Portal cavernoma cholangiopathy: diagnosis, imaging, and intervention

Abstract

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.

CT imaging findings of abdominopelvic vascular compression syndromes: what the radiologist needs to know

“…Portal biliopathy is defined as an abnormal dilatation of the extra-and/or intrahepatic biliary ducts, as well as the cystic duct and gallbladder, due to the presence of chronically thrombosed portal system evolved into "portal cavernoma" [42][43][44]. Portal cavernoma (Fig.…”

“…18) is the cavernous transformation of the venous drainage of the common bile duct formed by the paracholedochal veins (plexus of Petren) and the epicholedochal veins (plexus of Saint), located along the ductal wall [45]. There are two mechanisms responsible for portal biliopathy: an outer mechanical compression of the biliary system by portal cavernous transformation and/or peribiliary fibrosis secondary to inflammatory or ischemic changes caused by thrombosis of small veins in the bile duct walls [44,46]. Indeed, portal biliopathy has been classified into three types: varicoid, fibrotic, and mixed.…”

“…About 70-100% of patients with radiological evidence of portal biliopathy are initially asymptomatic [44]. Portal biliopathy becomes clinically manifest only in a minority of patients with chronic cholestasis and jaundice [42,43,46].…”

Portal Cavernoma Mimicking Pancreatic Malignancy

“…Symptoms usually appear during late stages of portal hypertension, indicating long-term obstruction. [5][6][7] Portal cavernoma on sonography appears as an anechoic structure, described as a sponge-like mass. Magnetic resonance imaging with MRCP can detect the wavy appearance of the biliary duct related to the extrinsic compression by the portal cavernoma, which is a common finding.…”

“…Magnetic resonance imaging with MRCP can detect the wavy appearance of the biliary duct related to the extrinsic compression by the portal cavernoma, which is a common finding. 7 The biliary abnormalities in PCC can be caused by direct compression of the biliary tree by the portal cavernoma or by a large collateral vessel. These changes are welldefined for non-cirrhotic patients, in whom malignant causes have been excluded.…”