Budd–Chiari syndrome: illustrated review of current management

Horton, John D.; Miguel, Francisco L. San; Ortíz, Jorge

doi:10.1111/j.1478-3231.2008.01684.x

Cited by 77 publications

(97 citation statements)

References 56 publications

(130 reference statements)

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“…Its aetiology has been associated with the presence of thrombophilia and myeloproliferative diseases. The rarity of the condition has not allowed for concise reports of its worldwide prevalence [129].…”

Section: Liver Diseasesmentioning

confidence: 99%

Thrombin generation in different cohorts: Evaluation of the haemostatic potential

Chaireti¹

2013

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Section: Liver Diseasesmentioning

confidence: 99%

Thrombin generation in different cohorts: Evaluation of the haemostatic potential

Chaireti¹

2013

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“…According to possible sites of obstruction, BCS may be divided into hepatic vein obstruction, inferior vena cava obstruction and a mixture of hepatic vein and inferior vena cava obstructions. Development of effective vascular surgery and interventional treatment approaches for the treatment of BCS associated with diffuse hepatic vein obstruction is difficult (3,4). Multiple studies have shown that portal vein-vena cava surgical shunt does not increase the survival of BCS patients (5)(6)(7)(8).…”

Section: Introductionmentioning

confidence: 99%

Development of a canine model with diffuse hepatic vein obstruction (Budd-Chiari syndrome) via endovascular occlusion

Shen

Zhang

Wang

et al. 2013

Molecular Medicine Reports

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Abstract. The aim of the present study was to develop a reliable and reproducible canine model to mimic human diffuse hepatic vein obstruction (Budd-Chiari syndrome, BCS). A total of 24 canines were divided into an experimental (n=18) and a control (n=6) group. Under the guidance of digital subtraction angiography, a balloon catheter was delivered to the target hepatic vein (the common trunk of the left hepatic and middle hepatic veins) via the right external jugular vein. The balloon was inflated to completely block the vessels. For the canines in the experimental group, a mixture of N-butyl-cyanoacrylate (NBCA) and lipiodol (3-5 ml) was injected via the balloon catheter. Canines in the control group were injected with equal volumes of normal saline. Liver function and pathology were examined at 4, 6 and 8 weeks following surgery. BCS was successfully established in all members of the experimental group and there were no serious complications in either group. The left and middle hepatic veins and common trunk were completely obstructed at 4, 6 and 8 weeks following surgery in the experimental group, while in the control group, the hepatic vein remained unobstructed at 4 weeks. There was hepatocyte congestion and edema at 4 weeks following surgery in the experimental group and the edema became aggravated following 6 weeks. At 8 weeks following surgery, there was necrosis of hepatocytes and significant thickening of the hepatic vein tunica intima in addition to an increased number of elastic fibers. In conclusion, the present study demonstrates that a reliable and reproducible canine model of BCS can be developed by endovascular obstruction of the hepatic vein.

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“…1,2 The most common underlying disorders are myeloproliferative, such as polycythemia vera and essential thrombocytosis. 4,5 Other causes include factor V Leiden mutation, oral contraceptive use, protein C and S deficiencies, antiphospholipid syndrome, antithrombin III deficiency, paroxysmal nocturnal hemoglobinuria, cancer, Behçet syndrome, trauma, and idiopathic disorders. 4,6 Treatment options for BCS include anticoagulation, percutaneous interventional techniques, transjugular intrahepatic portosystemic shunt (TIPS), surgical shunts, and liver transplant.…”

Section: Introductionmentioning

confidence: 99%

“…4,5 Other causes include factor V Leiden mutation, oral contraceptive use, protein C and S deficiencies, antiphospholipid syndrome, antithrombin III deficiency, paroxysmal nocturnal hemoglobinuria, cancer, Behçet syndrome, trauma, and idiopathic disorders. 4,6 Treatment options for BCS include anticoagulation, percutaneous interventional techniques, transjugular intrahepatic portosystemic shunt (TIPS), surgical shunts, and liver transplant. 4 Therapeutic procedures are introduced in order of increasing invasiveness, based on the patient's response to previous therapy.…”

Section: Introductionmentioning

confidence: 99%

“…4,6 Treatment options for BCS include anticoagulation, percutaneous interventional techniques, transjugular intrahepatic portosystemic shunt (TIPS), surgical shunts, and liver transplant. 4 Therapeutic procedures are introduced in order of increasing invasiveness, based on the patient's response to previous therapy. 1,[7][8][9] Orthotopic liver transplant (OLT) should be considered in patients with fulminant BCS with irreparable necrosis of the liver parenchyma, as well as in patients with chronic forms of BCS accompanied by established cirrhosis and hepatic decompensation.…”

Section: Introductionmentioning

confidence: 99%

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Doğrul

Yankol²,

Mecit³

et al. 2016

Exp Clin Transplant

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Objectives: Budd-Chiari syndrome is a low-prevalence, life-threatening disorder characterized by hepatic venous outflow obstruction at the hepatic venules, the large hepatic veins, the inferior vena cava, or the right atrium. Orthotopic liver transplant should be considered for patients with fulminant and chronic forms of the syndrome. Materials and Methods: Fourteen patients received 15 orthotropic liver transplants at our center from September 2006 to March 2013. This study retrospectively reviewed the prospectively collected data from these 14 patients. Results: The mean age of the patients was 33 years; only 1 patient was female. The severity of liver disease was Child-Pugh score A in 1 patient, B in 4 patients, and C in 9 patients. Mean calculated Model for EndStage Liver Disease score was 18 (range, 6-30). The cause of Budd-Chiari syndrome was factor 5 Leiden mutation in 3 patients, polycythemia vera in 2 patients, factor 2 and 3 deficiency in 1 patient, fulminant essential throm bocytosis in 1 patient, and protein C deficiency in 2 patients. We performed 15 transplants in 14 patients. Five grafts were obtained from deceased donors, and 10 grafts were from livingrelated donors. Mean graft-to-recipient weight ratio was 1,12 for patients receiving a living-donor liver transplant. Median follow-up was 29 months. Patient survival rates were 87%, 71%, and 71% at 1, 3, and 5 years. Conclusions: Liver transplant is an option for treating Budd-Chiari syndrome in cases of fulminant presentation and cirrhosis. Living-donor liver trans plant is a viable choice in countries where procuring organ donations is still a problem. To manage the long-term medical therapy and follow-up for these patients, a careful evaluation is necessary to determine the cause of Budd-Chiari syndrome. Anticoagulant and antiaggregant therapy remains the mainstay of treatment for this syndrome.

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Budd–Chiari syndrome: illustrated review of current management

Cited by 77 publications

References 56 publications

Thrombin generation in different cohorts: Evaluation of the haemostatic potential

Thrombin generation in different cohorts: Evaluation of the haemostatic potential

Development of a canine model with diffuse hepatic vein obstruction (Budd-Chiari syndrome) via endovascular occlusion

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