Focus on Controversies in Budd-Chiari Syndrome Management

: 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 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 timing of treatment. In fact, guidelines regarding management of BCS suggest a step-wise strategy starting with medical therapy, arriving to revascularization or TIPS as 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 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 be the trigger of 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.