Results of Liver Transplantation in Adult Polycystic Liver Disease: Report of a Single Center Experience

Introduction: Polycystic liver disease (PCLD) occurs either in an isolated form (Autosomal Dominant Polycystic Liver Disease [ADPLD]) or in association with Autosomal Dominant Polycystic Kidney Disease (ADPKD). It remains an uncommon and controversial indication for liver transplantation (LT). Objectives: 1. to assess the results of LT in patients with massive PCLD; 2. to determine whether previous hepatic surgery is associated with a higher rate of complications following LT; 3. to examine the evolution of renal function after LT in order to determine whether pre-emptive renal transplantation is justified in case of ADPKD without (pre-)terminal renal failure. Methods: We retrospectively reviewed the medical charts of 19 patients (15 females) who underwent LT for PCLD between 1999 and 2008. Fifteen patients had ADPKD (12 females). Three received a combined liver and kidney transplantation (LKT) for associated terminal (n = 1) or pre-terminal renal failure. Two patients had previous kidney transplantation (KT) and 7 had undergone hepatic surgery. Results: Median duration of follow-up is 30 months [5-112]. All patients are alive and relieved of their symptoms. Their median Karnofsky score is 90% [80-100]. Intervention tended to be longer (10:30h vs. 7:20h, p=0.098) in the group who had previous surgery. There was no significant difference in intra-operative blood transfusion, severity of postoperative complications, length of ICU or hospital stay. Median pre-LT GFR of ADPLD and ADPKD patients (excluding those who underwent LKT or previous KT) is 89.5ml/min/1.73m2 [69-114] and 69ml/min/1.73m2 [33-120] respectively (p=0.129). Median GFR 1 year post-LT is 68ml/min/1.73m2 [57-95] and 51ml/min/1.73m2 [29-85] respectively (p=0.089). Median decrease in GFR at one year post-LT is 17.5 ml/min/1.73m2 and 18 ml/min/1.73m2 respectively (p=0.694). Conclusions: LT is an effective treatment for selected patients with massive PCLD. Prior conservative surgery tends to increase the length of the transplant operation. Renal function decreases at an identical rate after LT in ADPLD and ADPKD patients with pre-LT GFR >30 ml/min/1.73m2. Thus, pre-emptive renal transplantation would not have been justified in the latter