Objective: To evaluate the efficacy and safety of percutaneous nephrolithotomy (PCNL) in the treatment of congenital autosomal dominant polycystic kidney disease (ADPKD) with renal calculi. Methods: A total of 11 cases of ADPKD with renal calculi were retrospectively studied. All cases were treated by PCNL and clinical parameters such as age, sex, stone burden, operation time, blood loss, complications and perioperative serum creatinine levels were collected and analyzed. All patients were routinely followed up for 36 months postoperatively and the efficacy and safety of PCNL in the management of ADPKD with renal calculi were assessed. Results: All procedures were successful without conversion to open surgery. The mean stone burden was 3.4 ± 0.7 cm (range 2.5-4.6 cm). The overall stone clearance rate was 81.8% (9/11). Two patients received extracorporeal shock wave lithotripsy postoperatively because of residual calculi. The mean operation time was 77 ± 23.5 min (range 45-128 min) and the mean blood loss was 147 ± 56 ml (range 80-260 ml). The mean preoperative serum creatinine level was 15.13 ± 3.30 mg/dl (range 9.90-20.14 mg/dl) and the mean postoperative serum creatinine level was 14.28 ± 2.24 mg/dl (range 10.76-17.28 mg/dl). Four cases had fever postoperatively (36.4%). Three patients had hemorrhage and two patients received blood transfusion. No patient had peripheral viscera injury. Statistical comparison showed that PCNL had no impact on perioperative renal function (p > 0.05). Conclusions: In spite of anatomical deformation and impairment of renal function, PCNL is an effective and safe procedure in managing ADPKD with renal calculi.

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