Journal of pediatric urology · 2026
Long-term outcomes of percutaneous nephrolithotomy and disease management in pediatric cystine stone patients: A 25-year single-institution experience.
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Kirli Elif Altinay, Kalender Goktug, Sahin Kadir Can, Onal Bulent
Аннотация
BACKGROUND: Cystine stones are a rare yet challenging cause of pediatric nephrolithiasis, marked by high recurrence and repeated surgical needs. Although endourological advances have improved the safety of percutaneous nephrolithotomy (PCNL), long-term renal outcomes remain strongly influenced by adherence to medical therapy.
OBJECTIVE: To evaluate the efficacy, safety, recurrence patterns, and long-term renal outcomes of PCNL for cystine stones in children across a 25-year institutional experience.
STUDY DESIGN: This retrospective review included pediatric patients with cystine stones who underwent PCNL between 2000 and 2024 at a tertiary referral center. Surgical success was defined as stone-free status. Complications were graded using the modified SATAVA and Clavien-Dindo systems. All patients received urine alkalization and thiol therapy, with adherence assessed by the Medication Possession Ratio. Long-term renal function and blood pressure were evaluated during follow-up.
RESULTS: A total of 127 PCNLs were performed in 94 renal units of 78 patients (median age at first PCNL 6 years [IQR 2-14]; 56% male, 44% female), with a median follow-up of 216 months (IQR 141-287). The overall stone-free rate was 64%, increasing to 81% when combined with simultaneous endoscopic procedures. Repeat PCNL for recurrence occurred in 42.5% of renal units (n = 40/94), and regrowth in 11.7% of the renal units (n = 11/94). Only nine (11.5%) of patients were fully adherent to medical therapy. Kaplan-Meier analysis showed a significantly shorter time to repeat PCNL in non-adherent renal units (log-rank p = 0.016); among those that experienced an event, the median time to first repeat PCNL was 15 months, whereas no repeat PCNL events were observed in adherent renal units. During follow-up, 18 patients (23%) developed hypertension and eight (10.3%) developed renal function decline, all confined to the non-adherent group.
DISCUSSION: Although PCNL provided high clearance with good procedural safety, long-term success was limited by poor adherence. Recurrence and renal deterioration rates were comparable to those reported in previous series. Major limitations include the retrospective, single-center design and incomplete metabolic data.
CONCLUSIONS: PCNL is a safe and effective option for pediatric cystine stones, but sustained medical adherence is essential to prevent recurrence and preserve renal function, ensuring durable long-term outcomes.
OBJECTIVE: To evaluate the efficacy, safety, recurrence patterns, and long-term renal outcomes of PCNL for cystine stones in children across a 25-year institutional experience.
STUDY DESIGN: This retrospective review included pediatric patients with cystine stones who underwent PCNL between 2000 and 2024 at a tertiary referral center. Surgical success was defined as stone-free status. Complications were graded using the modified SATAVA and Clavien-Dindo systems. All patients received urine alkalization and thiol therapy, with adherence assessed by the Medication Possession Ratio. Long-term renal function and blood pressure were evaluated during follow-up.
RESULTS: A total of 127 PCNLs were performed in 94 renal units of 78 patients (median age at first PCNL 6 years [IQR 2-14]; 56% male, 44% female), with a median follow-up of 216 months (IQR 141-287). The overall stone-free rate was 64%, increasing to 81% when combined with simultaneous endoscopic procedures. Repeat PCNL for recurrence occurred in 42.5% of renal units (n = 40/94), and regrowth in 11.7% of the renal units (n = 11/94). Only nine (11.5%) of patients were fully adherent to medical therapy. Kaplan-Meier analysis showed a significantly shorter time to repeat PCNL in non-adherent renal units (log-rank p = 0.016); among those that experienced an event, the median time to first repeat PCNL was 15 months, whereas no repeat PCNL events were observed in adherent renal units. During follow-up, 18 patients (23%) developed hypertension and eight (10.3%) developed renal function decline, all confined to the non-adherent group.
DISCUSSION: Although PCNL provided high clearance with good procedural safety, long-term success was limited by poor adherence. Recurrence and renal deterioration rates were comparable to those reported in previous series. Major limitations include the retrospective, single-center design and incomplete metabolic data.
CONCLUSIONS: PCNL is a safe and effective option for pediatric cystine stones, but sustained medical adherence is essential to prevent recurrence and preserve renal function, ensuring durable long-term outcomes.