Single-stage repair for female epispadias with urinary incontinence: A case report and literature review

BACKGROUND: Epispadias is a rare condition. Epispadias in females is two times less common than in males. Female epispadias range from 1 in 160,000 to 480,000 live births. Epispadias can be diagnosed through careful physical examination of the genital. Surgery is the management of epispadias. Surgical management of epispadias is quite tricky and requires expertise. The literature that discusses female epispadias is challenging to be found. In this paper, we would like to report surgical management of isolated female epispadias in Cipto Mangunkusumo Hospital, Jakarta. CASE PRESENTATION: A 7-year-old girl was admitted with a chief complaint of urinary incontinence since birth, during daytime and nighttime. Urinary incontinence was not induced by activities nor worsened by eating/drinking. Physical examination showed that the patient's external genitalia has underdeveloped labia minora, patulous urethra, bifid clitoris, and multiple hypopigmentation lesions. Laboratory results were in the normal range. The voiding cystourethrography (VCU) result revealed urine leaks during the filling phase. The bladder wall was normal, and no vesicoureteral reflux (VUR) appeared. The urethrocystoscopy shows a more vertical OUE, a wide-open bladder neck, and a urethral length of 1.5 cm. The patient underwent single-stage surgical procedures that consist of epispadias repair and bladder neck reconstruction through a subsymphyseal perineal approach. No complications occurred intra-operative and post-operative. At the 1-week and 6-months follow-up, the patient achieved urinary continence, and the surgical wound healed normally. DISCUSSION: Epispadias is a rare condition that could occur in various degrees, from mild to severe degrees. To a severe degree, there is a split at the entire urethral and involves the bladder neck causing constant incontinence for the patient. Epispadias cases are quite challenging to diagnose. The physician needs to separate the labia majora and perform the physical examination carefully. The objective goals of surgical management of epispadias are to achieve urinary continence, restoration of anatomy, function, and cosmetic appearance of urethra and genitalia. Single-stage reconstruction is the current surgical method used to repair isolated female epispadias. CONCLUSION: Females epispadias is a rare congenital anomaly that is quite often to miss diagnosed. The single-stage technique is the recommended surgical technique for isolated female epispadias. This patient has achieved urinary continence, and no complications occurred during and post-surgery.