The Davis classification [3] describes 3° of female epispadias; the mildest form being a patulous urethral opening, the intermediate form in which the urethra is dorsally split along most of its length, and the severest form characterized by cleft involving the entire length of the urethra and the sphincteric mechanism with associated incontinence [1, 3]. The examination of the external genitalia may reveal a bifid clitoris, depressed mons covered by a smooth, glabrous area of skin and poorly developed labia minora. The pubic symphysis is usually closed or may be represented by a narrow fibrous band. The vagina and internal genitalia are however usually normal [1, 3].
The incidence of VUR is reported to be between 30 and 75%, and a grade I reflux was observed in our patient. Reflux occurs because the ureterovesical junction is inherently deficient, and the ureter is often laterally placed in the bladder with a straight course [2, 6].
The incontinence seen in female epispadias varies from continuous dribbling without bladder filling, to episodes of daytime stress incontinence. Often, the bladder capacity is reduced and the bladder wall thinned out as a consequence of lack of filling [1, 7]. Our patient had more of daytime stress incontinence, which may account for the normal bladder capacity observed during the radiological evaluation.
The goal of surgery is to achieve continence, preserve the upper tracts, and reconstruct a functional and cosmetically acceptable external genitalia [1]. Various techniques for urogenital reconstruction and continence have been described. These include staged reconstruction, transvaginal plication of the urethra and bladder neck, muscle transplantation, urethral twisting, cauterization of the urethra, bladder flap, Marshall-Marchetti vesicourethral suspension, modified needle suspension, single-stage perineal urethroplasty, and bladder neck plication via a perineal approach [1, 2, 4, 5, 8,9,10,11,12,13]. A single-stage perineal urethroplasty beginning with an inverted “Y” incision was used in this patient, with good outcome [5]. The technique improved the urethral resistance, which in addition to good bladder capacity and intact bladder neck, is an important factor for continence. The continence rate following surgical reconstruction of female epispadias varies from 67 to 87.5% in literature [1, 14].
The delayed presentation in this patient may be due to a combination of the severity of the incontinence, repeated misdiagnosis, and social stigma associated with incontinence. Our patient achieved urinary continence with an overall improvement in her hygiene, self-esteem, and social interaction. The parental satisfaction following surgical intervention was also significant. The absence of facilities for urethrocystoscopy and urodynamic studies is one of the challenges encountered in the care of this patient.
