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, , , McPhee, I. Bruce, Cundy, Peter, & Leonard, Helen (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.

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Aim - 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. 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.

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28 citations in Scopus
22 citations in Web of Science®
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ID Code: 92333
Item Type: Contribution to Journal (Journal Article)
Refereed: Yes
ORCID iD:
Izatt, Mareeorcid.org/0000-0002-4926-3077
Askin, Geoffreyorcid.org/0000-0002-1734-0082
Measurements or Duration: 7 pages
Keywords: Rett Syndrome, Scoliosis surgery, Survival, early onset scoliosis, life expectancy, spinal fusion, surgical fusion
DOI: 10.1111/dmcn.12984
ISSN: 0012-1622
Pure ID: 33025252
Divisions: Past > Institutes > Institute of Health and Biomedical Innovation
Past > QUT Faculties & Divisions > Science & Engineering Faculty
Copyright Owner: Consult author(s) regarding copyright matters
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Deposited On: 27 Jan 2016 05:33
Last Modified: 10 Feb 2025 13:36