Experience with isolated female epispadias repair: case report from Northern Tanzania

Female epispadias is a rare congenital anomaly resulting from disrupted mesodermal migration during embryogenesis, leading to anterior urethral displacement, a wide bladder neck, and a bifid clitoris without bladder exstrophy. Diagnosis is clinical, and defects are classified according to the degree of meatal displacement.

The first case involved a 5-month-old infant with a congenital abnormal urethral opening, dorsal urethral cleft, cephalad-displaced meatus, and a bifid clitoris, with normal imaging. She underwent urethroplasty without bladder neck reconstruction and recovered uneventfully. The second case was a 4-year-old girl presenting with an abnormal urethral opening and lifelong urinary incontinence. Examination revealed a dorsal urethral cleft, cephalad-displaced meatus, and a bifid clitoris, with no visible bladder mucosa. She underwent perineal urethrocervicoplasty and achieved continence. Both patients demonstrated good bladder capacity, minimal residual urine, preserved renal function, and satisfactory cosmetic outcomes at 3-6 months of follow-up.

Most girls with isolated epispadias are incontinent and require individualized urethral and bladder neck reconstruction tailored to age and defect severity. Surgical approaches vary, but single-stage perineal urethrocervicoplasty may offer favorable cosmetic outcomes and improved continence compared with traditional techniques such as the Young-Dees procedure.

Early recognition and tailored surgical reconstruction are essential for isolated female epispadias. Age-appropriate staged or single-stage repairs can achieve continence, preserve upper urinary tract function, improve cosmetic appearance, and enhance quality of life. Long-term follow-up is recommended.