Primary liver transplantation for biliary atresia after percutaneous transhepatic cholangio-drainage

We herein report a case of primary living-donor liver transplantation (LDLT) for delayed diagnosis of biliary atresia under percutaneous transhepatic cholangio-drainage (PTCD). The case was a 10 months old female infant who was born in other Asian country and had been treated for cytomegalovirus hepatitis. After she moved to Japan at 9 months of age, she visited the referring hospital because of high fever. Severe jaundice and liver dysfunction were detected, and she was transferred to our hospital. Because of intrahepatic bile duct dilatation detected by computed tomography (CT), a PTCD tube was inserted to reduce jaundice, and liver biopsy was performed under general anesthesia. CT and pathologic findings led to the diagnosis of biliary atresia with liver cirrhosis. The patient was scheduled to undergo primary LDLT without Kasai portoenterostomy because of the progression of irreversible liver cirrhosis. While waiting for improvement of the severe fatty liver in her mother, a candidate donor, she underwent bile juice re-administration via the nasogastric tube. During this period, the total bilirubin did not increase above that at admission, and the albumin gradually improved. She underwent LDLT at 15 months of age, and her postoperative course was uneventful.