Uterus didelphys with cervical agenesis associated with adenomyosis, a leiomyoma and ovarian endometriosis. A case report.

BACKGROUND: Elevated level of serum CA-125 was detected in a 48-year-old woman who was diagnosed with a lateral fusion defect in association with congenital agenesis of the uterine cervix. This unusual case combined two developmental anomalies of the müllerian duct.

CASE REPORT: A 48-year-old woman consulted our outpatient department due to persistent abdominal pain for six months. Bimanual pelvic examination showed absence of the cervix, an anteverted uterus and a 6-cm, left adnexal mass. Ultrasound and computed tomography revealed a uterus didelphys with a 3-cm cystic mass over the right ovary. Serum level of CA-125 was 641.4 U/mL. The patient underwent exploratory laparotomy, and total abdominal hysterectomy with bilateral salpingo-oophorectomy was performed. Pathology confirmed adenomyosis and a leiomyoma of the uterus with functional endometrium in conjunction with endometriosis of the right ovary.

CONCLUSION: Multiple müllerian anomalies associated with adenomyosis and endometriosis should be considered in patients presenting with primary amenorrhea. Thorough evaluation, careful planning, fertility preservation and postoperative outcomes should be reviewed.