Abstract
Budd-Chiari syndrome (BCS) is a very rare vascular complication of Behçet’s disease (BD) which often leads to death as a result of portal hypertension and liver failure. We report a 45-year-old BD patient who presented with BCS. Diagnosis was confirmed with CT scan and contrast-enhanced MR angiography which showed ascites, short-segment stenosis of the inferior vena cava (IVC), and middle and left hepatic venous thrombosis. Percutaneous transluminal angioplasty (PTA) of the obstructed segment in the IVC was performed and resulted in dramatic reduction of portal venous pressure. Our experience indicates that PTA may be a safe and effective therapeutic modality for BCS in BD which is caused by short segmental obstruction of the IVC.
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Kiraz S, Ertenli I, Ozturk MA, Haznedaroglu IC, Celik I, Calguneri M (2002) Pathological haemostasis and “prothrombotic state” in Behçet’s disease. Thromb Res 105:125–133
Bayraktar Y, Balkanci F, Bayraktar M, Calguneri M (1997) Budd-Chiari syndrome: a common complication of Behçet’s disease. Am J Gastroenterol 92:858–862
Shimizu T, Ehrlich GE, Inaba G, Hayashi K (1979) Behçet disease (Behçet syndrome). Semin Arthritis Rheum 8:223–260
Ringe B, Lang H, Oldhafer KJ, Gebel M, Flemming P, Georgii A, Borst HG, Pichlmayr R (1995) Which is the best surgery for Budd-Chiari syndrome: venous decompression or liver transplantation? A single-center experience with 50 patients. Hepatology 21:1337–1344
Slakey DP, Klein AS, Venbrux AC, Cameron JL (2001) Budd-Chiari syndrome: current management options. Ann Surg 233:522–527
Pelage JP, Denys A, Valla D, Sibert A, Sauvanet A, Belghiti J, Menu Y (2003) Budd-Chiari syndrome due to prothrombotic disorder: mid-term patency and efficacy of endovascular stents. Eur Radiol 13:286–293
Cakaloglu Y, Okten A, Yalcin S (1991) Serum-ascites albumin concentration gradient (A-GRAD) in the prediction of portal hypertension in ascitic patients. Gastroenterology 100:1484–1485
Fisher NC, McCafferty I, Dolapci M, Wali M, Buckels JA, Olliff SP, Elias E (1999) Managing Budd-Chiari syndrome: a retrospective review of percutaneous hepatic vein angioplasty and surgical shunting. Gut 44:568–574
Hendrick RE (1987) Sampling time effects on signal-to-noise and contrast-to-noise ratios in spin-echo MRI. Magn Reson Imaging 5:31–37
Wu T, Wang L, Xiao Q, Wang B, Li S, Li X, Zhang J (2002) Percutaneous balloon angioplasty of inferior vena cava in Budd-Chiari syndrome-R1. Int J Cardiol 83:175–178
Townsend (2001) Anatomy and development of liver. In: Sabiston textbook of surgery. Saunders, Phladelphia, pp 1002–1003
O’Duffy JD (1990) Vasculitis in Behçet’s disease. Rheum Dis Clin North Am 16:423–431
Ozoran K, Dugun N, Gurler A, Tutkak H, Tokgoz G (1995) Plasma von Willebrand factor, tissue plasminogen activator, plasminogen activator inhibitor, and antithrombin III levels in Behçet’s disease. Scand J Rheumatol 24:376–382
Haznedaroglu IC, Ozdemir O, Ozcebe O, Dundar SV, Kirazli S (1996) Circulating thrombomodulin as a clue of endothelial damage in Behçet’s disease. Thromb Haemost 75:974–975
Aydintug AO, Tokgoz G, D’Cruz DP, Gurler A, Cervera R, Duzgun N, Atmaca LS, Khamashta MA, Hughes GR (1993) Antibodies to endothelial cells in patients with Behçet’s disease. Clin Immunol Immunopathol 67:157–162
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Han, S.W., Kim, G.W., Lee, J. et al. Successful treatment with stent angioplasty for Budd-Chiari syndrome in Behçet’s disease. Rheumatol Int 25, 234–237 (2005). https://doi.org/10.1007/s00296-004-0495-4
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DOI: https://doi.org/10.1007/s00296-004-0495-4