Document Detail


Pyloric atresia associated with epidermolysis bullosa: report of two cases and review of the literature.
MedLine Citation:
PMID:  22864723     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
The coexistence of pyloric atresia (PA) and epidermolysis bullosa (EB) is a rare but well-known surgical emergency in neonates. PA/EB is described by the association of atresia of the pylorus and bullous lesions on the skin. Ninetyone cases have been reported in the literature to date. We present two new cases and evaluate the association of PA/ EB, its etiopathogenesis and the clinical properties. Case 1: A three-day-old female presented with nonbilious vomiting and bullous lesions 2-3 cm in diameter on the extremities. Abdominal X-ray showed a single air-fluid level in the left upper quadrant. At laparotomy, we found PA and performed a pyloro-pylorostomy. The patient died due to sepsis complication of EB two months after surgery. Case 2: A two-day-old male presented with severe dermal bullous lesions on the trunk, neck and extremities. His stomach was dilated and there was no gas distally. We found PA and performed gastroduodenostomy. Initially, he tolerated the feeding well, but he died due to severe sepsis on the postoperative 23rd day. Almost all neonates born with the PA/EB result in a fatal outcome in the first few years. The complications related to EB are usually the cause of death. Even after successful repair of PA, skin lesions lead to death due to infection.
Authors:
Unal Bıçakcı; Burak Tander; Fatma Cakmak Çelik; Ender Arıtürk; Rıza Rızalar
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Ulusal travma ve acil cerrahi dergisi = Turkish journal of trauma & emergency surgery : TJTES     Volume:  18     ISSN:  1306-696X     ISO Abbreviation:  Ulus Travma Acil Cerrahi Derg     Publication Date:  2012 May 
Date Detail:
Created Date:  2012-08-06     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101274231     Medline TA:  Ulus Travma Acil Cerrahi Derg     Country:  Turkey    
Other Details:
Languages:  eng     Pagination:  271-3     Citation Subset:  IM    
Affiliation:
Department of Pediatric Surgery, Ondokuz Mayıs University Faculty of Medicine, Samsun, Turkey.
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