ESPE Abstracts

Introduction: The complicated surgical reconstruction of verilized female patients with congenital adrenal hyperplasia (CAH) aims to achieve normal external genital appearance and to maintain normal urogenital and sexual functions.

Objective: To study the urological complications including urinary incontinence, urinary tract infection (UTI) and voiding dysfunction besides assessment of cosmetic outcome after surgical correction using partial urogenital sinus mobilization in female children with classic CAH.

Materials and Methods: A prospective study included 18 female patients with classic CAH due to 21- hydroxylase enzyme deficiency. They were recruited over a period of 3 years. All the patients included in the study had virilized atypical genitalia which were not operated before with Praderʼs score of virilization ranged from III to V. Additionally, they were all toilet trained. All patients were subjected to preoperative assessment of urological condition by urine analysis, urine culture, pediatric urinary incontinence questionnaire, frequency volume charts and ultrasound for residual volume were done. A single team of surgeons performed single stage genital reconstruction using partial urogenital sinus mobilization. Post-operative assessment after three month using the same tools together with uroflowmetry and examination under anesthesia (EUA) were done to assess postoperative urological and cosmetic outcome.

Results: The age of patients at time of surgery ranged from 3 to 15 years, with follow up duration ranged from 25 to 40 months (mean± SD 33.3 ± 5.01). Eight patients were classified as Praderʼs score 3 (44.4 %) and 10 patients as Praderʼs 4 (55.6 %). Cosmetic appearance was assessed after 3 months, wound healing was found good in 14 patients (77.8%), satisfactory in 3 patients (16.7%) and poor in 1 patient (5.6%). One patient missed follow up. Postoperative pediatric urinary incontinence questionnaire and 3-day voiding diary were done and showed no abnormalities in 16 patients (94.1%). Urgency incontinence was found in one patient (5.9%) which resolved after two months. All the patients had negative urinary stress test (100%). Urine analysis and culture were positive for infection in five patients (29.4%) after three month of surgery. Repeated urine cultures were negative except for one patient who had asymptomatic UTI. Ultrasound for assessment of residual urine showed negligent amount of urine in all patients with good bladder emptying (100%).

Conclusion: Surgical reconstruction of female CAH patients with partial urogenital sinus mobilization does not increase the rate of urinary complications in the form of continence and UTI. Additionally, patients had excellent cosmetic outcome.