The term portal cavernoma cholangiopathy refers to the biliary tract abnormalities that accompany extrahepatic portal vein obstruction (EHPVO) and subsequent cavernous transformation of the portal vein. EHPVO is a primary vascular disorder of the portal vein in children and adults manifested by longstanding thrombosis of the main portal vein. Nearly all patients with EHPVO have manifestations of portal cavernoma cholangiopathy, such as extrinsic indentation on the bile duct and mild bile duct narrowing, but the majority are asymptomatic. However, progressive portal cavernoma cholangiopathy may lead to severe complications, including secondary biliary cirrhosis. A spectrum of changes is seen radiologically in the setting of portal cavernoma cholangiopathy, including extrinsic indentation of the bile ducts, bile duct stricturing, bile duct wall thickening, angulation and displacement of the extrahepatic bile duct, cholelithiasis, choledocholithiasis, and hepatolithiasis. Radiologists must be aware of this disorder in order to provide appropriate imaging evaluation and interpretation, to facilitate appropriate treatment and to distinguish this entity from its potential radiologic mimics.

Supra-mesocolic surgery (SMS) is complicated in patients with portal vein cavernoma (PC) and portal decompression is recommended. The aim of this study was to report a large single centre of SMS in patients with PC without portal decompression.

Between 2006 and 2013, all patients who met inclusion criteria were analyzed retrospectively. The primary endpoint was the feasibility rate, surgical and postoperative outcome. The secondary endpoints were the long-term outcome of patients who underwent biliary bypass for cholangitis. Risk factors for complications were studied.

Thirty patients underwent 51 procedures. Pancreatitis was the main etiology of PC (19/30) and biliary obstruction was mainly related to the underlying disease and not to portal cholangiopathy (12/14). All planned procedures were successfully completed. Fourteen patients underwent biliary bypass. Median blood loss (250 ml), transfusion (n = 7), mortality (n = 0), overall morbidity (n = 12) and the median hospital stay (10 days). Good long-term control of cholangitis was achieved in the 9 patients alive with available follow-up. Significant risk factors for complications were a previous abdominal wall scar, previous intra-abdominal surgical field and liver fibrosis.

SMS can be safely performed in patients with PC. In patients with risk factors for complications, portal decompression should be discussed.

Portal biliopathy (PB) refers to the biliary abnormalities of the biliary ducts observed in patients with extrahepatic portal hypertension. Although majority of patients are asymptomatic, approximately 20% of these patients present with biliary symptoms (pain, pruritus, jaundice, cholangitis). The pathogenesis of PB is uncertain but compression by dilated veins into or around common bile duct may play the main role. CT-scan, MR cholangiopancreatography with MR portography should be the initial investigations in the evaluation of PB. Treatment is limited to symptomatic cases and is dictated by clinical manifestations and complications of the disease. Treatment of PB could be done by endoscopy (sphincterotomy, stone extraction or biliary stenting of the common bile duct) or surgery (definitive decompression by porto-systemic shunt followed by bilioenteric anastomosis, if necessary). This review describes pathogenesis, clinical features, investigation and management of portal biliopathy.

The term, portal biliopathy, denotes various biliary abnormalities, such as stenosis and/or dilatation of the bile duct, in patients with portal hypertension. These vascular abnormalities sometimes bring on an obstructive jaundice, but they are not clear which vessels participated in obstructive jaundice. The aim of present study was clear the bile ductal changes in patients with portal hypertension in hopes of establishing a therapeutic strategy for obstructive jaundice caused by biliary varices.

Three hundred and thirty-seven patients who underwent intraductal ultrasound (IDUS) during endoscopic retrograde cholangiography for biliary abnormalities were enrolled. Portal biliopathy was analyzed using IDUS.

Biliary varices were identified in 11 (2.7%) patients. IDUS revealed biliary varices as multiple, hypoechoic features surrounding the bile duct wall. These varices could be categorized into one of two groups according to their location in the sectional image of bile duct: epicholedochal and paracholedochal. Epicholedochal varices were identified in all patients, but paracholedochal varices were observed only in patients with extrahepatic portal obstruction.

IDUS was useful to characterize the anatomy of portal biliopathy in detail.

Portal vein obstruction may be due to portal vein thrombosis (PVT) or its sequale, the portal cavernoma. PVT is a common complication in liver cirrhosis, however, it may also occur as a primary vascular disorder, in absence of any liver disease.

To review the current knowledge on nomenclature, etiology, pathophysiology, clinical presentation, diagnostic workup and management of adult patients with obstruction in the portal vein, either as a primary vascular disease in adults, or as a complication of liver cirrhosis.

A structured search in PubMed was performed using defined keywords (portal vein obstruction, extra-hepatic portal vein obstruction, PVT and portal cavernoma), including full text articles and abstracts in English language.

Several causes, operating both at local and systemic level, might play an important role in the pathogenesis of PVT. Frequently, more than one risk factor could be identified; however, occasionally no single factor is discernible. Diagnosis of portal vein obstruction depends on clinical presentation, imaging and laboratory investigations. Prompt treatment greatly affects the patient's outcome.

Portal vein obstruction occurring either due to thrombosis in the portal vein or due to the portal cavernoma, can contribute to significant morbidity and mortality in patients with or without cirrhosis. In recent years our understanding of etio-pathogenesis of portal vein obstruction has evolved tremendously, which has led to significant improvement in treatment outcomes. There are still areas where more studies are needed to better clarify the management issues of portal vein obstruction.

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.

Secondary sclerosing cholangitis (SSC) is an aggressive and rare disease with intricate pathogenesis and multiple causes. Understanding the specific cause underlying each case of SSC is crucial in the clinical management of the disease. Radiologic imaging can help diagnose SSC and hence institute management in a timely manner. Management may encompass simple interventions, such as supportive therapy, antibiotics, and monitoring, or more serious measures, such as surgery, endoscopic intervention, or liver transplantation. Patients with AIDS cholangiopathy have limited therapeutic options and worsened survival. The disease should always be highly suspected in patients with primary sclerosing cholangitis with questionable diagnosis.

Portal biliopathy (PBP) denotes intra- and extrahepatic biliary duct abnormalities that occur as a result of portal hypertension and is commonly seen in extrahepatic portal vein obstruction (EHPVO). The management of symptomatic PBP is still controversial.

Prospectively collected data for surgically managed PBP patients from 1996 to 2007 were retrospectively analysed for presentation, clinical features, imaging and the results of surgery. All patients were assessed with a view to performing decompressive shunt surgery as a first-stage procedure and biliary drainage as a second stage-procedure if required, based on evaluation at 6 weeks after shunt surgery.

A total of 39 patients (27 males, mean age 29.56 years) with symptomatic PBP were managed surgically. Jaundice was the most common symptom. Two patients in whom shunt surgery was unsuitable underwent a biliary drainage procedure. A total of 37 patients required a proximal splenorenal shunt as first-stage surgery. Of these, only 13 patients required second-stage surgery. Biliary drainage procedures (hepaticojejunostomy [n= 11], choledochoduodenostomy [n= 1]) were performed in 12 patients with dominant strictures and choledocholithiasis. One patient had successful endoscopic clearance of common bile duct (CBD) stones after first-stage surgery and required only cholecystectomy as a second-stage procedure. The average perioperative blood product transfusion requirement in second-stage surgery was 0.9 units and postoperative complications were minimal with no mortality. Over a mean follow-up of 32.2 months, all patients were asymptomatic. Decompressive shunt surgery alone relieved biliary obstruction in 24 of 37 patients (64.9%) and facilitated a safe second-stage biliary decompressive procedure in the remaining 13 patients (35.1%).

Decompressive shunt surgery alone relieves biliary obstruction in the majority of patients with symptomatic PBP and facilitates endoscopic or surgical management in patients who require second-stage management of biliary obstruction.

The association of cholelithiasis and portal cavernoma is rarely described in adult or pediatric patients. We report 2 cases of gallstone associated with portal cavernoma in 2 girls. The first one suffered from Evans syndrome associated with congenital immune deficiency. The portal cavernoma was discovered with gallstone after splenectomy indicated because of high steroid dependence. In the second case, the cavernoma complicated neonatal umbilical catheterism. The gallstone was asymptomatic and discovered on annual ultrasonography. Septicemia, profound thrombocytopenia, and acute anaemia led to rapid death in the first case. However, the progression was favourable under celioscopic treatment in the second one. Our original observations suggest systematically searching for gallstone in children with portal cavernoma.

Portal vein thrombosis (PVT) is an important cause of portal hypertension. It may occur as such with or without associated cirrhosis and hepatocellular carcinoma. Information on its management is scanty.

To provide an update on the modern management of portal vein thrombosis. Information on portal vein thrombosis in patients with and without cirrhosis and hepatocellular carcinoma is also updated.

A pubmed search was performed to identify the literature using search items portal vein thrombosis-aetiology and treatment and portal vein thrombosis in cirrhosis and hepatocellular carcinoma.

Portal vein thrombosis occurs because of local inflammatory conditions in the abdomen and prothrombotic factors. Acute portal vein thrombosis is usually symptomatic when associated with cirrhosis and/or superior mesenteric vein thrombosis. Anticoagulation should be given for 3-6 months if detected early. If prothrombotic factors are identified, anticoagulation should be given lifelong. Chronic portal vein thrombosis usually presents with well tolerated upper gastrointestinal bleed. It is diagnosed by imaging, which demonstrates a portal cavernoma in place of a portal vein. Anticoagulation does not have a definite role, but bleeds can be treated with endotherapy or shunt surgery. Rarely liver transplantation may be considered.

Role of anticoagulation in chronic portal vein thrombosis needs to be further studied.

Portal biliopathy is a late and serious complication of extrahepatic portal venous obstruction usually manifesting with jaundice. Surgery and endoscopic therapy are the usual modalities of treatment for this condition. Endoscopic management contains inherited risk of hemobilia treatment of which is yet to be standardized.

Retrospective analysis of data from 2002 to 2007 for nonsurgical management of portal biliopathy was carried out. We encountered 4 cases of hemobilia during this period. The management and outcome of these 4 patients was analyzed.

Median age at presentation was 39 years (22 to 50 y). All the patients had cholestatic jaundice and pain as presenting symptoms without prior history of gastrointestinal bleed. The median serum bilirubin and alkaline phosphatase values were 5 mg/dL (4.8 to 11.3 mg/dL ) and 494 IU/mL (342 to 645 IU/mL), respectively. Endoscopic retrograde cholangiography documented changes of portal biliopathy along with choledocholithiasis in all the 4 patients. An uneventful endoscopic sphincterotomy was followed by significant hemobilia during attempted stone extraction by Dormia basket/balloon. Patients were resuscitated with standard measures and injection terlipressin was started at a dose of 1 mg 4 times daily. Control of bleeding was achieved within 12 hours of infusion in all 4 patients and there was no bleed-related mortality.

All our patients had symptomatic portal biliopathy as their first manifestation of underlying extrahepatic portal venous obstruction. Common bile duct stone extraction in patients with portal biliopathy carries a high risk of hemobilia even with balloon sweeping. Terlipressin is an effective pharmacologic treatment for hemobilia in patients with portal biliopathy.

Biliary obstruction as a consequence of portal biliopathy, because of extrahepatic portal vein occlusion is an uncommon cause of biliary disease in the western world. We reviewed all patients presenting to the Regional Liver Transplant Unit in Birmingham, UK with symptomatic portal biliopathy between 1992 and 2005 and report the presentation, investigation, management and outcome of these complex patients.

Thirteen patients with symptomatic portal biliopathy were followed up for a median of 2 years (range 1-18 years). Jaundice was the presenting feature in all cases and was associated with bile duct stones or debris in 77% (10 of 13) of cases. Successful treatment of biliary problems was achieved by biliary decompression in six cases (metallic stent=three, plastic stent=one, combined procedure=one and sphincterectomy=one) and portal decompression in three cases (transjugular intrahepatic portosystemic shunt=two, meso-caval shunt=one). Successful biliary drainage could not be achieved endoscopically or by portal decompression in one case that was accepted for combined liver and small bowel transplantation. Three patients had spontaneous resolution without recurrence over the follow-up period. Ten patients (77%) experienced gastrointestinal bleeding. Two deaths over the follow-up period occurred; both were associated with portal hypertensive bleeding.

Endoscopic management (sphincterectomy and stone extraction or stent insertion) is effective initial therapy for patients with symptomatic portal biliopathy. In the case of persistent biliary obstruction porto-systemic shunting (transjugular intrahepatic portosystemic shunt or surgical) should be considered, however, the extent of vascular thrombosis precludes this in most cases.

Chronic portal obstruction can lead to formation of portal cavernoma (PC). Half of all patients with PC will develop cholestasis, termed portal biliopathy, and some will progress to symptomatic biliary obstruction. Because of the high hemorrhage risk associated with biliary surgery in patients with PC, the optimal therapeutic strategy is controversial.

Retrospective review of a single hepatobiliary center experience, including 64 patients with PC identified 19 patients with concurrent symptomatic biliary obstruction. Ten patients underwent initial treatment with a retroperitoneal splenorenal anastomosis. For the remaining 9 patients, portal biliopathy was managed without portosystemic shunting (PSS). Outcomes, including symptom relief, the number of biliary interventions, and survivals, were studied in these 2 groups.

Within 3 months of PSS, 7 of 10 patients (70%) experienced a reduction in biliary obstructive symptoms. Five of these 10 patients subsequently underwent uncomplicated biliary bypass, and none has recurred with biliary symptoms or required biliary intervention with a mean follow-up of 8.2 years. For patients without PSS, repeated percutaneous and endobiliary procedures were required to relieve biliary symptoms. Four of the 9 patients with persistent PC required surgical intrahepatic biliary bypass, which was technically more challenging. With a mean follow-up of 8 years, 1 of these 9 patients died of severe cholangitis, 1 remained jaundiced, and 7 were asymptomatic.

This study, which represents the largest published experience with the surgical treatment of patients with symptomatic portal biliopathy, indicates that retroperitoneal splenorenal anastomosis improves outcomes and should be the initial treatment of choice.

Secondary sclerosing cholangitis (SSC) is a disease that is morphologically similar to primary sclerosing cholangitis (PSC) but that originates from a known pathological process. Its clinical and cholangiographic features may mimic PSC, yet its natural history may be more favorable if recognition is prompt and appropriate therapy is introduced. Thus, the diagnosis of PSC requires the exclusion of secondary causes of sclerosing cholangitis and recognition of associated conditions that may potentially imitate its classic cholangiographic features. Well-described causes of SSC include intraductal stone disease, surgical or blunt abdominal trauma, intra-arterial chemotherapy, and recurrent pancreatitis. However, a wide variety of other associations have been reported recently, including autoimmune pancreatitis, portal biliopathy, eosinophillic and/or mast cell cholangitis, hepatic inflammatory pseudotumor, recurrent pyogenic cholangitis, primary immune deficiency, and AIDS-related cholangiopathy. This article offers a comprehensive review of SSC.

Portal biliopathy is due to compression of the common bile duct by varicose veins constituting portal cavernoma. Usually asymptomatic, it can occasionally be responsible for jaundice or cholangitis. We report a case of portal cavernoma secondary to pylephlebitis complicating acute appendicitis, followed eleven years later by occurrence of cholestatic jaundice. Diagnosis of portal biliopathy was done by imaging and confirmed by endoscopic retrograde cholangiography with insertion of a plastic stent into common bile duct. This stent was periodically changed and allowed regression of jaundice with a 3-year follow-up. Through a review of the literature, both clinical and therapeutic characteristics of portal biliopathy were studied.

Initial diagnosis of cavernous transformation of the portal vein (portal cavernoma) is rarely made in adults. Its main clinical manifestation is upper gastrointestinal hemorrhage due to variceal bleeding. More rarely, diagnosis is made from obstructive jaundice. In children, this condition is frequently associated to prehepatic portal hypertension and congenital anomalies, the most frequent of which are atrial septal defects or malformations of the biliary tract or of the inferior vena cava. We describe here a case of a 23-year-old female presenting with massive hematemesis due to the presence of esophageal and small intestinal varices. She had a cavernous transformation of the portal vein with prehepatic portal hypertension associated with heretofore unreported malformations such as right pulmonary hypoplasia, cardiac dextroposition, and right renal ectopia. A unifying hypothesis (e.g. an intrauterine vascular insult) to explain the pathogenesis of these defects seems unlikely. Appropriate tests failed to identify specific functional abnormalities in these organs. Although she bled more than once, the combination of sclerotherapy and beta-blockers has been, thus far, able to control the major clinical consequences of this disease.

Symptomatic biliary obstruction caused by cavernous transformation of the portal vein is an extremely rare disorder for which there is no consensus as to optimal treatment. The results of endoscopic treatments in a small group of patients is reviewed.

A total of 10 patients (5 men, 5 women; mean age 36.1 years, range 17-48 years) with severe biliary strictures were treated between 1995 and 2001. Biliary sphincterotomy was performed in all patients. Four patients also underwent balloon dilation, nasobiliary drainage, and stone or sludge extraction by using a balloon. All patients had stent insertion.

The mean duration of therapy was 3.3 years (range 1-7 years). There was no complication directly related to the endoscopic procedures except for hemobilia that occurred in one patient during stent removal. Cholangitis developed in 5 patients during the therapy period and was treated endoscopically. In 4 patients, significant improvement in the biliary stricture was observed and stents were removed in 3. These patients were followed without stent insertion for one year.

Endoscopic management of biliary stricture caused by cavernous transformation of the portal vein appears to be effective and safe.

Although biliary symptoms appear to be uncommon in patients with portal cavernoma, almost all of them have endoscopic retrograde cholangiographic abnormalities. The mechanisms underlying the biliary changes are explained poorly. This study in patients with portal cavernoma had 3 aims: (1) to assess the manifestations related to biliary involvement; (2) to evaluate with magnetic resonance (MR) imaging the aspect and frequency of cholangiographic changes; and (3) to clarify the mechanisms underlying biliary involvement. From December 1999 to July 2001, 25 consecutive adults with portal cavernoma without cancer or cirrhosis were studied with MR cholangiography coupled with MR portography. Seven patients presented with clinical manifestations of biliary disease. MR cholangiography findings were stenosis in 21 patients, with upstream dilatation in 16 and displacement in 13. MR cholangiography coupled with MR portography showed in all cases that the biliary abnormalities were secondary to a mass effect directly related to pressure by the cavernoma. In conclusion, in patients with portal cavernoma, clinical manifestations of biliary disease are most frequent; the cholangiographic abnormalities are very common and usually are caused by a mass effect by pressure from the veins composing the cavernoma. MR cholangiography coupled with MR portography is an effective noninvasive examination for simultaneous visualization of bile ducts and their relationship to the cavernoma.

Portal cavernoma is a network of veins whose caliber, initially millimetric or microscopic, is increased and which contain hepatopedal portal blood. It results from occlusion, thrombotic and always chronic, of the extra-hepatic portal system. Diagnosis is mainly done by imaging. Clinical signs of portal cavernoma are usually related to extra-hepatic portal hypertension (hematemesis due to rupture of oeso-gastric varices, splenomegaly, rectal bleeding from ano-rectal varices, growth retardation in children) and sometimes to the cause of portal hypertension (abdominal pain, venous bowel infarction). Occurrence of portal thrombosis is often the conjunction of a local cause and a prothrombotic disorder which must be systematically detected. Biliary consequences of cavernoma are related to compression of common bile duct and are usually asymptomatic. In case of jaundice or cholangitis, portal decompression by portosystemic shunt can be performed to treat both biliary symptoms and portal hypertension.

Extrahepatic portal venous obstruction can be associated with bile duct abnormalities, the entity being called portal biliopathy. Three cases are reported of extrahepatic bile duct strictures in patients with portal biliopathy who developed haemobilia during endotherapy. Although endoscopic therapy with stent placement can be successful in patients with portal biliopathy and could also lead to permanent stricture resolution, procedure-related haemobilia is not as uncommon as previously held. Shunt surgery could be a better option in fit patients, since it could provide definitive treatment in a young patient with an otherwise normal life expectancy.

In patients with portal hypertension, particularly with extrahepatic portal vein obstruction, portal biliopathy producing biliary ductal and gallbladder wall abnormalities are common. Portal cavernoma formation, choledochal varices and ischemic injury of the bile duct have been implicated as causes of these morphological alterations. While a majority of the patients are asymptomatic, some present with a raised alkaline phosphatase level, abdominal pain, fever and cholangitis. Choledocholithiasis may develop as a complication and manifest as obstructive jaundice with or without cholangitis. Endoscopic sphincterotomy and stone extraction can effectively treat cholangitis when jaundice is associated with common bile duct stone(s). Definitive decompressive shunt surgery is sometimes required when biliary obstruction is recurrent and progressive.