Functional endometrial tissue with vaginal agenesis

Vaginal agenesis in association with an intact and functioning or partially functioning uterus is not a well-described problem. The literature advocates attempting to preserve the uterus and surgically create a neovagina. Two patients are presented who suffered severe mobidity after the latter approach. We believe that there are three possibilities with patients who have functional endometrial tissue with vaginal agenesis: namely, an imperforate hymen, a transverse vaginal septum or complete vaginal agenesis with the absence or malformation of the cervix. If an imperforate hymen and transverse vaginal septum are ruled out, we believe it is the best interest of the patient to remove the Müllerian structures during the initial surgery.