Pyloric Atresia Associated with Epidermolysis Bullosa:A Report of 4 Survivals in 5 Cases

Objective: pyloric atresia (pa) is a rare congenital anomaly that constitutes approximately 1% of allintestinal atresias, and its incidence is approximately 1 in 100,000 live births. pa may occur as anisolated condition or associated with other abnormalities, the most common being junctionalepidermolysis bullosa (eb). evidence suggests that pa-eb is a distinct entity. in this report, wepresent 5 cases of pyloric atresia associated with junctional epidermolysis bullosa, 4 of whomsurvived after surgery.cases presentation: prospective evaluation of 5 patients with pyloric atresia associated withepidermolysis bullosa undergoing therapeutic surgery. biopsy of the fresh bulla was compatible withjunctional eb in all 5 patients. all patients underwent laparatomy after stabilization. four neonatesunderwent gastroduodenostomy, and one patient had excision of membrane and pyloroplasty. out of5 neonates, 4 survive and one died from fulminant septicemia 12 days after operation.conclusion: although association of pa with eb has been reported to be fatal, recently there havebeen encouraging reports of survival among these patients. these 5 patients underwent surgery andsurvived, and are doing well on follow up.