| Abstract
Background: Medical advancements have resulted in better survival and life expectancy
among those with spina bifida, but a significantly increased risk of perinatal and
postnatal mortality for individuals with spina bifida remains.
Objectives: To examine stillbirth and infant and child mortality among those affected
by spina bifida using data from multiple countries.
Methods: We conducted an observational study, using data from 24 population‐ and
hospital‐based surveillance registries in 18 countries contributing as members of the
International Clearinghouse for Birth Defects Surveillance and Research (ICBDSR).
Cases of spina bifida that resulted in livebirths or stillbirths from 20 weeks' gestation
or elective termination of pregnancy for fetal anomaly (ETOPFA) were included.
Among liveborn spina bifida cases, we calculated mortality at different ages as number
of deaths among liveborn cases divided by total number of liveborn cases with
spina bifida. As a secondary outcome measure, we estimated the prevalence of spina
bifida per 10 000 total births. The 95% confidence interval for the prevalence estimate
was estimated using the Poisson approximation of binomial distribution.
Results: Between years 2001 and 2012, the overall first‐week mortality proportion
was 6.9% (95% CI 6.3, 7.7) and was lower in programmes operating in countries with
policies that allowed ETOPFA compared with their counterparts (5.9% vs. 8.4%). The
majority of first‐week mortality occurred on the first day of life. In programmes where
information on long‐term mortality was available through linkage to administrative
databases, survival at 5 years of age was 90%‐96% in Europe, and 86%‐96% in North
America.
Conclusions: Our multi‐country study showed a high proportion of stillbirth and infant
and child deaths among those with spina bifida. Effective folic acid interventions
could prevent many cases of spina bifida, thereby preventing associated childhood
morbidity and mortality |