A Case of Budd-Chiari Syndrome Secondary to Tumor Thrombosis.

Budd-Chiari syndrome (BCS) is a rare constellation of conditions due to obstruction of venous flow from anatomical levels ranging from the hepatic veins to the confluence of the inferior vena cava (IVC) and right atrium. The resulting retrograde flow of blood leads to hepatomegaly, ascites, and liver failure among other features. Our case highlights the clinical features, diagnostic challenges, and management of a patient with a tumor thrombus from a metastatic prostate adenocarcinoma in a 67-year-old male leading to BCS. This patient, with a past history of prostate adenocarcinoma and aortic valve replacement on chronic warfarin anticoagulation, presented with acutely worsening abdominal pain and a distended abdomen, and imaging revealed an IVC filling defect. Subsequent imaging with a piflufolastat prostate-specific PET showing increased uptake in the IVC elucidated the diagnosis of tumor thrombosis. Management considerations include aggressive therapy and optimization of quality of life. The patient was offered both options, and options including surgical shunting, bypasses, and anticoagulation were discussed. After shared decision-making, the patient and family opted to choose the pathway of palliative radiation and anticoagulation.

Clinical Manifestations and Outcomes of Budd-Chiari Syndrome in Children: A Single-Centre Study.

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. The patients' medical records from December 2020 to July 2023 were obtained from Sisoft Healthcare Information System. In this study, we employ a set of predetermined questions to retrieve relevant data retrospectively and then organise it in Excel spreadsheets. SPSS version 26 (Armonk, NY: IBM Corp.) was used to analyse the data. Categorical variables are shown as frequencies (%), while continuous variables are reported as mean±SD. Results Of 37 (n) patients diagnosed with BCS, 19 (51.35%) were male and 18 (48.65%) were female. The mean age of presentation was 9.8±4.1 years. Ascites are the predominant clinical manifestation (100%), followed by hepatomegaly (37.8%). A total of 45.9% of patients had deranged liver function tests. Chronic BCS is the predominant mode of presentation. Protein C deficiency was present in nine patients (24.3%), two patients (5.4%) had protein S deficiency and two patients (5.4%) had antithrombin III deficiency. Hepatic veins exhibited the highest incidence of obstruction (73.0%). Liver biopsies were done in 15 (40.54%) patients to determine the staging of fibrosis. Eight patients (21.62%) had undergone radiological interventions, two patients had liver transplants and the rest were treated with medications, including anticoagulants. Conclusion BCS can present in acute, subacute or chronic forms. Ascites and hepatomegaly should raise the suspicion of BCS in children. Common radiological findings are non-visualisation of the hepatic veins. BCS has a wide range of aetiologies and treatment options. Protein C deficiency is the most predominant procoagulant disorder. Radiological interventions during the acute and subacute forms of BCS usually have excellent results. Liver transplant remains the definite treatment.

Innovation in direct intrahepatic portosystemic shunt; a developing country experience.

Anticoagulants are the first-line treatment option for patients with Budd-Chiari syndrome (BCS); however, intervention is required when this fails. Although, the ultimate treatment is liver transplant, other radiological procedures are for the management of the disease and bridge to definitive therapy. TIPS (trans jugular intrahepatic portosystemic shunt) is a method used by interventional radiologists to create a shunt between portal vein and hepatic vein. At times it is technically not possible, in such cases, direct intrahepatic portosystemic shunt (DIPS) is performed. This patient underwent a successful DIPS procedure for BCS along with balloon dilatation (venoplasty) for inferior vena cava (IVC) stenosis.

Venous thrombosis of the liver: current and emerging concepts in management.

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. Management decisions are influenced by a variety of factors including the chronicity, extent, and etiology of thrombosis. In this review we examine both portal vein thrombosis and hepatic vein thrombosis (and the associated Budd-Chiari Syndrome). We consider those factors which most impact presentation and most influence treatment. In so doing, we see how the particulars of specific cases introduce nuance into clinical decisions. At the same time we attempt to organize our understanding of such cases to help facilitate a more systematic approach. Critically, we must recognize that although increasing evidence is emerging to help guide our management strategies, the available data remain limited and largely retrospective. Indeed, current paradigms are based largely on observational experiences and expert consensus. As new and more rigorous studies emerge, treatment strategies are likely to be continually refined, and paradigm shifts are sure to occur.

Direct reopening of the occluded hepatic veins of Budd-Chiari syndrome: verification of our operative method by the perioperative course of esophageal varices.

OBJECTIVE: A total of 69 patients with Budd-Chiari syndrome (BCS) were operated by direct approach under cardiopulmonary bypass (CPB). To assess the operative procedure, the perioperative course of esophageal varices (EVs) was evaluated. PATIENTS AND METHODS: Of the 69 patients, 59 (22 females) were enrolled in this study because they had complete follow-up data for endoscopic evaluation of EVs. Their mean age was 46.3 ± 13.0 years (range 21-73.3 years). EVs were found in 52 patients. Under partial cardiopulmonary bypass, the inferior vena cava (IVC) was incised. The obstruction of the IVC was excised, and the occluded hepatic veins were reopened. The incised IVC was reconstructed with an auto-pericardial patch. RESULTS: Postoperatively, the repaired IVC was patent in all patients. The average number of patent hepatic veins (HVs) increased from 1.23 ± 0.81 to 2.21 ± 0.97/patient. The pressure gradient between the IVC and right atrium (RA) decreased from 12.4 ± 5.52 to 4.46 ± 3.21 mmHg. The indocyanine green clearance test (ICG) at 15 min decreased from 31.57 ± 17.44 to 22.27 ± 15.23%. EVs had disappeared in 13 patients at discharge and in 6 patients at late postoperative follow-up. CONCLUSION: Our operative procedure for BCS is useful for decreasing portal pressure, which is reflected by disappearance of EVs. Therefore, the high risk of EV rupture could be avoided by reopening the occluded HVs.

Accuracy of computed tomographic angiography in the diagnosis of patients with inferior vena cava partial obstruction in Budd-Chiari syndrome.

BACKGROUND AND AIM: The diagnosis of the partially obstructed inferior vena cava (IVC) in Budd-Chiari syndrome (BCS) patients has received little attention. We aimed to evaluate the diagnostic accuracy of computed tomographic angiography (CTA) for patients with BCS and a partially obstructed IVC. METHODS: A total of 329 patients with BCS and an obstructed IVC were endovascularly treated with balloon dilation and/or stent placement. All patients underwent a CTA examination prior to endovascular treatment, and the data were retrospectively reviewed. The presence of a round, oval, irregular shape or jet sign low-density area without enhancement within the enhanced proximal IVC was considered a sign of a partially obstructed IVC. Digital subtraction angiography was used as the gold standard. RESULTS: The results from the digital subtraction angiography revealed a partially obstructed IVC in 108 BCS patients and a complete obstruction in 221 patients. The CTA discovered a partially obstructed IVC in 99 patients and a completely obstructed IVC in 230 patients. From the CTA results, 15 were false negatives, and six were false positives. The patient-based evaluation yielded an accuracy of 93.6%, a sensitivity of 86.1%, specificity of 97.3%, positive predictive value of 93.9%, and negative predictive value of 93.5% for the detection of BCS patients with a partial IVC obstruction. CONCLUSIONS: Computed tomographic angiography offered a high diagnostic accuracy and sensitivity in BCS patients with a partially obstructed IVC. The low-density area within the enhanced proximal IVC above the membrane in artery phase can be considered a reliable indicator of a stenotic IVC in BCS patients.

Effects from right hepatic vein and inferior vena cava position on mechanism of Budd-Chiari syndrome / 医用生物力学

Objective To investigate the effects from various angles between inferior vein cava (IVC) and right hepatic vein (RHV) on pathogenesis of IVC membranous obstruction for patients with Budd-Chiari syndrome (BCS). Methods The normal 3D solid model of IVC and hepatic veins was reconstructed using MRI angiograms, and the angle between IVC and RHV was 56°. The two models with IVC-RHV angle of 30° and 120° were established, respectively, based on the reconstructed model. The distributions of wall shear stress, static pressure and blood velocity of the 3 models were calculated by numerical simulation. Results The wall shear stresses, static pressure and blood velocity of the 3 models displayed significantly differences. Compared with the normal 56° model, the 30° model showed a higher wall pressure and lower blood velocity, while the 120° model presented a lower wall pressure and blood velocity with turbulence of blood flowing, and such hemodynamic changes would increase the risk of thrombosis. The 56° model had the fastest blood velocity. Conclusions Numerical simulation of the flow in IVC and RHV can promote to discover the pathogenesis of BCS, and help to predict risk of IVC membranous obstruction, and provide theoretical references for BCS treatment.

Síndrome de Budd Chiari: Reporte de tres casos y revisión de la literatura / Budd Chiari Syndrome: Three Case Reports and a Literature Review

Se describen los casos de tres pacientes de sexo femenino a quienes se les hizo diagnóstico de síndrome de Budd Chiari. En una paciente la presentación del síndrome fue subaguda, pudiendo ser manejada exitosamente con la colocación de TIPS. Otra con mutación del factor V Leyden asociada desarrolló disfunción hepática progresiva y requirió de trasplante hepático. En dos de los tres casos se identificó una enfermedad hematológica como trastorno de base, y en uno el uso de anticonceptivos orales como factor de riesgo. Las tres pacientes fueron sometidas a terapia anticoagulante y el manejo quirúrgico fue definido de acuerdo a su condición clínica. Sin embargo, en un caso la presentación fue aguda con falla hepática y muerte.

This article describes the cases of three female patients who were diagnosed with Budd-Chiari syndrome. One patient was subacute and could be successfully managed by placement of a transjugular intrahepatic portosystemic stent (TIPS). Another patient who had the Factor V Leiden mutation developed associated progressive liver dysfunction and required liver transplantation. A hematologic disease was identified as the underlying disorder in two of the three cases. For one patient, the use of oral contraceptives was a risk factor. Since all three patients were undergoing anticoagulant therapy, surgical management was determined according to each patient’s clinical condition. Nevertheless, the one patient who that presented acute hepatic failure did not survive.