Portal Cavernoma Cholangiopathy: Consensus Statement of a Working Party of the Indian National Association for Study of the Liver

摘要: Portal cavernoma cholangiopathy (PCC) is defined as abnormalities in the extrahepatic biliary system including cystic duct and gallbladder with or without 1st 2nd generation ducts a patient portal cavernoma. Presence of cavernoma, typical cholangiographic changes on endoscopic magnetic resonance cholangiography absence other causes these like bile injury, primary sclerosing cholangitis, cholangiocarcinoma etc are mandatory to arrive diagnosis. Compression by porto-portal collateral veins involving paracholedochal epicholedochal venous plexuses cholecystic ischemic insult due deficient blood supply prolonged compression collaterals bring about changes. While former reversible after porto-systemic shunt surgery, latter not. Majority patients PCC asymptomatic approximately 21% symptomatic. Symptoms could be form long standing jaundice chronic cholestasis, pain cholangitis stones. Endoscopic retrograde has no diagnostic role because it invasive associated risk complications, hence reserved for therapeutic procedures. Magnetic portovenography noninvasive comprehensive imaging technique, modality choice mapping vascular patients. progressive condition symptoms develop late course hypertension only severe advanced cholangiopathy. Asymptomatic do not require any treatment. Treatment symptomatic can approached phased manner, coping first clearance nasobiliary stent placement acute sphincterotomy stone removal; second, decompression creating portosystemic shunt; third, persistent obstruction performing second-stage drainage surgery such hepaticojejunostomy choledochoduodenostomy. Patients have good prognosis successful surgery.