Uterus didelphys and vaginal duplication with unilateral obstruction presenting as a newborn abdominal mass

Vaginal duplication with uterus didelphys is a rare anomaly in which unilateral obstruction of either vaginal orifice may result in hydrocolpos but with normal-appearing external genitalia in the female newborn. We recently treated 2 female infants with this anomaly who presented with a lower abdominal mass that persisted after bladder drainage. Both infants had evidence of urological abnormalities on the side ipsilateral to the obstructed vagina. One infant had renal agenesis and 1 had severe renal dysplasia. Owing to the high incidence of associated renal abnormalities excretory urography, voiding cystourethrography and abdominal ultrasound are essential to the evaluation. Precise diagnosis and careful evaluation are mandatory to avoid unnecessary loss of essential normal müllerian structures.