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Semin Liver Dis 2002; 22(1): 005-014
DOI: 10.1055/s-2002-23202
Copyright © 2002 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA. Tel.: +1(212) 584-4662

Hepatic Vein Thrombosis (Budd-Chiari Syndrome)

Dominique-Charles Valla
  • Service d'Hépatologie, Hôpital Beaujon; Université Paris VII; and Unité INSERM U481, Clichy, France
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Publication History

Publication Date:
27 March 2002 (online)

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ABSTRACT

Hepatic vein thrombosis is caused by one or several thrombogenic conditions, of which myeloproliferative disorders are the most frequent. Thrombosis and its fibrous sequelae can affect the veins diffusely or locally. Severity is determined by the extent and velocity of the thrombotic process. Development of venous collateral vessels is an important compensatory mechanism. Some patients can be totally free of symptoms. Major complications are intractable ascites, liver insufficiency, and gastrointestinal bleeding. Diagnosis can be made via ultrasonography or magnetic resonance imaging in a majority of cases. The main prognostic factors are age, Child-Pugh score, and response of ascites to diuretics. Medical therapy includes control of causal factors, anticoagulation, and nonspecific treatment of complications. Procedures aiming to restore outflow of hepatic blood are indicated in patients with uncontrolled manifestations. Percutaneous angioplasty, followed by portosystemic shunt (including a transjugular intrahepatic portosystemic stent shunt) and eventually liver transplantation can be proposed in a graded manner. The current 10-year survival rate is about 75%.

KEYWORD

Hepatic vein thrombosis - thrombogenic disorders - portosystemic shunt - angioplasty - transplantation

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