Abstract

Budd-Chiari syndrome (BCS) is the liver disease resulting from hepatic venous outflow obstruction comprising a triad of abdominal discomfort, hepatomegaly and ascites. Advances in the management of this disorder over the last three decades have dramatically improved survival.

We present a review of the management of BCS followed by a case which illustrates some key points in the diagnosis and treatment of this condition.
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References

1. Valla D-C. Budd-Chiari syndrome and veno-occlussive disease / sinusoidal obstruction syndrome. Gut 2008; 57(10): 1469–1478.
2. Ludwig J, Hashimota E, McGill DB et al. Classification of hepatic venous outflow obstruction: ambiguous terminology of the Budd-Chiari syndrome. Mayo Clin Proc 1990; 65: 51–55.
3. Valla D. Hepatic venous outflow tract obstruction etiopathogenesis: Asia versus the West. J Gastroenterol Hepatol 2004; 19: S204–211.
4. Okuda K. Inferior vena cava thrombosis at its hepatic portion (obliterative hepatocavopathy). Semin Liver Dis 2002; 22: 15–26.
5. Sherlock S, Dooley J. Diseases of the Liver and Biliary System, 11th edition. Blackwell Science, 2005.
6. Murad SD, Plessier A, Hernandez-Guerra M et al. Etiology, management and outcome of the Budd-Chiari syndrome. Ann Intern Med 2009; 151: 167–175.
7. James C, Ugo V, Le Couedic JP et al. A unique clonal JAK2 mutation leading to constitutive signalling causes polycythaemia vera. Nature 2005; 434: 1144–1148.
8. Hussein K, Bock O, Kreipe H. Histological and molecular classification of chronic myeloproliferative disorders in the age of JAK2: persistence of old questions despite new answers. Pathobiology 2007; 74; 72–80.
9. De Stefano V, Fiorini A, Rossi E et al. Incidence of the JAK2 V617F mutation among patients with splanchnic or cerebral venous thrombosis and without overt chronic myeloproliferative disorders. J Thromb Haemost 2007; 5: 708–714.
10. Kiladjian JJ, Cervantes, F, Leebeek FWG et al. The impact of JAK2 and MPL mutations on diagnosis and prognosis of splanchnic vein thrombosis: a report on 241 cases. Blood 2008; 111: 4922–4929.
11. Regina S, Herault O, D’Alteroche L et al. JAK2 V617F is specifically associated with splanchnic vein thrombosis. J Thromb Haemost 2007; 5: 859–861.
12. Colaizzo D, Amitrano L, Lannaccone L et al. Gain-of-function gene mutations and thromboembolism: distinct roles in different clinical settings. J Med Genet 2007; 44: 412–416.
13. Patel RK, Lea NC, Heneghan MA et al. Prevelance of the activating JAK2 tyrosine kinase mutation V617F in the Budd-Chiari syndrome. Gastroenterology 2006; 130: 2031–2038.
14. Wanless IR, Peterson P, Das et al. Hepatic vascular disease and portal hypertension in polycythaemia vera and agnogenic myeloid metaplasia: a clinicopathological study of 145 patients examined at autopsy. Hepatology 1990; 12: 1166–1174.
15. Valla D, Lee MG, Poynard T et al. Risk of hepatic vein thrombosis in relation to recent use of oral contraceptives: a case-control study. Gastroenterology 1986; 90: 807.
16. Hadengue AE, Poliquin M, Vilgrain V et al. The changing scene of heptic vein thrombosis: recognition of asymptomatic cases. Gastroenterology 1994; 106: 1042–1047.
17. Miller WJ, Federle MP, Straub WH et al. Budd-Chiari syndrome: imaging with pathological correlation. Abdom Imaging 1993; 18: 329–335.
18. Brancatelli G, Vilgrain V, Federle MP et al. Budd-chiari syndrome: Spectrum of imaging findings. American Journal of Reoentgenology 2007; 188: W168–W176.
19. Kim TK, Chung JW, Han JK, et al. Hepatic changes in benign obstruction of the hepatic inferior vena cava: CT findings. AJR 1999; 173: 1235–1242.
20. Cho O-K, Koo J-H, Kim Y-S, Rhim H-C, Koh B-H, Seo H-S. Collateral pathways in Budd-Chiari syndrome: CT and venographic correlation. AJR 1996; 167: 1163–1167.
21. Soyer P, Rabenandrasana A, Barge J, et al. MRI of Budd-Chiari syndrome. Abdom Imaging 1994; 19: 325–329.
22. Mathieu D, Vasile N, Menu Y, et al. Budd-Chiari syndrome: dynamic CT. Radiology 1987; 165: 409–413.
23. Noone TC, Semelka RC, Siegelman ES, et al. Budd-Chiari syndrome: spectrum of appearances of acute, subacute, and chronic disease with magnetic resonance imaging. J Magn Reson Imaging 2000; 11: 44–50.
24. Runyon BA, Montano AA, Akriviadis et al. The serum-ascites albumin gradient is superior to the exudate-transudate concept in the differential diagnosis of ascites. Ann Intern Med 1992; 117: 215.
25. Pare P, Talbot J, Hoefs JC. Serum-ascites albumin concentration gradient: a physiological approach to the differential diagnosis of ascites. Gastroenterology 1983; 85(2): 240–244.
26. Patel RK, Lea NC, Heneghan MA et al. Prevelance of the activating JAK2 tyrosine kinase mutation V617F in the Budd-Chiari syndrome. Gastroenterology 2006; 130: 2031–2038.
27. Chait Y, Condat B, Cazals-Hatem D et al. Reelevance of the criteria commonly used todiagnose myeloproliferative disorder in patients with splanchnic vein thrombosis. Br J Haematol 2005; 129: 553–560.
28. Tavill AS, Wood EJ, Kreel L et al. The Budd-Chiari syndrome: correlation between hepatic scintigraphy and the clinical, radiological and pathological findings in nineteen cases of hepatic venous outflow obstruction. Gastroenterology 1975; 68: 509–518.
29. Mahmoud AE, Mendoza A, Meshikhes AN et al. Clinical spectrum, investigations and treatment of Budd-Chiari syndrome. QJM 1996; 89: 37–43.
30. Zeitoun G, Escolona S, Hadengue A et al. Outcome of Budd-Chiari syndrome: a multivariate analysis of factors related to survival including surgical portosystemic shunting. Hepatology 1999; 30: 80–89.
31. 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: 338-343;
32. Murad SD, Valla DC, de Groen PC et al. Determinants of survival and the effects of portosystemic shunting in patients with Budd-Chiari syndrome. Hepatology 2004; 39: 500–508.
33. Plessier A, Sibert A, Consigny Y et al. Aiming at minimal invasiveness as a therapeutic strategy for Budd-chiari syndrome. Hepatology 2006; 44: 1308–1316.
34. Eapen CE, Velissaris D, Heydtmann M et al. Favourable medium term outcome following hepatic vein recannalisation and/or transjugular intrahepatic portosytemic shunt for Budd Chiari syndrome. Gut 2006; 55: 978–884;
35. Moucari R, Rautou PE, Cazals-Hatem D et al. Hepatocellular carcinoma in Budd-Chiari syndrome: characteristics and risk factors. Gut 2008; 57: 828–835.