Article

Liver transplantation for Budd-Chiari syndrome: A European study on 248 patients from 51 centres.

Transplantation Unit, Department of Surgery, University Hospitals, 1211 Geneva 14, Switzerland.
Journal of Hepatology (Impact Factor: 10.4). 04/2006; 44(3):520-8. DOI: 10.1016/j.jhep.2005.12.002
Source: PubMed

ABSTRACT The results of liver transplantation for Budd-Chiari syndrome (BCS) are poorly known and the role and timing of the procedure are still controversial. The aim of this study was to investigate the results of transplantation for BCS, focusing on overall outcome, on prognostic factors and on the impact of the underlying disease.
An enquiry on 248 patients representing 84% of the patients transplanted for BCS in the European Liver Transplantation Registry between 1988 and 1999.
Of the 248 patients, 70.4% were female and 29.6% male. The mean age was 35.7 years. The overall actuarial survival was 76% at 1 year, 71% at 5 years and 68% at 10 years. 77% of deaths occurred in the first 3 months: 47% were due to infection and multiple organ failure, and 18% to graft failure or hepatic artery thrombosis. Late mortality (>1 year) occurred in nine patients, due to BCS recurrence in four of them. The only pre-transplant predictors of mortality on multivariate analysis (Cox) were impaired renal function and a history of a shunt.
Liver transplantation for BCS is an effective treatment, irrespective of the underlying cause, and should be considered before renal failure occurs.

Download full-text

Full-text

Available from: Andrew K Burroughs, Jun 17, 2015
0 Followers
 · 
116 Views
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: We analyzed sonomorphological signs of Budd-Chiari syndrome (BCS) and their potential for prognosis prediction. Forty-five consecutive patients were included. Analysis included the frequencies of sonomorphological signs and their predictive value for diagnosis of BCS, mean values of laboratory and color Doppler data in different therapeutic groups and survival. Specific ultrasound signs were identified at the level of the hepatic veins in 71% of the patients and in 33% at the level of the caval vein, i.e. thrombosis, stenosis, fibrotic cord or insufficient recanalization of the vessels. The frequent non-specific signs were splenomegaly (78%), inhomogeneous liver parenchyma (76%), intrahepatic collaterals (73%), caudate lobe hypertrophy (67%), ascites (56%) and extrahepatic collaterals (44%). The combination of specific signs and "caudate lobe hypertrophy" offered the highest predictive value to identify patients with BCS (p=0.014) with a specificity of 100%. Mean survival was significantly different between the patients with or without portal hypertension (n=25, 41.1+/-7.6, 95% CI (26.2-55.9) versus n=20, 89.4+/-4.5, 95% CI (80.5-98.2), p=0.004) and with or without portal vein thrombosis (n=12, 29.8+/-10.7, 95% CI (8.9-50.7) versus n=33, 79.3+/-6.1, 95% CI (67.4-91.1), p=0.003). We present a comprehensive description of sonomorphological signs in BCS. The combination of ultrasound signs "altered hepatic and/or caval veins" and "caudate lobe hypertrophy" was the best strategy to diagnose BCS. Patients with portal vein thrombosis or portal hypertension have a poor prognosis.
    Journal of Hepatology 07/2008; 49(4):572-80. DOI:10.1016/j.jhep.2008.04.025 · 10.40 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Budd-Chiari syndrome (BCS) is characterized by hepatic venous outflow obstruction at any level from the small hepatic veins to the atriocaval junction. BCS is a complex disease with a wide spectrum of aetiologies and presentations. This article reviews the current literature with respect to presentation, management and prognosis of the disease. Medical, interventional and surgical management of BCS is discussed. Particular attention is paid to interventional and surgical aspects of management. The review is augmented by images, which provide a clinical corollary to the text.
    Liver international: official journal of the International Association for the Study of the Liver 05/2008; 28(4):455-66. DOI:10.1111/j.1478-3231.2008.01684.x · 4.41 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Because of the rarity of Budd-Chiari syndrome (BCS), the flow chart of management comes from expert opinion and is not evidence based. To report an update on the management of BCS. I performed a review on published papers on BCS in an attempt to speculate in particular on the timing and the choice of treatment. Some authors suggest that the management of BCS should follow a step-wise strategy. Anticoagulation and medical therapy should be the first-line treatment. Revascularization or transjugular intrahepatic portosystemic shunt should be performed in case of no response to medical therapy. Orthotopic liver transplant should be used as a rescue therapy. The biggest criticism of this flow chart is that it is based on the assumption that patients with BCS should receive further treatment only when hemodynamic effects on portal hypertension become clinically evident, thus paying little attention to the chronic ischemic liver damage effects on hepatic function and to the possibility of preventing liver failure by relieving impaired hepatic veins outflow. Recently, I presented a proposal of a new algorithm for the management of BCS, in which medical therapy alone is suggested only for patients without any sign of portal hypertension, irrespective of whether early interventional treatment is suggested when either any symptoms or signs of portal hypertension appear, with the aim of preventing hepatic fibrosis development, disease progression, and finally improving outcome. Given that the benefit of treatments for BCS is not under debate, guidelines for the management of BCS should be re-evaluated and updated, with particular attention to both the timing and the choice of treatment.