Right esophageal lung in a preterm child with VACTERL association and Mayer-Rokitansky-Kuster-Hauser syndrome

We report on a preterm girl (birth weight 1,200 g) with a right esophageal lung in esophageal atresia type VIag (according to the extended classification of Kluth). Additionally, the child suffered from an atrioseptal defect, a dextrocardia with a left descending aorta, a duodenal atresia, a high type of anal atresia (VACTERL association), agenesis of the left kidney, and agenesis of the vagina, uterus, and ovarian tubes (Mayer-Rokitansky-Kuster-Hauser syndrome or incomplete MURCS association). The child was treated with an emergency gastrostomy because of increasing abdominal dilatation. Thereafter, the parents refused further surgical treatment, and the child was maintained on basic therapy. After an uneventful period of 4 weeks, the child died of an acute massive aspiration. This case shows that sufficient spontaneous ventilation is possible in esophageal lung as long as a gastrostomy is kept on suction to prevent overinflation of the affected lung and the stomach. Ethical aspects have to be considered when treatment is planned in cases of prematurity and associated malformations when a chance of good survival is rather limited. The stepwise approach as proposed in the present case appears to be the only possible therapeutic regimen that can be offered in this complicated condition.