A mullerian anomaly ‘‘without classification’’: Septate uterus with double cervix and longitudinal vaginal septum

Abstract

The incidence of congenital uterine anomalies in the general population is estimated to be 0.001e10% [1]. The most commonly seen anomalies are septate and arcuate uteri, which represent 75% of all mullerian anomalies. The majority of mullerian duct anomalies are considered to be sporadic or multifactorial in nature; however, polygenic and genetic patterns of inheritance have been described [2,3]. There are two theories to explain the development of mullerian duct anomalies, the classical unidirectional regression theory, and an alternative bidirectional theory in which it is hypothesized that the process proceeds simultaneously in both the cranial and the caudal directions. Anomalies such as a complete septum with a duplicated cervix or an isolated vertical upper vaginal septum could only be explained by the second theory [4,5]. We report the case of a woman with a complete uterine septum, and a double cervix with a longitudinal vaginal septum, and search the available literature for similar cases. A 28-year-old nulligravida woman was admitted to our hospital due to being unable to conceive. She had been married for 2 years and had never used any contraceptive methods. She had regular menses but had dysmenorrhea and sometimes also dyspareunia. A longitudinal vaginal septum from the midportion of the vagina to the cervix, and the presence of two separate cervixes were identified following a speculum examination. Transvaginal ultrasound showed a normal uterine contour, then hysterosalpingography was performed. When a contrast media was injected into the right cervix, the uterine cavity was filled, however, the fallopian tubes were not. When the contrast media was then injected into the left cervix and uterine cavity, the left fallopian tube and pelvic cavity filled with contrast media, but the right fallopian tube was not filled with contrast media. Following