Current Urologic Management of Cloacal Exstrophy
Since 1980 the authors have treated 12 infants with cloacal exstrophy (10 classical and 2 variants). Eleven patients had repair, and are all surviving. The initial phases of management that led to improved survival have previously been reported. Quality of life is now a major focus for the cloacal exstrophy patient. During the past 10 years, nine of the 11 patients had lower urinary tract reconstructive procedures. This review evaluates experience with reconstructive efforts to achieve bowel and bladder control and to improve the quality of life in this complex group of patients.
Through review of patient charts and by patient interviews, data were collected to evaluate the ability to provide urinary and bowel control. A continence score was applied to provide a measure of success: voluntary control, 3; control with an enema program or intermittent catheterization, 2; incontinence with a well-functioning stoma, 1; and incontinence without a stoma, 0. The best continence score is 6 (genitourinary and gastrointestinal). Surgical complications, urodynamic and metabolic sequelae of continent urinary diversion were reviewed.
At the time of the authors’ previous report, eight of 11 patients had a continence score of 2 or less. Currently, eight of 11 patients have a score of 3 or better (five with enteric stoma and continent urinary diversion, two with enema program and continent urinary diversion, and one with enema program and continent bladder). Urinary diversion procedures have included two gastric augmentations and five gastric reservoirs, two of which have a required subsequent bowel augmentation. Gastric augmentations carry a definite risk of metabolic problems with three of our patients demonstrating significant episodes of metabolic alkalosis. In addition, results of urodynamic monitoring suggests that gastric reservoirs may be less compliant than reservoirs formed using other bowel segments.
Modern principles of continent urinary diversion have been successfully applied to the cloacal exstrophy patient further improving their quality of life. Use of gastric flaps with preservation of intestinal length has been central to urologic reconstructive efforts. Use of stomach alone for formation of urinary reservoirs may produce suboptimal compliance, and composite ileogastric construction should be considered if the gastric flap is of marginal size.
Considerable progress has been made in treating the patient with cloacal exstrophy since the first report of a child surviving surgical correction was made in 1960.1 The foundation of this success has rested on advances in neonatal medicine, intensive nutritional support, and use of staged reconstructive efforts based on a greater understanding of the anomaly.2–4 With survival rates approaching 90%,5 reports describing treatment of the cloacal exstrophy patient have now shifted from a focus on improving survival to improving quality of life. We previously reported our results with a series of 11 patients who had completed the initial phases of reconstruction with definitive treatment of the gastrointestinal tract.6 The expectation at that time was to reconstruct, when appropriate, the lower urinary tract to achieve continence. Although functional bladder closure may be achieved in the cloacal exstrophy population,7 the majority of patients will require bladder augmentation to improve capacity and compliance and construction of a catheterization continence mechanism.8 Furthermore, in those patients in whom the bladder is too rudimentary to be of reconstructive value or in whom the bladder is committed to vaginal reconstruction, a continent urinary reservoir must be constructed. The stomach has been described as the ideal graft source in these patients because they have limited enteric length. Augmentation gastrocystoplasty and gastric reservoir construction have provided a method of achieving urinary continence while preserving the intestinal length that is important to fluid and electrolyte balance and to nutrition.9 We have reevaluated our patient series after lower urinary tract reconstruction.
KeywordsIntermittent Catheterization Urinary Continence Imperforate Anus Continence Score Continent Urinary Diversion
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