CURRENT UROLOGIC MANAGEMENT OF CLOACAL EXSTROPHY - EXPERIENCE WITH 11PATIENTS

Purpose: Since 1980 the authors have treated 12 infants with cloacal e xstrophy (10 classical and 2 variants). Eleven patients had repair, an d are all surviving. The initial phases of management that led to impr oved survival have previously been reported. Quality of life is now a major focus for the cloacal exstrophy patient. During the past 10 year s, nine of the 11 patients had tower urinary tract reconstructive proc edures. This review evaluates experience with reconstructive efforts t o achieve bowel and bladder control and to improve the quality of life in this complex group of patients. Methods: 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; incontinenc e 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 contine nt urinary diversion were reviewed. Results: At the time of the author s' previous report, eight of 11 patients had a continence score of 2 o r less. Currently, eight of 11 patients have a scare of 3 or better (f ive with enteric stoma and continent urinary diversion, two with enema program and continent urinary diversion, and one with enema program a nd continent bladder). Urinary diversion procedures have included two gastric augmentations and five gastric reservoirs, two of which have r equired subsequent bowel augmentation. Gastric augmentations carry a d efinite risk of metabolic problems with three of our patients demonstr ating significant episodes of metabolic alkalosis. In addition, result s of urodynamic monitoring suggests that gastric reservoirs may be les s compliant than reservoirs formed using other bowel segments. Conclus ions: Modern principles of continent urinary diversion have been succe ssfully applied to the cloacal exstrophy patient further improving the ir quality of life. Use of gastric flaps with preservation of intestin al length has been central to urologic reconstructive efforts. Use of stomach alone for formation of urinary reservoirs may produce suboptim al compliance, and composite ileogastric construction should be consid ered if the gastric flap is of marginal size. Copyright (C) 1997 by W. B. Saunders Company.