Budd-Chiari Syndrome

ArticleinSeminars in Liver Disease 28(3):259-69 · September 2008with9 Reads
DOI: 10.1055/s-0028-1085094 · Source: PubMed
Abstract
Primary Budd-Chiari syndrome is related to thrombosis of hepatic veins or the terminal portion of the inferior vena cava. This rare disease is usually caused by multiple concurrent factors, including acquired and inherited thrombophilias. Half of the patients with primary Budd-Chiari syndrome are affected with a myeloproliferative disease, the recognition of which is largely based on the assessment of V617F Janus tyrosine kinase 2 (JAK2) mutation in peripheral granulocytes. A diagnosis of Budd-Chiari syndrome should be considered in any patient presenting with acute or chronic liver disease, as clinical manifestations are extremely diverse. Spontaneous outcome in symptomatic patients is poor. Diagnosis can be made in most patients noninvasively when imaging shows venous obstruction and/or collaterals. A treatment strategy is recommended where anticoagulation is given first, followed by angioplasty when appropriate, then TIPS in patients not responding to previous measure, and finally liver transplantation. This strategy has achieved 5-year survival rates close to 90%.
    • "The exact incidence and prevalence of BCS in the general population are unknown. The high pressure in the occluded HV leads to an enlarged liver and spleen, impaired liver function, refractory ascites, esophageal variceal bleeding, and even acute liver failure in severe cases [1]. BCS associated with IVC occlusion is more common than BCS with HV occlusion [2]. "
    [Show abstract] [Hide abstract] ABSTRACT: Background Budd–Chiari syndrome (BCS) with hepatic vein (HV) occlusion is manifested by severe liver damage in acute cases and esophageal variceal bleeding or refractory ascites in chronic cases, which is difficult to differentiate from cirrhotic portal hypertension. Aims To evaluate the clinical efficacy and safety of HV angioplasty and transjugular intrahepatic portosystemic shunt (TIPS) in the treatment of BCS with HV occlusion. Methods Between May 1995 and December 2014, 60 patients with HV occlusive BCS underwent HV angioplasty or TIPS. BCS was subacute or chronic in 55 patients and acute in 5 patients. HV angioplasty was performed in 18 patients with HV occlusion, combined HV and IVC angioplasty in 9 patients with HV and IVC occlusion, TIPS in 12 patients with HV occlusion, and modified TIPS in 21 patients with extensive HV occlusion. Results The interventional procedure was successfully performed in all 60 patients. The portal pressure decreased from 41.23 ± 10.46 cmH2O preoperatively to 26.68 ± 6.46 cmH2O postoperatively, while the portal flow velocity increased from 14.31 ± 10.43 to 52.16 ± 13.68 cm/s in patients undergoing TIPS or modified TIPS. During hospitalization, two patients died from hepatic failure, and acute shunt occlusion occurred in two other patients during subsequent treatment with repeated intervention. During 82.25 ± 46.16 months of follow-up, three patients underwent re-intervention with a stenotic shunt, and other three with repeated dilation of the stenotic HV. Conclusion HV angioplasty and TIPS yield excellent long-term outcomes in patients with HV occlusive BCS.
    Full-text · Article · May 2016
    • "Doppler ultrasound has a diagnostic sensitivity of more than 75% and is the first line investigation [1]. If an experienced sonographer is not available, MR imaging and CT evaluation are used for diagnostic confirma- tion [1,48]. Venography is recommended if the diagnosis remains uncertain or for the characterization of anatomy prior to treatment . "
    Article · Jan 2016 · United European Gastroenterology Journal
    • "7 Irrespective of the cause of hepatic venous outflow obstruction, increased hepatic sinusoidal pressure and portal hypertension quickly ensues, resulting in venous congestion and ischemic damage to the surrounding sinusoidal hepatocytes. 8 If hepatic sinusoidal 12 Department of Liver and Biliopancreatic Disorders, University Hospital Gasthuisberg KU Leuven, Leuven, Belgium [10.4.2015–4:20pm]123456789101112 //blrnas3.glyph.com/cenpro/ApplicationFiles/Journals/SAGE/3B2/UEGJ/Vol00000/150041/APPFile/SG-UEGJ150041.3d (UEG) [PREPRINTER stage] pressure is not relieved by therapeutic interventions or the development of a venous collateral system, then nodular regeneration, fibrosis and ultimately cirrhosis occur. "
    [Show abstract] [Hide abstract] ABSTRACT: Budd-Chiari syndrome (BCS) is a rare and potentially life-threatening disorder characterized by obstruction of the hepatic outflow tract at any level between the junction of the inferior vena cava with the right atrium and the small hepatic veins. In the West, BCS is a rare hepatic manifestation of one or more underlying prothrombotic risk factors. The most common underlying prothrombotic risk factor is a myeloproliferative disorder, although it is now recognized that almost half of patients have multiple underlying prothrombotic risk factors. Clinical manifestations can be diverse, making BCS a possible differential diagnosis of many acute and chronic liver diseases. The index of suspicion should be very low if there is a known underlying prothrombotic risk factor and new onset of liver disease. Doppler ultrasound is sufficient for confirming the diagnosis, although tomographic imaging (computed tomography (CT) or magnetic resonance imaging (MRI)) is often necessary for further treatment and discussion with a multidisciplinary team. Anticoagulation is the cornerstone of the treatment. Despite the use of anticoagulation, the majority of patients need additional (more invasive) treatment strategies. Algorithms consisting of local angioplasty, TIPS and liver transplantation have been proposed, with treatment choice dictated by a lack of response to a less-invasive treatment regimen. The application of these treatment strategies allows for a five-year survival rate of 90%. In the long term the disease course of BCS can sometimes be complicated by recurrence, progression of the underlying myeloproliferative disorder, or development of post-transplant lymphoma in transplant patients.
    Full-text · Article · Apr 2015
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