Surgical fusion of early onset severe scoliosis increases survival in Rett syndrome: a cohort study

Article title

Downs J, Torode I, Wong K, Ellaway C, Elliott EJ, Izatt MT, Askin GN, McPhee BI, Cundy P, Leonard H. 2016. Surgical fusion of early onset severe scoliosis increases survival in Rett syndrome: a cohort study. Developmental Medicine & Child Neurology. 58(6):632-8. Download snapshot, see article abstract.

Background

Scoliosis is common in individuals with Rett syndrome and spinal fusion may be recommended if it is severe. Our study investigated the impacts of spinal fusion on survival and the risk of developing respiratory infections in females with Rett syndrome.

What we did

We analysed data on 140 females with Rett syndrome who developed severe scoliosis from information families had provided to the Australian Rett Syndrome Database with additional data from hospital medical records and from the Australian Institute of Health and Welfare National Death Index database.

What we found

Life expectancy was improved for those who underwent the spinal fusion surgery compared to those who did not, particularly in those whose scoliosis developed before they were eight years old. The surgery was also associated with fewer respiratory infections that needed hospitalisation among those whose scoliosis developed early.

What does it mean

With appropriate cautions in regards to the health status of girls with Rett syndrome, undergoing spinal fusion surgery may increase life expectancy. Our findings provide families and clinicians with a framework from which they can make decisions about whether or not to undergo a spinal fusion.