Portal vein thrombosis following hepatic portoenterostomy

Life-threatening ascites developed in a 4-month-old infant with biliary atresia 1 month after hepatic portoenterostomy. Although initially ascribed to progressive liver failure, the ascites was subsequently found to be partially caused by portal vein thrombosis. Documented recanalization of the portal venous system was accompanied by resolution of the ascites. Refractory ascites in patients with biliary atresia generally signals end-stage cirrhosis and consideration of liver transplantation. Portal vein patency should be evaluated before referral of such patients for this procedure.