Portal Hypertensive Biliopathy Research Articles

Transjugular intrahepatic portosystemic shunt for the treatment of portal hypertensive biliopathy with cavernous transformation of the portal vein: a case report

BackgroundPortal hypertensive biliopathy (PHB) was caused by anatomical and functional abnormalities in the intrahepatic and extrahepatic bile ducts secondary to portal hypertension. Currently, there is no consensus regarding to the optimal treatment for PHB. Transjugular intrahepatic portosystemic shunt (TIPS) is the treatment choice for the management of symptomatic PHB, however, it could be very difficult in patients with PHB and cavernous transformation of portal vein.Case presentationWe report a case of PHB, successfully managed with TIPS. A 23-year-old man with liver cirrhosis presented with jaundice. Magnetic resonance cholangiopancreatography (MRCP) showed multiple tortuous hepatopetal collateral vessels compressing the common bile duct (CBD) and leading to the dilated proximal bile duct. He was diagnosed with PHB and treated with TIPS. A guidewire was inserted into the appropriate collateral vessel through transsplenic approach to guide intrahepatic puncture and TIPS was performed successfully. After the operation, portal vein pressure decreased and the symptoms of biliary obstruction were relieved significantly. In addition, the patient showed no jaundice at a follow-up of one year.ConclusionsFor PHB patients presenting for cavernous transformation of the portal vein, which precludes the technical feasibility of TIPS, a combined transjugular/transsplenic approach could be an alternative option.

Portal hypertensive biliopathy presenting as acute jaundice with suspected intrahepatic biliary strictures and stones.

Portal hypertensive biliopathy presenting as acute jaundice with suspected intrahepatic biliary strictures and stones.

Portal hypertensive biliopathy and bile duct varices presenting as jaundice.

Portal hypertensive biliopathy and bile duct varices presenting as jaundice.

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.

Long‐term Outcomes and Health Perceptions in Pediatric‐onset Portal Hypertension Complicated by Varices

Outcomes of pediatric-onset portal hypertension are poorly defined. We aimed to assess population-based long-term outcomes of pediatric-onset portal hypertension complicated by varices. All children with esophageal varices (n = 126) were identified from 14,144 single nationwide referral center endoscopy reports during 1987 to 2013, and followed up through national health care and death registers. A questionnaire was sent to survivors (n = 94) of whom 65 (69%) responded. Nineteen underlying disorders included biliary atresia (35%), extrahepatic portal vein obstruction (35%), autosomal recessive polycystic kidney disease (7%), and other disorders (23%). During median follow-up of 15.2 (range 0.5-43.1) years patients underwent median 9 (1-74) upper gastrointestinal endoscopies. Esophageal varices were first observed at a median age of 4.0 (0.3-18.2) years, 112 (89%) patients underwent median 6 (1-56) sclerotherapy/banding sessions, and 61 (48%) experienced median 2 (range 1-20) variceal bleeding episodes. Forty-eight surgical shunt procedures were performed to 41 (36%) patients and 38% underwent liver transplantation. Portal hypertensive biliopathy was diagnosed in 4 patients. Hepatopulmonary syndrome necessitated liver transplantation in 2 patients, hepatic encephalopathy in 2, and hepatorenal syndrome in 1. No patient died of variceal bleeding. Patient-reported perception of health on a scale of 1 to 10 was 9 (range 4-10), and 86% reported no current symptoms attributable to esophageal varices. Pediatric-onset portal hypertension is a heterogeneous disease with significant long-term morbidity, requiring multimodal approach with considerable resources and continuation of follow-up in adulthood. Although mortality to variceal bleeding was avoided, bleeding episodes recurred also in adulthood, while patient-reported health of long-term survivors was encouraging.

Portal Hypertensive Biliopathy in Adult Patients: Findings and Interventional Radiologic Treatment-A Single-Center Experience.

The aim of this study was to evaluate the morphologic appearance, the clinical scenario, and the outcomes of patients with portal hypertensive biliopathy (PHB), particularly in the symptomatic subgroup treated with interventional radiology (IR) procedures. The outcome of 20 patients with PHB were retrospectively reviewed over a 5-year period. In all cases, the extrahepatic portal vein occlusion (EHPVO) and the compensatory cavernomatosis was the cause of PHB. Eight out of 20 patients had severe symptoms (jaundice and bleeding). Five out of these eight patients were successfully treated with IR procedures. PHB is a rare but serious complication of PH from EHPVO. IR treatments are highly effective in controlling symptoms. Moreover, IR procedures, as drainage and transjugular intrahepatic portosystemic shunt placement, are the first-line treatment in cases of life-threatening bleeding from ruptures of the varices.

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.

Endoscopic Treatment of Portal Hypertensive Biliopathy: Intervene with Caution!

Portal hypertensive biliopathy (PHB) is defined as biliary abnormalities associated with portal hypertension, particularly caused by extra hepatic portal venous obstruction. Clinical spectrum ranges from no symptoms to biliary obstruction and life threatening cholangitis. PHB is an unusual cause of hemobilia. We present three cases of PHB presented to us with varied clinical features and managed with endoscopic interventions. Here, we highlight the outcome of endotherapy in patients of PHB. We also review the literature regarding the pathogenesis, clinical features and management of PHB.

Biliary collateral veins and associated biliary abnormalities of portal hypertensive biliopathy in patients with cavernous transformation of portal vein

PurposeThe purpose was to investigate magnetic resonance imaging (MRI) features of biliary collateral veins and associated biliary abnormalities of portal hypertensive biliopathy (PHB). Materials and methodsThirty-six patients including 18 patients with abnormal biliary changes and 18 patients as control group were involved in this study. MRI features of biliary collateral veins were analyzed. ResultsStenosis with dilated proximal bile ducts occurred in 33.3% of patients, 27.8% of patients had irregular ductal walls, 22.2% of patients had thickened ductal walls, 16.7% of patients had angulated ductal walls, and 44.4% of patients had thickened gallbladder walls. ConclusionsBiliary collateral veins and associated biliary abnormalities of PHB can be detected by MRI.

English

Portal biliopathy (PB) is defined as biliary obstruction associated with enlarged collaterals in the setting of chronic extrahepatic portal vein occlusion (portal vein thrombosis EHPVO) or cavernous transformation of the portal vein. Although patients with portal biliopathy normally have asymptomatic biliary dilatation or an increase in liver enzyme levels, they may rarely experience jaundice, cholangitis or choledocholithiasis. The present case is of 17 year old male presenting with obstructive jaundice, malena, cholelithiasis secondary to portal venous thrombosis and cavernous transformation of portal vein due to CBD stricture secondary to Portal Hypertensive Biliopathy (PHB).

Hepatobiliary and Pancreatic: Portal hypertensive biliopathy presenting as acute cholangitis

Journal of Gastroenterology and HepatologyVolume 28, Issue 8 p. 1257-1257 Education and Imaging Hepatobiliary and Pancreatic: Portal hypertensive biliopathy presenting as acute cholangitis FS Macaluso, FS Macaluso Sezione di Gastroenterologia, DiBiMIS, ItalySearch for more papers by this authorM Maida, M Maida Sezione di Gastroenterologia, DiBiMIS, ItalySearch for more papers by this authorM Dioguardi Burgio, M Dioguardi Burgio Sezione di Scienze Radiologiche, University of Palermo, ItalySearch for more papers by this authorG Brancatelli, G Brancatelli Sezione di Scienze Radiologiche, University of Palermo, ItalySearch for more papers by this author FS Macaluso, FS Macaluso Sezione di Gastroenterologia, DiBiMIS, ItalySearch for more papers by this authorM Maida, M Maida Sezione di Gastroenterologia, DiBiMIS, ItalySearch for more papers by this authorM Dioguardi Burgio, M Dioguardi Burgio Sezione di Scienze Radiologiche, University of Palermo, ItalySearch for more papers by this authorG Brancatelli, G Brancatelli Sezione di Scienze Radiologiche, University of Palermo, ItalySearch for more papers by this author First published: 22 July 2013 https://doi.org/10.1111/jgh.12295Citations: 1Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL No abstract is available for this article.Citing Literature Volume28, Issue8August 2013Pages 1257-1257 RelatedInformation

Portal hypertensive biliopathy: A single center experience and literature review

Portal hypertensive biliopathy (PHB) is characterized by anatomical and functional abnormalities of the intrahepatic, extrahepatic and pancreatic ducts, in patients with portal hypertension associated to extrahepatic portal vein obstruction and less frequently to cirrhosis. These morphological changes, consisting in dilatation and stenosis of the biliary tree, are due to extensive venous collaterals occurring in an attempt to decompress the portal venous blockage. It is usually asymptomatic until it progresses to more advanced stages with cholestasis, jaundice, biliary sludge, gallstones, cholangitis and finally biliary cirrhosis. Imaging modalities of the biliary tree such as Doppler ultrasound, computed tomography, magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography are essential to establish the diagnosis and the need of therapeutical interventions. Once the diagnosis is established, treatment with ursodesoxycholic acid seems to be beneficial. Decompression of the biliary tree to dilate, remove stones or implant biliary prosthesis by endoscopic or surgical procedures (hepato-yeyunostomy) usually resolves the cholestatic picture and prevents septic complications. The ideal treatment is the decompression of the portal system, with transjugular intrahepatic porto-systemic shunt or a surgical porto-systemic shunt. Unfortunately, few patients will be candidates for these procedures due to the extension of the thrombotic process. The purpose of this paper is to report the first 3 cases of PHB seen in a Colombian center and to review the literature.

Portal hypertensive biliopathy: review of pathophysiology and management

Portal hypertensive biliopathy is described as abnormalities of the walls of the biliary tree secondary to portal hypertension. In literature it has also been named as "Cholangiopathy associated with portal hypertension", "Portal biliopathy" and "Portal cavernoma associated cholangiopathy". It is usually asymptomatic and found incidentally, but rarely patients do present with symptoms of abdominal pain, jaundice, asthenia and fever. Treatment is warranted in symptomatic cases only, and is dictated by the clinical manifestations and complications of the disease process. Due to presence of underlying severe portal hypertension, endoscopic biliary intervention is usually the first line of management, and is relatively safe and often sufficient. When surgery is resorted to, a porto-systemic shunt prior to biliary bypass procedure provides early relief of obstructive biliary symptoms and often precludes the need for a biliary bypass surgery. This review describes the pathophysiology, presentation, progression and management approaches to portal biliopathy.

Role of a Short-Term Stent-Trial in a Patient with Biliary Stricture and Portal Hypertensive Biliopathy: Long-Term Outcome Result

In portal hypertensive biliopathy (PHB) secondary to portal thrombosis, multiple factors can lead to symptoms and alteration of liver tests. The compression of the common bile duct (CBD) by peribiliary varices, portal vein thrombus or cavernoma, or the presence of biliary stone and an associated ischemic stricture are proposed to be involved in the pathogenesis of symptoms (jaundice, abdominal pain, fever) and alterations of liver tests. We present a case of a young HCV-infected male patient in which multiple factors in PHB as mentioned above were present. Clinical and biochemical evaluations after a short-term biliary stent and after its removal (stent-trial) showed the major role of the ischemic-associated stricture and rule out other factors in our patient. Long-term data support this result.

Portal hypertensive biliopathy: Can we prevent it?

Portal hypertensive biliopathy: Can we prevent it?

Perforators of common bile duct wall in portal hypertensive biliopathy (with videos)

Extrahepatic obstruction of the portal vein (EHPVO) with resulting portal hypertension may cause extensive collateral venous circulation at the porta hepatis. Portal hypertensive biliopathy (PHB) refers to abnormalities of the biliary tract in patients due to these portoportal collaterals. 1,2 These collateral veins are related to 2 preformed venous systems near the extrahepatic bile ducts: the paracholedochal (PACD) veins of Petren, and the epicholedochal (ECD) venous plexus of Saint. The PACD venous plexus of Petren runs parallel to the CBD, and the ECD plexus of Saint veins form a reticular mesh on the surface of the CBD. 3,4 The PACD collaterals, if dilated, may cause extrinsic compression and protrusion into the thin and pliable CBD, and the ECD collaterals, if dilated, may make the normallysmoothintraluminalsurfaceoftheCBDirregular. 5,6 Detection of these dilated venous collateral vessels is important for making the diagnosis of PHB. Detection of large PACD and ECD collaterals has been done by color Doppler US (CDUS). 7 Smaller collaterals are identified by EUS, color Doppler EUS (CD-EUS), and intraductal EUS. 8-10 On magnetic resonance imaging, PACD collaterals and ECD collaterals are identified separately. 11,12

Massive hemobilia during extraction of a covered self-expandable metal stent in a patient with portal hypertensive biliopathy

Massive hemobilia during extraction of a covered self-expandable metal stent in a patient with portal hypertensive biliopathy

Intracholedochal varices in portal hypertensive biliopathy

Portal hypertensive biliopathy (PHB) is associated with CBD stones in some cases. Removal of stone can cause bleeding. The exact cause of bleeding has been assumed to be from two groups of Epicholedochal (ECD) venous plexus and para-choledochal (PCD) venous plexus. The presence of these two plexuses does not explain the cause of bleeding during therapeutic ERCP. This case shows the presence of intracholedochal collaterals by Color Doppler Ultrasound (CDUS) and Endoscopic Ultrasound (EUS) which can be the cause of bleeding during ERCP.

Vascular Biliopathy as a Cause of Common Bile Duct Obstruction Successfully Treated by Mesocaval Shunt and Endoscopic Retrograde Cholangiopancreatography Biliary Stent Placement

Common bile duct stenosis owing to extrahepatic portal varices is termed "portal hypertensive biliopathy" (PHB) and is a rare occurrence. We report a case of PHB owing to portal vein thrombosis with cavernous transformation successfully managed by mesocaval shunt and endoscopic retrograde cholangiopancreatography (ERCP) biliary stent placement. A 44-year-old male, who presented with hematemesis, melena, jaundice, and abdominal pain, underwent gastroscopy, which revealed bleeding gastric varices. Computed tomography with arterial and venous imaging demonstrated portal vein thrombosis with cavernous transformation and extensive extrahepatic varices within the porta hepatis causing common bile duct obstruction from extrinsic compression. Biliary decompression was achieved with ERCP, and a small common bile duct stone was retrieved. A mesocaval shunt with a 16 mm Dacron graft successfully treated the portal hypertension. PHB is rare. We report a case successfully treated by mesocaval shunt and ERCP.

Portal hypertensive biliopathy: a rare cause of childhood cholestasis

Portal hypertensive biliopathy (PHB) is defined as abnormal biliary changes that take place most likely secondary to extrahepatic portal vein obstruction (EHPVO) with portal hypertension. This condition may be asymptomatic or could lead to a cholestatic state, which is not well-described in children. We report a child who developed a cholestatic nature with portal hypertension some time after having neonatal surgery for duodenal atresia. We discuss the differential diagnosis and management of this rare condition. Symptomatic PHB has been only rarely reported in children. It should be suspected in patients with portal hypertension and having features of biliary obstruction. Hepaticojejunostomy may have a therapeutic role in selected patients in whom endoscopic or percutaneous manipulation of the biliary tree is unsuccessful and who have not responded to a surgical portal-systemic shunt procedure.