Flexible ureteroscopy in children with von Willebrand disease

Background and Purpose: Treatment options for urolithiasis include shockwave lithotripsy (SWL), percutaneous nephrostolithotomy (PCNL), and ureteroscopy. While these treatment options are becoming the standard of care in the pediatric population, children with a bleeding dyscrasia must be approached differently. We report our flexible ureteroscopic experience in children with von Willebrand disease (vWd). Patients and Methods: A review of a prospectively maintained database of stone patients was performed. Children with a bleeding diathesis were identified. Stone burden, operative access, intraoperative complications, postoperative complications, stone-free status, and perioperative medical management were evaluated. Results: During a 7-year period, a total of five children with a bleeding disorder underwent 7 ureteroscopic and 10 cystoscopic procedures for urinary calculi. The mean patient age was 72.4 months (range 49-123 mos) at the time of the procedure. There were three boys and two girls. Mean follow-up was 29.7 months (range 8-79 mos). Mean stone burden was 6.1 mm (range 3-14 mm) with an average of 1.3 stones per patient. Four patients were known to have vWd before surgical intervention and were pretreated with desmopressin acetate. The remaining patient was found to have vWd based on significant bleeding after ureteral stent placement. No ureters were actively dilated. Flexible ureteroscopy was performed in all cases regardless of stone location. Stone clearance was 100% (7/7). Complications occurred in 6% of procedures (1/17). Conclusions: Ureteroscopy is a safe and efficacious modality in the management of urolithiasis in children with a bleeding abnormality. Perioperative medical management of the bleeding diathesis may help reduce complications.