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  • Title: Congenital absence of the portal vein in liver transplantation for biliary atresia.
    Author: Andreani P, Srinivasan P, Ball CS, Heaton ND, Rela M.
    Journal: Int J Surg Investig; 2000; 2(1):81-4. PubMed ID: 12774342.
    Abstract:
    BACKGROUND: Extrahepatic biliary atresia is the most common indication for liver transplantation in children. Coexistent congenital anomalies are common (25%), but the combination of polysplenia syndrome (10%) and absence of portal vein is rare. AIMS: We report a case of successful reduced size liver transplantation on a 13-month-old girl with extrahepatic biliary atresia, polysplenia syndrome and congenital absence of the portal vein. METHODS: Technical and post-operative problems related to absence of the portal vein and polysplenia are described and the literature reviewed. RESULTS: The donor portal vein was successfully anastomosed to the confluence of the superior mesenteric vein and splenic vein. The child is well with good graft function at a follow-up of 20 months. CONCLUSION: Absence of the portal vein and polysplenia syndrome is not a contraindication for liver transplantation although an increased post-operative morbidity may be expected.
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