Budd-Chiari syndrome (B-CS) is a complicated hepatic vascular disease caused by hepatic venous outflow obstruction.There are significant differences in the pathogenesis and treatment of B-CS between China and Western countries.Given the characteristics of B-CS in our country,Budd-Chiari Syndrome and Hepatic Vascular Diseases Professional Committee of Chinese Research Hospital Association organizes domestic experts in this field to formulate the "Chinese multidisciplinary collaborative expert consensus for the diagnosis and treatment of Budd-Chiari syndrome（2021 version）".This consensus elaborates the research status of epidemiology,pathogenesis,disease classification,clinical manifestations,diagnosis and treatment of B-CS in China.Conducting basic research on pathogenesis and clinical research with high level evidence are important work direction in the future.This consensus is expected to provide guidance for clinicians to make optimal therapeutic schedules,so as to further standardize and improve the comprehensive diagnosis and treatment and basic research level of B-CS in China.
Budd-Chiari Syndrome/therapy* ; China ; Consensus ; Humans

OBJECTIVE: To assess segmental liver stiffness (LS) with MRI before and after endovascular intervention in patients with Budd-Chiari syndrome (BCS). MATERIALS AND METHODS: Twenty-three patients (13 males and 10 females; mean age, 42.6 ± 12.6 years; age range, 31–56 years) with BCS as a primary liver disease were recruited for this study. Two consecutive magnetic resonance elastography (MRE) examinations were performed before the endovascular treatment. Fifteen patients who underwent endovascular intervention treatment also had follow-up MRE scans within three days after the procedure. LS was measured in three liver segments: the right posterior, right anterior, and left medial segments. Inter-reader and inter-exam repeatability were analyzed with intraclass correlation coefficients (ICCs) and Bland-Altman analysis. Segmental LS and clinical characteristics before and after the intervention were also compared. RESULTS: Within three days of the endovascular intervention, all three segmental LS values decreased: LS of the right posterior segment = 7.23 ± 0.88 kPa (before) vs. 4.94 ± 0.84 kPa (after), LS of the right anterior segment = 7.30 ± 1.06 kPa (before) vs. 4.77 ± 0.85 kPa (after), and LS of the left medial segment = 7.22 ± 0.87 kPa (before) vs. 4.87 ± 0.72 kPa (after) (all p = 0.001). There was a significant correlation between LS changes and venous pressure gradient changes before and after treatments (r = 0.651, p = 0.009). The clinical manifestations of all 15 patients significantly improved after therapy. The MRE repeatability was excellent, with insignificant variations (inter-reader, ICC = 0.839–0.943: inter-examination, ICC = 0.765–0.869). Bland-Altman analysis confirmed excellent agreement (limits of agreement, 13.4–19.4%). CONCLUSION: Segmental LS measured by MRE is a promising repeatable quantitative biomarker for monitoring the treatment response to minimally invasive endovascular intervention in patients with BCS.
Budd-Chiari Syndrome ; Elasticity Imaging Techniques ; Female ; Follow-Up Studies ; Humans ; Liver Diseases ; Liver ; Magnetic Resonance Imaging ; Male ; Venous Pressure

OBJECTIVE: This study aimed to illustrate the magnetic resonance venography (MRV) manifestations of obstructed hepatic veins (HVs), the inferior vena cava (IVC), and accessory hepatic veins (AHVs) in patients with Budd-Chiari syndrome (BCS) and to evaluate the visualization capacity of MRV in the diagnosis of BCS. MATERIALS AND METHODS: Fifty-two patients with chronic BCS were included in this study. All patients were examined via MRV performed with a 3T system following injections of gadolinium-diethylene triamine pentaacetic acid (Gd-DTPA) or Gd-ethoxibenzyl-DTPA. HV and IVC lesions were classified, and their characteristics were described. HV cord-like occlusions detected via MRV were compared using ultrasonography (US). Digital subtraction angiography (DSA) was performed as a contrast in the MRV detection of IVC lesions. The HVs draining collaterals, mainly AHVs, were carefully observed. HV lesions were classified as segmental stenosis, segmental occlusion, membranous stenosis, membranous occlusion, cord-like occlusion, or non-visualized. Except for patent IVCs, IVC lesions were classified as segmental occlusion, segmental stenosis, membranous occlusion, membranous stenosis, and hepatomegaly-induced stenosis. RESULTS: All patients (52/52, 100%) showed HV lesions of different degrees. MRV was inferior to US in detecting cord-like occlusions (6 vs. 19, χ2 = 11.077, p < 0.001). Dilated AHVs, including 50 (50/52, 96.2%) caudate lobe veins and 37 (37/52, 71.2%) inferior HV and AHV lesions, were well-detected. There were no significant differences in detecting segmental lesions and thrombosis between MRV and DSA (χ2 = 0.000, p1 = 1.000, p2 = 1.000). The capacity of MRV to detect membranous lesions was inferior to that of DSA (7 vs. 15, χ2 = 6.125, p = 0.013). CONCLUSION: In patients with BCS, MRV can clearly display the lesions in HVs and the IVC, as well as in AHVs, and it has diagnostic and therapeutic value.
Angiography ; Angiography, Digital Subtraction ; Budd-Chiari Syndrome* ; Constriction, Pathologic ; Diagnosis ; Hepatic Veins* ; Humans ; Magnetic Resonance Imaging ; Phlebography* ; Thrombosis ; Ultrasonography ; Veins ; Vena Cava, Inferior*

No abstract available.
Budd-Chiari Syndrome* ; Dermatitis*

PURPOSE: The purpose of this study was to describe the long-term effects of stenting in patients with hepatic venous outflow obstruction (HVOO), who underwent living donor liver transplantation (LDLT). METHODS: Between January 2000 and December 2009, 622 adult patients underwent LDLT at our hospital, and of these patients, 21 (3.3%) were diagnosed with HVOO; among these patients, 17 underwent stenting. The patients were divided into early or late groups according to the time of their HVOO diagnoses (cutoff: 60 days after liver transplantation). RESULTS: The median follow-up period was 54.2 months (range, 0.5–192.4 months). Stent insertion was successful in 8 of 10 patients in the early group and 6 of 7 in the late group. The 5-year primary patency rates were 46% and 20%, respectively. In both groups, patients with recurrent HVOO at the beginning showed kinking confirmed by venography. Patients who carried their stents for more than 3 years maintained long-term patency. There was no significant difference in spleen size between groups; however, when the groups were compared according to whether they maintained patency, spleens tended to be smaller in the patency-maintained group. CONCLUSION: Unlike stenosis, if kinking is confirmed on venography, stenting is not feasible in the long term for patients with LDLT.
Adult ; Budd-Chiari Syndrome ; Constriction, Pathologic ; Diagnosis ; Follow-Up Studies ; Hepatic Veins* ; Humans ; Liver Transplantation* ; Liver* ; Living Donors* ; Phlebography ; Spleen ; Stents*

We report a case of percutaneous transhepatic stent placement for the treatment of hepatic venous outflow obstruction after extracorporeal hepatic resection and autotransplantation. A 63-year-old woman with a large mass in the liver was asymptomatic with no hepatic virus infection. Because the tumor was unresectable by conventional means, we used extracorporeal hepatic resection and autotransplantation for operation. Two days after surgery, hepatic venous outflow obstruction of the right and right inferior hepatic veins was suspected on computed tomography. After failure of the transjugular approach, hepatic venous stenting was performed successfully via the percutaneoustranshepatic approach.
Autografts* ; Budd-Chiari Syndrome ; Female ; Hepatic Veins ; Humans ; Liver ; Middle Aged ; Stents* ; Transplantation, Autologous*

Klinefelter's syndrome is the most common congenital abnormality that causes primary hypogonadism. It is associated with diseases that predominantly affect women, such as systemic lupus erythematosus (SLE), and it can sometimes cause veno-occlusive disease. We experienced a case of Budd-Chiari syndrome (BCS) in a 33-year-old man with Klinefelter's syndrome presented with hematemesis and edema in both lower extremities. The clinical and laboratory findings were compatible with SLE, antiphospholipid syndrome, and BCS. To the best of our knowledge, this is the first case report to describe a simultaneous presentation of these four clinical syndromes in a single patient.
Adult ; Antiphospholipid Syndrome* ; Budd-Chiari Syndrome* ; Congenital Abnormalities ; Edema ; Female ; Hematemesis ; Humans ; Hypogonadism ; Klinefelter Syndrome* ; Liver Cirrhosis ; Lower Extremity ; Lupus Erythematosus, Systemic*

No abstract available.
Budd-Chiari Syndrome* ; Complement System Proteins* ; Hemoglobinuria, Paroxysmal* ; Korea*

Klinefelter's syndrome is the most common congenital abnormality that causes primary hypogonadism. It is associated with diseases that predominantly affect women, such as systemic lupus erythematosus (SLE), and it can sometimes cause veno-occlusive disease. We experienced a case of Budd-Chiari syndrome (BCS) in a 33-year-old man with Klinefelter's syndrome presented with hematemesis and edema in both lower extremities. The clinical and laboratory findings were compatible with SLE, antiphospholipid syndrome, and BCS. To the best of our knowledge, this is the first case report to describe a simultaneous presentation of these four clinical syndromes in a single patient.
Adult ; Antiphospholipid Syndrome ; Budd-Chiari Syndrome ; Congenital Abnormalities ; Edema ; Female ; Hematemesis ; Humans ; Hypogonadism ; Klinefelter Syndrome ; Liver Cirrhosis ; Lower Extremity ; Lupus Erythematosus, Systemic

PURPOSE: The purpose of current study is to evaluate the response of the patients with portal vein thrombosis (PVT) or hepatic vein thrombosis (HVT) in hepatocellular carcinoma (HCC) treated with three-dimensional conformal radiation therapy (3D-CRT). In addition, survival of patients and potential prognostic factors of the survival was evaluated. MATERIALS AND METHODS: Forty-seven patients with PVT or HVT in HCC, referred to our department for radiotherapy, were retrospectively reviewed. For 3D-CRT plans, a gross tumor volume (GTV) was defined as a hypodense filling defect area in the portal vein (PV) or hepatic vein (HV). Survival of patients, and response to radiation therapy (RT) were analyzed. Potential prognostic factors for survival and response to RT were evaluated. RESULTS: The median survival time of 47 patients was 8 months, with 1-year survival rate of 15% and response rate of 40%. Changes in Child-Pugh score, response to RT, Eastern cooperative oncology group performance status (ECOG PS), hepatitis C antibody (HCVAb) positivity, and additional post RT treatment were statistically significant prognostic factors for survival in univariate analysis (p = 0.000, p = 0.018, p = 0.000, p = 0.013, and p = 0.047, respectively). Of these factors, changes in Child-Pugh score, and response to RT were significant for patients’ prognosis in multivariate analysis (p = 0.001 and p = 0.035, respectively). CONCLUSION: RT could constitute a reasonable treatment option for patients with PVT or HVT in HCC with acceptable toxicity. Changes in Child-Pugh score, and response to RT were statistically significant factors of survival of patients.
Budd-Chiari Syndrome ; Carcinoma, Hepatocellular* ; Hepatic Veins* ; Hepatitis C ; Humans ; Multivariate Analysis ; Portal Vein* ; Prognosis ; Radiotherapy ; Retrospective Studies ; Survival Rate ; Thrombosis* ; Tumor Burden ; Venous Thrombosis