An update on management of Budd-Chiari syndrome

Area of Science:

• Hepatology
• Vascular Surgery
• Transplant Surgery

Background:

• Budd-Chiari syndrome (BCS) management guidelines are based on expert opinion due to its rarity.
• Current BCS treatment follows a stepwise approach: medical therapy, revascularization/TIPS, and liver transplantation (OLT) as rescue.
• Surgical intervention is reserved for specific cases, particularly high inferior vena cava obstruction unsuitable for endovascular methods.

Purpose of the Study:

• To propose an updated, evidence-informed algorithm for managing Budd-Chiari syndrome.
• To suggest an earlier therapeutic intervention in BCS management when clinical signs appear.
• To address concerns regarding treatment timing and recurrence risk after liver transplantation in BCS patients.

Main Methods:

• Review and synthesis of current expert opinions and limited evidence on BCS management.
• Development of a novel, step-wise algorithm for BCS treatment.
• Analysis of treatment efficacy and risks, including recurrence post-OLT.

Main Results:

• The proposed algorithm advocates for earlier therapeutic intervention in BCS management.
• It highlights the limited but crucial role of surgery in specific obstruction scenarios.
• Identifies areas of concern, such as optimal timing of interventions and post-transplant recurrence.

Conclusions:

• Current BCS management strategies require refinement due to rarity and lack of robust evidence.
• An earlier, algorithm-driven therapeutic approach may enhance BCS patient outcomes.
• Further research is needed to clarify optimal treatment timing and mitigate recurrence risks, particularly after OLT.