The aim of the present study was to explore the indications for and safety of orthotopic liver transplantation for polycystic liver disease (PLD). Orthotopic liver transplantation in 11 patients with PLD between May 2004 and September 2013 was retrospectively analyzed. Patient epidemiological, clinical and follow-up data were collected. The survival rate was calculated using the Kaplan-Meier method. Over the 10-year period, 11 patients received orthotopic liver transplantation (n=9) and combined liver-kidney transplantation (n=2) for PLD. The recipients' median age was 56 years [(interquartile range (IQR), 52–57 years], and 7 of the patients (63.6%) were classified as having Gigot type II PLD and 4 (36.4%) as having Gigot type III. A total of 8 (72.7%) patients had a severely decreased quality of life (Eastern Cooperative Oncology Group performance status score, ≥3). Only 3 cases (27.3%) were of Class C stage. The mean hospitalization duration was 45.4±15.3 days and the mean length of stay at the intensive care unit was 4.1±1.9 days. The peri-operative mortality was 18.2% and the morbidity was 54.5%. The median follow-up period was 111 months (IQR, 33–132 months). A total of 2 patients died of severe complications after combined liver-kidney transplantation. Furthermore, 1 patient died of ischemia cholangitis during the follow-up period. The actuarial 1-, 5- and 10-year survival rate during the follow-up period was 81.8, 81.8 and 65.5%, respectively. The mean physical component summary score was 87.1±6.9 and the mean mental component summary score was 81.5±6.4. In conclusion, liver transplantation is the only curative procedure for PLD, and the present study indicated that it is relatively and safe and leads to good long-term prognosis and high quality of life. Based on our experience and results, liver transplantation is a primary option for cases of PLD with progressive or advanced symptomatic disease where previous other forms of therapy to palliate symptoms have been insufficient.
展开▼
