Purpose or Case Report: Biliary atresia is an inflammatory cholangiopathy of the neonatal period that may lead to future fibrosis and obliteration of both intra and extrahepatic biliary ducts. It is an uncommon disease that can be further classified by morphology. The rare, atypical Type IIA variant results in absence of the common hepatic duct, but patent cystic and common bile duct, occasionally accompanied by a hilar cyst (so-called “cystic biliary atresia”) or otherwise normal-appearing gallbladder.

In this educational case exhibit, we present a premature, former 31-week newborn with neonatal hyperbilirubinemia who was ultimately diagnosed with a type IIA biliary atresia and treated with the Kasai procedure. Abdominal ultrasound showed a contracted gallbladder, and subsequent HIDA scans showed no radiotracer excretion into the common bile duct or small bowel. Surgery and GI were consulted, and the patient underwent the Kasai procedure. The intraoperative cholangiogram confirmed type IIA biliary atresia and postoperatively there was a general downward trend of transaminases and direct bilirubin. In our discussion, we will be reviewing this patient’s uncommon morphology of biliary atresia, key radiologic findings and surgical- pathologic correlation, along with subsequent care and management for this condition.
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