Uterus didelphys in a teenage girl: challenges in differential diagnosis

Abstract

Background: The malformations of female genital system are usually associated with abnormal Mullerian duct embryological development. A didelphys uterus, commonly called a “double uterus”; is the consequence of Mullerian duct fusion failure that has a broad spectrum of clinical manifestations. We report a case of a teenage girl presenting with lower abdominal pain after the menarche leading to the diagnosis of rare congenital condition. Case report: A 14-year-old girl presented with severe lower abdomen pain, nausea, and vomiting. Her symptoms have started at a menarche (since 13 years-old) and tend to get worse during irregular menstrual cycles. On the ultrasound examination (US) the uterus was normal in size and structure, pushed to the right side of the pelvis by a unilocular cystic mass 81x80x106 mm with thick irregular walls filled with ground-glass content. Another unilocular cystic formation 64x34x35 mm with incomplete septum and low-echogenicity content was seen above the mass. The latter formation was supposedly the left hydrosalpinx and the bigger mass was differentiated between adnexal pathologies considering the congenital malformation of the uterus (the hematometracolpos and obstructed left hemivagina were suspected). The magnetic resonance imaging confirmed the congenital uterine anomaly: the didelphic uterus with left hematometra due to the blind left upper vagina and left hydrosalpinx was diagnosed. The structure of both ovaries was normal. The patient was admitted for surgical treatment. While the blind vagina ended up near the normally structured right cervix with no contact to the right vagina, it was decided to do the laparoscopic hysterectomy of the left uterus. During the surgery, the left salpingectomy was performed after removal of adhesions. The normal left ovary was left intact. The surgery was continued removing the blood-filled left uterus and cervix, leaving a part of vaginal tissue adherent to the posterior part of the right cervix in the pouch of Douglas. The postoperative period was uneventful. 3 months after the surgery patients has normal menstrual cycles, and the US scan showed normally sized right uterus with bilateral ovaries, with no pelvic masses. Conclusion: With this case report, we wanted to emphasize the challenges of differential diagnosis of pelvic mases in the female adolescents after the menarche. While the benign adnexal pathology is diagnosed in most of the cases, the uncommon congenital uterine anomalies should be taken into consideration.