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Controversies in the Management of Budd–Chiari Syndrome

  • Andrea MancusoEmail author
Chapter

Abstract

The flowchart of Budd–Chiari Syndrome (BCS) management is not evidence-based and relies to experts’ opinion. The aim of this chapter is to enlighten the controversies about BCS management.

Guidelines about BCS management suggest following a step-wise strategy where sole medical therapy is the initial treatment, revascularization or TIPS the second step, and liver transplant the rescue therapy. However, sole medical therapy generally results in bad long-term outcome. The main debated issue of guidelines is that further intervention is suggested only when hemodynamic consequences of portal hypertension become evident. However, as a theory recently stated, liver fibrosis could be the final result of chronic micro-vascular ischemia. Consequently, in the context of BCS, impaired venous hepatic outflow could result in portal hypertension development so triggering hepatic fibrosis and liver failure through chronic liver ischemic injury. Moreover, treatment induced liver congestion relief might preserve liver function avoiding BCS complications development. Recently, early TIPS was suggested to possibly improve the outcome of BCS.

Future studies should be designed with the aim of evaluating whether the outcome of BCS could be improved with early intervention versus step-wise strategy. Furthermore, researchers should explore, using non-invasive tools, which subgroup of patients on only medical therapy would mostly benefit from early intervention.

Keywords

Budd–Chiari syndrome TIPS Liver transplant Outcome 

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Copyright information

© Springer Nature Singapore Pte Ltd. 2020

Authors and Affiliations

  1. 1.Medicina Interna 1ARNAS Civico - Di Cristina - BenfratelliPalermoItaly

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