Portal Cavernoma Cholangiopathy (PCC) occurs in a patient with portal vein thrombosis and cavernous transformation of the portal vein (cavernoma).
Criteria:
(1) extrahepatic portal vein obstruction (EHPVO), usually due to thrombosis
(2) cavernous transformation (cavernoma)
(3) biliary tract abnormalities in the region of, and proximal to, the cavernoma
Biliary tract abnormalities include:
(1) extrinsic compression of the bile duct with scalloping
(2) bile duct narrowing or stricture formation
(3) dilatation of intrahepatic ducts
(4) bile duct obstruction with secondary biliary cirrhosis
(5) bile wall thickening
(6) angulation and displacement
(7) choledocholithiasis and hepatolithiasis
(8) pericholedochal varices
Most patients are asymptomatic. A few patients may be symptomatic from cholestasis.
Differential diagnosis:
(1) cholangiocarcinoma
