Budd-chiari syndrome causing acute liver failure: A multicenter case series
Justin Parekh1, Vlad M Matei2, Alejandro Canas-Coto3
1Department of Surgery, University of Texas Southwestern Medical Center at Dallas, Dallas, TX.
Summary
Budd-Chiari syndrome (BCS) causing acute liver failure (ALF) is rare, often affecting young women. Prompt diagnosis and treatment, including anticoagulation, TIPS, or liver transplantation, are crucial for better outcomes in these critical cases.
Area of Science:
- Hepatology
- Gastroenterology
- Internal Medicine
Background:
- Budd-Chiari syndrome (BCS) is a rare hepatic venous outflow obstruction.
- BCS can rapidly progress to acute liver failure (ALF).
Purpose of the Study:
- To describe clinical features, treatments, and outcomes of ALF due to BCS.
- To compare findings with existing literature.
Main Methods:
- Retrospective analysis of 20 ALF-BCS patients from the Acute Liver Failure Study Group (ALFSG) registry.
- Literature review and comparison of 19 additional ALF-BCS cases.
Main Results:
- ALF-BCS predominantly affected white females in their fourth decade.
- Hypercoagulable state observed in 63% of patients.
- Treatments included anticoagulation (71%), TIPS (37%), and liver transplantation (37%); in-hospital mortality was ~60%.
Conclusions:
- BCS is a rare but critical cause of ALF requiring prompt diagnosis and management.
- Anticoagulation, evaluation for malignancy/thrombophilia, TIPS, and liver transplantation are key interventions.
- Outcomes may be improving with advanced treatments like TIPS and liver transplantation.
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