Article

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

Iranian Journal of Pediatrics 01/2007;
Source: DOAJ

ABSTRACT 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.

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