Endoscopic management of portal cavernoma cholangiopathy: practice, principles and strategy.

Portal cavernoma cholangiopathy (PCC) is the presence of typical cholangiographic changes in patients with a portal cavernoma due to chronic portal vein thrombosis, in the absence of other biliary tract diseases. Probably due to biliary stasis related to the cavernoma, there is a high incidence of biliary sludge and calculi in PCC, which trigger symptoms that resolve with appropriate interventions. Persistent and troublesome symptoms are usually due to biliary stenoses or strictures, which may occur with or without biliary calculi and may be short or long, solitary or multifocal, extrahepatic or intrahepatic. Experience with endoscopic interventions in PCC over the last twenty years has shown that it is the procedure of choice for bile duct calculi. Plastic stenting with repeated, timely, stent exchanges is the first line intervention for jaundice or cholangitis due to biliary strictures. If biliary obstruction does not resolve, portosystemic shunt surgery (PSS) or transjugular intrahepatic portosystemic stent shunt (TIPS) is performed to decompress the portal cavernoma. However, for patients with non-shuntable veins or blocked shunts, repeated plastic stent exchanges are the only option though there are reports of the use of biliary self-expandable metal stents in this situation. If symptomatic biliary obstruction persists after successful PSS or TIPS, second stage biliary surgery may be necessary. Recent experience suggests that treating biliary strictures in PCC on the lines of postoperative benign biliary strictures with balloon dilatation and repeated exchanges of plastic stent bundles may be effective therapy. Endoscopic management appears to be associated with an increased frequency of hemobilia, which usually responds to standard management. Recurrent cholangitis with formation of sludge and concretions may be a problem with repeated stent exchanges, especially if patient compliance is poor. In conclusion, the current understanding is that symptomatic PCC is best managed jointly by the endoscopist and surgeon with sequential interventions designed initially to establish and maintain biliary drainage, then to decompress the portal cavernoma and, finally, if required, second stage biliary surgery or endotherapy for biliary strictures. Endoscopic therapy occupies a central role in management before, during and after surgical therapy. Paradigms of endoscopic therapy continue to evolve as knowledge of pathogenesis and natural history improves and newer approaches and techniques are applied.