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Liver transplantation for the Budd-Chiari syndrome
1Department of Surgery, University Health Center of Pittsburgh, University of Pittsburgh, Pennsylvania.
Insights
Orthotopic liver transplantation is effective for Budd-Chiari syndrome with end-stage liver disease. While recurrence occurred in three deceased patients, most showed no evidence of recurrence post-transplant.
Area of Science:
- Hepatology
- Transplantation Surgery
- Vascular Surgery
Background:
- Budd-Chiari syndrome (BCS) is a rare condition causing liver damage due to hepatic vein obstruction.
- End-stage liver disease (ESLD) necessitates liver transplantation for survival.
- Orthotopic liver transplantation (OLT) is a potential treatment for BCS with ESLD.
Purpose of the Study:
- To evaluate the efficacy and outcomes of orthotopic liver transplantation in patients with Budd-Chiari syndrome and end-stage liver disease.
- To analyze technical considerations and complications associated with OLT for BCS.
- To assess the long-term survival rates and recurrence of BCS post-transplantation.
Main Methods:
- Retrospective analysis of 23 patients who underwent OLT for BCS with ESLD.
- Patient follow-up extended up to 14 years.
- Review of technical aspects, postoperative complications, and liver biopsy results.
Main Results:
- One-, 3-, and 5-year actuarial survival rates were 68.8%, 44.7%, and 44.7%, respectively.
- No serious complications were reported with postoperative anticoagulation.
- BCS recurrence was observed in three patients who died; no other recurrences were noted on postoperative biopsies.
Conclusions:
- Orthotopic liver transplantation is the most effective treatment for Budd-Chiari syndrome complicated by end-stage liver disease.
- OLT offers a viable option for managing BCS-related ESLD with acceptable survival rates.
- Careful patient selection and management are crucial for successful outcomes.
Abstract:
A retrospective study was performed that analyzed 23 patients who had an orthotopic liver transplantation for the Budd-Chiari syndrome with end-stage liver disease. Patient follow-up was as long as 14 years. The technical considerations relevant to the Budd-Chiari syndrome were discussed. There have been no serious complications of postoperative anticoagulation. Three patients, all of whom died, had recurrence of the Budd-Chiari syndrome. No other patient has had evidence of recurrent Budd-Chiari syndrome on postoperative liver biopsies. One-, 3-, and 5-year actuarial survival was 68.8%, 44.7%, and 44.7%, respectively. It was concluded that orthotopic liver transplantation is the most effective treatment for patients with the Budd-Chiari syndrome and end-stage liver disease.