Pathogenesis and treatment of Budd-Chiari syndrome combined with portal vein thrombosis.
OBJECTIVE
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.
Darwish Murad
Sarwa
S
Department of Gastroenterology and Hepatology, Erasmus University Medical Center Rotterdam, Rotterdam, The Netherlands.
Valla
Dominique-Charles
DC
de Groen
Piet C
PC
Zeitoun
Guy
G
Haagsma
Elizabeth B
EB
Kuipers
Ernst J
EJ
Janssen
Harry L A
HL
D000293:Adolescent; D000328:Adult; D000368:Aged; D000925:Anticoagulants / Q000627:therapeutic use*; D006502:Budd-Chiari Syndrome / Q000150:complications / Q000401:mortality / Q000473:pathology* / Q000628:therapy*; D015331:Cohort Studies; D003131:Combined Modality Therapy; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D011169:Portal Vein; D011170:Portasystemic Shunt, Surgical / Q000379:methods*; D011336:Probability; D016016:Proportional Hazards Models; D012189:Retrospective Studies; D018570:Risk Assessment; D012720:Severity of Illness Index; D018709:Statistics, Nonparametric; D016019:Survival Analysis; D016896:Treatment Outcome; D020246:Venous Thrombosis / Q000150:complications / Q000401:mortality / Q000473:pathology* / Q000628:therapy*
D000925:Anticoagulants