1986 Volume 53 Issue 4 Pages 409-415
Congenital pyloric atresia is a rare malformation that occurs with a frequency of about one per million births. One hundred fifty-one cases of the anomaly have been reported including 28 cases in Japan. This is a report on an additional case of membranous atresia that was treated successfully by incision of the diaphragm with pyloroplasty. The familial occurrence in siblings and the association of the abnormality with hereditary epidermolysis bullosa suggests a genetic etiology. Treatment should be surgical and the choice of an operative procedure should depend on the anatomical type of atresia. Excision or incision of the diaphragm with pyloroplasty and gastroduodenostomy offer the most promising opportunity for successful treatment. The mortality rate is 49.3% and the prognosis is dependent upon such factors as early diagnosis, the appropriate operative procedure, the extent of prematurity of the infant and association of other congenital anomalies or complications.