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Liver transplantation in patients with Budd-Chiari syndrome
1Department of General and Digestive Surgery, University Hospital 12 de Octubre, Madrid, Spain.
Insights
Orthotopic liver transplantation (OLT) is a viable option for Budd-Chiari syndrome patients with severe hepatic insufficiency. Early diagnosis of the obstruction
Area of Science:
- Hepatology
- Transplantation Surgery
- Vascular Surgery
Background:
- Budd-Chiari syndrome, characterized by hepatic vein obstruction, can lead to severe hepatic insufficiency.
- Orthotopic liver transplantation (OLT) is a potential treatment for end-stage liver disease caused by Budd-Chiari syndrome.
Purpose of the Study:
- To evaluate the role and outcomes of OLT in patients with Budd-Chiari syndrome.
- To investigate the diverse etiologies of hepatic vein obstruction in these patients.
Main Methods:
- Retrospective analysis of 405 OLT patients, identifying 3 cases (0.7%) with Budd-Chiari syndrome.
- Review of pre-transplant morphologic studies to determine the cause of obstruction.
- Standard OLT surgical techniques were employed.
Main Results:
- Three patients underwent OLT for Budd-Chiari syndrome due to severe hepatic insufficiency (two chronic, one acute).
- Identified causes of obstruction included thrombi, membranous webs, and extrinsic compression by a hydatid cyst.
- Surgical approach for OLT was comparable to other liver disease etiologies.
Conclusions:
- Budd-Chiari syndrome is a rare but significant indication for OLT.
- Accurate pre-transplant etiological diagnosis is crucial for managing Budd-Chiari syndrome.
- Early and long-term anticoagulant therapy is recommended post-OLT to prevent recurrence.
Abstract:
Patients with Budd-Chiari syndrome (obstruction of the hepatic veins) and associated hepatic insufficiency may be candidates for orthotopic liver transplantation (OLT). In our series of 405 OLT patients, 3 were transplanted due to Budd-Chiari syndrome (0.7%). The indication for liver transplantation in these patients was severe hepatic insufficiency (chronic in two and acute in the third one). Morphologic study of the obstructions revealed apparently different causes, including thrombi, membranous webs in hepatic veins, and hydatid cyst compression. The surgical technique employed in these transplantations was similar to that for other etiologies. Due to its implications for the future course of OLT, it is important to determine the exact etiology of Budd-Chiari syndrome in the pretransplant period and to treat the patients with early and long-term anticoagulant therapy to avoid syndrome recurrence.