Portal cavernoma cholangiopathy-clinical characteristics

Abstract

Because of the presence of portal cavernoma, paracholedochal and pericholedochal varices, portal cavernoma cholangiopathy (PCC) has become an entity unique to patients with extrahepatic portal venous obstruction (EHPVO). Majority of patients with these abnormalities are asymptomatic and are incidentally detected to have the presence of biliary abnormalities on cholangiography. Minority of patients present with symptoms of chronic cholestasis with or without biliary pain or acute cholangitis related most often to the presence of biliary strictures or stones. Other than the age of the patient and duration of EHPVO, presence of gall stones and common bile duct stones are other risk factors for the causation of symptoms in patients with PCC. This review summarizes the clinical characteristics of asymptomatic and symptomatic patients with PCC giving details of the prevalence of symptoms, their risk factors and overall burden of symptomatic PCC.

Keywords: CBD, common bile duct; EHPVO, extrahepatic portal venous obstruction; ERC, endoscopic retrograde cholangiography; MRCP, magnetic resonance cholangiography; NCPF, non-cirrhotic portal fibrosis; PCC, portal cavernoma cholangiopathy; PHB, portal hypertensive biliopathy; PVT, portal vein thrombosis; extrahepatic portal venous obstruction; portal biliopathy; portal hypertension.