Published online Dec 7, 2016. doi: 10.3748/wjg.v22.i45.9909
Peer-review started: August 29, 2016
First decision: September 20, 2016
Revised: October 12, 2016
Accepted: November 14, 2016
Article in press: November 16, 2016
Published online: December 7, 2016
Portal biliopathy (PB) is defined as the presence of biliary abnormalities in patients with non-cirrhotic/non-neoplastic extrahepatic portal vein obstruction (EHPVO) and portal cavernoma (PC). The pathogenesis of PB is due to ab extrinseco compression of bile ducts by PC and/or to ischemic damage secondary to an altered biliary vascularization in EHPVO and PC. Although asymptomatic biliary abnormalities can be frequently seen by magnetic resonance cholangiopancreatography in patients with PC (77%-100%), only a part of these (5%-38%) are symptomatic. Clinical presentation includes jaundice, cholangitis, cholecystitis, abdominal pain, and cholelithiasis. In this subset of patients is required a specific treatment. Different therapeutic approaches aimed to diminish portal hypertension and treat biliary strictures are available. In order to decompress PC, surgical porto-systemic shunt or transjugular intrahepatic porto-systemic shunt can be performed, and treatment on the biliary stenosis includes endoscopic (Endoscopic retrograde cholangiopancreatography with endoscopic sphincterotomy, balloon dilation, stone extraction, stent placement) and surgical (bilioenteric anastomosis, cholecystectomy) approaches. Definitive treatment of PB often requires multiple and combined interventions both on vascular and biliary system. Liver transplantation can be considered in patients with secondary biliary cirrhosis, recurrent cholangitis or unsuccessful control of portal hypertension.
Core tip: Portal biliopathy in patients with portal vein thrombosis and portal cavernoma can be symptomatic in about 5%-38% of patients. Therapy includes endoscopic and surgical approaches aimed to improve both portal hypertension and biliary alterations and clinical manifestation. Usually, multiple and combined treatments are required to resolve portal biliopathy.