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An Update on the Management of Budd-Chiari Syndrome
A Sharma1, S N Keshava2, A Eapen3
1Hepatology Department, Christian Medical College, Vellore, Tamil Nadu, India.
Budd-Chiari syndrome (BCS), a rare hepatic venous outflow obstruction, is often underdiagnosed. Myeloproliferative neoplasms are the most common cause, with improved diagnosis and endovascular treatments enhancing patient survival.
Area of Science:
- Hepatology
- Vascular Medicine
- Hematology
Background:
- Budd-Chiari syndrome (BCS) is an uncommon condition characterized by hepatic venous outflow obstruction.
- It is frequently underdiagnosed, necessitating a high index of suspicion in patients with unexplained portal hypertension.
Purpose of the Study:
- To review the current understanding of Budd-Chiari syndrome (BCS) etiology, pathology, diagnosis, and management.
- To highlight recent advances and discuss unresolved issues in BCS treatment.
Main Methods:
- Literature review focusing on recent studies and diagnostic techniques.
- Analysis of current treatment modalities including endovascular interventions, anticoagulation, and liver transplantation.
- Discussion of the role of genetic studies in diagnosing underlying myeloproliferative neoplasms (MPN).
Main Results:
- Identifiable etiologies are found in over 80% of BCS cases, with myeloproliferative neoplasm (MPN) being the most frequent.
- Advances in cross-sectional imaging improve the accuracy of obstruction site delineation.
- Endovascular interventions and anticoagulation have significantly improved survival rates for BCS patients.
Conclusions:
- Early diagnosis of BCS, particularly in patients with unexplained portal hypertension and potential MPN, is crucial.
- Endovascular angioplasty for hepatic veins/inferior vena cava is underutilized but effective.
- Current management strategies focus on endovascular treatments and anticoagulation, with liver transplantation reserved for specific indications.
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