Urinary continence outcomes following vaginoplasty in patients with congenital adrenal hyperplasia

Abstract

Background: Feminizing genitoplasty techniques have diversified to encompass the spectrum of atypical genitalia in 46,XX congenital adrenal hyperplasia (CAH) patients. However, long-term outcomes evaluating postoperative continence following complex vaginoplasty remain scarce.

Objective: The aim was to review our surgical experience and assess postoperative urinary continence outcomes in CAH patients following complex, primary vaginoplasty.

Study design: We retrospectively reviewed CAH patients who underwent complex, primary vaginoplasty at a single center by a single surgeon from 1996 to 2013. We collected data on clinical characteristics, surgical techniques and postoperative urinary continence outcomes. In patients who had reached toilet-training age, continence outcomes were reviewed.

Results: Twenty-four patients met the inclusion criteria. The median age at the time of surgery was 8 months (interquartile range [IQR] 1 year-6 months). The median length of follow up was 6 years (IQR 10 years-3 years). These patients required complex reconstruction because of more extensive virilization: total urogenital mobilization (TUM, 10), pull-through vaginoplasty (9), partial urogenital mobilization (3), or pull-through vaginoplasty with partial urogenital mobilization (2). After surgery, 15 out of 24 (62.5%) reported age appropriate toilet-training without further complaints. Seven (29.2%) patients reported daytime incontinence or enuresis that resolved with conservative management. Two patients reported day and night incontinence requiring urethral bulking procedures following TUM.

Discussion: Twenty-two (91.7%) of our patients achieved continence without further surgical intervention. Of these patients, the TUM patients had serious incontinence issues. Our study represents a review of clinically significant cases from the female CAH population. The limitations of our study include a relatively small sample size and retrospective design.

Conclusion: Females with CAH and urogenital virilization most often achieved age appropriate toilet-training following vaginoplasty. Daytime incontinence and enuresis resolved without operative management in the vast majority of cases. TUM carries a risk of severe incontinence and may require further procedures to achieve urinary continence.

Keywords: Congenital adrenal hyperplasia; Feminizing genitoplasty; Voiding dysfunction.