Budd-Chiari syndrome: consensus guidance of the Asian Pacific Association for the study of the liver (APASL)

Budd Chiari syndrome (BCS) is a diverse disease with regard to the site of obstruction, the predisposing thrombophilic disorders and clinical presentation across the Asia-Pacific region. The hepatic vein ostial stenosis and short segment thrombosis are common in some parts of Asia-Pacific region, while membranous obstruction of the vena cava is common in some and complete thrombosis of hepatic veins in others. Prevalence of myeloproliferative neoplasms and other thrombophilic disorders in BCS varies from region to region and with different sites of obstruction. This heterogeneity also raises several issues and dilemmas in evaluation and approach to management of a patient with BCS. The opportunity to recanalize hepatic vein in patients with hepatic vein ostial stenosis or inferior vena cava stenting or pasty among those membranous obstruction of the vena cava is a unique opportunity in the Asia-Pacific region to restore hepatic outflow closely mimicking physiology. In order to address these issues arising out of the diversity as well as the unique features in the region, the Asia Pacific Association for Study of Liver has formulated these guidelines for clinicians.

Automated summary

Budd Chiari syndrome presents a diverse range of disease manifestations, thrombophilic disorders, and clinical presentations across the Asia-Pacific region. This heterogeneity poses challenges in evaluation and management of patients with BCS, with differing prevalence of myeloproliferative neoplasms and thrombophilic disorders in different regions. The unique opportunities for recanalization or stenting in this region highlight the importance of tailored approaches for each individual case.