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 and Leonard, Helen (2015) Surgical fusion of early onset severe scoliosis increases survival in Rett syndrome: a cohort study. Developmental Medicine and Child Neurology, 58 6: 632-638. doi:10.1111/dmcn.12984

Author Downs, Jenny
Torode, Ian
Wong, Kingsley
Ellaway, Carolyn
Elliott, Elizabeth J.
Izatt, Maree T.
Askin, Geoffrey N.
Mcphee, Bruce I.
Cundy, Peter
Leonard, Helen
Title Surgical fusion of early onset severe scoliosis increases survival in Rett syndrome: a cohort study
Journal name Developmental Medicine and Child Neurology   Check publisher's open access policy
ISSN 1469-8749
Publication date 2015-12-11
Sub-type Article (original research)
DOI 10.1111/dmcn.12984
Open Access Status Not Open Access
Volume 58
Issue 6
Start page 632
End page 638
Total pages 7
Place of publication Chichester, West Sussex, United Kingdom
Publisher Blackwell Publishing
Language eng
Formatted abstract
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.
Q-Index Code C1
Q-Index Status Provisional Code
Institutional Status UQ

Document type: Journal Article
Sub-type: Article (original research)
Collections: Official 2016 Collection
School of Medicine Publications
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