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

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. (PsycINFO Database Record (c) 2016 APA, all rights reserved)Telethon Kids Institute, University of Western Australia, West Perth, WAU, Australia. Institutional Authors: Rett Syndrome Spinal Fusion Group. Other Publishers: Mac Keith Press. Release Date: 20151214. Correction Date: 20160530. Publication Type: Journal (0100), Peer Reviewed Journal (0110). Format Covered: Electronic. Document Type: Journal Article. Language: EnglishGrant Information: Leonard, Helen. Major Descriptor: Comorbidity; Rett Syndrome; Surgery. Classification: Neurological Disorders & Brain Damage (3297); Medical Treatment of Physical Illness (3363). Population: Human (10); Male (30); Female (40). Location: Australia. Age Group: Childhood (birth-12 yrs) (100); School Age (6-12 yrs) (180); Adolescence (13-17 yrs) (200); Adulthood (18 yrs & older) (300); Young Adulthood (18-29 yrs) (320); Thirties (30-39 yrs) (340). Methodology: Empirical Study; Followup Study; Longitudinal Study; Quantitative Study. References Available: Y. Page Count: 7. Issue Publication Date: Jun, 2016. Publication History: First Posted Date: Dec 11, 2015; Accepted Date: Oct 5, 2015. Copyright Statement: Mac Keith Press. 2015.Sponsor: National Institutes of Health, US. Grant: 5R01HD043100-05. Recipients: No recipient indicated <br />Sponsor: National Health and Medical Research Council. Grant: 303189; 1004384. Other Details: Project grants. Recipients: No recipient indicated <br />Sponsor: National Health and Medical Research Council. Grant: 572742. Other Details: Programme grant. Recipients: No recipient indicated <br />Sponsor: National Health and Medical Research Council. Grant: 572568. Date: from 2009 to 2014. Other Details: Senior Research Fellowship. Recipients: Leonard, Helen <br />Sponsor: National Health and Medical Research Council. Grant: 457084. Other Details: Practitioner Fellowship. Recipients: Elliott, Elizabeth J. <br />