Budd-Chiari syndrome: Epidemiology, clinical manifestations, and diagnosis
- Michelle Lai, MD, MPH
Michelle Lai, MD, MPH
- Assistant Professor, Harvard Medical School
- Beth Israel Deaconess Medical Center
- Section Editor
- Sanjiv Chopra, MD, MACP
Sanjiv Chopra, MD, MACP
- Editor-in-Chief — Gastroenterology/Hepatology
- Section Editor — General Hepatology; Gallbladder and Biliary Tract Disease
- Professor of Medicine
- Harvard Medical School
- Senior Consultant in Hepatology
- James Tullis Firm Chief
- Beth Israel Deaconess Medical Center
Budd-Chiari syndrome (BCS) 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) . Primary Budd-Chiari syndrome is present when there is obstruction due to a predominantly venous process (thrombosis or phlebitis), whereas secondary Budd-Chiari 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 epidemiology, clinical manifestations, and diagnosis of Budd-Chiari syndrome. The etiology and treatment of Budd-Chiari syndrome are discussed separately. (See "Etiology of the Budd-Chiari syndrome" and "Budd-Chiari syndrome: Management".)
Studies suggest that in non-Asian countries, Budd-Chiari syndrome is more common in women and usually presents in the third or fourth decade of life (although it may occur in children or older adults) [2,3]. By contrast, in Asia, there is a slight predominance of men, with a median age of 45 years at presentation. In the non-Asian countries, pure hepatic vein blockage is more common, whereas in Asia, pure inferior vena cava or combined inferior vena cava and hepatic vein blockage predominate [3,4].
In a ten year study of hospital admissions in Italy that included 287 patients with BCS, the incidence rates for males and females were 2.0 and 2.2 per million inhabitants, respectively . The median age was 50 years (interquartile range 36 to 68 years) and 54 percent of patients were female. In another series that included 237 patients who had been treated for BCS at four centers in the United States, the Netherlands, and France, the median age was 35 years (range 13 to 76 years) and 67 percent of patients were female . The location of the outflow obstruction was in the hepatic veins (62 percent), inferior vena cava (7 percent), or both (31 percent), and 34 patients (14 percent) had associated portal vein thrombosis.
Budd-Chiari syndrome is categorized by disease duration and severity [6,7]:
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- CLINICAL MANIFESTATIONS
- Acute liver failure
- Acute Budd-Chiari syndrome
- Subacute and chronic Budd-Chiari syndrome
- When to consider Budd-Chiari syndrome
- Establishing the diagnosis
- - General approach
- - Radiographic findings
- Doppler ultrasonography
- Cross-sectional imaging
- Sulfur-colloid scintigraphy
- - Liver biopsy
- Evaluating for predisposing conditions
- DIFFERENTIAL DIAGNOSIS
- SUMMARY AND RECOMMENDATIONS