Multicentric Study of the Andalusian Experience in Polycystic Liver Disease as Indication for Liver Transplantation

Background

The purpose of this study was to determine the morbidity and survival in patients with polycystic liver disease (PLD) undergoing liver transplantation (LT) in 4 Spanish hospitals.

Methods

A multicentric retrospective study using a prospective database was designed including 19 LTs after PLD diagnosis performed from January 1, 1990, to December 31, 2016. Pediatric patients were excluded from the analysis.

Results

Of the included patients, 63.2% were female, the overall average age was 52.16 ± 11.276 years, median time on the waiting list was 394 days (interquartile range [IQR], 96.25–464.50) and most of them were classified with Model for End-Stage Liver Disease scores of ≤17. Eleven patients received isolated LT, 1 patient had a previous kidney transplantation (KT), and 7 patients received combined liver-kidney transplantation, 4 of them with a previous nephrectomy. Complications include hepatopulmonary syndrome in 10.5%, paralytic ileus in 10.5%, transient renal dysfunction in 10.5%, and hepatorenal syndrome in 5.3%. The most common surgical complication was bleeding (15.8%). Three patients presented graft rejection, which was treated by means of immunosuppressive optimization (15.8%), with corticosteroid addition needed in 1 of them. Thrombosis of the hepatic artery occurred in 3 patients, requiring retransplantation in 2 of them. Most of the patients had improved renal function after the procedure. The mortality rate was 15.8%, related to tumors or sepsis, with an estimated 86% 5-year graft survival.

Conclusions

PLD as indication of LT presents a low complications rate and better graft survival and renal function, especially when KT is associated with LT.