Budd-Chiari syndrome: current perspectives and controversies

Area of Science:

• Hepatology
• Vascular Medicine
• Gastroenterology

Background:

• Budd-Chiari syndrome (BCS) is a rare condition characterized by hepatic venous outflow obstruction.
• Its diverse causes and varied clinical presentations necessitate considering BCS in patients with acute or chronic liver disease.

Purpose of the Study:

• To review recent advances in the classification, etiology, clinical presentation, diagnosis, and treatment of BCS.
• To highlight the evolving treatment strategies and the potential benefits of earlier intervention.

Main Methods:

• Literature review of recent studies and expert opinions on BCS.
• Analysis of current therapeutic modalities, including anticoagulation, thrombolysis, TIPS, surgery, and liver transplantation.

Main Results:

• BCS management has seen significant therapeutic improvements.
• A stepwise treatment strategy is established, but relies on expert opinion and retrospective data rather than prospective trials.
• Earlier use of transjugular intrahepatic portosystemic shunt (TIPS) is proposed for selected BCS cases due to high mortality with medical therapy alone.

Conclusions:

• Budd-Chiari syndrome requires a high index of suspicion due to its heterogeneous presentation.
• Current treatment strategies are evolving, with a growing emphasis on interventional procedures like TIPS.
• Further prospective studies are needed to optimize BCS management and improve survival rates.