Cloacal exstrophy (CE) is a severe midline congenital abnormality that requires numerous surgical corrections to achieve an acceptable quality of life. Candidates for urinary continence undergo multiple procedures, most often continent bladder diversions, to become socially dry. Here, the authors investigate the number of genitourinary interventions that patients with CE undergo to attain urinary continence.
A retrospective review of a prospectively maintained database of 1311 exstrophy epispadias complex patients was performed. Patients with CE who have had at least one continence procedure were included. A continence procedure was defined as bladder neck reconstruction with or without augmentation, bladder neck transection with continent urinary diversion, augmentation cystoplasty, or use of injectable bulking agents. Continence was defined as a dry interval greater than 3 hours without leakage at night.
In total, 140 CE and CE variant patients have been managed at the authors' institution. Of the 116 CE patients, 59 received at least one continence procedure, 14 were excluded for incontinent diversion or cystectomy, and the remaining 43 patients are awaiting a continence procedure. At the time of analysis, 42 (71%) patients who underwent a continence procedure were dry. The median number of total urologic procedures to reach urinary continence was 4 (range 2-10). This included 1 bladder closure (range 1-3), 2 urinary continence procedures (range 1-4), and 1 (range 0-4) "other" genitourinary procedures. The median time to urinary continence was 11.0 years (95% CI [9.2-14.2]).
A majority of CE patients who undergo a diversion procedure can achieve urinary continence. However multiple continence procedures are likely necessary. Of patients who are candidates for a continence procedure, half will be continent by the age of 11.
Level IV, Case series with no comparison group.
Journal of pediatric surgery. 2018 Feb 24 [Epub ahead of print]
Mahir Maruf, Matthew Kasprenski, John Jayman, Seth D Goldstein, Karl Benz, Timothy Baumgartner, John P Gearhart
Robert D. Jeffs Division of Pediatric Urology, The James Buchanan Brady Urological Institutions, The Johns Hopkins Hospital, Johns Hopkins Medical Institutions, Charlotte Bloomberg Children's Hospital, Baltimore, MD, USA., Division of General Pediatric Surgery, Department of Surgery, The Johns Hopkins Hospital, Baltimore, MD, USA., Robert D. Jeffs Division of Pediatric Urology, The James Buchanan Brady Urological Institutions, The Johns Hopkins Hospital, Johns Hopkins Medical Institutions, Charlotte Bloomberg Children's Hospital, Baltimore, MD, USA. Electronic address: .