The Diagnosis and Management of Budd-Chiari Syndrome

The Diagnosis and Management of Budd-Chiari Syndrome

Abstract and Introduction

Abstract

Budd–Chiari syndrome is a rare disorder caused by hepatic venous outflow obstruction and resulting hepatic dysfunction. Despite a lack of prospective randomized trials, much progress has been made in its management over the last 20 years. The main goals of treatment are to ameliorate hepatic congestion and prevent further thrombosis. The selective use of anticoagulation, vascular stents, transjugular intrahepatic portosystemic stent-shunt and liver transplant has resulted in a significant increase in survival. The diagnosis, initial management and long-term follow-up of patients with Budd–Chiari syndrome is reviewed. The concept of individualization of treatment and a stepwise approach to invasive procedures is also discussed.

Introduction

The Budd–Chiari syndrome (BCS) was first described by a British physician, William Budd in 1845 in his seminal work 'Diseases of the Liver'. He reported the case of a man who died in King's College Hospital, London, in February 1844. All the hepatic veins seemed thicker and more opaque than natural and on examining them closely found a thin false membrane on their inner surface which in the large veins could be readily stripped off. Budd did not associate any clinical features with this finding. The description of the clinical features of hepatic vein outflow obstruction is generally attributed to a pathologist, Hans Chiari (although he was not the first). In 1899, Chiari described an "obliterating endophlebitis of the hepatic veins" and its association with hepatomegaly, ascites and abdominal pain.

The BCS initially referred to symptomatic occlusion of the hepatic veins, but later also to obstructive changes in the hepatic portion of the inferior vena cava (IVC) and hepatic vein orifices. The term 'obliterative hepatocavopathy' has been used to describe obstruction primarily affecting the hepatic portion of the IVC. More recently, the nomenclature has been clarified following the meeting of an expert study group. BCS is defined as hepatic venous outflow obstruction at any level from the small hepatic veins (HV) to the junction of the IVC and the right atrium, regardless of the cause of obstruction. Outflow obstruction caused by the sinusoidal obstruction syndrome (formerly 'veno-occlusive disease') and cardiac disorders is excluded.

The focus of this review will be primary BCS where hepatic venous outflow obstruction occurs as a result of thrombosis and its sequelae. Hepatic venous outflow obstruction caused by external compression or invasion of the venous lumen is termed secondary BCS and is seen with malignant tumors or large cysts.