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Flexible ureteroscopy in children with von Willebrand disease
Matthew S Christman1, Justin Ziemba, Pasquale Casale
1Department of Urology, The Children's Hospital of Philadelphia, University of Pennsylvania, Philadelphia, Pennsylvania 19035, USA. christmanm@email.chop.edu
Insights
Flexible ureteroscopy is a safe and effective treatment for pediatric urolithiasis in children with bleeding disorders like von Willebrand disease (vWd). Proper perioperative management of bleeding abnormalities is key to minimizing complications.
Area of Science:
- Pediatric Urology
- Endourology
- Pediatric Nephrology
Background:
- Urolithiasis treatment options like SWL, PCNL, and ureteroscopy are standard for children.
- Children with bleeding dyscrasias require specialized management approaches.
- Von Willebrand disease (vWd) presents unique challenges in pediatric urolithiasis management.
Purpose of the Study:
- To evaluate the safety and efficacy of flexible ureteroscopy in pediatric patients with von Willebrand disease (vWd).
- To assess outcomes and complications associated with ureteroscopy in this specific patient population.
Main Methods:
- Retrospective review of a prospectively maintained database of pediatric stone patients.
- Identification of patients with bleeding diathesis, specifically vWd.
- Evaluation of stone burden, operative details, complications, stone-free status, and perioperative medical management.
Main Results:
- Seven flexible ureteroscopic procedures were performed on five children with vWd over seven years.
- Mean stone burden was 6.1 mm, with an average of 1.3 stones per patient.
- 100% stone clearance was achieved with a low complication rate of 6% (1/17 procedures).
Conclusions:
- Flexible ureteroscopy is a safe and effective treatment for pediatric urolithiasis in children with bleeding abnormalities.
- Perioperative medical management, including desmopressin acetate, is crucial for reducing complications.
- Ureteroscopy offers a viable solution for managing urinary stones in children with vWd.
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.
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