Portal biliopathy(PB) is defined as the presence of biliary abnormalities in patients with non-cirrhotic/nonneoplastic extrahepatic portal vein obstruction(EHPVO) and portal cavernoma(PC). The pathogenesis of PB is du...Portal biliopathy(PB) is defined as the presence of biliary abnormalities in patients with non-cirrhotic/nonneoplastic 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.展开更多
Background: Portal biliopathy producing intrahepa- tic and extrahepatic biliary ductai abnormalities from portal hypertension, particularly with extrahepatic portal vein obstruction (EHPVO) is common. A majority of th...Background: Portal biliopathy producing intrahepa- tic and extrahepatic biliary ductai abnormalities from portal hypertension, particularly with extrahepatic portal vein obstruction (EHPVO) is common. A majority of these patients are asymptomatic, but oc- casionally there is symptomatic biliary obstruction, and cholangitis and choledocholithiasis. Objective: To explore the principles of diagnosis and treatment of portal biliopathy. Data sources: To review the literature of portal bili- opathy. Conclusions: Endoscopic sphincterotomy, stone ex- traction and supportive drainage could effectively re- lieve cholangitis when jaundice is associated with common bile duct stones. Definitive decompressive portal-system vein shunting operation and choledo- cho-jejunostomy are sometimes required when biliary obstruction is recurrent and progressive.展开更多
Aim: To discuss the etiology, pathogenesis, diagnosis and management of PB, supported by a series of 9 cases from a personal series. Methods: Radiological images of 9 cases of PB were retrieved from different hospital...Aim: To discuss the etiology, pathogenesis, diagnosis and management of PB, supported by a series of 9 cases from a personal series. Methods: Radiological images of 9 cases of PB were retrieved from different hospitals and studied and information relating to PB was obtained from various internet databases including PUB Med, Google, Google Scholar and Educus. Results: Portal biliopathy (PB) is a rare complication of extra-portal venous obstruction. Most patients remain asymptomatic, but some may present with raised alkaline phosphatase level, abdominal pain, fever, and cholangitis. It tends to be associated with gallbladder (GB) wall abnormalities, a cavernous transformation of the portal vein and choledochal varices. Extrinsic compression of the common bile duct, (CBD), by dilated venous collaterals together with pericholedochal fibrosis from the inflammatory process can cause portal thrombosis, which may lead to biliary stricture and dilatation of the proximal biliary tree. These strictures set the scene for formation biliary stones and cholangitis. Liver function test abnormalities are common but remain non-specific. Imaging features are instrumental in the diagnosis of PB. This paper presents 9 of such cases of portal biliopathy, with discussions on their causation, the course of the disease process and management particularly minimally invasive procedure. The role of ultrasound (US), computed tomography (CT), magnetic resonance imaging (MRI), endoscopic retrograde cholangiopancreatography (ERCP) and direct puncture cholangiography is discussed. Surgical management is evaluated. An imaging atlas is provided. Conclusions: PB is a rare but significant complication of portal venous obstruction that has an insidious onset and may remain asymptomatic until late. PB has associated coagulopathies, and a variety of other disorders, which are discussed, illustrated and the diagnosis-elaborated. The role of MRI as a non-invasive imaging tool is emphasized.展开更多
Choledochal varices are a rare cause of hemobilia associated with chronic portal vein thrombosis.We present a case of chronic portal vein thrombosis comp licated with bleeding from choledochal varices.The presentation...Choledochal varices are a rare cause of hemobilia associated with chronic portal vein thrombosis.We present a case of chronic portal vein thrombosis comp licated with bleeding from choledochal varices.The presentation,clinical manifestations and management are described.展开更多
Portal biliopathy(PB)is a rare disorder,characterized by biliary ductal and gallbladder wall abnormalities seen in patients with portal hypertension.It most commonly occurs due to idiopathic extrahepatic portal vein o...Portal biliopathy(PB)is a rare disorder,characterized by biliary ductal and gallbladder wall abnormalities seen in patients with portal hypertension.It most commonly occurs due to idiopathic extrahepatic portal vein obstruction(EHPVO).The abnormalities consist mainly of bile duct compression,stenoses,fibrotic strictures and dilation of both extrahepatic and intrahepatic bile ducts,as well as gallbladder varices.PB may mimic cholangiocarcinoma,sclerosing cholangitis,or choledocholithiasis.Misdiagnosis can be avoided using appropriate imaging modalities to prevent complications.We present the magnetic resonance imaging(MRI)and magnetic resonance cholangiography(MRCP)features of three patients with PB.展开更多
文摘Portal biliopathy(PB) is defined as the presence of biliary abnormalities in patients with non-cirrhotic/nonneoplastic 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.
文摘Background: Portal biliopathy producing intrahepa- tic and extrahepatic biliary ductai abnormalities from portal hypertension, particularly with extrahepatic portal vein obstruction (EHPVO) is common. A majority of these patients are asymptomatic, but oc- casionally there is symptomatic biliary obstruction, and cholangitis and choledocholithiasis. Objective: To explore the principles of diagnosis and treatment of portal biliopathy. Data sources: To review the literature of portal bili- opathy. Conclusions: Endoscopic sphincterotomy, stone ex- traction and supportive drainage could effectively re- lieve cholangitis when jaundice is associated with common bile duct stones. Definitive decompressive portal-system vein shunting operation and choledo- cho-jejunostomy are sometimes required when biliary obstruction is recurrent and progressive.
文摘Aim: To discuss the etiology, pathogenesis, diagnosis and management of PB, supported by a series of 9 cases from a personal series. Methods: Radiological images of 9 cases of PB were retrieved from different hospitals and studied and information relating to PB was obtained from various internet databases including PUB Med, Google, Google Scholar and Educus. Results: Portal biliopathy (PB) is a rare complication of extra-portal venous obstruction. Most patients remain asymptomatic, but some may present with raised alkaline phosphatase level, abdominal pain, fever, and cholangitis. It tends to be associated with gallbladder (GB) wall abnormalities, a cavernous transformation of the portal vein and choledochal varices. Extrinsic compression of the common bile duct, (CBD), by dilated venous collaterals together with pericholedochal fibrosis from the inflammatory process can cause portal thrombosis, which may lead to biliary stricture and dilatation of the proximal biliary tree. These strictures set the scene for formation biliary stones and cholangitis. Liver function test abnormalities are common but remain non-specific. Imaging features are instrumental in the diagnosis of PB. This paper presents 9 of such cases of portal biliopathy, with discussions on their causation, the course of the disease process and management particularly minimally invasive procedure. The role of ultrasound (US), computed tomography (CT), magnetic resonance imaging (MRI), endoscopic retrograde cholangiopancreatography (ERCP) and direct puncture cholangiography is discussed. Surgical management is evaluated. An imaging atlas is provided. Conclusions: PB is a rare but significant complication of portal venous obstruction that has an insidious onset and may remain asymptomatic until late. PB has associated coagulopathies, and a variety of other disorders, which are discussed, illustrated and the diagnosis-elaborated. The role of MRI as a non-invasive imaging tool is emphasized.
文摘Choledochal varices are a rare cause of hemobilia associated with chronic portal vein thrombosis.We present a case of chronic portal vein thrombosis comp licated with bleeding from choledochal varices.The presentation,clinical manifestations and management are described.
文摘Portal biliopathy(PB)is a rare disorder,characterized by biliary ductal and gallbladder wall abnormalities seen in patients with portal hypertension.It most commonly occurs due to idiopathic extrahepatic portal vein obstruction(EHPVO).The abnormalities consist mainly of bile duct compression,stenoses,fibrotic strictures and dilation of both extrahepatic and intrahepatic bile ducts,as well as gallbladder varices.PB may mimic cholangiocarcinoma,sclerosing cholangitis,or choledocholithiasis.Misdiagnosis can be avoided using appropriate imaging modalities to prevent complications.We present the magnetic resonance imaging(MRI)and magnetic resonance cholangiography(MRCP)features of three patients with PB.
