Authors:
Downs J, Torode I, Wong K, Ellaway C, Elliott EJ, Izatt MT, ... Leonard H.
Authors notes:
Developmental Medicine and Child Neurology. 2015;58(6):632-8.
Keywords:
Surgical fusion, Spinal Fusion, Early onset severe scoliosis, Rett syndrome, 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 before adulthood.
Results: After adjusting for mutation type and age of scoliosis onset, the rate of death was lower in the surgery group compared to those without surgery.
Rate of death was particularly reduced for those with early onset scoliosis.
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.
Interpretation: With appropriate cautions, spinal fusion confers an advantage to life expectancy in Rett syndrome.
Authors:
Downs J, Torode I, Wong K, Ellaway C, Elliott EJ, Izatt MT, ... Leonard H.
Authors notes:
Developmental Medicine and Child Neurology. 2015;58(6):632-8.
Keywords:
Surgical fusion, Spinal Fusion, Early onset severe scoliosis, Rett syndrome, 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 before adulthood.
Results: After adjusting for mutation type and age of scoliosis onset, the rate of death was lower in the surgery group compared to those without surgery.
Rate of death was particularly reduced for those with early onset scoliosis.
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.
Interpretation: With appropriate cautions, spinal fusion confers an advantage to life expectancy in Rett syndrome.