Elsevier

Journal of Hepatology

Volume 39, Issue 4, October 2003, Pages 496-501
Journal of Hepatology

Clinicopathological forms and prognostic index in Budd-Chiari syndrome

https://doi.org/10.1016/S0168-8278(03)00323-4Get rights and content

Abstract

Background: A recent study in patients with Budd-Chiari syndrome showed the value of a prognostic index including age, Pugh score, ascites and serum creatinine. Surgical portosystemic shunt did not appear to improve survival.

Aims: To validate these findings in an independent sample; to evaluate a classification into three forms according to the presence of features of acute injury, chronic lesions, or both of them (types I, II or III, respectively); and to assess whether taking into account this classification would alter our previous conclusions.

Methods: Multivariate Cox model survival analysis, first on 69 new patients; second, on these 69 and 54 previous patients, all diagnosed since 1985.

Results: Previous prognostic index had a significant prognostic value (P<0.0001) which was further improved by taking into account type III form (P<0.001). Type III form was associated with the poorest outcome. No significant impact of surgical shunting on survival was disclosed.

Conclusions: The prognosis of Budd-Chiari syndrome can be based on age, Pugh score, ascites, serum creatinine and the presence of features indicating acute injury superimposed on chronic lesions (type III form). The idea that surgical shunting has no significant impact on survival is reinforced by these findings.

Introduction

Studying a cohort of adult patients with non-tumorous Budd-Chiari syndrome (BCS) and a patent portal vein, we recently brought out [1] that: (1) the outcome of the syndrome has improved in recent years (i.e. since 1985); (2) age, Pugh score, ascites score and serum creatinine at the time of diagnosis have independent prognostic values and can be combined into a clinically useful prognostic index; and (3) after adjustment on these prognostic factors, surgical portosystemic shunting (SPSS) had no detectable impact on survival. In this study, liver biopsy findings appeared of no prognostic value raising the issue of the absence of significance of pathological data [1]. Indeed, there are limitations to the use of pathological data for determining the presence of acute injury (grade) [2] and/or chronic lesions (stage) [3] mainly due to the heterogeneity in the distribution of lesions resulting in a high rate of sampling variability [4]. This prompted us to design a novel clinicopathological classification of BCS [5] taking into account all clinical, radiological, biochemical and histological information available at the time of diagnosis for grading and staging the disease. In this classification the three following clinicopathological forms are separated: type I form (acute injury only, corresponding to the onset of hepatic outflow obstruction); type II form (chronic lesions only, corresponding to the sequel of remote hepatic outflow obstruction); and type III form (acute injury superimposed on chronic lesions). The prognostic value of this novel classification has been demonstrated in children [5], but not yet in adults.

The aims of this work were: (1) in an independent sample of adult patients with BCS, to validate the prognostic value of the previously reported prognostic index; (2) to assess the prognostic significance of the novel clinicopathological classification in adult patients; and (3) to evaluate if adjustment for this classification would uncover a significant effect of SPSS on survival.

Section snippets

Patients and methods

Six specialized medical and/or surgical units participated in this retrospective survey. Consecutive adult patients with BCS seen between January 1985 and December 1997 were enrolled. As our first survey ended in December 1992, patients seen between January 1985 and December 1992 (i.e. 54 patients) by the three departments which participated to the previous survey were not included in this series. Criteria of inclusion were similar to those defined in our previous study. Briefly, patients were

Characteristics of patients

Out of the 87 unselected adult patients with BCS admitted to the six units participating to this study, 73 patients fitted the criteria of inclusion. Fourteen patients were not included for the following reasons: three had cancer; three had portal vein thrombosis; one had both; three were treated with thrombectomy; and four others were treated with angioplasty plus hepatic vein stenting. No liver transplantation was performed as a first treatment during the survey period. Out of these 73

Discussion

This study in patients with BCS confirms the conclusions from our previous multivariate analysis regarding overall survival, the validity of the previously reported prognostic index, and the absence of significant impact of SPSS on overall survival [1]. In addition, the present study shows that, similar to children, in adult BCS patients, features of simultaneous acute injury and chronic lesions (type III clinicopathological forms) are independently associated with a poor outcome as compared to

Acknowledgements

We are thankful to The members of the Observatoire National du Syndrome de Budd-Chiari for their continuous support. We gratefully acknowledge the unrestricted financial support provided by the Société Nationale Française de Gastro-Entérologie and Laboratoires Choay.

References (36)

  • G. Zeitoun et al.

    Outcome of Budd-Chiari syndrome. A multivariate analysis of factors related to survival including surgical portosystemic shunting

    Hepatology

    (1999)
  • R. Parker

    Occlusion of the hepatic veins in man

    Medicine

    (1959)
  • D. Valla et al.

    Obstruction of the hepatic veins or inferior vena cava

    Dig Dis

    (1996)
  • D. Coste-Zeitoun et al.

    Classification du syndrome de Budd-Chiari en trois formes: un nouvel outil pronostique

    Gastroenterol Clin Biol

    (1998)
  • Y. Menu et al.

    BCS. US evaluation

    Radiology

    (1985)
  • V. Deutsch et al.

    BCS: study of angiographic findings and remarks on etiology

    Am J Roentgenol

    (1972)
  • G. Zeitoun et al.

    Does surgery influence the prognosis of Budd-Chiari syndrome? A retrospective multivariate survival analysis

    Hepatology

    (1995)
  • C. Denié et al.

    Acute Budd-Chiari syndrome: spontaneously reversible exacerbation of hepatocellular necrosis but bad long-term prognosis

    Hepatology

    (1995)
  • Cited by (149)

    • Reply

      2023, Clinical Gastroenterology and Hepatology
    • Outcome of Budd-Chiari Syndrome Patients Treated With Direct Oral Anticoagulants: An Austrian Multicenter Study

      2023, Clinical Gastroenterology and Hepatology
      Citation Excerpt :

      The fact that we only observed procedure-related bleedings in 4.5% following DOAC treatment compared with 10.3% after LMWH or VKA additionally points toward a safe use of DOAC in the peri-interventional and perioperative setting if paused according to the label. The overall outcome of BCS patients in our study is favorable with a TFS of 92% at 5 years and 83% at 10 years compared with earlier studies that reported 5-year survival rates of 65%–82%.21–23 Indeed, our results including a substantial number of patients treated with DOAC are in line with studies focusing on the outcome of BCS patients after interventional treatment or TIPS implantation with reported 5-year survival rates of up to 78%–87% (Supplementary Table 1).24–26

    • Vascular Disorders

      2023, MacSween's Pathology of the Liver, Eighth Edition
    View all citing articles on Scopus
    View full text