Surgical fusion of early onset severe scoliosis increases survival in the child with rett syndrome: A population based study
Downs, Jenny, Torode, Ian, Wong, Kingsley, Ellaway, Carolyn, Elliot, Elizabeth J., Christodoulou, John, Jacoby, Peter, Thomson, Margaret R., Izatt, Maree T., Askin, Geoffrey N., McPhee, Bruce, Bridge, Corinne, Cundy, Peter, & Leonard, Helen J. (2015) Surgical fusion of early onset severe scoliosis increases survival in the child with rett syndrome: A population based study. In Williams, Richard (Ed.) 26th Annual Scientific Meeting of the Spine Society of Australia, 10-12 April 2015, National Convention Centre, Canberra, A.C.T.
Rett Syndrome is a rare genetic neurodevelopmental disorder usually affecting females. Scoliosis is a common comorbidity and spinal fusion may be recommended if severe. Little is known about long term outcomes. We examined the impact of spinal fusion on survival and risk of severe lower respiratory tract infection (LRTI) in Rett Syndrome.
Data were ascertained from hospital medical records, the Australian Rett Syndrome Database, a longitudinal and population-based registry of Rett Syndrome cases established in 1993, and the Australian Institute of Health and Welfare National Death Index database. An extended Cox regression model was used to estimate the effect of spinal surgery on survival in females who developed severe scoliosis (Cobb angle > 45 degrees). Generalized estimating equation modelling was used to estimate the effect of spinal surgery on the odds of developing severe LRTI.
Severe scoliosis was identified in 140 cases (60.3%) of whom slightly fewer than half (48.6%) developed scoliosis prior to eight years of age. Scoliosis surgery was performed in 98 (69.0%) of those at a median age of 13 years 3 months (IQR 11 years 5 months – 14 years 10 months). After adjusting for mutation type and age of scoliosis onset, the rate of death was lower in the surgery group (HR 0.30, 95% 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). Spinal fusion was not associated with reduction in the occurrence of a severe LRTI overall (OR 0.60, 95%CI 0.27, 1.33, P=0.206) but was associated with a large reduction in odds of severe LRTI among those with early onset scoliosis (OR 0.32, 95%CI 0.11, 0.93, P=0.036).
With appropriate cautions, spinal fusion confers an advantage to life expectancy in Rett syndrome.
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|Item Type:||Conference Item (Presentation)|
|Keywords:||Rett Syndrome, scoliosis, scoliosis progression, spinal fusion, spine deformity|
|Subjects:||Australian and New Zealand Standard Research Classification > MEDICAL AND HEALTH SCIENCES (110000)
Australian and New Zealand Standard Research Classification > MEDICAL AND HEALTH SCIENCES (110000) > CLINICAL SCIENCES (110300) > Orthopaedics (110314)
|Divisions:||Current > Schools > School of Chemistry, Physics & Mechanical Engineering
Current > Institutes > Institute of Health and Biomedical Innovation
Current > QUT Faculties and Divisions > Science & Engineering Faculty
|Copyright Owner:||Copyright 2015 The Authors|
|Deposited On:||23 Sep 2015 00:00|
|Last Modified:||27 Sep 2015 05:05|
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