Journal
JOURNAL OF PEDIATRIC SURGERY
Volume 39, Issue 1, Pages -Publisher
W B SAUNDERS CO
DOI: 10.1016/j.jpedsurg.2003.09.044
Keywords
extrahepatic biliary atresia; caudal regression syndrome; vertebral agenesis; infant of diabetic mother; biliary atresia splenic malformation
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Although most cases of extrahepatic biliary atresia are thought to result from perinatal obliterating inflammation, some are associated with a faulty morphogenesis. The authors report on a baby girl of a mother with ill-managed insulin-dependent diabetes mellitus. The baby presented with sacro-coccygeal agenesis, clubfoot, and ano-urinary incontinence. In addition, there was polysplenia, no inferior vena cava, and the portal vein was grossly distorted. Progressive conjugated hyperbilirubinemia prompted liver biopsy at 4 weeks of age, showing intracanalicular cholestasis without fibrosis. The diagnosis of extrahepatic biliary atresia was confirmed during laparotomy performed for hepatoportojejunostomy. Some cases of extrahepatic biliary atresia might be part of a spectrum of malformations associated with maternal diabetes mellitus. (C) 2004 Elsevier Inc. All rights reserved.
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