Management of Budd-Chiari syndrome

Area of Science:

• Hepatology
• Vascular Surgery
• Transplantation Medicine

Background:

• Thrombotic hepatic vein occlusion causes liver dysfunction and failure.
• Underlying conditions like Factor V Leiden mutation are associated with Budd-Chiari Syndrome (BCS).

Purpose of the Study:

• To review a single-center experience with 18 BCS patients treated between 1992 and 2003.
• To outline a tailored approach for BCS management based on disease stage and clinical deterioration.

Main Methods:

• Review of 18 consecutive patients with Budd-Chiari Syndrome (BCS).
• Treatment modalities included liver transplantation, stenting, TIPSS, and surgical shunting.
• Anticoagulant therapy was used in select cases.

Main Results:

• Twelve patients underwent liver transplantation, with higher complication rates compared to other liver diseases.
• Interventional procedures like stenting and TIPSS were employed.
• Conservative management was sustained for up to 8 years in some patients.

Conclusions:

• BCS management requires a staged approach, prioritizing conservative and interventional methods to preserve liver function.
• Liver transplantation is the definitive treatment when venous outflow cannot be restored and liver function deteriorates.
• Maintaining venous outflow through interventions is crucial to delay or avoid transplantation and its associated complications.