Congenital uterine malformations result from disruptions in the development, formation, Mullerian fusion, or para-mesonephric ducts during fetal life (1). The incidence of uterus congenital malformations in the general population is 0.001–10% (2). The prevalent form of the structural uterine anomaly was the septate uterus, with the highest reproductive failure rate. A rare type of development is a septate uterus with cervical duplication and a complete longitudinal vaginal sep-tum. This anomaly was first introduced by McBean and Brumsted (3). The existence of this anomaly challenges the classical theory of the Mullerian developmental (4). The traditional embryologic hypothesis is that Mullerian development proceeds in a unidirectional (from caudal to cranial fashion), and as a result, the septum is later absorbed. (5). We report the case of a woman with a septate uterus and cervical duplication and a longitudinal vaginal septum that does not fit into the current assortment system of Mullerian anomalies by the American Fertility Society (AFS).

 

A 26-year-old woman, Gravida 2 Aborted 2, married for 4 years presented with complaints of secondary infertility. In the history taken from the patient, her menarche happened at age 14. She has regular menstrual cycles with intervals of 28 to 31 days and a duration of 4 to 6 days. The patient had no history of dysmenorrhea and dyspareunia. There was no history of pelvic infection disease, abnormal uterine bleeding, and no complaint of excess waste hair growth and secretion from the breast. She has been pregnant spontaneously two times and aborted for less than ten weeks. She had no history of specific medical diseases or surgery. Gynecological examination revealed a longitudinal vaginal septum that extended to the cervix with two separate cervical os that the left cervical os was smaller than the right. Transvaginal ultrasound showed a normal uterine characteristic and the adnexes were normal (Figure 1), then hysterosalpingography was performed. In hysterosalpingography, they were unable to inject contrast media from the left cervix and injection from the right cervix showed that right tube was open (Figure 2). The patient underwent a laparoscopy and hysteroscopy. On laparoscopy, a single fundus of uteri with normal adnexa was observed. endometriotic lesion was evident in the middle part of the right fallopian tube and the left ovarian surface (Figure 3). On hysteroscopy with findings of a complete septate uterus with double cervix and a vertical vaginal septum that the left side was smaller than the right (Figure 4). The depth of the uterine cavity was 8 centimeters. During the laparoscopy, Methylene blue was injected through both cervices respectively. By injecting methylene blue from the right cervix, there was a fall in the right tube, but when injected into the left cervix, there was no drop in the left tube.

Figure 1. Transvaginal ultrasound	Figure 2. Laparoscopy
Figure 3. Hysterosalpingography	Figure 4. Vertical vaginal septum