Budd-Chiari Syndrome: Illustrated Review of Current Management

John D. Horton; Francisco L. San Miguel; Jorge A. Ortiz


Liver International. 2008;28(4):455-466. 

In This Article


Outcomes in patients with BCS have improved since 1985.[19] Approximately 25% of patients remain asymptomatic after treatment, and spontaneous resolution has been reported.[16] Several authors have investigated prognostic indicators in patients with BCS.

Khuroo[29] analysed 47 consecutive patients with BCS and found the following factors to adversely affect survival: florid clinical presentation, male sex, no TIPS performed and CTP score.

Langlet updated previous work on their prognostic index (PI) and found that ascites score, Pugh score, age, creatinine and type III presentation (acute injury superimposed on chronic lesions) were all independent predictors of survival. After including 123 patients who underwent surgical shunts, this analysis was unable to show a survival benefit to surgical shunts whether performed early (within 2 months after diagnosis) or not. Short-term and long-term survival was good regardless of treatment modality in patients with PI<5.1. However, the authors caution that this study was underpowered to detect a possible benefit to surgical shunts in patients who present with type I BCS (acute injury only, corresponding to the onset of hepatic outflow obstruction).[19] A weakness of the PI is that only two of its variables incorporate objective data.

Murad reported the results of an international multi-institutional study, which included 237 patients (205 included in multivariate analysis) treated with a variety of modalities. This cohort achieved transplant-free survival rates of 82%, 69% and 62% at 1, 5 and 10 years respectively. The following factors were found by multivariate analysis to be independent predictors of 5-year transplant-free survival: presence of ascites, presence of encephalopathy, INR and bilirubin. Based on these variables, the authors constructed an equation that assigned patients into one of three classes ( Table 2 ). The 5-year transplant-free survival was 89%, 74% and 42% for class I, class II and class III respectively.[44] The strengths of this analysis were that it included more patients than the Langlet study, and its variables were either objective or binary. The degree of ascites and encephalopathy were not variables. Only the presence or absence of ascites and encephalopathy was considered in the multivariate analysis. A possible weakness of this study is that it excluded patients who underwent transplant.


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