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

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.

Objective: 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.

Conclusions: Absence of the portal vein and polysplenia syndrome is not a contraindication for liver transplantation although an increased post-operative morbidity may be expected.