Double atresic uterus associated with agenesis of cervix and third upper of vagina

Background. Double atresic uterus (DAU) associated with agenesis of cervix and third upper of vagina is an uncommon congenital anomaly of the female genital tract. DAU occurs when the midline fusion of the mullerian ducts is completely arrested, while cervical and proximal vaginal agenesis is due to caudal mullerian aplasia. Aim is to show a rare case of twenty two year old woman with two atresic uteri localized on the right and left iliac fossa, each with unilateral adnexa and without cervix; agenesis of third proximal of the vagina is associated too. Methods. Patient complained of dyspareunia, primary amenorrhea, and reported slight pelvic pain monthly. Following gynecological examination, pelvic ultrasounds, abdominal computed tomography (TC), nuclear magnetic resonance (NMR) and laparoscopy were performed. Results. Gynecological examination showed agenesis of third proximal of the vagina, and two separated uteri without vaginal connection; ultrasounds revealed that both uteri had retrograde menstruation through the fallopian tubes; TC and NMR confirmed and allowed a better definition of genital anomalies. We expected a widespread endometriosis, but laparoscopy excluded it and allowed visualization of two abnormal uteri; a cross-shaped incision on the vaginal apex followed by digital enlargement under laparoscopic check was done. This resulted in lengthening of vagina and disappearing of dyspareunia. Conclusions. This case report involves mainly both quality of life (QOL) and fertility. QOL improves when the patient can ameliorate sex and avoid pain. Provided that the channeling of two separated atresic uteri with an abnormal vagina is practically impossible; fertility may be attempted with assisted fertilization. Such a case of congenital multiple malformations has never been published so far