A rare case of female hypospadias associated with congenital heart disease

Female hypospadias is a rare lower urogenital female anomaly. Hypospadias is primarily a congenital defect of males in which the urethra opens on the underside (ventral aspect) of the penis and is one of the most common congenital anomalies in the USA, occurring in about one of every 125 live male births. The corresponding defect in females is opening of the urethra into the vagina, and is quite rare and often associated with other genitourinary anomalies such as Cloacal malformation, female pseudo hermaphrodism, nonneurogenic neurogenic bladder and urethral duplication. Early diagnosis requires a high index of suspicion. Early treatment plays an important role in reducing the psychological and psychosocial impacts, decreases indolent kidney damages and prevents urinary incontinence. Although, interestingly, sometimes, with conservative management and close follow-up (like in our case), symptoms and signs may get better spontaneously over time. The authors report on a 4-year-old girl with hypospadias presented by hydronephrosis and recurrent urinary tract infection in infancy associated with congenital heart disease who was closely observed until spontaneous resolution of her hypospadias.