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Redefining Budd-Chiari syndrome: A systematic review
Naomi Shin1, Young H Kim1, Hao Xu1
1Naomi Shin, Young H Kim, Department of Radiology, University of Massachusetts Medical School, Worcester, MA 01655, United States.
Insights
Hepatic vein thrombosis (classical Budd-Chiari syndrome) and hepatic vena cava-Budd Chiari syndrome (HVC-BCS) appear to be distinct conditions. This systematic review highlights key differences in patient demographics, clinical presentation, etiology, and management strategies for these hepatic venous outflow disorders.
Area of Science:
- Hepatology
- Vascular Medicine
- Gastroenterology
Background:
- Budd-Chiari syndrome (BCS) encompasses hepatic vein thrombosis (HVT) and hepatic vena cava-Budd Chiari syndrome (HVC-BCS).
- Distinguishing between classical BCS (HVT) and HVC-BCS is crucial for appropriate diagnosis and treatment.
- Previous literature has sometimes conflated these two distinct clinical entities.
Purpose of the Study:
- To systematically review current evidence to determine if classical BCS and HVC-BCS represent the same or different clinical disorders.
- To identify and compare key characteristics, including etiology, clinical presentation, and management, of classical BCS and HVC-BCS.
Main Methods:
- A systematic review of observational studies published between January 2000 and February 2015 was conducted.
- Databases searched included Cochrane Library, CINAHL, MEDLINE, PubMed, and Scopus.
- Studies included adult subjects with primary BCS, hepatic vein outflow tract obstruction, inferior vena cava (IVC) obstruction, or HVT.
Main Results:
- Classical BCS predominantly affects women with pure hepatic vein obstruction, presenting acutely with abdominal pain, ascites, and hepatomegaly.
- HVC-BCS more commonly affects men, with obstruction in both the IVC and hepatic veins, presenting chronically with abdominal pain and abdominal wall varices.
- Myeloproliferative neoplasms (MPN), particularly JAK2V617-F mutation, are a major cause of classical BCS (16%-62%), but less frequent in HVC-BCS (4%-5%).
- Classical BCS responds to medical management, while HVC-BCS often requires percutaneous recanalization as first-line treatment.
Conclusions:
- Current data suggest that classical BCS and HVC-BCS are clinically distinct disorders.
- These conditions share the common feature of disrupted hepatic venous outflow but differ significantly in their clinical profiles.
- Further research may elucidate more precise diagnostic and therapeutic approaches for each entity.
Aim:
To re-examine whether hepatic vein thrombosis (HVT) (classical Budd-Chiari syndrome) and hepatic vena cava-Budd Chiari syndrome (HVC-BCS) are the same disorder.
Methods:
A systematic review of observational studies conducted in adult subjects with primary BCS, hepatic vein outflow tract obstruction, membranous obstruction of the inferior vena cava (IVC), obliterative hepatocavopathy, or HVT during the period of January 2000 until February 2015 was conducted using the following databases: Cochrane Library, CINAHL, MEDLINE, PubMed and Scopus.
Results:
Of 1299 articles identified, 26 were included in this study. Classical BCS is more common in women with a pure hepatic vein obstruction (49%-74%). HVC-BCS is more common in men with the obstruction often located in both the inferior vena cava and hepatic veins (14%-84%). Classical BCS presents with acute abdominal pain, ascites, and hepatomegaly. HVC-BCS presents with chronic abdominal pain and abdominal wall varices. Myeloproliferative neoplasms (MPN) are the most common etiology of classical BCS (16%-62%) with the JAK2V617-F mutation found in 26%-52%. In HVC-BCS, MPN are found in 4%-5%, and the JAK2V617-F mutation in 2%-5%. Classical BCS responds well to medical management alone and 1(st) line management of HVC-BCS involves percutaneous recanalization, with few managed with medical management alone.
Conclusion:
Systematic review of recent data suggests that classical BCS and HVC-BCS may be two clinically different disorders that involve the disruption of hepatic venous outflow.
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