OBJECTIVES: Combined Budd-Chiari syndrome and Portal Vein Thrombosis (BCS-PVT) is a challenging clinical condition with as yet unknown outcome. The aim of the present study was to investigate etiology, treatment options, and prognosis of patients with BCS-PVT. METHODS: Patients diagnosed with nonmalignant BCS between 1984 and 2001 were identified in a large international study and classified into isolated BCS (n = 204), BCS-PVT without spleno-mesenteric vein thrombosis (SMVT; n = 15), and BCS-PVT with SMVT (n = 18). RESULTS: Multifactorial etiology was present in 58% of patients with combined BCS-PVT. Number of etiological factors increased significantly with the extent of thrombosis (p= 0.002). Main treatment options included anticoagulation and portosystemic shunting, of which extended TIPS showed the most beneficial results. Five-year survival was 59% (95% CI 39-80%) in BCS-PVT versus 85% (95% CI 76-88%) in isolated BCS (p= 0.11). Survival tended to be worse in BCS-PVT patients with SMVT as compared to patients without SMVT (RR = 3.47, p= 0.11). CONCLUSIONS: In BCS, extension of thrombosis into the splanchnic venous bed was significantly related to the number of etiological factors, and was associated with poor outcome. These results strongly support a liberal use of anticoagulants, which so far had been widely debated. Alternatively, derivative shunt procedures appear difficult, yet not impossible.

Additional Metadata
Persistent URL dx.doi.org/10.1111/j.1572-0241.2006.00353.x, hdl.handle.net/1765/59510
Journal American Journal of Gastroenterology
Citation
Darwish Murad, S, Valla, D.C, de Groen, P.C, Zeitoun, G, Haagsma, E.B, Kuipers, E.J, & Janssen, H.L.A. (2006). Pathogenesis and treatment of Budd-Chiari syndrome combined with portal vein thrombosis. American Journal of Gastroenterology, 101(1), 83–90. doi:10.1111/j.1572-0241.2006.00353.x