[Liver transplantation for polycystic liver disease: 11 cases report and literature review].

Objective

To explore the indications and safety of orthopedic liver transplantation for polycystic liver disease (PLD).

Methods

Data of 11 patients with PLD who underwent orthotopic liver transplantation between 2004 and 2013 was retrospectively analyzed. Demographic, clinical and follow-up data were collected for statistical analysis. The survival rate was calculated by Kaplan-Meier method.

Results

Over a period of 10 years, the patients received modified piggyback orthopedic liver transplantation (n=9) or combined liver-kidney transplantation (n=2) for PLD. The recipients' median age was 56 years. Seven patients were classified as Gigot type Ⅱ PLD, and four were classified as Gigot type Ⅲ PLD. Eight patients had severe decreased mobility (Eastern Cooperative Oncology Group, ECOG≥3). Only three cases were Child-Pguh Class C patients and the model for end-stage liver disease (MELD) score was>20. The mean hospitalization duration was (45.4±15.3) days, and the mean length of stay in intensive care unit was (4.1±1.9) days. The perioperative mortality was 18.2% and morbidity of complications was 63.6%. The median follow-up period was 111 months. Two patients died of severe complications after combined liver-kidney transplantation. One patient died of ischemia cholangitis during follow-up. The actuarial 1-, 5-and 10-year survival rate during the follow-up period was 82.2%, 81.8%, and 65.5%, respectively.

Conclusions

Liver transplantation is the only curative and safe procedure for PLD, and it provides a good long-term prognosis and high quality of life for PLD patients. Liver transplantation could be a primary option in treating progressive or advanced PLD.