A Case of Congenital Imperforate Anus and Absent Vagina with a Functioning Uterus

Vaginoplasty and anoplasty were undertaken in a case (aged 8 years) of imperforate anus and perineal fistula with absence of the vagina by making use of the fistula and rectum and by the pull-through procedure, respectively. The surgical procedures employed are considered to be most appropriate for correction of these deformities. Three years later, however, the patient developed hematometra and hematosalpinx due to a functional uterus present. She underwent hysterectomy combined with left salpingo-oophorectomy. If normal uterus is noted to be present upon laparotomy, it seems important to establish spatial communication between the stump of the vagina constructed and the corpus uteri, to prevent future development of hematometra.