Therapeutic approaches for portal biliopathy: A systematic review

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.

Diagnosis and treatment of portal biliopathy

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.

Portal Biliopathy: A Review of Imaging Features of Nine Patients

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.

Portal biliopathy,magnetic resonance imaging and magnetic resonance cholangiopancreatography findings:a case series

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.

How can portal vein cavernous transformation cause chronic incomplete biliary obstruction?

Biliary disease in the setting of non-cirrhotic portal vein thrombosis(and similarly in portal vein cavernous transformation) can become a serious problem during the evolution of disease.This is mostly due to portal biliary ductopathy.There are several mechanisms that play a role in the development of portal biliary ductopathy,such as induction of fibrosis in the biliary tract(due to direct action of dilated peribiliary collaterals and/or recurrent cholangitis),loss of biliary motility,chronic cholestasis(due to fibrosis or choledocholithiasis) and increased formation of cholelithiasis(due to various factors).The management of cholelithiasis in cases with portal vein cavernous transformation merits special attention.Because of a heterogeneous clinical presentation and concomitant pathophysiological changes that take place in biliary anatomy,diagnosis and therapy can become very complicated.Due to increased incidence and complications of cholelithiasis,standard treatment modalities like sphincterotomy or balloon sweeping of bile ducts can cause serious problems.Cholangitis,biliary strictures and hemobilia are the most common complications that occur during management of these patients.In this review,we specifically discuss important issues about bile stones related to bile duct obstruction in non-cirrhotic portal vein thrombosis and present evidence in the current literature.

Portal ductopathy:Clinical importance and nomenclature

Non-cirrhotic portal hypertension(PHT)accounts for about 20%of all PHT cases,portal vein thrombosis(PVT) resulting in cavernous transformation being the most common cause.All known complications of PHT may be encountered in patients with chronic PVT.However,the effect of this entity on the biliary tree and pancreatic duct has not yet been fully established.Additionally,a dispute remains regarding the nomenclature of common bile duct abnormalities which occur as a result of chronic PVT.Although many clinical reports have focused on biliary abnormalities,only a few have evaluated both the biliary and pancreatic ductal systems.In this review the relevant literature evaluating the effect of PVT on both ductal systems is discussed,and findings are considered with reference to results of a prominent center in Turkey,from which the term"portal ductopathy"has been put forth to replace"portal biliopathy".

Choledochal varices bleeding:A case report

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.