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The Budd-Chiari syndrome revisited
M Shill1, J M Henderson, A S Tavill
1Friedman Center for Digestive and Liver Disorders, Mt. Sinai Medical Center, Case Western Reserve University, Cleveland, OH 44106.
Insights
Budd-Chiari syndrome, a hepatic venous outflow obstruction, is often linked to thrombogenic disorders. Advances in Doppler ultrasound and interventional procedures offer new diagnostic and management strategies for this condition.
Area of Science:
- Hepatology
- Vascular Surgery
- Interventional Radiology
Background:
- Budd-Chiari syndrome involves hepatic venous outflow obstruction.
- A significant percentage of cases are associated with underlying thrombogenic disorders.
Purpose of the Study:
- To review recent advances in the diagnosis and treatment of Budd-Chiari syndrome.
- To highlight the role of thrombogenic disorders in its etiology.
Main Methods:
- Doppler ultrasound for initial imaging of hepatic veins and flow.
- Angiography with pressure measurements and liver biopsy for management decisions.
- Interventional techniques including angioplasty and shunt placement.
Main Results:
- Doppler ultrasound is crucial for diagnosing hepatic vein abnormalities.
- Nonsurgical options like angioplasty and transjugular intrahepatic portosystemic shunts are effective.
- Decompressive shunts and liver transplantation remain vital for specific patient groups.
Conclusions:
- Early diagnosis and identification of thrombogenic causes are key.
- A multimodal approach combining imaging, interventional radiology, and surgery improves outcomes.
- Liver transplantation is a life-saving option for advanced disease.
Abstract:
Major advances in diagnosis and treatment of the Budd-Chiari syndrome over the past decade have made it worthwhile to revisit this intriguing condition. During this time it has become evident that an underlying predisposing cause, in particular a hematological, thrombogenic disorder, is present in up to 70% of patients presenting with hepatic venous outflow obstruction. Doppler ultrasound has proven extremely valuable as the initial imaging modality to establish the presence or absence of normal hepatic veins and phasic flow. Angiography with vena caval pressure measurements and liver biopsy remain essential in decision making with regard to surgical and nonsurgical management. Nonsurgical management has expanded to include angioplasty of stenoses and webs, although the placement of transjugular intrahepatic portosystemic shunts offers a useful alternative to operative shunts in selected circumstances. In many situations, however, decompressive side-to-side type shunts have a definitive role in patients with preserved liver function and structure. Other patients with severe necrosis, fibrosis, or cirrhosis may be offered a life-saving alternative only by orthotopic liver transplantation.