The postmortem findings in 672 Nigerian children with lethal congenita
l malformations are reviewed. Eighty-six percent of the patients were
less than 1 year of age and the male-to-female sex ratio was 1.3:1. Th
e alimentary tract was the most common anatomic site involved (30%), i
ntestinal and biliary atresia being the two lesions most frequently id
entified in this location. Twenty-six percent of the children had card
iovascular malformations, of which ventricular septal defect, patent d
uctus arteriosus, and tetralogy of Fallot were most important. There w
as an accentuated male predominance among cases with urogenital malfor
mations due to the high frequency of posterior urethral valve. Hydroce
phalus, spina bifida, and anencephaly were the most common lesions obs
erved in the craniospinal axis. One-fifth of the children had complex
malformations of multiple organ systems, 20% of which were associated
with chromosomal anomalies, rubella, and other malformation syndromes.