Prospective Evaluation of Feminizing Genitoplasty Using Partial Urogenital Sinus Mobilization for Congenital Adrenal Hyperplasia

Abstract

We present our experience with partial urogenital sinus mobilization in girls with congenital adrenal hyperplasia, with particular attention to vaginal caliber, introitus position, urinary continence and genital appearance. We present data on a prospective evaluation of 24 girls with congenital adrenal hyperplasia who underwent feminizing genitoplasty using urogenital sinus mobilization with preservation of the pubourethral ligaments during a 4-year period. Urogenital sinus length determined by cystoscopy and degree of external genitalia virilization, defined according to Prader classification, were evaluated before reconstruction. At followup patients were examined while under sedation for evaluation of overall external genitalia cosmesis and calibration of the vagina. Urinary continence status and voiding efficiency were assessed clinically in toilet trained patients by voiding diary, and measurement of bladder capacity and post-void residual by ultrasound. Patient age at operation ranged from 1 to 16 years (median 28.5 months), with a mean followup of 25 months (8 to 47). Degree of virilization was Prader type III in 3 children (12.5%), type IV in 16 (66.7%) and type V in 5 (20.8%). Urogenital sinus length was 2.5 cm or less in 17 children. Cosmetic results were good in 21 patients (87.5%) and satisfactory in 3 (12.5%). The vaginal and urethral openings were separate and identified at the surface of the vestibule in 21 girls. Adequate caliber of the mobilized vagina was achieved in 23 patients (95.8%). None of the 20 toilet trained children had urinary incontinence or recurrent urinary tract infections, with normal bladder emptying in 18 and small post-void residual in 2 (10%). Urinary continence and excellent cosmetic appearance with adequate exteriorization of the vaginal and urethral openings can be achieved in most children with urogenital sinus anomaly treated with partial urogenital sinus mobilization.