26-Year-Old Survivor of Kasai Procedure With Native Liver

A 26-year old woman with history of congenital biliary atresia and Kasai procedure as a newborn presented with recurrent nausea and abdominal pain. Physical examination showed anicteric sclera and tender right upper quadrant. Elevated liver function tests suggested obstruction and hepatitis. Percutaneous transhepatic cholangiogram showed generalized ductal dilation, innumerable intrahepatic bile duct stones, and multiple filling defects. No anastomotic stenosis was noted. The patient was treated for acute symptoms and referred to a liver transplant center. Long-term survivors of the Kasai procedure with a native liver are rare, and this represents the third known U.S. case in the literature.