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Liver transplantation for polycystic liver disease
1Liver Transplant Group, Katholiek Universiteit Leuven, University Hospitals Leuven, Herestraat 49, B-3000 Leuven, Belgium. jacques.pirenne@uz.kuleuven.ac.be
Summary
Orthotopic liver transplantation (OLT) offers a definitive treatment for polycystic liver disease (PLD) causing severe handicaps. This study shows OLT provides excellent survival rates and improves quality of life, with combined kidney transplantation rarely needed.
Area of Science:
- Hepatology
- Transplantation Surgery
- Medical Genetics
Background:
- Polycystic liver disease (PLD) can lead to massive hepatomegaly, causing significant physical and social limitations.
- Existing data on orthotopic liver transplantation (OLT) for PLD are limited and show conflicting outcomes.
- Conservative surgical options like resection or fenestration are effective for large cysts but questionable for diffuse small cysts and carry risks.
Purpose of the Study:
- To review the experience and outcomes of OLT for patients with small, diffuse PLD.
- To evaluate the effectiveness of OLT as a curative treatment for severe hepatomegaly and associated handicaps caused by PLD.
- To assess the need for combined liver and kidney transplantation in PLD patients.
Main Methods:
- Retrospective review of 16 adult women who underwent OLT for small diffuse PLD between 1990 and 1999.
- Analysis of indications for transplantation, surgical procedures, perioperative data, complications, and long-term outcomes.
- Assessment of patient and graft survival, kidney function, and quality of life post-transplantation.
Main Results:
- OLT was performed alone in 13 patients; only 1 required a subsequent kidney transplant.
- High patient and graft survival rates of 87.5% were observed with a follow-up of up to 9 years.
- OLT significantly improved quality of life, enabling patients to return to active lifestyles, with satisfactory kidney function in most cases.
Conclusions:
- OLT provides a definitive treatment option for patients with extensive, small, diffuse PLD causing severe hepatomegaly and handicaps.
- Combined liver and kidney transplantation is rarely necessary for PLD patients.
- The benefits of OLT in improving quality of life and offering definitive palliation must be weighed against transplantation risks and immunosuppression.