Introduction: Two-stage surgical feminization is a part of the multidisciplinary rehabilitation of girls with external genital virilization. The first stage involves clitoroplasty and labioplasty with preservation of urogenital sinus (US) in girls with third degree of virilization according to Prader classification and higher. US eliminating is performed by second stage in the puberty. Persistence US may cause dysfunction of urodynamics and infections of the lower urinary tract
Aim: To assess the functional state of the lower urinary tract in girls with disorders of sex development (DSD) and US.
Materials and Methods: The study included 35 girls and women from 11 to 32 years old with DSD with US. Most of them have congenital adrenal hyperplasia (32), fewer girls have partial gonadal dysgenesis (1) and idiopathic virilization (2). Patients were examined before second stage surgical feminization in 519 years after the first stage. Concomitant pathology of the urogenital tract was detected in 23 (65%) patients. Urinary tract infection was verified in 17 (48%) base on anamnesis and changes in urine tests. Cystoscopy was performed part of patients with US, but in all cases revealed signs of chronic urinary tract infection presented as follicular, granular cystitis and bladder mucosal metaplasia. Bladder dysfunction was diagnosed in 5 (14%) as stress urinary incontinence, enuresis and hyporeflex bladder base on history, urination rhythm and analysis of questionnaire ICIQ-SF on urinary incontinence influence on quality of life. Trapped menstrual secretions presented as hematometra, hematocolpos, and urine accumulation and stagnation in the vagina in anamnesis or as a result of preoperative studies were diagnosed in 10 (28%). Part of these girls (6) with hematometra/hematocolpos previously were operated yet. One of them was operated twice. Combination of the listed complications were observed in five patients (14%).
Conclusion: Urogenital sinus in girls with DSD, which was observed in all examined patients, is a risk factor for the development of urinary tract infection, hydrocolpos/hydrometra disorders and bladder dysfunction. This circumstance requires change in surgical feminization tactics in girls with disorders of sex development, taking into account the anatomical components of genitalia malformations.
19 - 21 Sep 2019
European Society for Paediatric Endocrinology