= Abstract =

Congenital Pyloric Atresia with Junctional Epidermolysis Bullosa - a case report

Ma Hae Cho, M.D., Seok Joo Han, M.D.*

Department of Pediatric Surgery, College of Medicine, Ha llym Un iversity

Department of Pediatric Surgery, College of Medicine, Yonsei University*

Seoul, Korea

The association of pyloric atresia and epidermolysis bullosa(EB) in newborn is rare and inheritant as an autosomal recessive trait. We report a newborn girl with pyloric atresia and epidermolysis bullosa. Blisters were noted on her skin at birth, especially in pressure-exposed area, and later on the oral mucosa. Junc­tional epidermolysis bullosa was confirmed by light microscopy and electron mi­croscopy. Radiography revealed pyloric atresia. Segmental resection of 1.5 cm and gastroduodenostomy were carried out at 4 days of age. Protein loosing enteropathy developed after oral feeding. The frequency of episodes of nonscarred blisters and the severity and duration improved significantly with time. The protein loosing enteropathy was persistent, and at 1 year of age, her growth is markedly retarded.

Index Words: Pyloric atresia, Epidermolysis bullosa

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Fig. 1. Preoperative roentgenograph at 3 hours after birth reveals a single bubble of the stomach.

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Fig. 2. Photomicrograph of the transitional area(A) and denuded mucosa(B) at pylorus(H & E, x 40).

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Fig. 3. Junctional epidermolysis bullosa on the left cheek of the face.

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- 81 -

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