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Current urologic management of cloacal exstrophy: experience with 11 patients
E A Smith1, J R Woodard, B H Broecker
1Department of Surgery, Emory University School of Medicine, Egleston Children's Hospital, Atlanta, GA, USA.
Insights
Reconstructive surgery significantly improved bowel and bladder control in cloacal exstrophy patients, enhancing their quality of life. Modern continent urinary diversion techniques are key to these positive outcomes.
Area of Science:
- Pediatric Surgery
- Urology
- Reconstructive Surgery
Background:
- Cloacal exstrophy is a complex congenital anomaly requiring multidisciplinary management.
- Historically, survival has been the primary focus, but quality of life is now paramount.
- Reconstructive efforts aim to achieve bowel and bladder control in these patients.
Purpose of the Study:
- To evaluate the effectiveness of reconstructive procedures in improving bowel and bladder control.
- To assess the impact of these procedures on the quality of life for patients with cloacal exstrophy.
- To review surgical complications and sequelae of continent urinary diversion.
Main Methods:
- Review of patient charts and interviews to assess urinary and bowel control.
- Application of a standardized continence score (0-6) to measure success.
- Analysis of surgical complications, urodynamic data, and metabolic sequelae.
Main Results:
- Eight of 11 patients improved their continence score to 3 or better.
- Successful continent urinary diversion was achieved in multiple patients using various techniques.
- Gastric augmentations carried a risk of metabolic alkalosis, and gastric reservoirs showed potential compliance issues.
Conclusions:
- Modern continent urinary diversion principles have improved quality of life for cloacal exstrophy patients.
- Gastric flaps and preservation of intestinal length are crucial for urologic reconstruction.
- Composite ileogastric construction may be necessary for optimal urinary reservoir compliance.
Purpose:
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.
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; 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.
Results:
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 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.
Conclusions:
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.