Determinants of survival in children with congenital abnormalities: a long-term population-based cohort study.

Background: Today more children with birth defects survive early childhood because of improved medical care; however, little information is available about patterns of long-term mortality and survival in this population. In particular, it is not clear whether other birth characteristics, apart from birth defects, have any role in their mortality.

Methods: Two large cohorts of children with and without birth defects were followed for up to 17 years. More than 45,000 children with birth defects, and 45,000 matched children without birth defects born in Ontario between 1979 and 1986 were followed. Throughout the study period long-term survival rates and the risk of death were compared between the 2 cohorts. Birth characteristics were also examined to determine their effect on the risk of death.

Results: During the study the deaths of 3620 and 301 children with and without birth defects, respectively, were recorded, indicating that those with birth defects had a 13 times higher rate of mortality (relative risk [RR], 12.9, 95% confidence interval [CI], 12.1-13.7). Mortality rates in the birth-defects cohort remained higher even after 10-15 years. In both groups children of low gestational age and low birth weight had a higher risk of death. There was a strong dose-response relationship between the number of defects and the risk of death.

Conclusions: Children born with abnormalities face many challenges throughout their lifetimes. If they survive the high mortality risk of the first year of life, they still have to face the considerably higher risk of death in the years to come. In addition to birth defects, other birth characteristics play an independent role in their mortality. These indicators could be used to identify high-risk children.