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Budd-Chiari syndrome: consensus guidance of the Asian Pacific Association for the study of the liver (APASL)
Akash Shukla1, Ananta Shreshtha2, Amar Mukund3
1Department of Gastroenterology, King Edward Memorial Hospital and Seth Gordhandas Sunderdas Medical College, Mumbai, India. drakashshukla@yahoo.com.
Insights
Budd Chiari syndrome (BCS) presents diversely in the Asia-Pacific. Guidelines address regional variations in obstruction sites, thrombophilic disorders, and management strategies for this complex liver condition.
Area of Science:
- Hepatology
- Vascular Medicine
- Gastroenterology
Background:
- Budd Chiari syndrome (BCS) exhibits significant heterogeneity in Asia-Pacific regarding obstruction sites, thrombophilic disorders, and clinical manifestations.
- Common presentations include hepatic vein ostial stenosis, short segment thrombosis, membranous obstruction of the vena cava, and complete hepatic vein thrombosis.
Purpose of the Study:
- To provide clinical guidelines for managing Budd Chiari syndrome (BCS) in the Asia-Pacific region.
- To address the diagnostic and therapeutic challenges posed by the heterogeneity of BCS in this region.
Main Methods:
- Development of clinical guidelines by the Asia Pacific Association for Study of Liver.
- Consideration of regional variations in BCS presentation and underlying thrombophilic disorders.
- Focus on unique therapeutic opportunities for restoring hepatic outflow.
Main Results:
- Guidelines address the diverse etiologies and presentations of BCS across the Asia-Pacific.
- Management strategies consider specific regional patterns of venous obstruction.
- Emphasis on interventional procedures like hepatic vein recanalization and vena cava stenting.
Conclusions:
- Standardized guidelines are crucial for managing the diverse spectrum of Budd Chiari syndrome in the Asia-Pacific.
- Addressing regional heterogeneity in BCS is key to optimizing patient outcomes.
- The guidelines aim to standardize evaluation and management approaches for clinicians.
Abstract:
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.
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