Hyaline cartilage at porta hepatis in extrahepatic biliary atresia: metaplasia or choristoma.

We report a case of a 5-month-old child who presented with the features of obstructive jaundice, anaemia and had no associated congenital anomalies. The child was suspected to have biliary atresia on hepatobiliary iminodiacetic acid (HIDA) scan and underwent Kasai portoenterostomy. Microscopic examination of the atretic gallbladder revealed islands of mature hyaline cartilage, lymphoid aggregates and fibrosis. There was an abrupt transition between the cartilage and the adjacent stroma. Only rare case reports have documented this finding with hypothesis ranging from metaplasia occurring secondary to the associated chronic inflammation or whether it is a choristoma. These cases need to be documented to elucidate the pathways of occurrence and may lead to have a better and detailed understanding of the disease process.