Management of nephrolithiasis in autosomal dominant polycystic kidney disease - A single center experience

Abstract

Purpose:To evaluate available options for the management of nephrolithiasis in patients with autosomal dominant polycystic kidney disease (ADPKD).Materials and Methods:Case files of all the patients with ADPKD treated in our hospital in the last 18 years were evaluated. Their demographic details, clinical presentations, investigations, treatments, and outcomes were critically analyzed.Results:There were a total of 19 patients (23 renal units) with nephrolithiasis among 452 consecutive cases of ADPKD. Male-to-female ratio was 3.75:1. The mean age of the patients was 43.3 years (range 23 to 60 years). The most common presentations were pain and hematuria (27.7% each). Mean serum creatinine was 7.2 mg/ dl (range 0.8-18.1 mg/dl) at presentation. The mean stone size was 115 mm2 (range 36 to 980 mm2). The majority of the stones were calyceal (n = 10). Ten renal units (nine patients) required intervention, while the rest were treated conservatively. Treatment offered included open nephrectomy for non-functioning infected kidney (n = 1), extracorporeal shock wave lithotripsy (ESWL, n = 3), ureterorenoscopy (URS, n = 3), and percutaneous nephrolithotomy (PCNL, n = 3). All patients undergoing URS and PCNL had complete clearance, while those undergoing ESWL had a residual stone. Two failed ESWL patients required an auxiliary procedure (retrograde intrarenal surgery, RIRS) and the other was kept under observation. Mean follow-up after treatment was 4.2 years (one month to six years). None of the patients had major complications.Conclusion:Careful selection of the endourological procedure can give good results in patients of ADPKD with nephrolithiasis.

Percutaneous Nephrolithotomy in Autosomal Dominant Polycystic Kidney Disease: Is it Different from Percutaneous Nephrolithotomy in Normal Kidney?

“…There are few papers and case reports on the PNL in ADPKD with limited number of patients and experience. The PNL in ADPKD create some difficulty such as distorted pelvicaliceal system leading to difficult puncture and dilatation, cyst puncture, cyst bleeding, cyst infection and associated parenchymal calcifications misleading to presence of stones under the fluoroscopy [1,2,4,5,6,7,8]. Umbreit et al [9] found that PNL to be safe and effective in ADPKD with large stone burden despite the increased operative complexity, need of multiple punctures and repeat nephroscopy.…”

“…The important complications which occur due to this disease are the hypertension, renal failure, nephrolithiasis, hemorrhage and infections [1]. The nephrolithiasis occurs in about 8-36% of the patients which increases the morbidity and further accelerates the onset of renal failure [2].…”

“…Most of the patients show a stable period of mild to moderate renal failure, which later on hastened by onset of hypertension, infection and nephrolithiasis [1]. Nephrolithiasis has been reported in 20-28% of patients, whom 50% are symptomatic for stone disease and 20% require definite urologic intervention [1,2].…”

“…The causes of stone formation in ADPKD are due to both anatomic and metabolic factors. The management of nephrolithiasis includes oral alkali dissolution therapy, extracorporeal shock wave lithotripsy (ESWL) and surgical treatment [1,2,3]. There was time when open surgeries including nephrectomy were done for treatment of nephrolithiasis.…”

Polycystic Kidney Disease with Renal Calculi Treated by Percutaneous Nephrolithotomy: A Report of 11 Cases

“…Although extracorporeal shock wave lithotripsy is least invasive and its stone-free rate is similar to those achieved by percutaneous nephrolithotomy (PCNL) and ureteroscopy, its limitations lie in long-term complications like renal dysfunction and hypertension, which make its efficacy questionable [7]. PCNL has been reported for treatment of very few cases recently and its effect is acceptable [8,9]. Nevertheless, the deformed anatomy and the impairment of renal function of ADPKD with renal calculi still make minimally invasive PCNL a very challenging procedure.…”