Budd-Chiari Syndrome

Aurélie Plessier; Dominique-Charles Valla

Disclosures

Semin Liver Dis. 2008;28(3):259-269. 

In This Article

Abstract and Introduction

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%.

Introduction

In the past, the term Budd-Chiari syndrome has been used to designate various entities, which was occasionally misleading. Recently, however, international panels have agreed that Budd-Chiari syndrome (BCS) be used as an eponym for hepatic venous outflow tract obstruction, whatever the level or the mechanism of obstruction.[1,2] Cardiac and pericardial diseases are excluded from this definition as well as sinusoidal obstruction syndrome occurring in the context of an exposure to toxic substances. BCS is further separated into secondary BCS when related to compression or invasion by a lesion originating outside the veins (benign or malignant tumor, abscess, cyst, etc.); and primary BCS when related to a primarily venous disease (thrombosis or phlebitis). Obstruction of the hepatic venous outflow tract can be classified according to its location: small hepatic veins (HVs), large HVs, inferior vena cava (IVC), and combined obstruction of large HVs and IVC.[3]

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