Portal cavernoma cholangiopathy: consensus statement of a working party of the Indian national association for study of the liver

Abstract

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

Keywords: CBD, common bile duct; CHD, common hepatic duct; CT, computed tomography; EHPVO, extrahepatic portal venous obstruction; ERC, endoscopic retrograde cholangiography; EUS, endoscopic ultrasound; GRADE, Grading of Recommendations, Assessment, Development and Evaluation; INASL, Indian National Association for Study of the Liver; MRC, magnetic resonance cholangiography; MRI, magnetic resonance imaging; NCPF, non-cirrhotic portal fibrosis; PSS, portosystemic shunt; PVT, portal vein thrombosis; UDCA, ursodeoxycholic acid; USG, ultrasound; cholestasis; extrahepatic portal venous obstruction; gallbladder varices; obstructive jaundice; portal hypertensive biliopathy.

Figure 1

(A) Endoscopic cholangiography shows common bile duct stricture (arrows). (B) MR cholangiography confirms common bile duct stricture seen on endoscopic cholangiography (arrows). (C) MR venography shows that common bile duct stenosis is caused by pressure from a large collateral. Blood vessels appear in white in flow sensitive sequence (large arrows).

Figure 2

Algorithm for the management of a patient with portal cavernoma cholangiopathy. EHPVO, extrahepatic portal venous obstruction; CBD, common bile duct; LFT, liver function tests; MR, magnetic resonance; PCC, portal cavernoma cholangiopathy; *EUS with Doppler for evaluation of biliary obstruction when other imaging modalities are unrevealing; †endoscopic treatment includes endoscopic sphincterotomy, stone extraction with or without the help of mechanical lithotripsy, plastic biliary stent placement, etc; ‡endoscopic treatment includes endoscopic sphincterotomy and stricture dilatation, followed by stone extraction with or without the help of mechanical lithotripsy. P1, phase 1; P2, phase 2 and P3, phase 3. (Modified from Dhiman RK, et al. Gut 2007;56:1001–8).