Birth defects in children with newborn encephalopathy

This study was designed to investigate birth defects found in association w ith newborn encephalopathy. All possible birth defects were ascertained in a population-based study of 276 term infants with moderate or severe enceph alopathy and 564 unmatched term control infants. A strong association betwe en birth defects and newborn encephalopathy was found with defects affectin g 27.5% of children with encephalopathy and 4.3% of control children (odds ratio 8.55; 95% confidence interval 5.25 to 13.91; p<0.001). In 11.8% of in fants with a birth defect the defect was not diagnosed until after the newb orn period, illustrating one of the difficulties in attempting to exclude i nfants with birth defects from studies of newborn encephalopathy. The major ity of defects (89%) were not specific anomalies of the CNS. In 36.8% of ch ildren with encephalopathy who had a birth defect, the defect was considere d to be the probable cause of the encephalopathy. Infants with birth defect s who had encephalopathy had a poorer prognosis than those without: they we re twice as likely to die by the age of 2 years and three times more likely to have cerebral palsy. This study catalogues the spectrum of birth defect s associated with newborn encephalopathy and illustrates the importance of their inclusion when investigating both the aetiology and outcome of this c ondition.