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Case Reports
. 2008 Jan-Feb;40(1):313-5.
doi: 10.1016/j.transproceed.2007.11.027.

Cadaveric liver transplantation in biliary atresia splenic malformation syndrome with the absence of retrohepatic inferior vena cava, preduodenal portal vein, and intestinal malrotation: a case report

E Sen-Oran  1 , Y Yankol, B Tuzun, B Kocak, T Kanmaz, K Acarli, M Kalayoglu
Affiliations
Case Reports

Cadaveric liver transplantation in biliary atresia splenic malformation syndrome with the absence of retrohepatic inferior vena cava, preduodenal portal vein, and intestinal malrotation: a case report

E Sen-Oran et al. Transplant Proc. 2008 Jan-Feb.

Abstract

A 9-month-old female infant with biliary atresia underwent cadaveric liver transplantation due to progressive cholestatic hepatitis following a Kasai operation. She had biliary atresia splenic malformation syndrome (BASM) composed of an absent retrohepatic inferior vena cava with an azygous connection, preduodenal portal vein, polysplenia, and intestinal malrotation. A portal vein thrombosis developed on the 4th postoperative day requiring immediate treatment by thrombectomy. The patient is well with normal liver function at 3 months follow-up. Although BASM may render the transplantation more difficult, the presence of BASM is no longer a contraindication to liver transplantation.

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