REVIEWS   

Acta Phlebologica 2014 April;15(1):5-12

Copyright © 2014 EDIZIONI MINERVA MEDICA

language: English

The Budd-Chiari Syndrome: a review

Boucelma M.

University of Algiers Internal Medicine, Mohamed Lamine Debaghine Hospital, Algiers, Algeria

The Budd-Chiari syndrome is a heterogenous group of disorders characterized by hepatic venous out flow obstruction at the level of the hepatic venous out flow obstruction at the level of the hepatic veinules, the large hepatic veins, the inferior vena cava (IVC) or the right atrium. It has a dismal outcome if left untreated. Most of the IVC. Currently, a prothrombotic risk factor, either inherited or acquired, can be identified in the majority of patients. A combination of risk factors is present. Myeloproliferative are occurring in approximately half of the patients. Recent discovery of the V617F mutation in Janus tyrosine kinase-2 gene (JAK2) of peripheral blood granulocytes has significantly contributed to the diagnosis of myeloproliferative disorders. Typical presentation consists of abdominal pain, hepatomegaly and ascites, although symptoms may vary significantly. Doppler ultrasound, computed tomography or magnetic resonance imaging’s are successful in demonstrating the obstacle. Anticoagulation is indicated for all patients with BCS and additional therapy depends on the severity of symptoms and the extent of venous obstruction. A transjugular intrahepatic portosystemic shunt (TIPS) is proving to be a good therapeutic option, ultimately a liver transplantation should be considered.