Survival and causes of death in adults with spina bifida in Sweden: a population-based case-control study

Abstract

Objective: To analyse survival rates and causes of death in adults with spina bifida in Sweden compared with a matched control group. Design and methods: This population-based study included 11,900 adults born between 1950 and 1997. Three national Swedish registers were used to identify individuals with a diagnosis of spina bifida and a matched control group without spina bifida in the period 1990–2015. International Classification of Diseases codes were used to identify causes of death. Survival analysis was conducted and causes of death in the 2 groups were compared. Results: There was a lower probability of survival for people with spina bifida in all age groups (p < 0.001) compared with the control group. The most prevalent causes of death in people with spina bifida were congenital, respiratory, nervous, cardiovascular, genitourinary, and injuries. People with spina bifida had a higher probability of dying from congenital (p < 0.001), respiratory (p = 0.002), genitourinary (p < 0.002), and nervous-related (p < 0.001) and lower probability of injury-related deaths (p < 0.001). Conclusion: Adults with spina bifida in Sweden have a lower survival rate compared with the general population, with the frequency of certain causes of death differing between the two groups. In order to reduce excess premature mortality, prevention and careful management of potentially fatal conditions are essential throughout a patient’s lifespan.

Keywords

• spina bifida
• adults
• transition to adult care
• mortality
• survival