OBJECTIVES: To establish risks for development of hepatocellular carcinoma (HCC) in children with biliary atresia (BA), the most common chronic liver disease of childhood. STUDY DESIGN: In our tertiary referral center database we have identified children with BA who had development of or have been incidentally found to have HCC. Their demographic, clinical, radiologic, and histologic features were analyzed. RESULTS: Between 1990 and 2008, 387 infants were diagnosed with BA at our center. Of these, three (0.8 %) who underwent operation at a median age of 68 (range 66 to 71) days had development of a histologically proven HCC detected at a median age of 2.1 (range 1.8 to 4.9) years. Another two, referred later, were diagnosed with HCC on their liver explants at ages 1.1 and 17.75 years, respectively. Overall, two had elevated serum levels of alpha-fetoprotein. All five children underwent successful liver transplantation at a median age of 2.1 years (range 1.1 to 17.75) and remain well after a median of 2.5 (range 2 to 5.7) years. CONCLUSION: HCC develops in a small percentage of children with BA. Serum alpha-fetoprotein levels and ultrasound screening are helpful but not absolute markers of the malignant change. In the absence of the extrahepatic involvement, liver transplantation represents an effective treatment.
OBJECTIVES: To establish risks for development of hepatocellular carcinoma (HCC) in children with biliary atresia (BA), the most common chronic liver disease of childhood. STUDY DESIGN: In our tertiary referral center database we have identified children with BA who had development of or have been incidentally found to have HCC. Their demographic, clinical, radiologic, and histologic features were analyzed. RESULTS: Between 1990 and 2008, 387 infants were diagnosed with BA at our center. Of these, three (0.8 %) who underwent operation at a median age of 68 (range 66 to 71) days had development of a histologically proven HCC detected at a median age of 2.1 (range 1.8 to 4.9) years. Another two, referred later, were diagnosed with HCC on their liver explants at ages 1.1 and 17.75 years, respectively. Overall, two had elevated serum levels of alpha-fetoprotein. All five children underwent successful liver transplantation at a median age of 2.1 years (range 1.1 to 17.75) and remain well after a median of 2.5 (range 2 to 5.7) years. CONCLUSION: HCC develops in a small percentage of children with BA. Serum alpha-fetoprotein levels and ultrasound screening are helpful but not absolute markers of the malignant change. In the absence of the extrahepatic involvement, liver transplantation represents an effective treatment.