Budd-Chiari Syndrome Research Articles

Budd-Chiari syndrome as an initial manifestation of incomplete systemic lupus erythematosus

This article describes a case of a 26-year-old female with a history of Evan’s syndrome who presented with severe exertional dyspnoea and abdominal discomfort. The patient was diagnosed with chronic Budd-Chiari syndrome, a rare vascular disorder characterized by obstruction of the hepatic vein. We discuss the risk factors, the clinical manifestations, and diagnostic methods for Budd-Chiari syndrome, as well as the possible association with an underlying incomplete systemic lupus erythematosus. The importance of close follow-up and timely diagnosis for preventing disease progression and reducing mortality is emphasized. The article concludes by highlighting the need for further monitoring to identify any symptoms or signs suggesting a progression to complete lupus erythematosus.

A calcitonin secreting pancreatic neuroendocrine tumor with liver metastasis and hepatic vein thrombosis: a case report

A calcitonin secreting pancreatic neuroendocrine tumor with liver metastasis and hepatic vein thrombosis: a case report

Sternotomy and extracorporal circulation for fulminant Budd-Chiari syndrome due to leiomyosarcoma of the inferior vena cava

BackgroundBudd–Chiari syndrome is a rare and severe vascular liver disease. We presented patient with fulminant liver failure secondary to leiomyosarcoma of the IVC and thrombosis.Case presentationA 44-year-old female presented with fulminant liver failure secondary to inferior vena cava (IVC) thrombosis. Contrast-enhanced computed tomography subsequently revealed a thrombus within the IVC, extending cranially to the right atrium and caudally to the renal veins. The patient’s condition, characterized by early comatose symptoms, necessitated surgical intervention. Under extracorporeal circulation, a right atriotomy with thrombus lesion removal and descending thrombectomy of the IVC was performed. Hepatic congestion resolved after the thrombus was removed. A pathological examination of the excised thrombus revealed the presence of high-grade leiomyosarcoma.ConclusionsIn cases where a thrombus extends from the IVC to the right atrium, urgent surgical intervention with extracorporeal circulation should be considered.

The histological grading of fibrosis in Budd-Chiari syndrome: A chronic liver disease, different from others.

Budd-Chiari syndrome (BCS) is an uncommon disease caused by hepatic venous outflow obstruction. They can result in centrilobular fibrosis, nodular regenerative hyperplasia and cirrhosis. Assessing liver fibrosis is crucial for determining the stage of BCS, predicting disease progression and guiding treatment decisions. Although this pathology has been known for decades, no useful grading system was assigned. This study aims to introduce a histologic fibrosis grading system for BCS patients. All patients from 2017 to 2022 (Sanjay Gandhi Postgraduate Institute of Medical Sciences [SGPGIMS]), Lucknow diagnosed with BCS for whom liver biopsy was performed were included in the study. The Budd-Chiari syndrome-Hepatic Fibrosis system (BCS-HFS) was implemented to grade fibrosis. The fibrosis grade was compared with the fibrosis percentage area and a correlation was found with the hemodynamic variables (hepatic venous pressure gradient[HVPG])and the prognostic scores. There were 56 patients with BCS. The median age was 27years, with a male-female ratio of 1.8:1. There was a significant difference in the fibrosis percentage, hemorrhage percentage and model for end-stage liver disease (MELD) score among the BCS-HFS grades (p < 0.05). There was a significant correlation between BCS-HFS and HVPG (ρ = 0.699, p < 0.001) and the MELD prognostic score (ρ = 0.474, p < 0.001). BCS-HFS is applicable for grading fibrosis in BCS. It can help in uniform histopathology reporting and for further prospective and comparative studies.

Acute Onset Budd-Chiari Syndrome in the Postpartum Period: A Case of Missed Diagnosis Leading to Rapid Deterioration

Acute Onset Budd-Chiari Syndrome in the Postpartum Period: A Case of Missed Diagnosis Leading to Rapid Deterioration

Guidelines for the diagnosis and treatment of idiopathic portal hypertension, extrahepatic portal obstruction, and Budd-Chiari syndrome in Japan.

This is the English version of the guidelines for the diagnosis and treatment of idiopathic portal hypertension, extrahepatic portal obstruction, and Budd-Chiari syndrome, which were established and revised in 2018 by the Aberrant Portal Hemodynamics Study Group under the jurisdiction of the Ministry of Health, Labor, and Welfare in Japan. These guidelines are excerpts, and the full version consists of 86 clinical questions and explanations, totaling 183 pages in Japanese.

Pseudo hepatic vein thrombosis in a newborn with infracardiac total anomalous pulmonary venous connection.

We report an interesting incidental liver finding during ECG-gated cardiac computed tomography (CT) in a newborn with infracardiac total anomalous pulmonary venous connection to the portal vein. This case shows a unique abnormality in hepatic perfusion that was initially mistaken for hepatic vein thrombosis. We review the altered hepatic blood flow distribution in this pathologic anatomy to help explain the observed hepatic perfusion abnormality on CT. This understanding will enable an imager to anticipate hepatic perfusion patterns in similar patients, potentially avoiding misdiagnosis and unnecessary further testing.

Chronic Budd-Chiari syndrome as an initial manifestation of primary antiphospholipid syndrome in a young patient.

Chronic Budd-Chiari syndrome as an initial manifestation of primary antiphospholipid syndrome in a young patient.

Systematic review of treatments for the gastrointestinal manifestations of systemic lupus erythematosus

ObjectivesTo comprehensively assess and present the evidence for treatments used in the management of the gastrointestinal manifestations of SLE. MethodsA systematic search of the literature from January 1990 to June 2022 was performed using the following databases: MEDLINE, EMBASE, PubMed and Cochrane. Key words relating to the gastrointestinal system, SLE, and treatment were used. Where there was sufficient evidence for the treatment of a manifestation, we excluded case series with <10 cases and case reports. However, for rarer manifestations with insufficient higher-level evidence, smaller case series and case reports were included. ResultsA total of 29 studies including 767 patients were included in the review; six cohort studies, 11 case-control studies, and 11 case series. Specific gastrointestinal manifestations included enteritis (5 studies), mesenteric vasculitis (3 studies), acute pancreatitis (5 studies), chronic pancreatitis (1 study), intestinal pseudo-obstruction (IPO) (2 studies), hepatitis (4 studies), protein-losing enteropathy (PLE) (6 studies), acute acalculous cholecystitis (2 studies), and Budd-Chiari Syndrome (1 study). Evidence for the treatment of Ascites (13 case reports), peritonitis (3 case reports), and miscellaneous GI manifestations (11 case reports) are included as a supplemental file. Most studies demonstrated a benefit from pulsed intravenous methylprednisolone (IVMP) in severe or life-threatening manifestations, and oral prednisolone for less severe manifestations. However, the quality of evidence was low, with a high risk of bias in all studies. ConclusionThis review highlights the need for standardised disease definitions and terminology, as well as consideration of including gastrointestinal manifestations in disease scoring systems. There is a significant need for high-quality clinical trials in the treatment of the gastrointestinal manifestations of SLE, which will likely need to be multi-centre. We hope that this review will promote awareness of the gastrointestinal manifestations of SLE, and serve as a practical guide for evidence-based treatment.

Complex Antiphospholipid Syndrome Successfully Controlled for 17 Years with Personalized Enoxaparin Therapy.

This patient presented with rare manifestations of antiphospholipid syndrome (APS), such as acalculous cholecystitis, Budd-Chiari syndrome, and Addison's disease, all linked to ischemic events caused by vascular thrombosis. These atypical presentations highlight the varied and severe clinical spectrum of APS, requiring physicians to be vigilant about unusual complications.After multiple severe thrombotic and haemorrhagic events, the patient was successfully managed with long-term enoxaparin, with dose adjusted according to anti-factor Xa activity (which allowed for a significant dose reduction in comparison with the predicted empiric dose).The patient remained event-free for 17 years, reflecting the importance of personalizing anticoagulation therapy.

Early relaparotomy in recipients after living donor liver transplantation: causes, risk factors, and consequences.

Despite advancements in surgical methodologies and the extensive perioperative and postoperative care administered to recipients, the prevalence of complications requiring early relaparotomy following living donor liver transplantation (LDLT) remains persistent. This study sought to analyze the determinants influencing relaparotomy occurrences in the initial 30 days following LDLT. Additionally, it was aimed to evaluate the impact of early laparotomy on both graft and patient survival within this distinct patient cohort. The study encompassed recipients (n = 535) aged 18 years and older who underwent primary LDLT at our institution from January 2019 to December 2021. Exclusion criteria involved patients necessitating early retransplantation. Early relaparotomy was specified as surgical intervention within the initial 30 days following LDLT. The study enrolled a total of 535 patients, among whom 85 (15.9%) underwent early relaparotomy. The median age of the patients was 54 (range: 41-60) years, with a predominant male representation (66.2%). Univariate analysis comparing the laparotomy and nonrelaparotomy groups revealed statistically significant differences in the creatinine (p = 0.043) and sodium (p = 0.025) levels, graft side (p < 0.001), etiology (p = 0.005), and blood loss (p = 0.012).In the multivariate analysis, creatinine (p = 0.039; OR = 1.668; 95% CI = 1.027-2.709) and left lobe graft (p < 0.0001; OR = 3.611; 95% CI = 1.960-6.652) emerged as independent risk factors for relaparotomy. The primary causes of early relaparotomy following LDLT include postoperative bleeding, biliary leakage, and vascular complications. Preoperative elevation in creatinine and sodium levels, the presence of Budd-Chiari syndrome, utilization of a left lobe graft, and intraoperative blood loss are identified as risk factors associated with early relaparotomy after LDLT. Patients undergoing early relaparotomy exhibit inferior survival rates compared to those who do not.

Transjugular Intrahepatic Portosystemic Shunt for Budd-Chiari Syndrome: A Single-Centre Experience.

Background: Despite several challenges in clinical management, there has been significant progress in understanding the aetiology, natural history and outcomes of Budd-Chiari syndrome (BCS) treatments. This study aims to evaluate the outcomes of transjugular intrahepatic portosystemic shunt (TIPS) using covered stent in management of BCS. Methods: We conducted a retrospective analysis of 70 BCS patients who underwent TIPS using covered stents between January 2010 and December 2022 at a single tertiary liver transplant centre. Patients' clinical features, laboratory parameters, and imagine findings were collected before and after TIPS. The primary endpoint was overall survival. Results: TIPS was performed on 70 patients with BCS out of a total of 88 patients. The remaining patients (18) underwent liver transplantation. The mean age was 37.7 ± 11.2 years at time of diagnosis and the majority were female (64.35). The most common symptoms and signs at presentation were abdominal pain, jaundice, ascites, and variceal bleeding. Over a median followup of 76 months, the survival rates at 1, 3, and 5 years were 98.8%, 97.9%, and 97.7%, respectively. Patients who underwent TIPS alone had better survival that patients with BCS who required liver transplantation (LTx) (p = 0.003). Conclusions: In our study TIPS provided a highly effective treatment option for BCS patients. The long-term favourability of the outcome was not impacted by the need for repeated TIPS revision. Use of covered stents was instrumental in reducing shunt dysfunction rates. Prospective and larger studies are needed to further optimize therapeutic strategies in this challenging population.

Characteristics and Outcome of Primary Budd Chiari Syndrome due to Myeloproliferative Disorder in Egyptian Patients, A Single Center Study

Abstract Background Budd–Chiari syndrome (BCS) is a rare life-threatening disorder caused by obstruction of the hepatic venous outflow tract at any level between the small hepatic veins and the right atrium, hence also known as hepatic venous outflow tract obstruction (HVOTO). Primary BCS is often related to multiple thrombophilic conditions, including primary myeloproliferative disorders. Aim of the Work The present study aimed to assess characteristics of Primary Budd Chiari syndrome due to Myeloproliferative disorder in Egyptian patients and the outcome of the radiologic endovascular intervention (TIPS or angioplasty) Patients and Methods This was a retrospective cohort study that was carried out in The Tropical Medicine department, at Ain Shams University Hospitals. A total of 37 patients were enrolled, including 12 males and 25 females, with an age of 15-49 years (mean: 32 years) with primary BCS and MPDs during the interval between January 2006 and March 2023. Results JAK2 was positive in 64.9% of BCS patients, more in ET patients. ET was more frequent in BCS due to MPDs. Furthermore, these results validate routine inclusion of JAK2V617F in the diagnostic workup of BCS. Rate of stent occlusion was high 33.3% due MPDs thrombophilic burden nature. Conclusion MPDs are major aetiology of BCS. TIPS placement is an effective treatment for portal hypertension, but in any case, long-term high intensity anticoagulation treatment is essential in order to prevent recurrent thrombosis.

JAK2 Mutation Assessment in Thrombotic Events at Unusual Anatomical Sites: Insights from a High-Altitude Cohort.

Thrombosis stands as a significant contributor to both morbidity and mortality in individuals afflicted with myeloproliferative neoplasms. This retrospective study investigated the association between JAK2 mutations and venous thrombosis at unusual sites, and in young individuals with ischemic stroke, residing at high altitudes in the Aseer region, Saudi Arabia. Data were collected from two high-altitude referral hospitals over three years (2020-2022). Records of all JAK2 mutation tests were reviewed. Those requested as part of evaluation of thrombosis events, without known myeloproliferative neoplasms (MPNs) were analysed. Among the 208 JAK2 tests, 40 (19.2%) were linked to thrombotic event evaluations. The cohort, with a median age of 41, included 17 (42.7%) males and 23 females, with 57.5% having completely normal complete blood counts (CBC). Thrombotic events were divided between splanchnic vein thrombosis (36.6%) and cerebral thrombosis (34.1%), while the remaining cases involved unprovoked deep vein thromboses/pulmonary embolisms and portal vein thrombosis. Only 2 (5%) participants tested positive for JAK2 mutations: a 17-year-old male diagnosed concurrently with polycythemia vera after renal vein thrombosis and a 31-year-old woman with hepatic vein thrombosis and a normal CBC. This study reveals that JAK2 mutations are infrequently found in high-altitude patients with unprovoked DVT, PE, or atypical thrombosis. While JAK2 testing is notably relevant for splanchnic vein thrombosis, its routine use for other thrombotic events, particularly with normal CBC results, remains uncertain. Given the study's limitations, further prospective research with larger cohorts is needed to refine guidelines for JAK2 mutation testing in various thrombotic contexts.

S2901 Dialysis Catheter-Associated Budd Chiari Syndrome Presenting as Ischemic Colitis

S2901 Dialysis Catheter-Associated Budd Chiari Syndrome Presenting as Ischemic Colitis

S4522 A Case of Acute Budd Chiari Syndrome Treated With Portosystemic Shunting

S4522 A Case of Acute Budd Chiari Syndrome Treated With Portosystemic Shunting

Budd Chiari syndrome (BCS) and Myeloproliferative Neoplasms (MPN): A Moroccan Experience Center

Background and Objectives: Budd–Chiari syndrome is a vascular disorder of the liver which can cause fulminant liver injury and lethal portal hypertension-related complications. It is a rare disease and can be primary or secondary. The objective of our work is to detail evolution, treatment of patients with BCS and MPN according to the experience of a Moroccan center. Patients and Methods: This is a retrospective and descriptive study in the university hepato-gastroenterology department including all patients with BCS and MPN with portal hypertension (PH) over a period of 29 years. All our patients benefited from an etiological work-up and morphological explorations. Results: Out of a total 29 patients had BCS, 4 had MPN with a prevalence of 10%. Clinically, the signs of decompensated PH were predominant. Imaging confirmed BCS. The etiological work-up showed that all our patients had essential thrombocytemia. We had also association of other prothrombotic factors in 50 % of cases and a portal thrombosis in 25% of cases. Our patients had received treatment for the causative disease and treatment of thrombosis associated with the treatment of PH complications. The evolution was marked by the death of 2 patients (50%). Conclusion: The strong association between MPN and BCS is well established. The knowledge of the molecular mutations underlying MPN has dramatically improved in the last decade, allowing early diagnosis of MPN in a significant portion of BCS patient.

Application of liver biopsy in etiological diagnosis of unexplained portal hypertension: Porto-sinusoidal vascular disease should not be ignored.

The diagnostic value of liver biopsy has been confirmed in patients with abnormal liver test results; however, little data are available on its application in patients with portal hypertension. This study aimed to investigate the utility of liver biopsy for the etiological diagnosis of unexplained portal hypertension, and explore the clinical and pathological characteristics of each etiology. A retrospective observational analysis was conducted on 1367 patients who underwent liver biopsy at the Second Hospital of Nanjing from 2017 to 2019. Of these, 188 patients with unexplained portal hypertension were enrolled. The clinical and pathological characteristics were collected and reassessed in a multidisciplinary team meeting. Among these patients, 174 (92.6%, 174/188) had a definite etiological diagnosis through liver biopsy. The main etiologies were autoimmune hepatitis in 47 patients (25%, 47/188), autoimmune hepatitis-primary biliary cirrhosis overlap syndrome in 41 patients (21.8%, 41/188), and porto-sinusoidal vascular disease (PSVD) in 40 patients (21.3%, 40/188). Compared to liver cirrhosis, PSVD patients were younger and the liver function damage of which was subtler. The widths of portal vein diameter were widest in PSVD but the liver stiffness measurement were almost normal. Splenomegaly was common in PSVD, but ascites were less frequent than in autoimmune hepatitis (25.0% vs 51.1%, P = .013). Based on the histological patterns, we found that cholestatic liver diseases such as primary biliary cirrhosis, autoimmune hepatitis-primary biliary cirrhosis overlap syndrome, and progressive familial intrahepatic cholestasis could lead to non-cirrhotic portal hypertension, while vascular liver diseases such as PSVD and Budd-Chiari syndrome could also show fibrous proliferation as the disease progresses. Liver biopsy is safe and valuable for etiological diagnosis of unexplained portal hypertension. Cirrhosis is the leading cause of portal hypertension, and porto-sinusoidal vascular diseases should also be considered. Clinical features may be helpful in suggesting the cause; however, pathological examination is still indispensable for disease diagnosis and progression assessment.

Hepatocellular Carcinoma Associated with Secondary Budd-Chiari Syndrome.

Hepatocellular Carcinoma Associated with Secondary Budd-Chiari Syndrome.

Interventions in Budd-Chiari syndrome: an updated review.

Budd Chiari syndrome is a potentially treatable disease, and imaging is the key to its diagnosis. Clinical presentations may vary, ranging from asymptomatic to fulminant disease. Subacute BCS is the most common type encountered in clinical practice, characterized by ascites, hepatosplenomegaly, dilated abdominal wall veins, and varicosities in the lower limb and scrotum. While hepatic vein thrombosis is the leading cause in the West, membranous and short segmental occlusion are predominant in the Asian populations. These geographical variations have an impact on the treatment algorithm in managing BCS. Anticoagulation alone often fails to prevent disease progression, demanding further interventional therapy. Interventional therapy carries a lower morbidity and mortality than surgery. Anatomical recanalization and portosystemic shunting form the basis of endovascular management. Membranous or short-segment occlusion are best treated by angioplasty, which restores the physiological venous outflow and possibly disease reversal. Suboptimal results with angioplasty require stenting. Transjugular intrahepatic shunt (TIPS) or direct IVC to portal vein shunt (DIPS) decompresses the portal pressure and reduces the sinusoidal congestion, which in turn diminishes hepatocellular damage and hepatic fibrosis. Despite its ability to modify the disease course, TIPS carries several procedure and shunt-related complications, mainly hepatic encephalopathy. Thus, anatomical recanalization precedes TIPS in the traditional step-up approach in managing BCS. However, this concept is challenged by some authors, necessitating future reseach. TIPS is a valid bridge therapy in BCS with acute live failure awaiting liver transplantation. Despite all, interventional therapies fail in a subset of BCS patients, leaving them with only option of liver transplantation.