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Cystectomy in the Pediatric Exstrophy Population: Indications and Outcomes.

Joan S Ko1, Kathy Lue1, Daniel Friedlander1

  • 1Division of Pediatric Urology, James Buchanan Brady Urological Institute, Charlotte Bloomberg Children's Hospital, The Johns Hopkins University School of Medicine, Baltimore, MD.

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Summary

Cystectomy in bladder exstrophy patients is a rare but viable option for poor-quality bladders. This study shows cystectomy can lead to urinary continence, even in complex cases.

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Area of Science:

  • Pediatric Urology
  • Reconstructive Surgery

Background:

  • Bladder exstrophy is a rare congenital condition requiring complex surgical management.
  • Cystectomy is a seldom-used, final surgical option for patients with a non-functional bladder template.

Purpose of the Study:

  • To investigate the long-term outcomes and indications for cystectomy in patients with bladder exstrophy.
  • To assess the feasibility of achieving urinary continence after cystectomy and urinary diversion in this population.

Main Methods:

  • A retrospective review of 1298 patients with exstrophy-epispadias complex was conducted.
  • Data on 18 patients who underwent cystectomy between 1970 and 2015 were analyzed, including demographics, indications, surgical history, and outcomes.
  • Urinary diversion methods and continence status were specifically evaluated.

Main Results:

  • Eighteen patients (15 classic bladder exstrophy) underwent cystectomy, most commonly due to intrinsically poor bladder quality, size, or compliance.
  • Urinary diversion varied, including cutaneous ureterostomies, bowel conduits, and continent urinary diversions.
  • Eight patients who underwent a continence procedure achieved dryness at a median follow-up of 25.3 months.

Conclusions:

  • Cystectomy is indicated for bladder exstrophy patients with small, poor-quality bladders unresponsive to other treatments.
  • Achieving urinary continence is possible following cystectomy and appropriate urinary diversion.
  • This approach offers a solution for patients with otherwise unsalvageable bladder templates.