Zealand Standard Research Classification > MEDICAL AND HEALTH SCIENCES (110000) > CLINICAL SCIENCES (110300) > Orthopaedics (110314)
Divisions: Current > Schools > School of Chemistry, Physics & Mechanical Engineering
Current > Institutes > Institute of Health and Biomedical Innovation
Current > QUT Faculties and Divisions > Science & Engineering Faculty
Copyright Owner: Copyright 2015 Mac Keith Press
Deposited On: 27 Jan 2016 05:33
Last Modified: 10 Jun 2016 05:46

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