Congenital pouch colon in girls: Genitourinary abnormalities and their management

To discuss the assessment and management of genitourinary (GU) tract abnormalities in 21 girls with Types I-III congenital pouch colon (CPC), studied over a period of 10 years.

Assessment included clinical and radiological assessment, examination under anesthesia (EUA), endoscopy of the lower GU tract, and evaluation of the surgical findings, operative procedures for the GU anomalies, and the results of management.

Initial examination of the external genitalia showed a "clover-leaf" appearance (n = 6) and a single perineal opening (n = 6). In 9 patients, the openings of the urethra and double vagina were seen, of which a vestibular fistula was seen in 5 and an anterior perineal fistula in 1. Seventeen patients (81%) had urinary incontinence (UI) - partial in 10, and complete in 7. Renal function tests, X-ray sacrum, and abdominal US were normal in all patients. Micturating cystourethrogram (n = 9) showed a wide, bladder neck incompetence (BNI) with reduced bladder capacity in seven patients. EUA and endoscopy revealed a septate vagina in all patients and the urethral opening at a "high" position (n = 14) or at a relatively normal or "low" position (n = 7). In 8 patients, the intervaginal septum was thick and fleshy. Endoscopy showed a short, wide urethra, an open incompetent bladder neck, poorly developed trigone, and reduced bladder capacity in the patients with UI. The fistula from the colonic pouch opened in the proximal urethra (n = 4), high in the vestibule (n = 3), low in the vestibule (n = 8), perineum just posterior to the vestibule (n = 1), and undetermined (n = 5). Vaginoscopy (n = 8) showed normal cervices in all and cervical mucus in 4 patients. The subtypes of CPC were Type I CPC (n = 4), Type II CPC (n = 16), and Type III CPC (n = 1). All 21 patients had uterus didelphys. In four patients with UI, during tubular colorraphy, a segment of the colonic pouch was preserved for later bladder augmentation if required. A Young-Dees bladder-neck repair (BNR) was performed in four older girls for treatment of UI, with marked improvement in urinary continence in two girls, some improvement in one girl with complete urinary incontinence, and minimal improvement in one child. Division of the intervaginal septum was performed in three girls.

GU abnormalities in girls with CPC need to be assessed and managed by a tailored protocol. UI is frequent, and its correction may require BNR. A segment of the colonic pouch can be preserved for possible future bladder augmentation. All girls have a septate vagina, often widely separated, and uterus didelphys. Gynecologic assessment and monitoring is required throughout adult life. Considering the wide opening of the vestibule, surgical management of the urogenital component by division of the intervaginal septum and if required, the vagino-fistula septum on each side results in a normal or a hypospadiac urethral opening and an adequate vaginal inlet.