Congenital absence of the portal vein in liver transplantation for biliary atresia

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.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.Technical and post-operative problems related to absence of the portal vein and polysplenia are described and the literature reviewed.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.Absence of the portal vein and polysplenia syndrome is not a contraindication for liver transplantation although an increased post-operative morbidity may be expected.