Mixed Müllerian and gonadal dysgenesis: A case report

Abstract

Background: Globally, the incidence of cervical agenesis is estimated at 0.01% and constitutes 3% of all Müllerian anomalies.

Case presentation: A 17-year-old nulliparous presented to the gynecology clinic with a transverse vaginal septum and cyclic lower abdominal pain after two failed vaginal surgical procedures to restore genital tract patency. The examination under anesthesia revealed a 3cm short vagina, and a decision was made for vaginoplasty. Magnetic resonance imaging of the pelvis revealed a bulky uterus. Intraoperatively, locating the cervix through the neo-vagina was difficult, and laparotomy was opted for. The left adnexa and cervix were absent, with an atrophic right ovary and a hydrosalpinx on the fallopian tube. Creating a uterovaginal anastomosis was difficult as the uterus was high in the pelvis. Postoperatively, the patient was counseled, and hysterectomy and right oophorectomy were done four months later.

Conclusion: Vaginoplasty with total hysterectomy is an alternative management method in failed uterovaginal anastomosis.