title: Therapeutic approaches for portal biliopathy: A systematic review.
abstract: 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 toab extrinsecocompression 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.
authors.affiliation: Irene Franceschet, Alberto Zanetto, Alberto Ferrarese, Patrizia Burra, Marco Senzolo, Gastroenterology Unit, Department of Surgery, Oncology and Gastroenterology, University Hospital of Padua, 35128 Padua, Italy.
authors.forename: Irene
authors.initials: I
authors.lastname: Franceschet
authors.affiliation: Irene Franceschet, Alberto Zanetto, Alberto Ferrarese, Patrizia Burra, Marco Senzolo, Gastroenterology Unit, Department of Surgery, Oncology and Gastroenterology, University Hospital of Padua, 35128 Padua, Italy.
authors.forename: Alberto
authors.initials: A
authors.lastname: Zanetto
authors.affiliation: Irene Franceschet, Alberto Zanetto, Alberto Ferrarese, Patrizia Burra, Marco Senzolo, Gastroenterology Unit, Department of Surgery, Oncology and Gastroenterology, University Hospital of Padua, 35128 Padua, Italy.
authors.forename: Alberto
authors.initials: A
authors.lastname: Ferrarese
authors.affiliation: Irene Franceschet, Alberto Zanetto, Alberto Ferrarese, Patrizia Burra, Marco Senzolo, Gastroenterology Unit, Department of Surgery, Oncology and Gastroenterology, University Hospital of Padua, 35128 Padua, Italy.
authors.forename: Patrizia
authors.initials: P
authors.lastname: Burra
authors.affiliation: Irene Franceschet, Alberto Zanetto, Alberto Ferrarese, Patrizia Burra, Marco Senzolo, Gastroenterology Unit, Department of Surgery, Oncology and Gastroenterology, University Hospital of Padua, 35128 Padua, Italy.
authors.forename: Marco
authors.initials: M
authors.lastname: Senzolo
mesh_terms: D015746:Abdominal Pain / Q000209:etiology; D001649:Bile Duct Diseases / Q000209:etiology / Q000628:therapy*; D002760:Cholangiopancreatography, Endoscopic Retrograde; D049448:Cholangiopancreatography, Magnetic Resonance; D002761:Cholangitis / Q000209:etiology; D002764:Cholecystitis / Q000209:etiology; D002769:Cholelithiasis / Q000209:etiology; D003251:Constriction, Pathologic; D006801:Humans; D006975:Hypertension, Portal / Q000150:complications / Q000628:therapy*; D041781:Jaundice, Obstructive / Q000209:etiology; D011169:Portal Vein / Q000002:abnormalities; D011170:Portasystemic Shunt, Surgical; D019168:Portasystemic Shunt, Transjugular Intrahepatic
keywords: Endoscopic retrograde cholangiopancreatography; Magnetic resonance cholangiopancreatography; Portal biliopathy; Portal cavernoma; Porto-systemic shunt