Management of urinary incontinence in girls with congenital pouch colon.

PURPOSE: This study describes the management of urinary incontinence (UI) in eight girls with congenital pouch colon (CPC) associated with anorectal malformation (ARM).

METHODS

From 2013 to 2015, six girls with CPC and UI underwent bladder neck reconstruction (BNR). Four girls had complete UI (CUI) and two girls partial UI (PUI). From 2019 to 2023, four girls, including two with failed BNR, underwent bladder neck closure (BNC) and augmentation cystoplasty (AC) with a continent stoma. Subtypes of CPC were Complete CPC (n = 7) and Incomplete CPC (n = 1). All girls had a double vagina; short, wide urethra; and reduced bladder capacity with an open, incompetent bladder neck (BNI). During BNR, a neourethra was constructed from a 1.5-2 cm-wide and 1.5-3-cm-long trigonal strip. During BNC, AC was performed using a 20 cm ileal segment (n = 3) and by a colonic pouch segment, preserved during earlier colorraphy (n = 1). Continent stoma included a Monti's channel (n = 3) and appendicovesicostomy (n = 1).

RESULTS

BNR produced moderate improvement of UI (n = 2), while UI was still very severe (n = 4). During BNC, intraoperative complications included iatrogenic vaginal tears (n = 4). Early complications included partial dehiscence of the ileocystoplasty (n = 1), partial adhesive small bowel obstruction (n = 1), and difficulty in stomal catheterization with prolonged drainage from the pelvic drain (n = 1). Late complications included unilateral grade II vesicoureteric reflux (n = 2) and vesicovaginal fistula (VVF) (n = 2) needing trans-vaginal closure in one girl. Urinary stones (n = 2) with stomal leakage of urine in one girl needed open cystolithotomy twice (n = 1), and endoscopic lithotripsy (n = 1). At follow-up, all patients have high overall satisfaction with the procedure and their continence status.

CONCLUSIONS

BNC with AC and a catheterizable stoma satisfactorily achieves continence in girls with CPC and UI, vastly improving quality of life. If lower urinary tract (LUT) anatomy is favorable, BNR with/without AC can be the initial surgical procedure. BNC should be the primary procedure in girls with unfavorable LUT anatomy and for failed BNR.

LEVEL OF EVIDENCE: IV.