Abnormality of gastric outlet development. An autosomal-recessive genetic determination is probable in familial occurrences. Epidermolysis bullosa, aplasia cutis congenita, esophageal atresia, omphalocele. Esophageal atresia and duodenal atresia. Good in isolated pyloric atresia.
Mortality is due to association with epidermolysis bullosa letalis.
We know of only two cases in which pyloric atresia was diagnosed prenatally. Her past obstetrical history included a primary cesarean section for dystocia. Large dilatation of the stomach on a transverse view. The diaphragm and other abdominal organs appeared normal. A longitudinal view of the chest showed a thin, irregular tube, full of a clear fluid that seemed to be connected to the stomach.