title: Surgical fusion of early onset severe scoliosis increases survival in Rett syndrome: a cohort study.
abstract: 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.
  
  
  
  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.
authors.affiliation: Telethon Kids Institute, The University of Western Australia, Perth, WA, Australia.|School of Physiotherapy and Exercise Science, Curtin University, Perth, WA, Australia.
authors.forename: Jenny
authors.initials: J
authors.lastname: Downs
authors.affiliation: Department of Orthopaedics, Royal Children's Hospital, Melbourne, Vic., Australia.
authors.forename: Ian
authors.initials: I
authors.lastname: Torode
authors.affiliation: Telethon Kids Institute, The University of Western Australia, Perth, WA, Australia.
authors.forename: Kingsley
authors.initials: K
authors.lastname: Wong
authors.affiliation: Discipline of Genetic Medicine, The University of Sydney, Sydney, NSW, Australia.|Discipline of Paediatrics and Child Health, The Children's Hospital at Westmead, The University of Sydney, Sydney, NSW, Australia.|The Sydney Children's Hospitals Network (Westmead), Sydney, NSW, Australia.
authors.forename: Carolyn
authors.initials: C
authors.lastname: Ellaway
authors.affiliation: Discipline of Paediatrics and Child Health, The Children's Hospital at Westmead, The University of Sydney, Sydney, NSW, Australia.|The Sydney Children's Hospitals Network (Westmead), Sydney, NSW, Australia.
authors.forename: Elizabeth J
authors.initials: EJ
authors.lastname: Elliott
authors.affiliation: Paediatric Spine Research Group, Lady Cilento Children's Hospital, Queensland University of Technology, Brisbane, Qld, Australia.
authors.forename: Maree T
authors.initials: MT
authors.lastname: Izatt
authors.affiliation: Paediatric Spine Research Group, Lady Cilento Children's Hospital, Queensland University of Technology, Brisbane, Qld, Australia.
authors.forename: Geoffrey N
authors.initials: GN
authors.lastname: Askin
authors.affiliation: Department of Surgery, University of Queensland, Brisbane, Qld, Australia.
authors.forename: Bruce I
authors.initials: BI
authors.lastname: Mcphee
authors.affiliation: Discipline of Orthopaedics and Trauma, University of Adelaide, Adelaide, SA, Australia.|Department of Orthopaedic Surgery, Women's and Children's Hospital, Adelaide, SA, Australia.
authors.forename: Peter
authors.initials: P
authors.lastname: Cundy
authors.affiliation: Telethon Kids Institute, The University of Western Australia, Perth, WA, Australia.
authors.forename: Helen
authors.initials: H
authors.lastname: Leonard
mesh_terms: D000293:Adolescent; D000328:Adult; D001315:Australia / Q000453:epidemiology; D002648:Child; D015331:Cohort Studies; D016208:Databases, Factual; D005260:Female; D006801:Humans; D017063:Outcome Assessment, Health Care / Q000706:statistics & numerical data; D012042:Registries; D012141:Respiratory Tract Infections / Q000453:epidemiology / Q000209:etiology / Q000401:mortality*; D015518:Rett Syndrome / Q000150:complications / Q000453:epidemiology / Q000401:mortality*; D012306:Risk; D012600:Scoliosis / Q000453:epidemiology / Q000209:etiology / Q000601:surgery*; D013123:Spinal Fusion / Q000706:statistics & numerical data; D055815:Young Adult