3 research outputs found
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 all intestinal atresias, and its
incidence is approximately 1 in 100,000 live births. PA may occur as an
isolated condition or associated with other abnormalities, the most
common being Junctional epidermolysis bullosa (EB). Evidence suggests
that PA-EB is a distinct entity. In this report, we present 5 cases of
pyloric atresia associated with Junctional epidermolysis bullosa, 4 of
whom survived after surgery. Cases Presentation: Prospective evaluation
of 5 patients with pyloric atresia associated with Epidermolysis
bullosa undergoing therapeutic surgery. Biopsy of the fresh bulla was
compatible with Junctional EB in all 5 patients. All patients underwent
laparatomy after stabilization. Four neonates underwent
gastroduodenostomy, and one patient had excision of membrane and
pyloroplasty. Out of 5 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 have been
encouraging reports of survival among these patients. These 5 patients
underwent surgery and survived, and are doing well on follow up