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Budd-Chiari syndrome: Management

Author
Michelle Lai, MD, MPH
Section Editor
Sanjiv Chopra, MD, MACP
Deputy Editor
Anne C Travis, MD, MSc, FACG, AGAF

INTRODUCTION

Budd-Chiari syndrome is defined as hepatic venous outflow tract obstruction, independent of the level or mechanism of obstruction, provided the obstruction is not due to cardiac disease, pericardial disease, or sinusoidal obstruction syndrome (veno-occlusive disease) [1]. Primary Budd-Chiari syndrome is present when there is obstruction due to a predominantly venous process (thrombosis or phlebitis), whereas secondary Budd-Chiari syndrome is present when there is compression or invasion of the hepatic veins and/or the inferior vena cava by a lesion that originates outside of the vein (eg, a malignancy).

This topic will review the treatment of Budd-Chiari syndrome. The etiology, clinical manifestations, and diagnosis of Budd-Chiari syndrome are discussed separately. (See "Etiology of the Budd-Chiari syndrome" and "Budd-Chiari syndrome: Epidemiology, clinical manifestations, and diagnosis".)

CATEGORIZATION

When patients are diagnosed with Budd-Chiari syndrome, they are categorized based on disease duration and severity [2,3]. The categories include (see "Budd-Chiari syndrome: Epidemiology, clinical manifestations, and diagnosis", section on 'Clinical manifestations'):

Acute (fulminant) liver failure: Characterized by acute liver injury with elevated transaminases, jaundice, hepatic encephalopathy, and an elevated prothrombin time/international normalized ratio; hepatic encephalopathy develops within eight weeks after the development of jaundice.

Acute (non-fulminant) Budd-Chiari syndrome: Clinical manifestations develop rapidly (over the course of weeks), with intractable ascites and hepatic necrosis.

                   

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Literature review current through: Nov 2016. | This topic last updated: Wed Nov 02 00:00:00 GMT+00:00 2016.
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