Urinary continence outcomes following vaginoplasty in patients with congenital adrenal hyperplasia

Summary 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. Table . Comparison of vaginoplasty techniques according to presurgical measurements of urogenital sinus and urethra, and postoperative urinary continence outcomes. Vaginoplasty Technique No. Urogenital Sinus Length (cm) Mean ± SD Urethral Length (cm) Mean ± SD Continence Enuresis Voiding Postponement Dysfunctional Voiding Continuous Incontinence Total Urogenital Mobilization 10 2.1 ± 0.6 1.7 ± 0.4 4 2 1 1 2 Pull-through 9 2.7 ± 1.0 1.4 ± 0.7 7 1 1 0 0 Partial Urogenital Mobilization 3 2 ± 0 2.1 ± 0.1 3 0 0 0 0 Pull-through with Partial Urogenital Mobilization 2 2.1 ± 1.0 1.6 ± 0 1 1 0 0 0