|
|
 |
 |
| |
 Printable version |
Primary Budd-Chiari syndrome |
Sang Thrombose Vaisseaux. Volume 22, Number 4, 201-8, avril 2010, Mini-revue
|
 Résumé
Texte intégral
|
Author(s) : Pierre-Emmanuel Rautou, Aurélie Plessier, Bertrand Condat, Dominique Valla |
Summary : Primary Budd-Chiari syndrome is characterized by a blocked hepatic venous outflow tract at various levels from small hepatic veins to the inferior vena cava, resulting from thrombosis or its fibrous sequelae. This rare disease mainly affects young adults. Multiple risk factors have been identified and they are often combined in the same patient. Myeloproliferative diseases of atypical presentation account for nearly 50% of patients\; their diagnosis can be made by showing the V617F mutation in the Janus tyrosine kinase-2 gene of peripheral blood granulocytes and, should this mutation be absent, by showing clusters of dystrophic megacaryocytes onbone marrow biopsy. Presentation and manifestations are extremely varied which means that the diagnosis must be considered in any patient with acute or chronic liver disease. Doppler-ultrasound, computed tomography or magnetic resonance imaging of hepatic veins and inferior vena cava are usually successful in demonstrating non-invasively the obstacle or its consequences, the collaterals to hepatic veins or inferior vena cava. A therapeutic strategy has been proposed with anticoagulation, correction of risk factors, diuretics and prophylaxis for portal hypertension as first linemeasures. Angioplasty may be used and transjugular intrahepatic portocaval shunts (TIPS) may be effective before ultimately liver transplantation. Treatment progression is dictated by the response to previous therapy. This strategy has achieved 5-year survival rates approaching 90%. Medium-term prognosis depends on the severity of liver disease. Long-term outcome may be jeopardized by transformation of underlying conditions and hepatocellular carcinoma. In women with well-controlled liver disease, Pregnancy is not contraindicated. However, women should be fully informed of the persistent risks of such pregnancies. |
Keywords : Budd-Chiari, thombosis, hepatic vein, survival, angioplasty, TIPS |
|
