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
- Section Editor — 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 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 the West, 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 West, 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].
One of the largest published series from the West included 237 patients who had been treated for Budd-Chiari syndrome at four centers (in the United States, the Netherlands, and France) between 1984 and 2001 . Two-thirds of the patients were female, and the median age was 35 years (range 13 to 76 years). 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 [5,6]:
- DeLeve LD, Valla DC, Garcia-Tsao G, American Association for the Study Liver Diseases. Vascular disorders of the liver. Hepatology 2009; 49:1729.
- Mahmoud AE, Mendoza A, Meshikhes AN, et al. Clinical spectrum, investigations and treatment of Budd-Chiari syndrome. QJM 1996; 89:37.
- Plessier A, Valla DC. Budd-Chiari syndrome. Semin Liver Dis 2008; 28:259.
- Darwish Murad S, Valla DC, de Groen PC, et al. Determinants of survival and the effect of portosystemic shunting in patients with Budd-Chiari syndrome. Hepatology 2004; 39:500.
- Menon KV, Shah V, Kamath PS. The Budd-Chiari syndrome. N Engl J Med 2004; 350:578.
- Ferral H, Behrens G, Lopera J. Budd-Chiari syndrome. AJR Am J Roentgenol 2012; 199:737.
- Fickert P, Ramschak H, Kenner L, et al. Acute Budd-Chiari syndrome with fulminant hepatic failure in a pregnant woman with factor V Leiden mutation. Gastroenterology 1996; 111:1670.
- Darwish Murad S, Plessier A, Hernandez-Guerra M, et al. Etiology, management, and outcome of the Budd-Chiari syndrome. Ann Intern Med 2009; 151:167.
- Hadengue A, Poliquin M, Vilgrain V, et al. The changing scene of hepatic vein thrombosis: recognition of asymptomatic cases. Gastroenterology 1994; 106:1042.
- Mitchell MC, Boitnott JK, Kaufman S, et al. Budd-Chiari syndrome: etiology, diagnosis and management. Medicine (Baltimore) 1982; 61:199.
- De BK, Sen S, Biswas PK, et al. Occurrence of hepatopulmonary syndrome in Budd-Chiari syndrome and the role of venous decompression. Gastroenterology 2002; 122:897.
- Miller WJ, Federle MP, Straub WH, Davis PL. Budd-Chiari syndrome: imaging with pathologic correlation. Abdom Imaging 1993; 18:329.
- Millener P, Grant EG, Rose S, et al. Color Doppler imaging findings in patients with Budd-Chiari syndrome: correlation with venographic findings. AJR Am J Roentgenol 1993; 161:307.
- Chawla Y, Kumar S, Dhiman RK, et al. Duplex Doppler sonography in patients with Budd-Chiari syndrome. J Gastroenterol Hepatol 1999; 14:904.
- Grant EG, Perrella R, Tessler FN, et al. Budd-Chiari syndrome: the results of duplex and color Doppler imaging. AJR Am J Roentgenol 1989; 152:377.
- Gupta S, Barter S, Phillips GW, et al. Comparison of ultrasonography, computed tomography and 99mTc liver scan in diagnosis of Budd-Chiari syndrome. Gut 1987; 28:242.
- Soyer P, Rabenandrasana A, Barge J, et al. MRI of Budd-Chiari syndrome. Abdom Imaging 1994; 19:325.
- Friedman AC, Ramchandani P, Black M, et al. Magnetic resonance imaging diagnosis of Budd-Chiari syndrome. Gastroenterology 1986; 91:1289.
- Lin J, Zhou KR, Chen ZW, et al. Vena cava 3D contrast-enhanced MR venography: a pictorial review. Cardiovasc Intervent Radiol 2005; 28:795.
- Cazals-Hatem D, Vilgrain V, Genin P, et al. Arterial and portal circulation and parenchymal changes in Budd-Chiari syndrome: a study in 17 explanted livers. Hepatology 2003; 37:510.
- Tang TJ, Batts KP, de Groen PC, et al. The prognostic value of histology in the assessment of patients with Budd-Chiari syndrome. J Hepatol 2001; 35:338.
- Dilawari JB, Bambery P, Chawla Y, et al. Hepatic outflow obstruction (Budd-Chiari syndrome). Experience with 177 patients and a review of the literature. Medicine (Baltimore) 1994; 73:21.
- Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 51-1987. Progressive abdominal distention in a 51-year-old woman with polycythemia vera. N Engl J Med 1987; 317:1587.
- Powell-Jackson PR, Melia W, Canalese J, et al. Budd-Chiari Syndrome: clinical patterns and therapy. Q J Med 1982; 51:79.
- Gupta S, Blumgart LH, Hodgson HJ. Budd-Chiari syndrome: long-term survival and factors affecting mortality. Q J Med 1986; 60:781.
- Murphy FB, Steinberg HV, Shires GT 3rd, et al. The Budd-Chiari syndrome: a review. AJR Am J Roentgenol 1986; 147:9.
- Maddrey WC. Hepatic vein thrombosis (Budd-Chiari syndrome). Hepatology 1984; 4:44S.
- 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