Purpose or Case Report: Ninety percent of biliary atresia cases occur with no associated anomalies. Ten percent of cases of biliary atresia are considered syndromic, occurring in association with a variety of congenital anomalies including heterotaxy, polysplenia, asplenia, congenital cardiac defects, intestinal malrotation, interrupted IVC, hepatic artery anomalies, and portal vein anomalies, including pre-duodenal portal vein. The incidence of hepatopulmonary syndrome is also increased in this group. Images from CT, MR, radiography, nuclear medicine, ultrasound and fluoroscopy will illustrate the variety of imaging appearances of the biliary atresia/splenic malformation syndrome.
Methods & Materials: After obtaining approval from our Institutional Review Board we retrospectively reviewed the imaging and clinical presentation of 16 pediatric patients with biliary atresia and splenic malformations.
Results:
Conclusions: Pediatric radiologists should be aware of the association of biliary atresia and splenic malformations, and should be suspicious for biliary atresia in the presence of liver malfunction or jaundice in patients with splenic abnormalities. Additional structural abnormalities such as malrotation and vascular anomalies should be investigated as they may complicate surgical interventions such as Kasai procedure or liver transplant.