Mullerian duct anomalies (MDAs) are congenital defects of the female genital system that arise from abnormal embryological development of the Mullerian ducts. A didelphys uterus, also known as a "double uterus," is one of the least common amongst the MDAs. Reported here is a case of a 16 year old female with a uterus didelphys with the obstructed left hemiuteri adherent in the anterior abdominal wall, and an endometriotic cyst on the same side. She underwent hysteroscopy-guided vaginoscopy, laparoscopic left hemihysterectomy, left oophorocystectomy. Cases such as these require careful preoperative planning and diagnostic imaging for more accurate diagnosis and, hence, for the most appropriate surgical procedure to be carried out. 3D ultrasonography and Magnetic Resonance Imaging have been the most widely used imaging techniques. The goals of management are to relieve the symptoms of obstruction and to restore the normal anatomy as much as possible in order to provide the best chance for future fertility.
Uterine Didelphys

Genetic errors and teratogenic events during embryonic development can lead to congenital abnormalities of the female reproductive tract. Many patients are asymptomatic, while some have major abnormalities that can cause severe impairment of menstrual and reproductive functions. This case report focuses on Müllerian anomaly Class III, uterine didelphys, along with obstructed hemivagina and ipsilateral renal agenesis (the Herlyn–Werner–Wunderlich syndrome [HWWs]). The patient presented with cyclic pelvic pain due to the hematometra and hematocolpos. She initially underwent resection of the vaginal septum but had a recurrence of obstructive symptoms more than a year after vaginal surgery was performed. This prompted further evaluation with magnetic resonance imaging revealing cystic dilatation of the right uterine horn and a hypoplastic right vagina appearing to end blindly. The patient subsequently underwent hemihysterectomy. The right hemiuterus was noted to have a depression on its thickened inferior aspect, but no cervix was identified. This case is a variant of the classic HWWS, as there was cervical atresia on the right uterine corpus. A thorough preoperative evaluation and accurate intraoperative assessment of patients with Müllerian anomalies can decrease misdiagnoses, guide appropriate intervention, and decrease the risk of future reproductive complications.
Cervical atresia ; uterine didelphys

A six year-old presented with a 10-month history of vaginal discharge. On initial gynecologic evaluation, a vaginal mass was noted. A thorough investigation included pelvic sonogram, KUB sonogram, pelvic examination under anesthesia. A close team-up with a pediatrician and a urologist was established. Intra-operative findings revealed uterine didelphys and a blind hemivagina. This report discusses the approach to the investigation of vaginal discharge in association with a vaginal mass in a 6-year old.
HEMIVAGINA ; MULLERIAN DEFECT ; UTERINE DIDELPHYS

Wunderlich-Herlyn-Werner syndrome is an uncommon but already well-established mullerian anomaly consisting of uterine didelphys, hemivaginal septum and ipsilateral renal agenesis. The discussion here would concentrate on the occurrence of foul-smelling vaginal discharge in some of these cases, its pathogenesis and its impact on generally-accepted assumptions on the embryogenesis of the mullerian system. Reported here are three such cases with discussions on the goals of therapy being relief of the obstructive symptoms, restoration of normal menstrual egress and sexual function, with preservation of reproductive potential.
UTERINE DIDELPHYS ; VAGINAL SEPTUM ; MULLERIAN ANOMALY