Budd-Chiari syndrome - Symptoms, Causes, Images, and Treatment Options

Diseases

Highlights & Basics

Key Highlights

Budd-Chiari syndrome (BCS) includes hepatic venous outflow obstruction at any level from the small hepatic veins to the junction of the inferior vena cava and the right atrium, regardless of the cause of the obstruction.
Classic triad of symptoms is abdominal pain, ascites, and hepatomegaly.
Seventy-five percent of patients have at least one underlying hypercoagulable state.
Color and pulsed Doppler ultrasonography is the first-line investigation for diagnosis. Patients should also undergo testing for hypercoagulable states to identify any predisposition to venous thrombosis.
The aim of therapy and interventional management is to relieve hepatic congestion and prevent necrosis, fibrosis, and ultimately liver failure, cirrhosis, and/or portal hypertension.

Hepatic venogram demonstrating "spider web" and thrombus in the inferior vena cava

Liver Transplantation Journal. 2006;12:S21-S22. Reprinted with permission of John Wiley & Sons, Inc

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Definition

Epidemiology

Etiology

Pathophysiology

Images

Hepatic venogram demonstrating "spider web" and thrombus in the inferior vena cava
Tests for prothrombotic conditions in patients with Budd-Chiari syndrome
Tests for prothrombotic conditions in patients with Budd-Chiari syndrome

Citations

Key Articles

Northup PG, Garcia-Pagan JC, Garcia-Tsao G, et al. Vascular liver disorders, portal vein thrombosis, and procedural bleeding in patients with liver disease: 2020 practice guidance by the American Association for the Study of Liver Diseases. Hepatology. 2021 Jan;73(1):366-413.[Abstract][Full Text]
de Franchis R, Bosch J, Garcia-Tsao G, et al. Baveno VII - Renewing consensus in portal hypertension. J Hepatol. 2022 Apr;76(4):959-74.[Abstract][Full Text]
Valla DC. The diagnosis and management of the Budd-Chiari syndrome: consensus and controversies. Hepatology. 2003;38:793-803.[Abstract]
Menon KV, Shah V, Kamath PS. The Budd-Chiari syndrome. N Engl J Med. 2004;350:578-585.[Abstract]
European Association for the Study of the Liver. EASL clinical practice guidelines: vascular diseases of the liver. J Hepatol. 2016;64:179-202.[Abstract][Full Text]

Other Online Resources

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Referenced Articles

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