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

No abstract available.
Adult ; Budd-Chiari Syndrome/complications/*pathology ; Carcinoma, Hepatocellular/complications/*pathology/therapy ; Humans ; Liver Neoplasms/complications/*pathology/therapy ; Liver Transplantation ; Magnetic Resonance Imaging ; Male ; Tomography, X-Ray Computed

OBJECTIVEDue to its minimal-invasive approach, endovascular procedure had replaced surgery in treating Budd-Chiari syndrome (BCS). The interventional therapy was a safe and effective treatment in adults with BCS and the cure rate was high. However Budd-Chiari syndrome in children and adolescents is rare. Published literature on interventional procedure for Budd-Chiari syndrome in children and adolescents is scarce. The aim of the study was to present results of percutaneous transluminal angioplasty (PTA) and stents placement in children and adolescents with BCS and to evaluate the efficacy and safety in these patients of this approach.

METHODTwenty-five patients [16 boys and 9 girls; average age of (14.5 ± 3.4) years old; age ranged from 5 to 17 years] with Budd-Chiari syndrome who were hospitalized from December 1990 to August 2012 were presented. All of them were diagnosed by color Doppler ultrasound scan while 12 of them had magnetic resonance venography (MRV) scan. All of the patients had undergone angiographic examination. Four cases with membranous obstruction of the inferior vena cava (IVC) were treated with PTA. One case with segmental block of IVC was treated with PTA and stent placement. Five cases with membranous obstruction of IVC and hepatic vein (/and accessory hepatic vein) were treated with PTA. Among 8 cases with membranous obstruction of hepatic veins, 6 cases were treated with PTA and the others with PTA and stent placement. Among 4 cases with blocks of 3 hepatic veins (HVs), one was treated with PTA, one with PTA plus catheter thrombolysis plus PTA, one with PTA and stent placement and the other one was unsuccessful. Three cases with obstruction of HV and accessory HV (AHV) were treated with PTA. Totally, 24 patients were treated with interventional approach and followed up.

RESULTThe procedure was successful in 24 patients. The involved veins (hepatic veins or IVC) were patented after interventional procedure. The pressure of hepatic vein was (42.1 ± 4.2) cm H2O (37-50 cm H2O) (1 cm H2O = 0.098 kPa) before the interventional therapy, while it was (17.3 ± 3.3) cm H2O (14-26 cm H2O) after it. The pressure of IVC was (30.6 ± 2.9) cm H2O (26-36 cm H2O) before the interventional therapy, while it was (18.8 ± 4.2) cm H2O (15-26 cm H2O) after it. The symptoms and signs vanished instantly after interventional procedure. There were no procedure-related complications. The rate of overall initial cure was 96%. The patients were followed up for a mean of 25.8 months (range 6 months to 8 years). Seven cases developed restenosis after first procedure. Five of them were treated with PTA, one with PTA plus catheter thrombolysis plus PTA, one with PTA and stent placement. All of the involved veins were patented again. Clinical symptoms were relieved. There were no procedure-related complications as well.

CONCLUSIONThe interventional procedure in children and adolescents with BCS is the same as in adults. Radiological therapeutic intervention is efficacious and safe in children and adolescents with BCS.

Adolescent ; Angioplasty ; Budd-Chiari Syndrome ; diagnostic imaging ; surgery ; therapy ; Catheterization, Peripheral ; Child ; Child, Preschool ; Female ; Follow-Up Studies ; Hepatic Veins ; diagnostic imaging ; surgery ; Humans ; Liver ; blood supply ; diagnostic imaging ; Male ; Phlebography ; methods ; Radiography, Interventional ; Retrospective Studies ; Stents ; Thrombolytic Therapy ; Treatment Outcome ; Vena Cava, Inferior ; diagnostic imaging ; surgery ; Venous Thrombosis ; therapy

Although alveolar echinococcosis (AE) can cause a serious disease with high mortality and morbidity similar to malign neoplasms. A 62-year-old woman admitted to a hospital located in Sivas, Turkey, with the complaints of fatigue and right upper abdominal pain. On contrast abdominal CT, a 54x70x45 mm sized cystic lesion was detected in the left lobe of the liver that was seen to extend to the posterior mediastinum and invade the diaphragm, esophagus, and pericardium. The cystic lesion was seen to be occluding the inferior vena cava and left hepatic vein at the level where the hepatic veins poured into the inferior vena cava. Bilateral pleural effusion was also detected. We discussed this secondary Budd-Chiari Syndrome (BCS) case, resulting from the AE occlusion of the left hepatic vein and inferior vena cava, in light of the information in literature.
Animals ; Anthelmintics/therapeutic use ; Budd-Chiari Syndrome/drug therapy/*etiology/parasitology ; Echinococcosis, Hepatic/*complications/drug therapy/parasitology ; Echinococcus multilocularis/isolation & purification ; Female ; Humans ; Middle Aged

BACKGROUNDBudd-Chiari syndrome (BCS) is a posthepatic portal hypertension caused by the obstruction of the lumen of the hepatic veins or the proximal inferior vena cava (IVC). This study aimed to evaluate the clinical experience of interventional therapy for Budd-Chiari syndrome.

METHODSIVC venography was carried out first, the obliteration or stenosis in the IVC was opened or dilated with the hard guided wire or Rups100 puncture needle and balloon, then a stent was routinely implanted for the type of obliteration or stenosis.

RESULTSThe procedure was successful in 821 out of 903 cases including IVC intervention in 760 cases, and hepatic vein intervention in 61 cases. An IVC stent was used in 517 cases and hepatic vein stent in 19 cases. There were no pulmonary embolisms, but acute renal failure occurred in eight cases, hepatic coma in two cases and acute heart failure in 43 cases. Two patients died in this group and five cases were complicated with acute IVC thrombosis. Follow up of 7 to 124 months was made in 679 cases with recurrence found in 59 cases.

CONCLUSIONSInterventional therapy is safe and effective with a fast recovery for most types of BCS. It is gradually becoming the first therapeutic choice.

Adolescent ; Adult ; Aged ; Angioplasty, Balloon ; adverse effects ; Budd-Chiari Syndrome ; surgery ; therapy ; Child ; Female ; Humans ; Male ; Middle Aged ; Phlebography ; Treatment Outcome ; Young Adult

Angioplasty, Balloon ; methods ; Budd-Chiari Syndrome ; diagnostic imaging ; etiology ; therapy ; Hepatic Veins ; diagnostic imaging ; pathology ; Humans ; Liver ; diagnostic imaging ; pathology ; Liver Circulation ; Liver Transplantation ; adverse effects ; Living Donors ; Male ; Middle Aged ; Radiography ; Radiology, Interventional ; Retrospective Studies ; Treatment Outcome ; Ultrasonography, Doppler, Color

Administration, Oral ; Adult ; Biomarkers ; analysis ; Budd-Chiari Syndrome ; chemically induced ; diagnosis ; pathology ; Cyproterone Acetate ; adverse effects ; therapeutic use ; Diagnosis, Differential ; Ethinyl Estradiol ; adverse effects ; therapeutic use ; Female ; Humans ; Liver ; diagnostic imaging ; pathology ; Menstruation Disturbances ; drug therapy ; Radiography

OBJECTIVETo investigate the causes and treatment of postoperative venous outflow obstruction after orthotopic liver transplantation (OLT).

METHODSClinical data of 776 patients after OLT were analyzed retrospectively between January 2000 and December 2006. The accumulated experiences in diagnosis and treatment of postoperative outflow obstruction after OLT were reviewed.

RESULTSVenous outflow obstruction occurred in 10 patients (1.29%) after OLT, among those 6 had supra-hepatic inferior vena cava (IVC) stenosis, 2 had IVC stenosis of the post-hepatic segment, and 2 had outflow obstruction of hepatic vein. The diagnosis was confirmed by inferior vena cavography in all the patients. Of the 10 patients, 8 received percutaneous transluminal angioplasty (PTA) or metallic stent replacement, and 2 underwent liver retransplantation (re-LT) when interventional therapy failed. Three patients died from outflow obstruction, so the outflow obstruction related mortality was 30% in the patients.

CONCLUSIONSComplications of outflow obstruction after OLT were associated with surgical technique like vascular anastomosis, various types of cavo-caval anastomosis and graft size mismatch between donor and recipient. Making an early diagnosis and giving timely treatment including interventional therapy or re-LT is the key to improve the prognosis of outflow obstruction.

Adolescent ; Adult ; Aged ; Budd-Chiari Syndrome ; etiology ; therapy ; Child ; Child, Preschool ; Female ; Humans ; Liver Transplantation ; methods ; Male ; Middle Aged ; Postoperative Complications ; etiology ; therapy ; Retrospective Studies

Budd-Chiari Syndrome ; classification ; etiology ; therapy ; Humans

Adult ; Aortic Aneurysm ; surgery ; Aortic Rupture ; surgery ; Budd-Chiari Syndrome ; therapy ; Heart Atria ; injuries ; Humans ; Male ; Sinus of Valsalva ; Stents ; adverse effects