Budd-Chiari syndrome
Michael A Zimmerman1, Andrew M Cameron, R Mark Ghobrial
1Division of Liver and Pancreas Transplantation, Department of Surgery, The Pfleger Liver Institute, The Dumont-UCLA Transplant Center, The David Geffen School of Medicine at the University of California Los Angeles, Los Angeles, CA 90095, USA.
Insights
Budd-Chiari syndrome (BCS) involves hepatic venous outflow occlusion due to anatomical issues or clotting disorders. Diagnosis and supportive care are key, with surgical shunting and liver transplantation offering viable treatment options.
Area of Science:
- Hepatology
- Vascular Medicine
- Gastroenterology
Background:
- Budd-Chiari syndrome (BCS) is characterized by hepatic venous outflow occlusion.
- It encompasses anatomical abnormalities and hypercoagulable disorders.
- Common symptoms include hepatomegaly, abdominal pain, and ascites.
Purpose of the Study:
- To outline the diagnostic approach for Budd-Chiari syndrome.
- To review supportive medical therapies for BCS.
- To evaluate surgical shunting and liver transplantation as treatment options.
Main Methods:
- Review of clinical manifestations.
- Analysis of diagnostic strategies.
- Evaluation of treatment data for surgical and transplant options.
Main Results:
- Budd-Chiari syndrome presents with varied clinical signs.
- Medical management focuses on supportive care.
- Surgical shunting and liver transplantation show promise.
Conclusions:
- Effective management of Budd-Chiari syndrome requires accurate diagnosis and tailored therapy.
- Surgical interventions and liver transplantation are critical treatment modalities for BCS.
- Further research can optimize outcomes for patients with hepatic venous outflow obstruction.
Abstract:
Budd-Chiari syndrome is a spectrum of disease states, including anatomic abnormalities and hypercoagulable disorders, resulting in hepatic venous outflow occlusion. Clinical manifestations observed in the majority of patients include hepatomegaly, right upper quadrant pain, and abdominal ascites. This article outlines the approach to clinical diagnosis and supportive medical therapy in patients who have BCS and reviews the clinical data supporting surgical shunting and liver transplantation as viable treatment options in this patient population.
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