Spectrum of Female Hypospadias: A Case Series

Female hypospadias is an extremely rare condition characterised by urethral meatus placed within the introitus on the anterior vaginal wall, along with foreshortening of the urethra. It is an often missed anomaly that usually co-exists with other genitourinary conditions like ectopic ureter, renal anomalies, septate vagina and bicornuate uterus. A case series of five girls who had hypospadias is being described. All the children had an early onset of recurrent episodes of urinary tract infections (UTIs). All of them had voiding dysfunction in the form of dribbling, straining or obstruction to their urinary outflow. On cystoscopic evaluation, all of them had trabeculated bladder, open bladder neck and poorly formed trigone, with the function of at least one kidney compromised on nuclear scintigraphy. Two children in the series had isolated female hypospadias with backpressure changes in the bladder. Two girls had associated anterior ectopic anus, and one girl had a septate vagina with a bicornuate uterus, suggesting abnormal urogenital sinus development. One child had a spinal abnormality, which would have contributed to the neurogenic bladder. Female children with recurrent UTIs and voiding problems should be carefully evaluated for primary urethral and bladder abnormalities. Children diagnosed with hypospadias-related neurogenic bladder have a high risk of progressing to renal failure at an early age and need bladder drainage procedures to prevent the same.