(129) Uterus didelphys and unilateral cervical agenesis: challenges of diagnosis and treatment in early adolescence.

Background: Cervical aplasia, subclassified into cervical agenesis and cervical dysgenesis, is a rare congenital anomaly of the reproductive tract caused by abnormal development of the Müllerian ducts. Surgical management includes reconstruction of the cervical canal and restoration of the continuity of the genital tract or hysterectomy. Symptoms of uterus didelphys concomitant with unilateral cervical agenesis occur after menarche and include severe dysmenorrhea due to progressive collection of the menstrual discharge in the obstructed hemiuterus.

Case: We present a case of a 12-year-old girl who presented with dysmenorrhea unresponsive to pain medication five months after menarche. She was previously diagnosed with agenesis of the right kidney. The ultrasound (US) showed uterus didelphys and normal ovaries, hematometra (20cm3) within the right uterine cavity, and another hematoma (6,7cm3) located below, likely in the cervix, to differentiate with hematoma in the obstructed proximal right hemivagina. Menstrual suppression with oral progestin (linestrenol) was recommended. Magnetic resonance imaging (MRI) confirmed the diagnosis of uterus didelphys with right distal cervical agenesis. Though conservative management of cervical agenesis may be related to substantial complications, the patient's mother has not accepted hemi-hysterectomy as a treatment alternative. The patient underwent one-step conservative surgery: opening of the right vaginal apex and the right cervix, creation of neocervical canal, and anastomosis with a vagina, without postoperative dilatation. At consecutive follow-up visits, re-obstruction was not observed, and the patient reported dysmenorrhea responsive to single doses of non-steroidal anti-inflammatory drugs (NSAIDs).

Comments: Reproductive organs should be routinely assessed at puberty in patients with congenital kidney anomalies. Detailed diagnosis of the müllerian anomaly constitutes the first critical step in management. Conservative surgery in patients with uterus didelphys and unilateral distal cervical agenesis can be a safe and effective alternative to hemi-hysterectomy.