Objectives: Surveillance of hepatic nodules for malignant transformation to hepatocellular carcinoma is important in the monitoring of patients with Biliary Atresia (BA). To-date, only two published case reports describe the finding of Hepatoblastoma (HB) in this setting. This study aimed to investigate this association of HB and BA, and to assess the utility of alpha-fetoprotein (aFP) as a marker in the diagnosis. Methods: A retrospective study of all patients who underwent isolated liver transplantation (LTx) for the primary diagnosis of BA at a single center, between January 1999 and June 2014, was conducted. Patient demographics, pre-LTx aFP levels and histologic examination of native liver explants were reviewed. Results: 102 (44% male, median age 11 months) patients underwent LTx for BA. Two (2%) explants examinations were confirmatory for a co-diagnosis of HB; both patients had abnormally elevated aFP. Overall, 56 (55%) patients had available pre-LTx aFP levels. Recipients with persistently abnormal aFP levels (n = 20, 36%) were older at hepatoportoenterostomy (107d vs. 68d, P = 0.02) and younger at LTx surgery (359d vs. 1713d, P
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