Symptomatic Portal Biliopathy Research Articles

Primary bile duct surgery for symptomatic portal biliopathy in patients with portal hypertension due to EHPVO: a single Centre experience

Primary bile duct surgery for symptomatic portal biliopathy in patients with portal hypertension due to EHPVO: a single Centre experience

Safety and efficacy of transjugular intrahepatic portosystemic shunt therapy for symptomatic portal biliopathy

Safety and efficacy of transjugular intrahepatic portosystemic shunt therapy for symptomatic portal biliopathy

An Audit of Extrahepatic Portal Vein Obstruction: Experience from Tertiary Referral Center.

Extrahepatic portal vein obstruction (EHPVO) is a common cause of portal hypertension in India. (1) To evaluate the clinical presentation and the natural history of EHPVO; (2) to describe the risk factors, rebleeding rates and development of portal biliopathy on follow-up; and requirement of surgery in EHPVO at a tertiary care center. Data from 318 consecutive patients with EHPVO from June 2012 to October 2020 were analyzed. All patients underwent liver biochemistry, ultrasonography (USG) abdomen, upper gastrointestinal (GI) endoscopy, and viral serology. Color Doppler, computed tomography (CT) abdomen and magnetic resonance cholangiopancreatography (MRCP) were done as indicated. Mean age of presentation was 15.08 years [standard deviation (SD) 12.74; 6 months-60 years; 210 males)]. The presenting features were upper GI bleed (n = 227) (age at first bleed 11 years; 4 months-56 years), left hypochondrium pain or lump (n = 67), and only lower GI bleed (n = 1). Incidentally detected EHPVO on USG was seen in 10.69% (n = 34) patients. Postbleed ascites were seen in 10.69% (n = 34) patients. Six patients had symptomatic portal biliopathy and 14 had hypersplenism. Around 14.77% (n = 47) of patients had a history of being delivered at home, while 3.45% (n = 11) had a history of umbilical sepsis. During follow-up, 35.3% (n = 82) of patients had rebled. On imaging, associated splenic vein (SV) collaterals and superior mesenteric vein (SMV) collaterals were seen in 35.84% (n = 114) and 11.01% (n = 35) patients, respectively. Gallbladder varices were seen in 44.3% (n = 106), while gallstones in 5.66% (n = 18). On endoscopy, 87.42% (n = 278) patients had esophageal varices, 18.86% (n = 60) had isolated fundic varices, and three had ectopic varices. Only two patients had rectal varices and colopathy. Emergency devascularization was required in 3.45% (n = 14) patients for the failure of variceal bleed control, 1.88% (n = 7) underwent splenectomy, and four patients had proximal splenorenal shunt (PSRS) surgery. Extrahepatic portal hypertension (EHPVO) is an important cause of portal hypertension (PHT) in our country. The majority of them present with GI bleed; postbleed ascites were seen only in ~10%. Rebleed occurs in one-third of cases. Gallbladder varices were common; portal biliopathy occurred in 10% and were usually asymptomatic.

Single-Stage Treatment for Portal Biliopathy in Selected Cases

Symptomatic portal biliopathy requires two-staged treatment, portosystemic shunt in first stage and intervention for biliary obstruction in second stage if not resolved by the first stage. We present our series of single-stage treatment in form of portosystemic shunt with segment III duct Roux-en-Y hepaticojejunostomy. We retrospectively analysed 3 out of 20 patients who underwent single-stage surgical intervention for extrahepatic portal vein obstruction with portal biliopathy. Three out of 20 patients who underwent single-stage portosystemic shunt with segment III duct Roux-en-Y hepaticojejunostomy were older than the rest and had atrophied left lobe of the liver with dilated segment III duct easily approachable at liver surface. There was no increase in major morbidity or mortality due to combination of procedures. It took longer than expected to resolve jaundice. Selected patients with portal biliopathy having atrophied left lobe and dilated superficial segment III duct can be treated with single-stage surgical treatment. It ensures the relief of biliary obstruction and possibly averts the need of second intervention.

Endoscopic Biliary Stenting for Portal Biliopathy Perforating Paracholedochal Collateral: A Rare Complication

AbstractExtrahepatic portal venous obstruction (EHPVO) usually presents with upper gastrointestinal bleed in the first decade of life. Symptomatic portal hypertensive biliopathy is seen in a minority of patients with EHPVO. With use of endoscopic intervention, biliary drainage is maintained in these patients. Various procedural complications have been linked while performing endoscopic retrograde cholangiography and stenting; however, these are managed conservatively. Here, we are highlighting a case of EHPVO with symptomatic portal biliopathy in which the patient bled from paracholedochal collateral after biliary stenting and was managed successfully with a multidisciplinary approach.

Surgical Management of Portal Biliopathy – A Prospective Analysis.

Background: Portal biliopathy denotes intrahepatic and extra hepatic biliary ductal abnormalities in portal hypertension. It is usually associated with extra hepatic portal vein obstruction (EHPVO). These patients are also prone to develop obstructive jaundice as a result of strictures and/or choledocholithiasis. Surgical management of obstructive jaundice in such patients becomes difficult in the presence of these collaterals. The aim of the study is to prospectively analyze the approach to management of patients with Symptomatic portal biliopathy. Subjects and Methods: The study was conducted at Narayana Medical College & Hospital, Chintareddy Palem, Nellore, Andhra Pradesh on surgical management of the patients of EHPVO with portal biliopathy presenting to the surgical clinic of this tertiary referral center between November 2016 and October 2017. The data was analyzed for presentation, clinical features, imaging and results of surgical management. Results: During the study period, total of 44 patients of EHPVO were referred for surgical management. Of these 14 patients (9 males, mean age 34.6 years) were diagnosed to have portal biliopathy. Ten patients had prior history of variceal bleed which was managed endoscopically. Jaundice was the most common symptom followed by right upper quadrant pain and recurrent cholangitis. Four patients had prior unsuccessful endoscopic management. ERCP/ MRCP was used for delineation of the biliary tree, which showed irregularity (14 patients), dominant strictures (8 patients), filling defects (5 patients), and intrahepatic biliary dilatation (7 patients). Proximal splenorenal shunt (PSRS) was performed in 13 patients. While in 1 patient peroperatively liver was found to be grossly nodular, hence gastro-esophageal devascularization with simultaneous biliary drainage was done. Of the 13 patients who underwent PSRS, all patients were intensively followed for 4-6 weeks with history and liver function tests. After 6 weeks, five patients showed clinical as well as biochemical improvement and they are being followed up regularly. Eight patients had persistent symptoms and abnormal liver function tests. These were the patients with dominant stricture and choledocholithiasis. Of these, 6 patients underwent Roux-en-Y hepaticojejunostomy. The average blood transfusion requirement at second surgery was 1 unit. Postoperative complications were minimal with no mortality. One patient and was lost to follow up and the remaining one is awaiting second surgery (RYHJ). Over a follow up of 3-28 months the patients are asymptomatic and well. Conclusion: Portal biliopathy with symptomatic biliary obstruction needs intervention. Surgical decompressive shunt followed by biliary drainage is the best possible treatment. While for most of the early biliary changes shunt alone is effective, patients with dominant stricture will need a biliary diversion which can be safely performed following Porto systemic shunt without increase in morbidity or mortality.

Endoscopic Biliary Stenting for Portal Biliopathy Perforating Paracholedochal Collateral: A Rare Complication

ABSTRACTExtrahepatic portal venous obstruction (EHPVO) usually presents with upper gastrointestinal bleed in the first decade. Symptomatic portal hypertensive biliopathy is seen in minority of patients with EHPVO. With the use of endoscopic intervention, biliary drainage is maintained in these patients. Various procedural complications have been linked while performing endoscopic retrograde cholangiography and stenting; however, these are managed conservatively. Here, we are highlighting a case of EHPVO with symptomatic portal biliopathy who bled from paracholedochal collateral after biliary stenting and managed successfully with multidisciplinary approach.

11. Endoscopic management of symptomatic portal biliopathy – our experience

11. Endoscopic management of symptomatic portal biliopathy – our experience

Noncirrhotic portal hypertension: Medical and endoscopic management.

Noncirrhotic portal hypertension: Medical and endoscopic management.

Clinical presentation of extrahepatic portal vein obstruction: 10-year experience at a tertiary care hospital in Pakistan.

ObjectiveTo evaluate the clinical presentation, possible etiological factors, management and outcome of patients in our hospital with extrahepatic portal vein obstruction (EHPVO).Materials and MethodsThis study included patients with EHPVO followed up in our department during last 10 years. Patients of cirrhosis with EHPVO were excluded. Patients’ clinical presentation, etiology of EHPVO, management and outcome results were analyzed.ResultsOf 30 patients, 19 (67.9%) were males. Median age was 12 years. Of 14 patients who underwent liver biopsy 9 had histological activity index stage of 1/6. History of omphalitis and pulmonary tuberculosis was present in one case each. Of 22 patients with the available thrombophilia profile, nine patients had a deficiency of protein C, five patients had a deficiency of protein S, one each had reduced level S of anti-thrombin III and factor V mutation. The predominant presenting symptom was hematemesis (15 patients, 53.6%). Seven patients (25%) had splenomegaly. Three patients (10.7%) had no esophageal varices on endoscopy. Three patients underwent splenectomy due to severe pancytopenia. Endoscopic retrograde cholangipancreatography was performed in four patients (14.3%) due to portal biliopathy. Common bile duct stenting was performed in all four patients. Of them, one patient underwent splenorenal shunt operation for indication of hemobilia. One patient died at the age of 40 years, due to cholangitis and sepsis.ConclusionsResults from this study show that the anticoagulant deficiency is a common cause of EHPVO in our setup. Hematemesis is a common presenting symptom. Some of these patients have symptomatic portal biliopathy.

Portal biliopathy

Biliary ductal changes are a common radiological finding in patients with portal hypertension, however only a small percentage of patients (5%-30%) develop symptomatic bile duct obstruction. The exact pathogenesis is not clear, but an involvement of factors such as bile duct compression by venous collaterals, ischemia, and infection is accepted by most authors. Although endoscopic retrograde cholangiopancreatography was used to define and diagnose this condition, magnetic resonance cholangiopancreatography is currently the investigation of choice for diagnosing this condition. Treatment is indicated only for symptomatic cases. Portosystemic shunts are the treatment of choice for symptomatic portal biliopathy. In the majority of patients, the changes caused by biliopathy resolve after shunt surgery, however, 15%-20% patients require a subsequent bilio-enteric bypass or endoscopic management for persistent biliopathy. There is a role for endoscopic therapy in patients with bile duct stones, cholangitis or when portosystemic shunt surgery is not feasible.

Terlipressin in Control of Acute Hemobilia During Therapeutic ERCP in Patient With Portal Biliopathy

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.

Right Porto-Ovarian H-Shunt for the Surgical Treatment of Symptomatic Portal Biliopathy: A Case Report and Literature Review

Portal hypertension, especially when it is caused by extrahepatic portal vein thrombosis, is commonly followed by the development of an abnormal periportal and pericholedochal variceal network, which form a portal cavernoma. This may exert extrinsic pressure on the adjacent biliary ducts and gallblader, causing morphologic abnormalities, termed portal biliopathy, which is usually leading to asymptomatic cholestasis, while less frequently it can be associated with obstructive jaundice, gallstone formation, and cholangitis. Endoscopic stone extraction can effectively treat portal biliopathy when cholangitis is associated with common bile duct stones. Portosystemic shunts are indicated in cases of disease recurrence as they can achieve regression of portal cavernoma and usually relieve symptomatic portal biliopathy. This case describes an alternative partial portosystemic shunt that utilizes the right ovarian vein as an autologous conduit for the surgical treatment of symptomatic portal biliopathy.

Symptomatic portal biliopathy: a single centre experience from the UK

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.

Extrahepatic Portal Vein Thrombosis

Noncirrhotic, nontumoral portal vein thrombosis (PVT) is the second most-frequent cause of portal hypertension in the world. General thrombophilic factors can be identified in approximately 60% of patients. PVT may manifest as an acute process. However, the acute episode more frequently is asymptomatic or paucisymptomatic and portal vein thrombosis is misdiagnosed until the development of complications secondary to portal hypertension, such as variceal bleeding or portal biliopathy. Although no randomized controlled trials have been performed, after the diagnosis of acute PVT early initiation of anticoagulation (within 30 days of the onset of symptoms) is recommended to achieve recanalization. In patients with portal cavernoma, anticoagulation is aimed to prevent the progression and recurrence of thrombosis. Because of the lack of data in this specific population, variceal bleeding is managed as in cirrhotic patients. Ursodeoxycholic acid has been proposed empirically for the treatment of patients with symptomatic portal biliopathy. Choledocholithiasis might be present, complicating a bile duct stenosis. Accordingly, an endoscopic retrograde cholangiopancreatography with sphincterotomy, extraction with balloon catheter, and stent placement is indicated. Mortality among patients with PVT is low (5-year mortality rate of 5 to 10%) and is mainly related to associated diseases rather than to complications of portal hypertension.

Therapeutic Strategies in Symptomatic Portal Biliopathy

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.

Evolving Consensus in Portal Hypertension Report of the Baveno IV Consensus Workshop on methodology of diagnosis and therapy in portal hypertension

Portal hypertension is associated with the most severe complications of cirrhosis, including ascites, hepatic encephalopathy, and bleeding from gastro-esophageal varices. Despite the progress achieved over the last decades, the 6-week mortality associated with variceal bleeding is still in the order of 10–20%. Awareness of the difficulty inherent to the evaluation of diagnostic tools and the design and conduct of good clinical trials for the treatment of portalhypertensionhas led to theorganization, since1986,of a series of consensus meetings. The first one was organized by Andrew Burroughs in Groningen, The Netherlands [1]. After Groningen, other meetings followed, in Baveno in 1990 (Baveno I) [2] and in 1995 (Baveno II) [3,4], in Milan in 1992 [5], in Reston, USA, in 1996 [6], in Stresa in 2000 (Baveno III) [7,8], again in Baveno in 2005 (Baveno IV) [9,10], and in Atlanta in 2007 [11]. The aims of these meetings were to develop definitions of key events in portal hypertension and variceal bleeding, to review the existing evidence on the natural history, the diagnosis and the therapeutic modalities of portal hypertension, and to issue evidence-based recommendations for the conduct of clinical trials and the management of patients. All these meetings were successful and produced consensus statements on some important points, although some issues remained unsettled. To continue the work of the previous meetings, a Baveno V workshop was held on May 21–22, 2010. The workshop was attended by many of the experts responsible for most of the major achievements of the last years in this field. Many of them had attended the previous meetings as well. The main fields of discussion of the Baveno V workshop were the same as in Baveno I–IV, i.e. the definitions of key events concerning the bleeding episode and the therapeutic options in patients with portal hypertension. For each of these topics, a series of consensus statements were discussed and agreed upon. As in Baveno IV, whenever applicable, the level of existing evidence was evaluated and the recommendations were ranked according to the Oxford System [12] (i.e.: level of evidence from 1 = highest

Fifteen-year follow up of endoscopic injection sclerotherapy in children with extrahepatic portal venous obstruction.

Endoscopic sclerotherapy has emerged as an effective treatment for bleeding esophageal varices both in adults and children but the long-term outcome is poorly defined in children. The authors report a 15-year follow up of sclerotherapy in children with extrahepatic portal venous obstruction. Between June 1982 and February 1992, 69 children with bleeding esophageal varices underwent sclerotherapy; variceal eradication was achieved in 63 (91.3%) patients, with procedure-related morbidity of 28.9% and mortality of 1.4%. Fifty-nine patients with variceal eradication were followed for between 10.4 and 20.1 years (mean, 15.1 +/- 3.1 years). After a median period of 3 years (range, 1.2-12.8 years), seven (11.9%) patients presented with recurrent bleeding (esophageal varices, four; gastric varices, two; and duodenal ulcer, one). Recurrent bleeding occurred in six of seven (85.7%) patients within the first 4 years of initial variceal eradication. Esophageal varices recurred in eight (13.6%) patients. Five of the seven patients with recurrent bleeding and all eight with recurrent varices were effectively treated with further sclerotherapy. Two patients with gastric variceal bleeding unresponsive to sclerotherapy underwent shunt surgery. Elective surgery was required in eight additional patients for reasons other than recurrent varices or bleeding. The authors conclude that (i) sclerotherapy is the ideal, safe and effective treatment for bleeding esophageal varices, that it prevented bleeding in 88.1% patients after variceal eradication and hence, should be included in primary management strategies; (ii) follow-up endoscopy should be performed on a yearly basis for the first 4 years after variceal eradication; and (iii) surgery is required as a complementary technique for patients with uncontrolled bleeding, painful splenomegaly, growth retardation and symptomatic portal biliopathy.

Bile duct obstruction due to portal biliopathy in extrahepatic portal hypertension: surgical management.

Varices can develop in and around the bile duct in the presence of portal hypertension, especially when it is caused by extrahepatic portal vein thrombosis. The term 'portal biliopathy' is used to describe changes in the bile duct due to these varices, which may cause bile duct obstruction. This paper reviews experience of the surgical management of patients with symptomatic portal biliopathy. Nine patients with extrahepatic portal vein obstruction with symptomatic portal biliopathy. were reviewed retrospectively. Eight patients presented with jaundice, two had abdominal pain and one had recurrent cholangitis. Endoscopic retrograde cholangiography revealed abnormality of the bile duct wall, with stricture in eight patients and bile duct calculi in two. Portasystemic shunting relieved jaundice in five of seven patients, and in two a second-stage hepaticojejunostomy was required. Symptomatic biliary obstruction in patients with extrahepatic portal hypertension may be relieved by a portasystemic shunt. Rarely biliary bypass may be required and is rendered safer by previous portasystemic shunting to decompress the pericholedochal varices. A direct approach to the biliary tract without a preliminary shunt may be hazardous and is frequently unnecessary.

Extrahepatic portal vein obstruction.

Extrahepatic portal vein obstruction (EHPVO) is an important cause of noncirrhotic portal hypertension, especially in Third World countries. The etiology and clinical presentation are different in children and adults. The portal vein is transformed into a cavernoma, resulting in portal hypertension and oesophagogastic varices. In addition, extensive collateral circulation develops, involving paracholecystic, paracholedochal and pancreaticoduodenal veins resulting in formation of ectopic varices, and portal biliopathy. Besides variceal bleeding, which is the commonest presentation, patients may have symptomatic portal biliopathy, hypersplenism, and growth retardation. Although the liver may appear normal, functional compromise develops in the long term. Variceal bleeding in EHPVO can be successfully managed by endoscopic obliteration of varices, which has low morbidity but requires repeated visits, or by portosystemic shunt surgery, which provides good control of bleeding, possibly helps growth retardation, hypersplenism, and protects against future development of portal biliopathy but is associated with surgical mortality and is sometimes not feasible due to nonavailability of a satisfactory vessel.