Budd–Chiari syndrome

BACKGROUND AND AIMS: EUS-guided portal pressure gradient (EUS-PPG) measurement is a novel method to evaluate portal hypertension severity. In this study, we determined the consistency between EUS-PPG and hepatic venous pressure gradient (HVPG) measurements in patients with acute or subacute portal hypertension. METHODS: Twelve patients were prospectively enrolled. EUS-PPG measurements were performed using a 22-gauge FNA needle and a central venous pressure measurement monitor...

INTRODUCTION: Budd-Chiari syndrome (BCS) is a rare condition affecting children. It is characterized by occlusion of venous outflow from liver at the level of hepatic veins (HV) or inferior vena cava (IVC). The management of BCS in children revolves around forming new collaterals for venous outflow or by elimination of blockage in the venous outflow tracts. These can be achieved by balloon venoplasty (BV), transjugular intrahepatic portosystemic shunting (TIPSS) or open shunt surgeries...

Acute Budd-Chiari syndrome is a rare condition characterised by obstruction of hepatic venous outflow. We describe the case of a 52-year-old man, with a congenital Morgagni diaphragmatic hernia, who presented with acute onset abdominal pain, shortness of breath, lactic acidosis, hyperbilirubinaemia and transaminasaemia. Computed tomography revealed strangulation of the diaphragmatic hernia and extrinsic compression of the inferior vena cava from the herniated viscera. Emergency surgery was carried out to repair the hernia with a biosynthetic mesh, with complete resolution of the Budd-Chiari syndrome...

BACKGROUND: Hereditary hemorrhagic telangiectasia (HHT) often involves the liver, and belongs to abnormal blood vessel disease. The etiology of Budd-Chiari syndrome (BCS) is not clear, but congenital vascular dysplasia is considered to be one of the causes. Liver cirrhosis due to hepatic hereditary hemorrhagic telangiectasia concomitant with BCS has not been reported. Here, we report a case of cirrhosis with hepatic hereditary hemorrhagic telangiectasia (HHHT) and BCS. CASE PRESENTATION: A 58-year-old woman with hepatic hereditary hemorrhagic telangiectasia showed decompensated liver cirrhosis, and abdominal imaging revealed Budd-Chiari syndrome...

OBJECTIVE: Interventional treatment is the main therapy for Budd-Chiari syndrome (BCS) with hepatic vein obstruction. The aim of this study was to investigate the long-term outcomes of endovascular management for BCS with chronic accessory hepatic vein (AHV) obstruction. METHODS: In total, 68 patients with primary BCS who underwent AHV dilation with or without stenting from January 2008 to December 2018 were included in this retrospective study. The technical success rate and complications were recorded...

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Background & Aims: Hepatobiliary phase (HBP) images can discriminate between benign and malignant liver lesions, but it is unclear if this approach can be used in patients with Budd-Chiari syndrome (BCS). Thus, we aimed to assess the diagnostic utility of HBP images in patients with BCS. Methods: This retrospective study included all patients admitted to our institution with a diagnosis of BCS and focal liver lesions on hepatobiliary contrast agent-enhanced MR imaging (HBCA-MRI) from 2000 to 2019...

OBJECTIVE: Our study aimed to evaluate the safety and efficacy of sequential interventional therapy for Budd-Chiari syndrome (BCS) caused by obstruction of the inferior vena cava (IVC) with fresh thrombus in the IVC. METHODS: Full medical records were obtained for 20 patients with BCS associated with fresh IVC thrombus who received sequential interventional therapy from 2014 to 2019 at our hospital. All patients underwent small-diameter percutaneous transluminal angioplasty (PTA) balloon catheter predilation combined with sequential catheter-directed thrombolysis and large-diameter PTA balloon dilation...

Venous thrombosis within the hepatic vasculature is associated with a distinct array of risk factors, characteristics, and potential complication. As such, it entails unique management considerations and strategies relative to the more common categories of venous thromboembolic disease. Although broadly divided into thrombosis of the afferent vasculature (the portal venous system) and efferent vasculature (the hepatic venous system), presentations and management strategies within these groupings are heterogeneous...

Epstein-Barr virus (EBV) and cytomegalovirus (CMV) infections are common and are associated with a variety of liver manifestations. EBV and CMV infections, in immunocompetent hosts, commonly manifest as acute hepatitis, with severity varying from asymptomatic, self-limited icteric hepatitis to acute liver failure. Atypical manifestations, such as cholestasis, chronic hepatitis, precipitation of acute-on-chronic liver failure, and autoimmune hepatitis, are reported with EBV infection, whereas cholestasis, portal vein thrombosis, and Budd-Chiari syndrome are reported with CMV infection...

Objective: To explore the correlation between prenatal clinical data with etiological diagnosis and neonatal outcome in isolated fetal ascites. Methods: Totally, 36 pregnancy cases diagnosed as isolated fetal ascites by ultrasound in Provincial Hospital Affiliated to Shandong University from June 22nd, 2016 to September 28th, 2018 were collected. Invasive prenatal diagnosis was performed by taking fetal cord blood, amniotic fluid, and fetal ascites respectively for cytogenetics, molecular genetics and biochemical examination and the impact of intrauterine therapeutic procedures on neonatal outcomes was evaluated as well...

Budd-Chiari syndrome may rarely occur as a complication of Behcet's disease, and presentation with thrombosis of both inferior vena cava (IVC) and hepatic veins is rarer still. We present a young woman with Behcet's disease who presented with acute Budd-Chiari syndrome, with thrombosis of IVC and all 3 hepatic veins. An IVC stent was placed, followed by a transjugular intrahepatic portosystemic shunt through the IVC stent. On follow-up, despite oral anticoagulants and oral steroids, she developed recurrent thrombosis twice within a 1-year span...

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BACKGROUND: Recently, as a possible therapy resolving solution, pentadecapeptide BPC 157 therapy, has been used in alleviating various vascular occlusion disturbances. BPC 157 was previously reviewed as novel mediator of Robert cytoprotection and endothelium protection in the stomach, and gut-brain axis, beneficial therapy in gastrointestinal tract, with particular reference to vascular recruitment, ulcerative colitis and tumor cachexia, and other tissues healing. Here we raised new hypothesis about BPC 157 therapy in the Budd-Chiari syndrome in rats, rapid bypassing of the suprahepatic inferior caval vein occlusion, and rats recovery with the active and effective pharmacotherapy treatment...

Budd-Chiari syndrome (BCS) is an uncommon disorder defined as an obstruction of the hepatic venous outflow. Percutaneous transluminal balloon angioplasty (PTA) is a less invasive treatment option for BCS patients. However, there are no reports regarding inferior vena cava (IVC) rupture caused by perforation route through a collateral vein during treatment of BCS. Here, we report a male patient with BCS who had a long segmental obstruction of the IVC and its collateral vessels. Here, IVC rupture occurred at the distal end of the obstructed IVC during a percutaneous angioplasty; the rupture was repaired successfully with an endovascular stent graft...

BACKGROUND AND AIMS: Infiltration of hepatic venous outflow in hepatic alveolar echinococcosis can lead to development of Budd-Chiari syndrome. Medical treatment of this condition is generally unsatisfactory. Radical hepatic resection is impossible for extensive parasitic involvement of liver. This is a case report on a patient who was successfully treated with percutaneous stenting of left hepatic vein followed by Ex vivo Liver Resection and Autotransplantation (ELRA). METHODS: Using the transjugular approach, a metal mesh stent was placed in the left hepatic vein of a 45-year-old man who presented with Budd-Chiari syndrome...

INTRODUCTION: Familial Mediterranean fever (FMF), the most frequent autoinflammatory disease, is caused by mutations in the MEFV gene. It is characterized by recurrent febrile attacks of polyserositis. Liver abnormalities may develop during its course, but they remain poorly defined. OBJECTIVE: To describe liver involvement in FMF patients. METHODS: A systematic search was conducted through PubMed/Medline and Embase from 1946 to January 2020...

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Prevention and control about the situation of 2019 coronavirus disease (COVID-19) are grim at present. In addition to supporting the frontline actively, medical workers in general surgery spare no efforts in making good diagnosis and treatment of specialized diseases by optimizing treatment process, providing medical advice online, mastering indications of delayed operation and emergency operation reasonably, etc. Budd-Chiari syndrome is a complex disorder, and severity of the disease varies, serious cases can be life threatening...