Therapy For Budd-Chiari Syndrome Research Articles

Diagnosis and therapy of Budd Chiari syndrome.

Budd-Chiari syndrome is described as a disorder characterized by the obstruction of hepatic venous outflow. The first description of the syndrome was done by George Budd in 1846. The etiology of the disease is multifactorial and requires differential diagnosing. The prognostic evaluation of patients with liver outflow obstruction differentiates special groups for further treatment procedures. The stepwise approach of Budd-Chiari syndrome allows the finding of the right technique on an individual basis for every patient.

Study on interventional treatment of Budd-Chiari syndrome in rat models

Objective To explore the feasibility of interventional therapy in rat models of Budd-Chiari syndrome (BCS). Methods A total of 50 male clean SD rats were divided into model group and control group using random number table method, with 25 rats in each group. In the model group, BCS rat model was constructed by adopting partial ligation of the inferior vena cava (IVC), and the control group only had separation of surrounding tissues of IVC. Liver function was studied 12 weeks after postoperative raising, and digital subtraction angiography (DSA) and interventional therapy were performed, coupled with liver histopathology staining. Results Twenty BCS rats survived till the 12th week of raising, with the survival rate reaching 80.0%, and 22 survived in the control group. Compared with the control group, ALT [(43.1±5.5) U/L vs. (62.6±4.6) U/L] and AST [(84.7±26.5) U/L vs. (161.7±25.8) U/L)] in serum of rats in the model group increased, the differences were statistically significant (P<0.05). HE staining showed that obvious hepatocyte necrosis and inflammatory cell infiltration were observed in BCS rats, and liver fibrosis was spotted via Masson staining. DSA examination found IVC obstruction in the model group, among which 14 (70.0%, 14/20) received interventional therapy after successful probing of IVC obstructed segment, and 7 had balloon dilatation with a diameter of 3.5 mm, with 6 (85.7%, 6/7) successfully dilatatedand the other 1 (14.3%, 1/7) failed; the remaining 7 had balloon dilatation with a diameter of 4.5 mm, with 2 (28.6%, 2/7) successfully dilatated, and the other 5 (71.4%, 5/7) died of IVC rupture. Conclusion The BCS rat models by partial ligation of IVC can well simulate the pathophysiological changes and angiopathy characteristics of IVC obstructive BCS patients, which provide a platform for the basic research of interventional therapy of BCS. Key words: Budd-Chiari syndrome; Model, animal; Vena cava, inferior; Rat, Sprague-Dawley; Interventional treatment

Clinical application of MSCT and MRI in diagnosis and interventional therapy of Budd-Chiari syndrome

Clinical application of MSCT and MRI in diagnosis and interventional therapy of Budd-Chiari syndrome

Interventional recanalization of Budd-Chiari syndrome due to old inferior vena cava thrombosis

Objective To evaluate the clinical safety and efficacy of interventional recanalization of Budd-Chiari syndrome (BCS) due to old inferior vena cava (IVC) thrombosis.Methods Between January 2008 and March 2013,23 patients with BCS due to old IVC thrombosis (16 males and 7 females) and with a mean age of (51.1 ± 11.7) years (range 32 ～71 years) underwent interventional recanalization.IVC balloon dilation was performed in 19 patients,and balloon dilation and stenting in 4 patients.All the patients received oral anticoagulant (warfarin) after interventional recanalization.On follow-up,color Doppler ultrasound was used to examine patency of the IVC and changes in the old IVC thrombus.Results Technical success was achieved in all 23 patients.There were no complications such as pulmonary embolism and no death.The mean pressure gradient between the IVC and the right atrium reduced from (27.0 ± 3.0) cmH2O (1 cmH2 O =0.098 kPa) before treatment to (3.5 ± 1.2) cm H2 O after treatment (t =45.8,P ＜ 0.05).The follow-up period ranged from 3 to 66 (mean ± SD,18.8 ± 16.5)months after interventional recanalization.Color Doppler ultrasound showed patency in the IVC lumen in 21 patients,and IVC reocclusion occurred in 2 patients.Of the 23 patients,complete resolution of the old thrombus was achieved in 15 patients and partial resolution was achieved in 8 patients.Conclusions Interventional recanalization is a safe and efficacious therapy for BCS due to old IVC thrombosis.The treatment achieved excellent clinical efficacy. Key words: Budd-Chiari syndrome; Inferior vena cava; Thrombus, old ; Recanalization

Clinical evaluation of interventional treatment for Budd-Chiari syndrome with hepatic vein thrombosis

Objective To evaluate the effect of interventional therapy for Budd-Chiari syndrome with hepatic vein thrombosis. Methods Twenty-five patients with Budd-Chiari syndrome complicated with hepatic vein thrombosis underwent catheter-directed urokinase thrombolysis, balloon dilation and/or stent placement. During follow-up, re-thrombosis and patency of the recanalized hepatic vein and inferior vena cava were evaluated by liver ultrasound. The pressure gradient of hepatic vein-right atrium or inferior vena cava-right atrium before and after interventional treatment was compared with paired t-test. ResultsTechnical success was obtained in 23 patients. Complete resolution and partial resolution of the thrombi were accomplished in 18 cases and 5 cases, respectively. The recanalized hepatic veins and inferior vena cava were patent. The mean pressure gradient of hepatic vein-right atrium dropped from (29±7) cm H2O to (8±3) cm H2O (1 cm H2O＝0.098 kPa) after the interventional treatment (t=13.7,P<0.01). The mean pressure gradient of inferior vena cava-right atrium dropped from (19±4) cm H2O to (5±2) cm H2O after the interventional treatment (t=13.3, P<0.01). Failures occurred in 2 patients. Over the follow-up period of 1 to 42 months[(18±10) months]after interventional treatment in the 23 patients, one late death occurred. Restenoses of hepatic veins were found in 2 patients, which were all redilated successfully. Neither restenosis of hepatic vein nor recurrence of thrombosis was found in the other 20 patients. Conclusion Interventional therapy could be effectively performed for the treatment of Budd-Chiari syndrome with hepatic vein thrombosis. Key words: Hepatic vein thrombosis; Angioplasty; Radiology,interventional

50 Years Ago in The Journal of Pediatrics: Budd-Chiari Syndrome

50 Years Ago in The Journal of Pediatrics: Budd-Chiari Syndrome

Update on the classification, assessment of prognosis and therapy of Budd–Chiari syndrome

Budd-Chiari syndrome (BCS) occurs as a result of obstruction of hepatic venous outflow at any level from the small hepatic veins to the junction of the inferior vena cava with the right atrium. Diagnosis can be difficult because of the wide spectrum of presentation of the disease and the varying severity of liver damage. The traditional classification of BCS--as fulminant, acute or chronic--is not prognostically useful. This makes assessing the benefit of therapy difficult, especially as there is no evidence from randomized studies. This article highlights advances in the prognosis and therapy of BCS. Identification of the site of venous obstruction has a major effect on prognosis. Portal-vein thrombosis occurs in 20-30% of cases, and acute presentation of BCS reflects an acute or chronic syndrome in 60% of BCS cases. BCS can be diagnosed and treated on a single occasion in the setting of the radiology department, with hepatic venography, transjugular liver biopsy, retrograde CO2 portography and inferior vena cava pressure measurements performed simultaneously with therapies such as dilation or stenting of webs in the inferior vena cava or hepatic veins, and placement of transjugular intrahepatic portosystemic shunts. Disruption of a portal vein thrombus can also be done during the same session. Surgical shunts have been superseded by the use of transjugular intrahepatic portosystemic shunts. Liver transplantation is reserved for fulminant and progressive chronic forms of BCS. Anticoagulation therapy must be used routinely, before and after specific therapy, regardless of whether a thrombophilic disorder is diagnosed.

Transjugular intrahepatic portosystemic shunt: current status

The transjugular intrahepatic portosystemic shunt (TIPS) was developed in the 1980s for treatment of complications of portal hypertension. Once it was shown that the shunt could be placed with relative ease, TIPS was rapidly applied to the treatment of many of the complications of portal hypertension. These complications include actively bleeding gastroesophageal varices, prevention of rebleeding from varices, control of refractory cirrhotic ascites and hepatic hydrothorax, and treatment of hepatorenal failure and hepatopulmonary syndrome. TIPS has also been used as therapy for Budd-Chiari syndrome and veno-occlusive disease. Despite these broad applications, TIPS has been compared with other forms of therapy in only 2 situations: prevention of rebleeding from varices and control of refractory cirrhotic ascites. In the trials, TIPS was shown to provide better control of these 2 complications of portal hypertension than standard forms of therapy. However, there was no improvement in survival and the incidence of encephalopathy was greater for patients receiving a TIPS. Thus, the use of TIPS for the control of ascites and prevention of rebleeding from varices should be limited to a select group of patients. There have been no controlled trials for the other indications listed. Despite the apparent efficacy of TIPS in many of these situations, its use should be limited to salvage therapy pending the publication of controlled trials showing it is a better treatment than other forms of therapy.