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

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

OBJECTIVETo assess a new intervention for reestablishing the second hepatic hilum by means of puncturing and stenting the liver tissue between the intrahepatic vena cava and a hepatic vein for the treatment of Budd-Chiari syndrome (BCS).

METHODSTwo patients with BCS, in which no second hepatic hilum structure was found in transhepatic venography, underwent an interventional procedure of canalizing and stenting the parenchyma tract between the intrahepatic vena cava and a hepatic vein. The procedures were performed in the percutaneous transhepatic and right jugular vein, respectively. A metallic stent with a 10 mm diameter was implanted to maintain tract patency.

RESULTSThe free hepatic vein pressure (FHVP) of both patients decreased from 37 mm Hg to 5 mm Hg and from 28 mm Hg to 4 mm Hg, respectively, after the procedure. The complication of hemorrhage due to puncture was observed in one patient. Both patients maintained hepatic improvements in 3-year follow-up. Both clinical conditions and laboratory values were significantly improved after the procedure. Furthermore, the stented canals (the reestablished second hepatic hilum) maintained patent with normal FHVP, which was confirmed by control venography.

CONCLUSIONThe new technique provides a simple, safe, effective, and relatively inexpensive treatment of Budd-Chiari syndrome. Long-lasting effectiveness is expected.

Adult ; Budd-Chiari Syndrome ; therapy ; Hepatic Veins ; Humans ; Male ; Middle Aged ; Stents ; Vena Cava, Inferior

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

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

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

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

OBJECTIVEReport the results of interventional or semi-interventional techniques for 173 patients with Budd-Chiari syndrome.

METHODThis group included 120 males and 53 females. The pathologic lesions composed of localized complete occlusion of inferior vena cava (IVC) (78), IVC stenosis (49), IVC membrane with a hole (37), membrane of hepatic vein (HV) (3), IVC thrombosis (4), IVC membrane with thrombosis (2) and IVC lesion with occlusion of HV (32). Treatment methods included that I: Percutaneous transinferior vena cava angioplasty (PTA) (76); II: IVC PTA with stent (59); III: Percutaneous transhepatic vein recanalization (3); IV: IVC thrombolysis through a catheter (4), V: Combined transcardiac and transfemoral venous membranotomy and balloon dilation (22); VI: V and stent (17); VII: Stenting during radical surgery (3); VIII: Additional operation after intervention (23).

RESULTSThe immediate technique success rate for intervention was 90.1%, for the semi-intervention was 100%. The IVC pressure was reduced from 3 to 29 cmH2O. Complications occurred in 8 cases. The death rate was 2.9%. A follow-up study showed the recurrence rates were 14.5% in IVC PTA group, 1.7% in IVC PTA with stent, 18.2% in combined technique without stent and no recurrence was found in other groups.

CONCLUSIONThe PTA is the first choice for localized lesions. When elastic recoil occurs, immediate stenting is suggested. The semi-interventional approach is advised for PTA failure and more complicated cases. For those with both IVC lesion and occlusion of HV, the additional operation is needed after IVC intervention.

Adolescent ; Adult ; Aged ; Angioplasty, Balloon ; Budd-Chiari Syndrome ; surgery ; therapy ; Catheterization ; Child ; Female ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Stents

OBJECTIVETo evaluate the clinical value of various kinds of interventional techniques in the treatment of Budd-Chiari syndrome (BCS).

METHODSMultiple techniques such as recanalization of the inferior vena cava (IVC) under the guidance of marker and multi-angled fluoroscopy, recanalization of the hepatic vein with a transjugular approach, PTA, Z-expandable metallic stent (Z-EMS) implantation and modified TIPSS were used to treat 103 patients with BCS.

RESULTSOf 103 patients with BCS, 59 patients with obstruction of IVC were treated using recanalization of IVC. Seventeen patients with hepatic vein obstruction had their hepatic veins recanalized. The rest of the patients were given other methods of interventional treatment. Of all the subjects, 101 successfully underwent their procedures, with a success rate of 98.06%; and only 2 failed to recanalization of the IVC. Fifty-three patients were treated using PTA for the first time, with a success rate of 100%. In the 48 patients undergoing Z-EMS implantation for the first time, the success rate was 95.8%. Five patients were treated with modified TIPSS. After these interventional treatments, the success rate was 100%. Two patients died 16 h and 72 h respectively after operation because of DIC and severe hemoptysis. Seventy-two patients were followed up for 1 - 94 months (with a mean of 42.3 months). The mean follow-up of a BCS patient treated with PTA was 52.1 months, resulting in a primary patent rate of 59.4% and a restenosis rate of 40.6%. The mean follow-up of BCS treated with stenting was 33.5 months, with a primary patent rate of 87.5% and a restenosis rate of 12.5%. Eight patients died 7 - 64 months after the interventional procedure.

CONCLUSIONRecanalization of IVC or the hepatic vein transjugularly, PTA, Z-EMS implantation and modified TIPSS can be regarded as safe and effective micro-invasive methods in the treatment of BCS.

Adolescent ; Adult ; Angioplasty, Balloon ; Budd-Chiari Syndrome ; therapy ; Female ; Hepatic Veins ; surgery ; Humans ; Male ; Middle Aged ; Portasystemic Shunt, Transjugular Intrahepatic ; Stents ; Vena Cava, Inferior ; surgery

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