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  • Title: 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.
    Author: Sen-Oran E, Yankol Y, Tuzun B, Kocak B, Kanmaz T, Acarli K, Kalayoglu M.
    Journal: Transplant Proc; 2008; 40(1):313-5. PubMed ID: 18261615.
    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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