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Pyloric atresia associated with epidermolysis bullosa, malrotation, and high anorectal malformation with recto-urethral fistula: a report of successful management
Journal article   Peer reviewed

Pyloric atresia associated with epidermolysis bullosa, malrotation, and high anorectal malformation with recto-urethral fistula: a report of successful management

S Agarwala, J K Goswami and D K Mitra
Pediatric surgery international, Vol.15(3-4), pp.264-265
01/05/1999
DOI: https://doi.org/10.1007/s003830050572
PMID: 10370040

Abstract

Anus, Imperforate - complications Epidermolysis Bullosa, Junctional - complications Humans Infant, Newborn Intestines - abnormalities Male Pylorus - abnormalities Rectal Fistula - complications Urethral Diseases - complications Urinary Fistula - complications
Pyloric atresia (PA) is an uncommon anomaly that may be associated with many other congenital anomalies, the commonest of which is junctional epidermolysis bullosa (JEB). Most of the cases of PA associated with JEB (Herlitz syndrome) reported have been fatal. A case of PA associated with JEB, malrotation, and a high anorectal malformation with a rectourethral fistula, which was hitherto undescribed, was successfully managed at our institution.

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