Hepatocellular carcinoma complicating biliary atresia after Kasai portoenterostomy

Kasai portoenterostomy (PE) increases the survival for children with biliary atresia (BA) and consequently postpones subsequential liver transplantation. All long-term survivors, however, develop complications of biliary cirrhosis. We report a case of hepatocellular carcinoma (HCC) in a 19-year-old male patient with BA and Kasai PE. The preoperative abdominal ultrasound and magnetic resonance imaging showed a large hepatic mass (diameter 10 cm). The serum α-fetoprotein level was within normal range. Pathologic findings of the mass, after orthotopic liver transplantation, demonstrated a well-differentiated HCC (T1N0M0). HCC is a rare complication of BA, but will intensively impair the survival. Therefore, clinicians should be alert to the development of HCC in this very young patient group. Repeated sequential magnetic resonance imaging of the native liver in patients with Kasai PE is necessary to monitor possible malignant transformation of liver nodules that may potentially develop as a result of chronic cholestatic liver disease.