Title:Focusing the Controversies in Budd-Chiari Syndrome Management
Volume: 1
Author(s): Andrea Mancuso*
Affiliation:
- Centro di Riferimento Regionale Malattie Rare, Sindrome di Budd-Chiari-e-Teleangectasia Emorragica Ereditaria, Medicina Interna 1, ARNAS Civico - Di Cristina - Benfratelli, Palermo, Italy
Keywords:
Budd-Chiari syndrome, TIPS, liver transplant, outcome, hepatocellular carcinoma, diagnosis.
Abstract:
Budd-Chiari Syndrome (BCS) is characterized by significant clinical and pathophysiological
aspects that seem to allow a sharp differentiation between a variant in the West from one in
the East.
The aim of this paper is to focus on the main issues and controversies about the management of BCS
in the West. The study discusses different treatment options and how research is trying to solve controversies
about debated topics, such as the timing of treatment. In fact, guidelines regarding management
of BCS suggest a step-wise strategy starting with medical therapy, arriving at revascularization
or TIPS as the second step, and culminating to liver transplant as rescue therapy.
However, long-term outcome is frequently dismal on sole medical therapy. In fact, it is a matter of
debate whether further intervention should be suggested only when hemodynamic consequences of
portal hypertension are evident. However, as recently hypothesized, chronic micro-vascular ischemia
due to impaired venous hepatic outflow could trigger liver fibrosis, resulting in portal hypertension
and progressive liver failure. Consequently, liver congestion relief through treatment might be useful
as a preventive tool. Recently, early TIPS proved to improve BCS outcome. A direct comparison of
early intervention versus step-wise strategy would seem advisable. Furthermore, further studies
should address whether non-invasive tools could predict which patients benefit from early intervention.
