Cystectomy in the Pediatric Exstrophy Population: Indications and Outcomes

To explore the long-term outcomes and indications for cystectomy in bladder exstrophy patients. Although rare, cystectomy is the final surgical alternative to bladder repair among these patients with a poor quality bladder template.

A prospectively maintained database of 1298 patients with exstrophy-epispadias complex was reviewed for patients who underwent cystectomy between 1970 and 2015 at the author's institution. Demographic data, indication for cystectomy, surgical history, post-operative outcomes, and continence status were collected.

Eighteen (6 male; 12 female) exstrophy patients (15 classic bladder exstrophy; 2 bladder exstrophy variants; 1 cloacal exstrophy) underwent cystectomy at a median age of 3.8 years. Six patients (33.3%) underwent primary cystectomy without attempted bladder closure. Eight (44.4%) had a history of failed primary closure with loss of capacity or inadequate growth after closure. Four (22.2%) had successful primary closure but underwent cystectomy secondary to poor bladder compliance with declining renal function or poor bladder growth/quality. Urinary diversion included 6 cutaneous ureterostomies, 4 bowel conduits (1 ileal; 3 colon), 6 continent urinary diversions with ileosigmoid reservoir, and 1 ureterosigmoidostomy. Of eight patients who underwent a continence procedure, all were dry at a median of 25.3 months after cystectomy.

Cystectomy was most commonly indicated in intrinsically diseased bladder templates that remained too small despite permitting time for interval growth. These bladders often were of poor quality and compliance and did not reduce into the pelvis on exam. It was, however, possible to achieve urinary continence in these patients with cystectomy and urinary diversion.

Urology. 2017 Sep 25 [Epub ahead of print]

Joan Ko, Kathy Lue, Daniel Friedlander, Timothy Baumgartner, Peter Stuhldreher, Heather N DiCarlo, John P Gearhart

The Johns Hopkins University School of Medicine, James Buchanan Brady Urological Institute, Division of Pediatric Urology, Charlotte Bloomberg Children's Hospital, Suite 7304, Baltimore, MD 21201, Tel: (410) 955-5358, Fax: (410) 614-8096., The Johns Hopkins University School of Medicine, James Buchanan Brady Urological Institute, Division of Pediatric Urology, Charlotte Bloomberg Children's Hospital, Suite 7304, Baltimore, MD 21201, Tel: (410) 955-5358, Fax: (410) 614-8096,. Electronic address: .

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