Portal Cavernoma Cholangiopathy

Abstract

Portal cavernoma cholangiopathy is a rare condition, a proper recognition of which is critical. Solid data on this condition are scarce. This review aims at updating current knowledge on its definition, manifestations, diagnosis, and management. A consensus has been reached to prefer the denomination portal cavernoma cholangiopathy for the bile duct irregularities associated with portal cavernoma. Such irregularities are characterized by stenosis with or without dilatation and are mostly related to the impinging on bile duct lumen by portoportal collaterals. While bile duct irregularities are found in over 80% of patients with portal cavernoma, clinical manifestations, and complications (cholecystitis and biliary stones, but rarely cholestasis) occur in only 5–35% of them. Diagnosis can be and should be based on findings at magnetic resonance cholangiography and portography. Differential diagnosis includes primary and secondary sclerosing cholangitis, and cholangiocarcinoma. Asymptomatic patients may be managed expectantly. Endoscopic procedures are to be used in the first line for complications. Combined endoscopic and surgical approaches including portosystemic shunting can be considered in refractory or recurrent cases. Portal cavernoma cholangiopathy mostly is a morphologic entity which is present in a majority of patients with portal cavernoma. The minority of patients with acute biliary complications should be treated as conservatively as possible. Late consequences of chronic cholestasis appear to be rare.