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Surgical fusion of early onset severe scoliosis increases survival in Rett syndrome: a cohort study
Author(s) -
Downs Jenny,
Torode Ian,
Wong Kingsley,
Ellaway Carolyn,
Elliott Elizabeth J,
Izatt Maree T,
Askin Geoffrey N,
Mcphee Bruce I,
Cundy Peter,
Leonard Helen
Publication year - 2016
Publication title -
developmental medicine and child neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.658
H-Index - 143
eISSN - 1469-8749
pISSN - 0012-1622
DOI - 10.1111/dmcn.12984
Subject(s) - rett syndrome , medicine , scoliosis , hazard ratio , spinal fusion , confidence interval , cobb angle , cohort , pediatrics , proportional hazards model , surgery , biology , biochemistry , gene
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.