Budd-Chiari

OBJECTIVES: Radiological intervention (RI) is the preferred treatment in children with Budd-Chiari syndrome (BCS). We studied the comparative long-term outcome of BCS children, with and without RI and utility of liver and splenic stiffness measurement (LSM, SSM) by 2-dimensional shear wave elastography (2D-SWE) in assessing response. METHODS: Sixty children (40 boys, median age 10.5 [6.5-15.25] years) with BCS (29 newly diagnosed, 31 follow-up) were evaluated. LSM and SSM by 2D-SWE and vascular patency were monitored pre- and postprocedure (≥ 6 months postprocedure) in those undergoing RI...

INTRODUCTION: The management of Budd-Chiari syndrome is determined on the basis of the severity of the disease. There are no standard guidelines regarding the management of Budd-Chiari syndrome in children, particularly in cases of liver transplantation. Therefore, we present a case of a pediatric patient with Budd-Chiari syndrome treated with liver transplantation. CASE PRESENTATION: A female patient aged 1 year and 8 months presented to the hospital with an enlarged stomach in the last 1...

INTRODUCTION: Cardiac functional abnormalities are common in patients with cirrhosis of the liver. Nonetheless, the effect of portal hypertension and liver disorder on cardiac abnormalities is yet to be investigated. The current study evaluated the contribution of cirrhotic and non-cirrhotic portal hypertension as the potential cause of cardiac abnormalities. METHODS: The present study was a cross-sectional observational study. After excluding known heart diseases, 128 patients with portal hypertension from different causes were enrolled in the study...

BACKGROUND AND AIM: Statin use has shown a reduction in hepatic decompensation and portal hypertension. Its association with portal vein thrombosis (PVT) incidence is unknown. We aim to compare the incidence of PVT in patients with and without statin use. METHODS: We excluded patients with a history of hepatocellular cancer, liver transplants, Budd-Chiari syndrome, and intra-abdominal malignancies. Patients with cirrhosis were followed from their first hepatologist clinical encounter (January 1, 2016, to January 31, 2021) for 180 days to determine PVT incidence...

The growth of several porto-portal collateral veins encircling an existing stenosed or obstructed entry vein is an uncommon condition known as portal cavernoma. It is traditionally shown as the entry vein thrombosis (portal vein thrombosis - PVT) outcome. A male of 25 years with stomach discomfort for three days that was acute, nonprogressive, and was not accompanied by fever, loose stools, or vomiting. After he had undergone abdominal ultrasonography, portal vein thrombosis was discovered, and based on no involvement of suprahepatic veins according to ultrasonography, Budd-Chiari syndrome was ruled out...

Splanchnic vein thrombosis (SVT), a thrombosis which involves the portal, mesenteric, and splenic veins, and the Budd-Chiari syndrome, represents an uncommon type of venous thromboembolism (VTE). Like with deep vein thrombosis of the lower extremities and pulmonary embolism, ample evidence suggests a significant association between SVT and cancer, particularly intra-abdominal solid malignancies (e.g. hepatobiliary and pancreatic cancers) and myeloproliferative neoplasms (MPN). Clinical symptoms of SVT in cancer patients can be ambiguous, and frequently attributed to the primary cancer itself...

BACKGROUND AND AIMS: The diagnosis of Budd-Chiari syndrome (BCS) is essentially radiological and is needed to plan appropriate therapy. We therefore conducted this proof of concept study to assess the utility of EUS in assessing anatomy of BCS. METHODS: A prospective multi-centre observational study including 50 consecutive patients with a diagnosis of BCS were enrolled. All patients underwent a detailed EUS examination by three independent endosonographers, blinded to the anatomical details of BCS and others' findings...

Objective: To summarize the clinical features and prognosis of Budd-Chiari syndrome with hepatopulmonary syndrome (HPS) in children. Methods: The clinical data of a child who had Budd-Chiari syndrome with HPS treated at the Department of Pediatrics of the First Affiliated Hospital of Zhengzhou University in December 2016 was analyzed retrospectively. Taking "Budd-Chiari syndrome" and "hepatopulmonary syndrome" in Chinese or English as the keywords, literature was searched at CNKI, Wanfang, China Biomedical Literature Database and PubMed up to July 2023...

UNLABELLED: The inferior vena cava (IVC) is an uncommon site for primary pathologies and secondary involvement is also infrequent, but involvement of the IVC can often drastically change management. It is therefore important to be cognizant of IVC pathologies. This review discussed common and rare neoplastic and non-neoplastic pathologies of the IVC as well as pathology mimics. Primary and secondary neoplasms can lead to tumour extension or bland thrombus formation and it is often important to distinguish between these two entities...

Objectives  Direct intrahepatic portosystemic shunt (DIPS) stent placement is an effective treatment for patients with Budd-Chiari syndrome (BCS); however, thrombotic occlusion of DIPS stent remains a cause of concern. The purpose of this study is to describe a novel technique of balloon-occluded-thrombolysis (BOT) for occluded DIPS stent, and compare it with the conventional catheter-directed-thrombolysis (CDT). Methods  In this retrospective study, the hospital database was searched for BCS patients who underwent DIPS revision for thrombotic stent occlusion between January 2015 and February 2021...

OBJECTIVE: To present the early results of a new technique for the treatment of renal cell carcinoma with intra-cardiac tumour extension and Budd-Chiari Syndrome. PATIENT AND METHODS: The first stage involves transdiaphragmatic debulking of the right heart, inferior vena cava and hepatic veins via median sternotomy, followed by a purse string suture placed in the IVC below the hepatic veins. The second stage is performed separately and involves en bloc resection of the affected kidney and inferior vena cava and vascular reconstruction via an abdominal incision...

Liver involvement is commonly seen in various haematological disorders. They present clinically with elevation of liver enzymes and organomegaly, with or without mass lesions. However, liver involvement may be silent in many hematological disorders or there may be specific findings in liver biopsy that can lead to the diagnosis of clinically inapparent hematological disorders. Present review highlights features of hepatic manifestations in various hematological diseases with special emphasis on histopathological findings...

Budd-Chiari syndrome (BCS) is an uncommon disease of the liver, characterised by obstruction of the hepatic venous outflow tract. The etiological spectrum of BCS as well as venous obstruction pattern show wide geographical and demographic variations across the globe. Compared to adults with BCS, children have primary BCS as the predominant etiology, earlier clinical presentation, and hence better treatment outcome. Underlying prothrombotic conditions play a key role in the etiopathogenesis of BCS, though work-up for the same is often unyielding in children...

OBJECTIVE: To investigate the value of multiparametric MR imaging to differentiate between small hepatocellular carcinoma (s-HCC) versus benign liver lesions in patients with Budd-Chiari syndrome. METHODS: 12 patients with benign hepatocellular lesions and 32 patients with small (<3 cm) HCCs were assessed. MRI images were reviewed by two radiologists blinded to the patient background information; lesion T1 and T2 signal intensities and ADC values were compared with the background liver...

Introduction Budd-Chiari syndrome (BCS) is a rare cause of ascites in children, and its clinical manifestation depends upon the extent and rapidity of the occlusion of hepatic veins. This study aimed to identify the clinical manifestations, causes, treatment options, and outcomes of BCS in children. Materials and methods A retrospective descriptive study of BCS in children under 15 years of age was conducted. This study was approved by the Pakistan Kidney and Liver Institute and Research Centre on June 23, 2023, with approval number 0128...

INTRODUCTION: In pediatric patients with Budd-Chiari syndrome (BCS), living donor liver transplantation (LDLT) raises substantial challenges regarding IVC reconstruction. CASE PRESENTATION: We present a case of an 8-year-old girl with BCS caused by myeloproliferative syndrome with JAK2 V617F mutation. She had a complete thrombosis of the inferior vena cava (IVC) with multiple collaterals, developing a Budd-Chiari syndrome. She underwent LDLT with IVC reconstruction with a cryopreserved pulmonary vein graft obtained from a provincial biobank...

Hepato-pancreato-biliary (HPB) emergencies in patients with cancer encompass an extensive array of various conditions, including primary malignancies that require prompt treatment, associated severe complications, and life-threatening consequences arising from treatment. In patients with cancer, the liver can be affected by chemotherapy-induced hepatotoxicity, veno-occlusive disease, Budd-Chiari syndrome, liver hemorrhage, and other complications arising from cancer therapy with all these complications requiring timely diagnosis and prompt treament...

We report the case of a male patient who had a history of early-onset protein-losing enteropathy, chronic diarrhea, and repeated thrombotic events since early childhood. He developed Budd-Chiari syndrome with consequent acute liver failure that required liver transplantation when he was 12 years old. The initial graft failed to function and he required retransplantation. Steroid-resistant rejection complicated the clinical course after the second transplant. Treatment with antithymocyte globulin stabilized graft function but abdominal symptoms and enteral protein loss persisted...

BCR::ABL1-negative myeloproliferative neoplasms (MPNs) are classically represented by polycythemia vera, essential thrombocythemia, and primary myelofibrosis. BCR::ABL1-negative MPNs are significantly associated with morbidity and mortality related to an increased risk of thrombo-hemorrhagic events. They show a consistent association with splanchnic vein thrombosis (SVT), either represented by the portal, mesenteric or splenic vein thrombosis, or Budd-Chiari Syndrome. SVT is also a frequent presenting manifestation of MPN...

Budd-Chiari syndrome (BCS) and sinusoidal obstruction syndrome (SOS) are two major vascular disorders of the liver, of which both can cause portal hypertension related complications, but their locations of obstruction are different. BCS refers to the obstruction from the hepatic vein to the junction between the inferior vena cava and right atrium, which is the major etiology of post-sinusoidal portal hypertension; by comparison, SOS is characterized as the obstruction at the level of hepatic sinusoids and terminal venulae, which is a cause of sinusoidal portal hypertension...