Clinical Liver Disease | 2021
Portal Cavernoma Cholangiopathy: Indian Perspective
BaCKGrOUnD Portal cavernoma cholangiopathy (PCC) refers to the imaging, endoscopic, and clinically noted changes in the intrahepatic extrahepatic and biliary ducts and gallbladder (GB) abnormalities in patients with extrahepatic portal vein (PV) obstruction (EHPVO) with portal cavernoma formation. The portal cavernoma is formed as a result of recanalization of a thrombosed PV, which results in the formation of multiple collateral channels. PCC is seen in 80% to 100% of patients with EHPVO and is largely asymptomatic. The diagnosis is based on biliary tract imaging based on magnetic resonance (MR) imaging (MRI), such as MR cholangiography (MRC) or endoscopic retrograde cholangiography (ERC). Commonly noted are intrahepatic biliary radicle dilatation (IHBRD), smooth indentations, or impressions on the common bile duct (CBD), duct angulations, ectasias, strictures, stones, filling defects, and GB and pericholedochal varices. There are several synonyms for PCC, and multiple differential diagnoses need to be considered as shown in Fig. 1. Changes like PCC can be identified in cirrhosis (0%33%) and noncirrhotic portal hypertension (9%40%), which are denoted as “portal biliopathy,” but these changes are limited to the intrahepatic biliary radicles (IHBRs).