Advances in pediatric surgery

Korean Association of Pediatric Surgeons (대한소아외과학회)
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Congenital Pyloric Atresia with Junctional Epidermolysis Bullosa-a case report

수포성 표피박리증을 동반한 선천성 유문부폐쇄증 1예

  • Cho, Ma-Hae (Department of Pediatric Surgery, College of Medicine, Hallym University) ;
  • Han, Seok-Joo (Department of Pediatric Surgery, College of Medicine, Yonsei University)
  • 조마해 (한림대학교 의과대학 외과학교실) ;
  • 한석주 (연세대학교 의과대학 외과학교실)
  • Published : 1997.06.30
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Abstract

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.

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