Endoscopic retrograde cholangiography in portal cavernoma cholangiopathy - results from different studies and...

Portal cavernoma cholangiopathy (PCC) refers to a constellation of secondary changes in the biliary tree in patients with chronic portal vein (PV) thrombosis and portal cavernoma formation. These findings of PCC are seen in the extra-hepatic bile duct(s), with or without involvement of the 1st or 2nd degree intra-hepatic bile ducts. Of all patients with chronic PV thrombosis, cholangiographic features of PCC are found in 80%-100%. The biliary changes are symptomatic in a smaller proportion of 5%-38% patients. Choledocholithiasis and hepatolithiasis occur in 5%-20%, independent of the occurrence of cholelithiasis. We review the published literature on cholangiographic description of PCC. We also propose standardized nomenclature for the cholangiographic findings, namely: extrinsic impressions/indentations, shallow impressions, irregular ductal contour, stricture (s), upstream dilatation, filling defects, bile duct angulation, and ectasia.