Background
Budd-Chiari syndrome (BCS) is a rare disease resulting from obstruction of the hepatic venous outflow tract that typically presents with abdominal pain, jaundice and ascites without frank liver failure. However, BCS may also evolve more rapidly to acute liver failure (ALF-BCS).
Aims
To describe the clinical features, treatment and outcomes of ALF due to BCS and compare our results with those in the published literature.
Methods
Twenty of the 2,300 patients enrolled in the Acute Liver Failure Study Group (ALFSG) registry since 1998, presented with a clinical diagnosis of BCS. An additional 19 cases of ALF-BCS in the English-language literature were reviewed and compared to the ALFSG cases.
Results
Most ALF-BCS patients were white (84%) and female (84%) in their fourth decade. A hypercoagulable state was noted in 63% of cases. BCS was diagnosed by Doppler ultrasonography or abdominal CT in all cases. Liver biopsies (6) all had evidence of severe peri-central necrosis. Treatments used included most commonly anticoagulation (71%), but also transjugular intrahepatic portosystemic shunt (TIPS) (37%) and orthotopic liver transplantation (37%). In-hospital mortality was approximately 60%.
Conclusions
Budd-Chiari syndrome is a rare cause of ALF and mandates prompt diagnosis and management for successful outcomes. Once the diagnosis is confirmed, prompt anticoagulation is recommended in conjunction with evaluation for malignancy or thrombophilic disorder. Mortality may have improved in recent years with use of TIPS and/or OLT compared to prior published reports.