Acta Scientific Medical Sciences (ISSN: 2582-0931)

Research Article Volume 4 Issue 3

Etiological Profile of 11 Cases of Bud-Chiari Syndrome

Salim Larbani*, Nabila Lyazidi, Mohamed Hamza Bouchouareb, Amir Hadji, Yasmine Hamma, Chafia Ouarab, Sadek Boumedine and Soraya Ayoub

Internal Medicine Department, University Hospital Centre Issad Hassani, Beni Messous Algiers, Algeria

*Corresponding Author: Salim Larbani, Internal Medicine Department, University Hospital Centre Issad Hassani, Beni Messous Algiers, Algeria.

Received: March 18, 2020; Published: April 07, 2020



Background and Aim: Budd-Chiari syndrome is a relatively rare condition with multiple etiologies and high risk of complications and death. The aim of our study were to investigate the etiology of BCS, especially auto-inflammatory diseases and rare associations, analyze different clinical presentations, treatment modalities and evolutionary profile.

Methods: Retrospective cohort study of patients hospitalized from 2000 to 2018 with confirmed diagnosis of BCS on Doppler ultrasound, with which we have conducted a large etiological survey.

Results: 11 patients were included; mean age 29 years (16 to 45 years); sex ratio (M/F) 0,83.
All patients were symptomatic at diagnosis with classic subacute to chronic clinic presentation, 6 patients had extravascular manifestations.
The etiological survey conclusion was: Behçet’s syndrome in 6 cases (most severe forms), liver chronic disease in 2 cases (hepatitis C and cryptogenic cirrhosis), protein S deficiency associated with coeliac disease in 1 case, paroxysmal nocturnal hemoglobinuria associated with antiphospholipid syndrome in 1 case and factor five Leiden mutation in 1 case. The treatment included anticoagulants in 7 cases, specific treatment combining corticosteroids and immunosuppressive drugs in patients with BS, Gluten-free diet in celiac disease.

Conclusion: Behçet’s syndrome was the first etiology in our study, it must be systematically searched in countries with high prevalence, especially in young patients with extravascular manifestations. The association of 2 or plus prothrombotic states is not rare and often insidious. The systematic search of this kind of association must be discussed.

Keywords: Budd-Chiari Syndrome; Behçet’s Syndrome; Portal Hypertension; Vasculitis; Thrombosis



  1. Valla DC. “Primary Budd-Chiari syndrome”. Journal of Hepatology 50 (2009): 195-203.
  2. Hadengue A., et al. “The changing scene of hepatic vein thrombosis: recognition of asymptomatic cases”. Gastroenterology 106 (1994): 1042-1047.
  3. Parker RGF. “Occlusion of the hepatic veins in man”. Medicine (Baltimore) 38 (1959): 369-402.
  4. Aydinli M and Bayraktar Y. “Budd-Chiari syndrome: etiology, pathogenesis and diagnosis”. World Journal of Gastroenterology 13 (2007): 2693-2696.
  5. Taylor RW., et al. “Leiomyosarcoma of the inferior vena cava presenting as Budd- Chiari syndrome”. Liver 7 (1987): 201-205.
  6. Schraut WH and Chilcote RR. “Metastatic Wilms’ tumor causing acute hepatic-vein occlusion (Budd-Chiari syndrome) ”. Gastroenterology 88 (1985): 576-579.
  7. Janssen HL., et al. “Factor V Leiden mutation, prothrombin gene mutation, and deficiencies in coagulation inhibitors associated with Budd-Chiari syndrome and portal vein thrombosis: results of a casecontrol study”. Blood 96 (2000): 2364-2368.
  8. Denninger MH., et al. “Budd-Chiari syndrome and factor V Leiden mutation”. Lancet 345 (1995): 525-526.
  9. Denninger MH., et al. “Cause of portal or hepatic venous thrombosis in adults: the role of multiple concurrent factors”. Hepatology 31 (2000): 587-591.
  10. Myones BL and McCurdy D. “The antiphospholipid syndrome: immunologic and clinical aspects. Clinical spectrum and treatment”. The Journal of Rheumatology 58 (2000): 20-28.
  11. Bismuth E., et al. “Hepatic vein thrombosis in Behcet’s disease”. Hepatology 11 (1990): 969-974.
  12. Bayraktar Y., et al. “Budd-Chiari syndrome: a common complication of Behcet’s disease”. The American Journal of Gastroenterology 92 (1997): 858-862.
  13. Yazici H., et al. “Behcet syndrome: is it one condition?” Clinical Reviews in Allergy and Immunology 43 (2012): 275-280.
  14. Kuniyoshi Y., et al. “Surgical treatment of Budd-Chiari syndrome induced by Behcet’s disease”. Annals of Thoracic and Cardiovascular Surgery 8 (2002): 374-380.
  15. Desbois AC., et al. “Immunosuppressants reduce venous thrombosis relapse in Behcet’s disease”. Arthritis and Rheumatology 64 (2012): 2753-2760.
  16. Yurdakul S., et al. “The prevalence of Behcet’s syndrome in a rural area in northern Turkey”. The Journal of Rheumatology 15 (1988): 820-822.
  17. Cakir N., et al. “Prevalence of Behcet’s disease in rural western Turkey: a preliminary report”. Clinical and Experimental Rheumatology 22 (2004): 53-55.
  18. Aydinli M and Bayraktar Y. “Budd-Chiari syndrome: etiology, pathogenesis, and diagnosis”. World Journal of Gastroenterology 13 (2007): 2683-2696.
  19. Sakr MA., et al. “Characteristics and outcome of primary Budd-Chiari syndrome due to Behçet’s syndrome”. Clinics and Research in Hepatology and Gastroenterology (2019).
  20. Calamia KT., et al. “Major vessel involvement in Behcet disease”. Current Opinion in Rheumatology 17 (2005): 1-8.
  21. Bayraktar Y., et al. “Budd-Chiari syndrome: a common complication of Behcet’s disease”. The American Journal of Gastroenterology 92 (1997): 858-862.
  22. Korkmaz C., et al. “Budd-Chiari syn-drome in the course of Behcet’s disease: clinical and laboratory analysis of four cases”. Joint Bone Spine 74 (2007): 245-248.
  23. Hatemi G., et al. “Update of the EULAR recommendations for the management of Behcet’s syndrome”. Annals of the Rheumatic Diseases 77 (2018): 808-818.
  24. Seyahi E. “Behcet’s disease: how to diagnose and treat vascular involvement”. Best Practice and Research: Clinical Rheumatology 30 (2016): 279-295.
  25. Tayer-Shifman OE., et al. “Major vessel thrombosis in Behcet’s disease: the dilemma of anticoagulant therapy e the approach of rheumatologists from different countries”. Clinical and Experimental Rheumatology 30 (2012): 735-740.
  26. Denninger MH., et al. “Cause of portal or hepatic venous thrombosis in adults: the role of multiple concurrent factors”. Hepatology 31 (2000): 587-591.
  27. Chait Y., et al. “Relevance of the criteria commonly used to diagnose myeloproliferative disorder in patients with splanchnic vein thrombosis”. British Journal of Haematology 129 (2005): 553-560.
  28. Deltenre P., et al. “Factor V Leiden related Budd-Chiari syndrome”. Gut 48 (2001): 264-268.
  29. Seyahi E., et al. “An outcome survey of 43 patients with Budd-Chiari syndrome due to Behcet’s syndrome followed up at a single, dedicated center”. Seminars in Arthritis and Rheumatism 44 (2015): 602-609.
  30. Zeitoun G., et al. “Outcome of Budd-Chiari syndrome: a multivariate analysis of factors related to survival including surgical portosystemic shunting”. Hepatology 30 (1990): 84-89.
  31. Sakr MA., et al. “The pattern of vascular involvement in Egyptian patients with Budd-Chiari syndrome: relation to etiology and impact on clinical presentation”. Annals of Hepatology 17 (2018): 638-644.
  32. Darwish MS., et al. “Etiology, management, and outcome of the Budd-Chiari syndrome”. Annals of Internal Medicine 151 (2009): 167-175.
  33. Darie C., et al. “Pseudotumeur cardiaque révélant une maladie de Behçet”. Revue de Médecine Interne 26 (2005): 420-424.


Citation: Salim Larbani., et al. “Etiological Profile of 11 Cases of Bud-Chiari Syndrome". Acta Scientific Medical Sciences 4.5 (2020): 03-08.


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