Introduction: Uterine didelphys is an uncommon Mullerian anomaly that often is associated with obstructive pathology. Without early intervention, obstruction can progress to potentially fatal complications including pyometra and sepsis. Due to the rarity and variability of this condition, treatment options have not been systematically studied.

Case Description: In this case report, we discuss a 16-year-old female who presented with acute sepsis secondary to pyometra in the setting of chronic dysmenorrhea and purulent vaginal discharge. On imaging, she was found to have uterus didelphys with right renal agenesis and a hypoplastic right cervix along with a pelvic fluid collection. During exam under anesthesia, no obstructing hemivagina was found, however the right cervical os was identified with purulent material draining from it and identified as the site of obstruction. The right cervical os was extended and its edges marsupialized. The patient recovered well postoperatively with a course of antibiotics and continued to be followed in an outpatient setting, where she was started on Depo Provera for menstrual suppression. She remained asymptomatic for months, but after discontinuing the Depo for some time, began to experience pelvic pain and purulent vaginal discharge again. While she agreed to resume Depo treatment, definitive treatment with right hemihysterectomy was discussed and eventually undertaken.

Discussion: Although hemihysterectomy may be required in many cases, the technique of marsupialization of a cervical os is unique and may be a new approach to patients with non-classic anatomic presentations of obstructive symptoms with uterine didelphys.