budd chiary syndrome

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...

Splanchnic or visceral vein thromboses (VVTs) are atypical thrombotic entities and include thrombosis of the portal vein, hepatic veins (Budd-Chiari syndrome), mesenteric veins, and splenic vein. All VVTs have in common high 30-day mortality up to 20% and it seems to be difficult to diagnose VVT early because of their rarity and their wide spectrum of unspecific symptoms. VVTs are often associated with myeloproliferative neoplasia, thrombophilia, and liver cirrhosis. VVT is primarily diagnosed by sonography and/or computed tomography...

BACKGROUND: Budd - Chiari syndrome (BCS) due to hepatic venous outflow obstruction is a rare cause of liver disease with dismal outcome, often amenable to catheter intervention. MATERIALS AND METHODS: This retrospective single-center study analyzed the clinical profile and medium-term outcome of interventional treatment with balloon angioplasty ± stenting in all pediatric BCS over a 10-year period. Clinical, laboratory, imaging, and interventional data were retrieved...

Budd-Chiari syndrome (BCS) is a rare condition characterized by the obstruction of hepatic venous outflow. It has various potential etiologies, with myeloproliferative neoplasms representing the most prevalent pathogenic association. Here, we present the case of a 51-year-old male who manifested abdominal pain and ascites. Subsequent clinical investigation revealed the presence of BCS secondary to a myeloproliferative syndrome, specifically polycythemia vera. This case emphasizes the importance of diagnosing BCS and conducting a thorough investigation into its underlying etiology...

INTRODUCTION AND IMPORTANCE: Surgical treatment of Budd-Chiari syndrome (BCS) includes endovenectomy followed by angioplasty of the inferior vena cava (IVC). Herein, we report a case of surgery using an open-chest approach in a patient with BCS. We modified the technique reported by Kuniyoshi et al. CASE PRESENTATION: A 45-year-old male, was diagnosed with BCS and referred to our hospital. We used an open-chest approach to remove stenosis in the IVC and angioplasty with a bovine pericardial patch...

OBJECTIVES: Polycythemia vera (PV) is classically thought to be associated with low erythropoietin (EPO) levels. Here, we present a review of the utility of using EPO levels in diagnosing polycythemia. METHODS: We conducted a systematic literature review of the Medline data through Pubmed and Google Scholar. We included the articles which described confirmed PV associated with elevated EPO level. Our search strategy included the following terms in Pubmed (((polycythemia vera[MeSH Terms]) OR (jak2 protein tyrosine kinase[MeSH Terms])) OR (Myeloproliferative Disorders[MeSH Terms])) AND (Erythropoietin[MeSH Terms]), and 'polycythemia vera with erythropoietin' in Google Scholar...

Splanchnic vein thrombosis includes Budd-Chiari syndrome and portal vein thrombosis. These diseases share common features: (i) they are rare diseases and (ii) they can lead to portal hypertension and its complications. Budd-Chiari syndrome and portal vein thrombosis in the absence of underlying liver disease share many risk factors, the most common being myeloproliferative neoplasms. A rapid and comprehensive workup for thrombosis risk factors is necessary in these patients. Long-term anticoagulation is indicated in most patients...

INTRODUCTION: The incidence of splanchnic vein thrombosis (SVT) in cancer patients has increased in recent years and its real clinical significance and management can be challenging. This study aimed to describe the clinical presentation and short-term outcomes of patients with cancer-associated SVT. MATERIAL AND METHODS: This was a retrospective observational study of consecutive patients with cancer-associated SVT diagnosed during the period 2015-2020. The primary objective was to describe the clinical presentation of SVT...

Budd-Chiari syndrome (BCS) patients with portal hypertension are often treated with a direct intrahepatic portosystemic shunt (DIPS) or transjugular intrahepatic portosystemic shunt (TIPS) and angioplasty. DIPS can be problematic, however, due to the technical difficulty of the procedure. To address this problem, we describe a method using the balloon used for inferior vena cava (IVC) dilatation as a puncture target to safely perform DIPS in a BCS patient with complete hepatic vein occlusion and stenosis of the IVC...

No abstract text is available yet for this article.

Hepatic caval stenosis is managed with stenting; however, stent placement can be complicated by migration, which can be life-threatening. The risk of migration can be mitigated by increasing the length of the stent, which increases contact with the vessel wall. We describe the cases of three patients with hepatic caval stenosis treated with two Z-stents sutured together. Each had an uncomplicated postoperative course and demonstrated clinical improvement. The use of sutured Z-stents can increase the stability of the stent and, therefore, decrease the morbidity associated with stent placement for hepatic caval stenosis...

BACKGROUND: Budd-Chiari syndrome (BCS) results when the outflow of the hepatic vein (HV) is obstructed. BCS patients exhibiting an accessory HV (AHV) that is dilated but obstructed can achieve significant alleviation of liver congestion after undergoing AHV recanalization. This meta-analysis was developed to explore the clinical efficacy of AHV recanalization in patients with BCS. MATERIALS AND METHODS: PubMed, Embase, and Wanfang databases were searched for relevant studies published as of November 2022, and RevMan 5...

A 24-year-old man was admitted to our hospital with abdominal distension. He was found to have acute liver failure and diagnosed with Budd-Chiari syndrome based on angiography and liver biopsy. Liver transplantation was deemed necessary when angiography showed extensive thrombotic occlusion of the hepatic veins and liver biopsy revealed submassive hepatic necrosis. The patient was found to have the JAK2V617F mutation, indicating a myeloproliferative neoplasm as the background disease. He developed hepatic encephalopathy but remained conscious on on-line hemodiafiltration...

Sharp recanalization for short-segment intravascular occlusion, using an endovascular route, has been described for inferior vena cava (IVC) occlusion. Often, the technical challenge to the endovascular management of Budd-Chiari syndrome (BCS) is the recanalization of the occluded hepatic vein or suprahepatic IVC. Presented here, the challenge was the level of occlusion of the suprahepatic IVC, with the resultant separation of both the patent IVC segments in a horizontal plane, making it technically challenging for sharp recanalization...

Splanchnic vein thrombosis (SVT) is a manifestation of venous thromboembolism in an unusual site. Portal, mesenteric, and splenic veins are the most common vessels involved in SVT which occurs mainly in patients with liver cirrhosis, although non-cirrhotic patients could be affected as well. Thrombosis of hepatic veins, also known as Budd-Chiari syndrome, is another manifestation of SVT. Prompt diagnosis and intervention are mandatory in order to increase the recalization rate and reduce the risk of thrombus progression and hypertensive complications...

Budd Chiari syndrome is an unusual vascular disease involving the hepatic vasculature and has significant mortality and morbidity if not treated early. Ultrasonography (USG), Doppler, computed tomography (CT), and magnetic resonance imaging (MRI) have classical imaging findings that can help make a reliable and quick diagnosis. Intervention radiology plays an important role in the treatment of these patients, helping avoid various complications and proper patient management. We are presenting a case report with classical imaging spectrum and highlighting successful intervention with hepatic vein stenting...

Point mutations in the DEAD-box helicase DDX24 are associated with vascular malformations such as multi-organ venous and lymphatic defect (MOVLD) syndrome and Budd-Chiari syndrome, with the pathogenesis largely uncharacterized. DDX24 is mainly located in the nucleolus, where nucleophosmin (NPM1) regulates nucleolar homeostasis via liquid-liquid phase separation (LLPS). However, the connection between DDX24 and NPM1 in vascular malformation remains elusive. Here we demonstrated that DDX24 formed biomolecular condensates in vitro and the mutated DDX24 protein, DDX24E271K , partitioned less into the nucleoli in tissues from patients with MOVLD syndrome and cultured endothelial cells (ECs), altering nucleolar morphology...

Since 1988, transjugular intrahepatic portosystemic shunt (TIPS) has been an effective therapy for portal hypertension in many settings. Thanks to continuous technical improvements and a wiser selection of patients, excellent results have been achieved with this therapeutic strategy. The historical indications for TIPS placement, in the context of liver cirrhosis, such as refractory ascites and variceal bleeding are now well established and known. However, in recent years, new indications are emerging. These have been investigated and approved in some studies but are not yet included in guidelines and clinical practice...