Extrahepatic Portal Vein Occlusion Research Articles

Beyond the Rex: an innovative meso-intrahepatic portal vein bypass for late-onset extrahepatic portal vein occlusion after living donor liver transplantation.

The Meso-Rex bypass (MRB) is recognized as an effective treatment for portal hypertension secondary to extrahepatic portal vein occlusion (EHPVO) both in the pediatric and adult population, within or outside the context of liver transplantation. It is the preferred surgical treatment in most centers because not only does it addresses the portal hypertension, but also restores physiologic portal hepatopetal flow. However, the Rex recess, the landmark for this technique, may not be safely accessible in some patients. We present a 22-year-old male who underwent living donor liver transplant (LDLT) for neonatal hepatitis. He presented with variceal bleeding due to EHPVO at 13 years after transplant. Various endoscopic, radiologic, and surgical interventions were employed to address the recurrent gastrointestinal bleeding, but results have been unsatisfactory. We performed a meso-intrahepatic portal vein bypass (MIPVB), an innovative alternative to the MRB, for this patient with extensive post-operative adhesions, perihilar collaterals, and cavernous transformation. MIPVB creation in patients where the Rex recess is inaccessible is technically challenging. But with a multidisciplinary team approach, meticulous preoperative planning, and close follow-up, the authors have demonstrated that it is a safe and feasible option for patients with late-onset EHPVO after liver transplantation.

Embolization with or without portal vein stenting for bleeding ectopic jejunal varices in hepatopetal portal collaterals due to extrahepatic portal vein occlusion or stenosis after hepatobiliary and pancreatic surgery

PurposeTo evaluate the efficacy and safety of embolization with or without portal vein stenting for bleeding ectopic jejunal varices in the hepatopetal portal collateral due to extrahepatic portal vein occlusion or stenosis after hepatobiliary and pancreatic surgery.Materials and methodsThis study included consecutive patients who underwent embolization for bleeding ectopic jejunal varices in the hepatopetal collateral due to extrahepatic portal vein occlusion or stenosis after hepatobiliary and pancreatic surgery between September 2012 and December 2020. The safety, technical and clinical success rates (no re-bleeding within 1 month) and re-bleeding-free survival after the first therapy and overall survival were assessed.ResultsFourteen sessions in 11 patients were included. Four patients (7 sessions) underwent variceal embolization only, and the remaining seven patients (7 sessions) underwent portal vein stenting and variceal embolization. Technical success was achieved in all 14 sessions (100%). Clinical success was achieved in 13 of 14 sessions (92.9%). No treatment-related serious complications including liver failure were observed. One-year and 2-year re-bleeding-free survival rate after the first endovascular therapy in all 11 patients was 90.9 and 60.6%, respectively. Two patients who experienced re-bleeding had repeat embolization treatment. There was no significant difference in re-bleeding-free survival after endovascular therapy between the combination with stenting and embolization group and the embolization-only group (p = 0.13).ConclusionEmbolization with or without portal vein stenting of bleeding ectopic jejunal varices in the hepatopetal portal collateral due to extrahepatic portal vein occlusion or stenosis after hepatobiliary and pancreatic surgery can be considered a safe, effective, and repeatable therapy for long-term hemostasis of uncontrollable bleeding.

Ectopic Variceal Bleeding from the Hepaticojejunostomy due to Extrahepatic Portal Vein Occlusion: How to Treat?

Atypical variceal bleeding, which primarily stems from extrahepatic portal vein obstruction (EHPVO), is a severe complication of pancreatic hepatobiliary surgery. This review provides insights into this condition's incidence, diagnosis, and management strategies. The treatment modalities for atypical variceal bleeding resulting from EHPVO range from endoscopic intervention to surgical procedures, including direct variceal ligation and shunt surgery. Here, we discuss the efficacy and potential limitations of each treatment approach. Additionally, we explored the utility and therapeutic advantages of the meso-Rex shunt, a particularly promising surgical technique for mitigating the hemodynamic and metabolic impacts of EHPVO.

Extrahepatic portal vein aneurysm in which the acute thrombogenic process triggered by trauma confirmed by abdominal ultrasonography: a case report.

Extrahepatic portal vein aneurysm (PVA) is a rare condition in which the extrahepatic portal vein is partially dilated into a sac-like or spindle-like shape. Usually, patients are followed, but surgery is considered in cases of rupture, thrombus, or enlargement. We report a case of thrombus formation in an extrahepatic portal vein aneurysm following trauma that resulted in regression of the aneurysm and extrahepatic portal vein occlusion. Immediately after the trauma, ultrasonography showed moderately hyperechoic structures and comet signs along the vessel wall of the aneurysm and turbulent blood flow in the aneurysm, like in a whirlpool. There were floating point-like echogenic features, which were presumed to be microthrombi. In other words, the trauma might have triggered Virchow's triad: changes in the vessel wall, changes in blood properties, and blood stagnation. This is a valuable case in which ultrasonography imaging revealed interesting changes during the thrombus formation process inside an extrahepatic portal vein aneurysm. The aneurysm's size was reduced by thrombus-induced organization, but the main trunk of the portal vein became deficient in blood flow, resulting in extrahepatic portal vein occlusion. This case is suggestive of the mechanism of extrahepatic portal vein occlusion.

Anesthetic Challenges in Pregnancy with Wilson’s Disease Associated with Massive Splenomegaly Complicated with Intramyometrial Carboprost: A Case Report

Wilson’s disease is a rare autosomal recessive condition, that affects the liver and brain mainly. Pregnancy in these patients is of high risk due to involvement of liver and high incidence of abortion, preeclampsia, HELLP syndrome. We report a case of a 24year old G2A1 (35+2 weeks) diagnosed with wilson’s disease 6 years ago, currently on tab zinc. She presented with thrombocytopenia, anemia, extrahepatic portal vein occlusion with splenomegaly and mild ascites. She was taken up for elective section at 35+2 weeks, under general anesthesia. With intramyometrial injection of carboprost, there was sudden desaturation and bronchospasm. However, we managed the case successfully with safe outcome of both the mother and child. There are only very few case reports of Wilson’s disease in pregnancy undergoing C-section under general anesthesia in the presence of hepatic dysfunction.

Reply: Discussing the place of TIPS in non-cirrhotic patients with chronic extra hepatic portal vein occlusion refractory to standard of care therapy.

Department of Radiology, Section of Vascular and Interventional Radiology, Northwestern University, Chicago, Illinois, USA CorrespondenceRiad Salem ([email protected])

Extrahepatic portal vein occlusion presenting with vascular, biliary, and visceral complications evaluated on multidetector computed tomography

Extrahepatic portal venous obstruction, predominantly a disease of childhood, is a chronic and long-standing disease characterized by thrombosis of the portal vein, leading to multiple collateral formation and other associated complications owing to long-standing nature of the disease. We present the case of a 22-year-old male with symptoms and signs of gastrointestinal bleed and pain in the left upper abdomen with similar episodes in the past.

Superior mesenteric vein to the right testicular vein shunt operation for jejunal varices bleeding associated with extrahepatic portal vein obstruction after pancreaticoduodenectomy: a case report

BackgroundCauses of extrahepatic portal vein obstruction include abdominal surgeries such as pancreaticoduodenectomy. We improved jejunal variceal bleeding due to extrahepatic portal vein occlusion after pancreaticoduodenectomy, by shunting of the testicular vein.Case presentationA 72-year-old man was diagnosed with extrahepatic bile duct cancer and underwent subtotal stomach-preserving pancreaticoduodenectomy 5 years ago. No postoperative complications occurred, adjuvant chemotherapy using gemcitabine hydrochloride was performed, and the patient remained recurrence-free. One year and 6 months post-operation, extrahepatic portal vein stenosis appeared, but no recurrence was noted. However, 4 years and 6 months later, recurrent gastrointestinal bleeding occurred, and the patient was diagnosed with an extrahepatic portal vein obstruction. Double-balloon enteroscopy showed capillary dilatation and varicose veins in the hepaticojejunostomy region, and venous bleeding from collateral blood vessels was diagnosed. A superior mesenteric vein to the right testicular vein shunt operation was performed, following which the gastrointestinal bleeding disappeared, and the anemia improved. Although transient hepatic encephalopathy occurred, conservative treatment relieved it. Double-balloon enteroscopy confirmed the disappearance of abnormal blood vessels.ConclusionsA portosystemic shunt operation using the right testicular vein effectively relieved refractory variceal bleeding around the hepaticojejunostomy site in the jejunum due to an extrahepatic portal vein obstruction after pancreaticoduodenectomy.

Mass-Like Extreme Wall Thickening of the Entire Common Bile Duct in a Case of Portal Cavernoma Cholangiopathy

AbstractPortal cavernoma cholangiopathy (PCC) refers to the cholangiographic abnormalities that occur in patients with portal cavernoma. These abnormalities may be either due to chronic portal vein thrombosis or extrahepatic portal vein occlusion. These abnormalities occur due to enlargement of the bridging tortuous paracholedochal, epicholedochal, and cholecystic veins exerting pressure on the bile ducts. Ischemic changes can also occur in the bile duct due to portal vein thrombosis, which affects the microvascular circulation or associated hepatic artery thrombosis. These may be either reversible with shunt procedures such as transjugular intrahepatic portosystemic shunt (TIPS) or irreversible in the advanced stage, leading to recurrent episodes of biliary pain, cholangitis, and cholestasis. Occasionally it may present as mass-like diffuse common bile duct (CBD) wall thickening, which may be confused with mimickers like primary CBD lymphoma, immunoglobulin G4-related sclerosing cholangitis, and even cholangiocarcinoma on imaging. Thus, we need to be aware of the mass-forming PCC imaging findings to avoid unnecessary invasive procedures like biopsy or surgical intervention. Here, we present a case of PCC, which presented as mass-like diffuse CBD wall thickening with patent lumen on ultrasound that led to further workup with contrast-enhanced computed tomography and magnetic resonance imaging. The wall thickening showed persistent delayed enhancement, no significant diffusion restriction, and there was also associated superior mesenteric vein thrombosis with multiple mesenteric collaterals. A positron emission tomography-CT scan also ruled out malignant disease as there was no uptake. Finally, a diagnosis of mass-forming PCC was made by combining imaging features and excluding other causes.

TIPS for Adults Without Cirrhosis With Chronic Mesenteric Venous Thrombosis and EHPVO Refractory to Standard-of-Care Therapy.

Extrahepatic portal vein occlusion (EHPVO) from portal vein thrombosis is a rare condition associated with substantial morbidity and mortality. The purpose of this study is to investigate the efficacy of transjugular intrahepatic portosystemic shunts (TIPS) for the treatment of chronic EHPVO, cavernomatosis, and mesenteric venous thrombosis in adults without cirrhosis who are refractory to standard-of-care therapy. Thirty-nine patients with chronic EHPVO received TIPS. Laboratory parameters and follow-up were assessed at 1, 3, 6, 12, and 24months, and every 6months thereafter. Two hepatologists adjudicated symptom improvement attributable to mesenteric thrombosis and EHPVO before/after TIPS. Kaplan-Meier was used to assess primary and overall TIPS patency, assessing procedural success. Adverse events, radiation exposure, hospital length-of-stay and patency were recorded. Cavernoma was present in 100%, with TIPS being successful in all cases using splenic, mesenteric, and transhepatic approaches. Symptom improvement was noted in 26 of 30 (87%) at 6-month follow-up. Twelve patients (31%) experienced TIPS thrombosis. There were no significant long-term laboratory adverse events or deaths. At 36months, freedom from primary TIPS thrombosis was 63%; following secondary interventions, overall patency was increased to 81%. TIPS in chronic, noncirrhotic EHPVO with cavernomas and mesenteric venous thrombosis is technically feasible and does not adversely affect liver function. Most patients demonstrate subjective and objective benefit from TIPS. Improvement in patency rates are needed with proper timing of adjuvant anticoagulation.

Early Percutaneous Intervention in Pediatric Chronic Extrahepatic Portal Vein Occlusion Is Feasible And Safe

Early Percutaneous Intervention in Pediatric Chronic Extrahepatic Portal Vein Occlusion Is Feasible And Safe

Clinical features and prognosis of primary myelofibrosis with hepatic vascular disease and/or portal hypertension

Objective To investigate the clinical features, liver histopathological features, treatment, and prognosis of primary myelofibrosis (PMF)-associated hepatic vascular disease and portal hypertension. Methods A retrospective analysis was performed for the clinical and pathological features of 68 patients who were diagnosed with PMF in 960 Hospital of the PLA Joint Logistics Support Force and Xijing Hospital of Digestive Diseases, Air Force Medical University, from July 2010 to December 2020, among whom 22 patients had hepatic vascular disease/portal hypertension as the main manifestation. The patients were divided into two groups according to the presence or absence of thrombosis, and treatment and prognosis were summarized. The Kaplan-Meier method was used to plot survival curves, and the log-rank test was used to compare long-term survival rate between the two groups. Results Among the 68 patients with PMF, 22 had hepatic vascular disease and/or portal hypertension, resulting in a prevalence rate of 32.35%, and among these 22 patients, 13 (59.1%) had extrahepatic portal vein occlusion, 1 (4.5%) had Budd-Chiari syndrome, and 8 (36.4%) had portal hypertension. Biopsy was performed for 7 patients, and pathological results showed extramedullary hematopoiesis in the liver and varying degrees of infiltration of lymphocytes, plasma cells, and eosinophils at the lobular and portal areas, but the lobular structure was normal. A total of 7 patients died during follow-up, among whom 5 died of complications associated with thrombosis or portal hypertension. The overall median survival time was 57.99 months for all patients; the median survival time was 45.33 months in patients with thrombosis and 64.00 months in patients without thrombosis, and although there was no significant difference between the two groups (χ2=3.035, P=0.081), the non-thrombosis group tended to have better survival and prognosis than the thrombosis group. Conclusion The possibility of PMF as the primary disease should be considered for patients with hepatic vascular disease and portal hypertension. Patients with PMF should be screened for hepatic vascular disease, and early intervention should be given. The patients without thrombosis tend to have better survival and prognosis than those with thrombosis.

Intrahepatic veno-venous collateralization and misrepresentative hepatic venous pressure gradients in children.

Accurate and reproducible means of measuring the portosystemic gradient are essential for risk stratification and treatment of portal hypertension. To report the reliability of hepatic venous pressure gradients in children with intrahepatic veno-venous collateralization. Between January 2012 and December 2019 (96months), 39 patients with native livers underwent wedge hepatic venography and hepatic venous pressure gradient measurements at a tertiary pediatric center. All archived images were reviewed for balloon isolation of the hepatic vein and hepatic vein-to-hepatic vein (HV-HV) collaterals. HV-HV collaterals were categorized as present on the basis of non-catheterized segmental venous opacification despite appropriate balloon isolation. Hepatic venous pressure gradient was defined as the difference of wedge and free hepatic venous pressures. Wedge portosystemic gradient was defined as the difference between wedge hepatic venous pressure and right atrial (RA) pressures. For patients subsequently undergoing portal venous catheterization, portosystemic gradient was defined as the difference between main portal vein and RA pressures. Thirteen of 39 (33.3%) patients demonstrated HV-HV collaterals on wedge hepatic venography. The mean hepatic venous pressure gradient was 5.2±3.8mmHg (range: 0-15mmHg). The mean hepatic venous pressure gradient was 3.6±2.6mmHg (range: 0-9mmHg) in the presence of HV-HV collaterals and 5.9±4.2mmHg (range: 1-15mmHg) in the absence of HV-HV collaterals (P=0.043). Twelve (30.8%) patients were found to have varices: 10 gastroesophageal, 1 rectal and 1 stomal. The mean hepatic venous pressure gradient in patients with varices was 5.4±47mmHg (range: 0-15mmHg). For patients with varices, mean hepatic venous pressure gradient was 3.0±2.7mmHg (range: 0-9mmHg) in the presence of HV-HV collaterals and 10.3±4.1mmHg (range: 5-15mmHg) in the absence of HV-HV collaterals (P=0.004). Four (10.3%) patients had extrahepatic portal vein occlusion: 3 with cavernous transformation and 1 with type Ib Abernethy malformation. All patients with extrahepatic portal vein occlusion demonstrated HV-HV collaterals compared with 8 of 35 (22.9%) patients without extrahepatic portal vein occlusion (P=0.002). Four of 39 (10.3%) patients underwent direct portal pressure measurements: 3 via transhepatic and 1 via trans-splenic portal access. All had demonstrated HV-HV collaterals on wedged imaging. One had extrahepatic portal vein occlusion. The mean time between wedge portosystemic gradient and portosystemic gradient measurement was 3.75days (range: 0-8days). The mean wedge portosystemic gradient was 4.5±3.1mmHg (range: 2-9mmHg) and the mean portosystemic gradient was 14.5±3.7mmHg (range: 12-20mmHg) (P=0.006). HV-HV collateralization is frequently observed in children undergoing wedged portal venography and leads to misrepresentative hepatic venous pressure gradients. All patients undergoing hepatic venous pressure gradient measurement should have wedged venography to identify HV-HV collaterals and to qualify measured pressures. Additional techniques to obtain representative pressures in the presence of HV-HV collaterals warrant further investigation.

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.

Management of Portal Hypertension in the Pediatric Population: A Primer for the Interventional Radiologist.

Interventional radiology's role in the management of portal hypertension in the pediatric population differs from the management of adult portal hypertension. In the pediatric population, portal hypertension is frequently secondary to thrombosis and cavernous transformation of the extrahepatic portion of the portal vein. Transjugular intrahepatic portosystemic shunt can be utilized to manage portal hypertension in children with intrinsic liver disease that results in cirrhosis and portal hypertension, and is often used as a bridge to transplant. While technically feasible in extrahepatic portal vein occlusion, the sequelae of portosystemic shunting are less desirable in a child. The Meso-Rex bypass procedure, which represents the mainstay of management for pediatric portal hypertension, provides surgical relief of extrahepatic portal vein obstruction and restores mesenteric venous blood flow to the liver. This article aims to review management of portal hypertension in children as it pertains to the interventional radiologist, including preoperative assessment, postoperative evaluation, and the management of complications of the Meso-Rex bypass.

Pediatric Transjugular Intrahepatic Portosystemic Shunts

AbstractTransjugular intrahepatic portosystemic shunt (TIPS) creation offers potentially life-saving portal decompression regardless of patient age or size, but has been underutilized in pediatric patients to date. Experience has therefore been limited, and the full clinical benefit in this population is yet to be realized. Those wishing to embark on pediatric TIPSs would benefit not only from significant experience in adult TIPSs, but also from an awareness of challenges posed by the unique pediatric population. We herein review the etiologies and manifestations of portal hypertension more common in children, highlight some of the technical nuances of creating TIPSs in smaller anatomy, and summarize the existing literature on the topic. As extrahepatic portal vein occlusion (EHPVO) occurs with greater frequency in pediatric patients, special attention is paid to this condition and its associated challenges.

Research advances in diagnosis and treatment of duodenal varices

Duodenal varices are an uncommon disease which occurs along the entire gastrointestinal tract outside the esophagus or stomach. Among ectopic varices, duodenal varices are the most common one. Cirrhosis, hepatic portal hypertension, extrahepatic portal vein occlusion and vasculitis contribute to the pathogenesis of ectopic variceal formation. The unusual pathophysiology, location, diversity and uncertain curative effect of DV are associated with its low detection rate and high mortality of patients. Therefore, the diagnosis and treatment of DV is one of the tough problems in clinical practice. This article reviews the current research advances in pathophysiology, diagnosis and treatment of duodenal varices to provide reference for clinical practice. Key words: Varicose veins; Duodenum; Diagnosis

A Study of Portal Hypertensive Polyps

Background: Portal hypertension induces mucosal changes like portal hypertensive gastropathy, duodenopathy and colonic mucosal abnormalities. These polyps are commonly found in the stomach than other parts of bowel. Portal hypertensive polyp usually occur in cirrhotic portal hypertension and are also in extra hepatic portal vein occlusion. Diagnostic criteria for portal hypertensive polyp is not yet clearly described. Hence it becomes essential to evaluate the prevalence of polyps in portal hypertensive patients. The aim was to estimate the prevalence of portal hypertensive polyps in patients with portal hypertension due to any etiology.Methods: This is a prospective observational study of patients with portal hypertensive polyps. All patients in age group of 18 to 80 years who had portal hypertension diagnosed by clinical, laboratory and imaging criteria who went upper gastro intestinal endoscopy from the period of May 2012 to April 2013 were included in the study. Diagnosis of portal hypertensive polyps was confirmed histologically. Patients with proton pump inhibitor use, gastric varices, h. pylori gastritis, history of inherited polyposis syndromes, patients who refused consent were excluded.Results: A total of 3,621upper GI endoscopies were done of which 428 patients had portal hypertension. Polyps were noted in 9 patients (2.1%). Out of 428 patients 366 had chronic liver disease and 62 had extrahepatic portal hypertension and out of them 1 was excluded due to Rapid urease test positivity. Histopathological examination showed four had extensive vascular proliferation and glandular hyperplasia (portal hypertensive polyp) two had hyperplastic polyp and other two had neuroendocrine tumour. Endoscopic appearances of polyps varied considerably, with sizes ranging from 5mm to 15 mm. Polyps were sessile or pedunculated, singular or multiple, found in the antrum, body of the stomach .There is no specific endoscopic features to identify portal hypertensive polyps.Conclusions: Portal hypertensive polyps are rare. There is no specific endoscopic features. Biopsy is necessary for diagnosis. Longterm studies are needed to characterise their significance.

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).

Creation of a percutaneous mesocaval shunt to control variceal bleeding in a child

Although transjugular intrahepatic portosystemic shunt (TIPS) placement is the standard procedure for the treatment of portal hypertension, it is often impossible to perform in patients with extrahepatic portal vein occlusion. In these patients, options for decompressing the liver are few. In this report, we present a novel solution for managing gastro-esophageal hemorrhage in a child with portal hypertension and extrahepatic portal vein occlusion, through the creation of a percutaneous mesocaval shunt.