Portal cavernoma cholangiopathy: diagnosis, imaging, and intervention

The term portal cavernoma cholangiopathy refers to the biliary tract abnormalities that accompany extrahepatic portal vein obstruction (EHPVO) and subsequent cavernous transformation of the portal vein. EHPVO is a primary vascular disorder of the portal vein in children and adults manifested by longstanding thrombosis of the main portal vein. Nearly all patients with EHPVO have manifestations of portal cavernoma cholangiopathy, such as extrinsic indentation on the bile duct and mild bile duct narrowing, but the majority are asymptomatic. However, progressive portal cavernoma cholangiopathy may lead to severe complications, including secondary biliary cirrhosis. A spectrum of changes is seen radiologically in the setting of portal cavernoma cholangiopathy, including extrinsic indentation of the bile ducts, bile duct stricturing, bile duct wall thickening, angulation and displacement of the extrahepatic bile duct, cholelithiasis, choledocholithiasis, and hepatolithiasis. Radiologists must be aware of this disorder in order to provide appropriate imaging evaluation and interpretation, to facilitate appropriate treatment and to distinguish this entity from its potential radiologic mimics.