Esophageal atresia with pulmonary agenesis

Two neonates with esophageal atresia and agenesis of the right lung are described. Review of the literature on the subject revealed an additional 11 cases of esophageal atresia associated with severe pulmonary hypoplasia. All of the affected infants died. Half of the deaths were caused by congenital heart disease, and the others were due to prematurity combined with various other severe anomalies and respiratory complications.