Budd-Chiari Syndrome: Illustrated Review of Current Management

John D. Horton; Francisco L. San Miguel; Jorge A. Ortiz

Disclosures

Liver International. 2008;28(4):455-466. 

In This Article

Abstract and Introduction

Budd-Chiari syndrome (BCS) is characterized by hepatic venous outflow obstruction at any level from the small hepatic veins to the atriocaval junction. BCS is a complex disease with a wide spectrum of aetiologies and presentations. This article reviews the current literature with respect to presentation, management and prognosis of the disease. Medical, interventional and surgical management of BCS is discussed. Particular attention is paid to interventional and surgical aspects of management. The review is augmented by images, which provide a clinical corollary to the text.

In 1845, an internist named George Budd described the classic triad of abdominal pain, hepatomegaly and ascites. In 1899, pathologist Hans Chiari documented the histopathological features of what is now known as the Budd-Chiari syndrome (BCS).[1,2] BCS is characterized by hepatic venous outflow obstruction at any level from the small hepatic veins to the atriocaval junction, regardless of the cause of obstruction.[3,4]

The natural history of BCS is poorly understood.[5] Described treatment modalities for BCS include anticoagulation and treatment of underlying disease, percutaneous interventional techniques, transjugular intrahepatic portosystemic shunt (TIPS), surgical shunts and liver transplantation. Because of the rarity of BCS, there have been no randomized prospective trials comparing medical with surgical treatment of this disease.[3] Currently, clinical judgment and local expertise play a large role in the management of BCS. This review article discusses the presentation, management and prognosis of BCS with particular attention focused on the interventional and surgical aspects of treatment.

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