Biliary atresia and noncardiac polysplenic syndrome: US and surgical considerations

Ten percent of children with biliary atresia have an associated complex of anomalies, including polysplenia, azygous continuation of the inferior vena cava, preduodenal portal vein, hepatic arterial anomalies, and bilaterally bilobed lungs. These abnormalities will not be detected if the preoperative workup is limited to hepatobiliary nuclear scanning. Ultrasonography is important in the preoperative evaluation of patients suspected of having biliary atresia. It is important to identify the associated abnormalities preoperatively because they have an impact on the initial portoenterostomy and may preclude subsequent orthotopic liver transplantation.