Congenital pouch colon in girls: Genitourinary abnormalities and their management

Abstract

Aims: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.Materials and Methods: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.Results: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.Conclusions: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.

Congenital pouch colon

“…[2744] Significantly, in males with CPC, where descent of the Müllerian ducts plays no role in the development of the genitourinary tract, the bladder neck is normal.…”

“…However, others may present later with moderate abdominal distension, poorly managed constipation with episodes of enterocolitis, perineal rashes, and often a doughy feeling over the abdomen due to retained feces. [182744] Urinary incontinence may be apparent. [2744] Abdominal X-rays in older girls may show a large soft-tissue shadow of retained fecal matter.…”

“…[182744] Urinary incontinence may be apparent. [2744] Abdominal X-rays in older girls may show a large soft-tissue shadow of retained fecal matter. We believe that examination of the external genitalia, in the clinic/EUA is diagnostic.…”

“…We believe that examination of the external genitalia, in the clinic/EUA is diagnostic. [2744] In doubtful cases, a dye-study through the vestibular fistula, if present, may be diagnostic. [213]…”

Anorectal agenesis with rectovaginal fistula: A rare/regional variant

“…Although finding of a vestibular fistula indicates low anomaly, it may still herald a high anomaly like CPC. [511] Presence of two openings can be due to vestibular anus with vaginal agenesis or RVF with anorectal agenesis. [4] EUA and endoscopy before definitive reconstructive surgery can confirm the diagnosis, and a preoperative magnetic resonance imaging scan may be useful.…”