Hepatolithiasis in a 52-year-old native liver survivor with postoperative biliary atresia

Hepatolithiasis is well known to be a late complication after biliary reconstruction. However, there were few reports regarding hepatolithiasis in late complications of long-term, native liver survivors in postoperative patients of biliary atresia. Here, we present a 52-year-old woman who underwent Kasai portoenterostomy for biliary atresia type Ⅲ at the age of 110 days. She was asymptomatic for over 30 years, and birthed three normal babies. Then she suffered from repeated cholangitis due to multiple hepatolithiasis since age 50. A lithotripsy via Roux-en-Y limb was performed but failed due to hepatic hilum strictures. Finally, she underwent living donor liver transplantation. Endoscopic lithotripsy and hepatectomy for hepatolithiasis often fail in biliary atresia patients due to unresolvable intrahepatic or hilar bile duct stenosis and liver dysfunction. Therefore, we consider that liver transplantation is curative and strongly recommended for symptomatic hepatolithiasis as a late complication after biliary atresia surgery.