Liver Transplantation for Polycystic Disease: Experience and Results of a Single-Center Series

Abstract

Polycystic liver disease (PLD) is a hereditary condition, and its symptoms are due to the growth of cysts. Liver transplantation (LT) is the only curative treatment. A retrospective single-center analysis was conducted on the 10 LTs performed for PLD between 2004 and 2023. Eighty percent of the patients were women, and 70% had polycystic hepatorenal involvement. Forty percent required a kidney transplant prior to LT, and in 30%, simultaneous hepatorenal transplantation was performed due to end-stage chronic kidney failure. In 70% of cases, hepatectomy was performed with vena cava preservation (the piggy-back technique). The median operative time was 235 minutes (range 190-398), and the mean weight of the explanted liver was 5050g (range 1840-9450). Two patients had major complications classified as ≥IIIa on the Clavien-Dindo scale: a hemorrhage from the phrenic artery and a hepatic artery anastomotic stenosis, both resolved through interventional radiology. The rate of surgical reoperation and postoperative mortality was zero. The median stay in the Intensive Care Unit was 4 days (range 3-7), and the median hospital stay was 17 days (range 10-25). No patient required re-transplantation. After a median follow-up of 43.5 months (range 8-284), both patient and graft survival were 100%. Overall, and based on our experience, LT provides excellent outcomes and it is the treatment of choice for PLD in patients with end-stage liver failure or clinically limiting symptoms.