Budd-Chiari syndrome overview

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Aditya Govindavarjhulla, M.B.B.S. [2]

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Overview

The Budd-Chiari syndrome is the clinical picture caused by occlusion of the hepatic vein or inferior vena cava. Its presents with the classical triad of abdominal pain, ascites and hepatomegaly. Examples of occlusion include thrombosis of hepatic veins and membranous webs in the inferior vena cava. The syndrome can be fulminant, acute, chronic, or asymptomatic.

Historical Perspective

Budd-Chiari syndrome (BCS) was first discovered by George Budd in 1845. In 1899, Hans Chiari was the first to describe the histopathology of Budd-Chiari syndrome (BCS).

Classification

Budd-Chiari syndrome may be classified into several subtypes based on etiology, disease duration, severity and anatomical location of the occlusion.An obstruction below 300µm in diameter is not considered as BCS by some authors.Budd-Chiari syndrome may be classified according to etiology into two subtypes: primary and secondary.Budd-Chiari syndrome may be classified according to disease duration and severity into four subtypes: acute, subacute, chronic, fulminant liver failure.Budd-Chiari syndrome may be classified according to the anatomical location of obstruction into 3 subtypes: type I - truncal type, type II - radicular type, type III - venooclusive type.

Epidemiology and Demographics

It occurs in 1 out of 100,000 individuals and is more common in females. Some 10-20% also have obstruction of the portal vein.

Diagnosis

CT

Computed tomography (CT) or magnetic resonance imaging (MRI) is sometimes employed although these methods are generally not as sensitive.

Treatment

Medical Therapy

A minority of patients can be treated medically with sodium restriction, diuretics to control ascites, anticoagulants such as heparin and warfarin, and general symptomatic management. The majority of patients require further intervention. Milder forms of Budd-Chiari may be treated with surgical shunts to divert blood flow around the obstruction or the liver itself. Shunts must be placed early after diagnosis for best results. The transjugular intrahepatic portosystemic shunt (TIPS) is similar to a surgical shunt. It accomplishes the same goal but has a lower procedure-related mortality, which has led to a growth in its popularity. Patients with stenosis or vena caval obstruction may benefit from angioplasty. Limited studies on thrombolysis with direct infusion of urokinase and tissue plasminogen activator (tPA) into the obstructed vein have shown moderate success in treating Budd-Chiari syndrome; however, it is not routinely attempted.