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PEDIATRICS Vol. 108 No. 3 September 2001, pp. 728-734

Increased Risk for Developmental Disabilities in Children Who Have Major Birth Defects: A Population-Based Study

Received Aug 4, 2000; accepted Mar 14, 2001.

Pierre Decouflé, Coleen A. Boyle, Leonard J. Paulozzi, and Joseph M. Lary

From the National Center on Birth Defects and Developmental Disabilities Centers for Disease Control and Prevention, Atlanta, Georgia.

Objective.  We sought to quantify the strength of associations between each of four specific developmental disabilities (DDs) and specific types of major birth defects.

Methods.  We linked data from 2 independent surveillance systems, the Metropolitan Atlanta Congenital Defects Program and the Metropolitan Atlanta Developmental Disabilities Surveillance Program. Children with major birth defects (n = 9142; born 1981-1991 in metro Atlanta) and 3- to 10-year-old children who were born between 1981 and 1991 in metro Atlanta and identified between 1991 and 1994 as having mental retardation, cerebral palsy, hearing impairment, or vision impairment (n = 3685) were studied. Prevalence ratio (PR), which is the prevalence of a DD in children with 1 or more major birth defects divided by the prevalence of the same DD in children without major birth defects, was measured.

Results.  Among the 9142 children who were born with a major birth defect, 657 (7.2%) had a serious DD compared with 0.9% in children with no major birth defect, yielding a PR of 8.3 (95% confidence interval: 7.6-9.0). In general, the more severe the DD, the higher was the PR. Birth defects that originated in the nervous system and chromosomal defects resulted in the highest PRs for a subsequent DD. For all other categories of birth defects, PRs were lowest when all major birth defects present were confined to a single category (ie, isolated defects). PRs for any DD increased monotonically with the number of coded birth defects per child or the number of different birth defect categories per child, regardless of the severity of the defect or whether defects of the nervous system, chromosomal defects, or "other syndromes" were counted.

Conclusions.  These data highlight the possible early prenatal origins of some DDs and suggest that both the number of coded birth defects present and the number of anatomic systems involved are strongly related to functional outcomes.  Key words:  birth defects, developmental disabilities, mental retardation, cerebral palsy, hearing impairment, vision impairment, surveillance systems.


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