Unicornuate uterus and ectopic (undescended) ovary

According to the literature the prevalence of congenital uterine anomalies varies between 3.4 and 5.0% and Müllerian anomalies are found more often in subfertile patients compared to fertile controls (Raga et al., 1997; Grimbizis et al., 2001). Congenital malformations of the Müllerian system are probably caused by multifactorial polygenic and familial factors. The prevalence of a unicornuate uterus is rather low (0.3% of the whole population, 0.6% of the infertile population, 0.2% of the fertile population) (Raga et al., 1997). However, of all Müllerain defects unicornuate uterus is found in 3 to 13% of women (Acién, 1997; Raga et al., 1997; Grimbizis et al., 2001). Unicornuate uterus is caused by a failure of one Müllerian duct to develop (unicornuate uterus without rudimentary horn) or to migrate to its proper location. During the third month of foetal life the developing ovaries descend from a position near the kidneys to their final position in the pelvis, this descent is guided by the gubernaculum (Van Voorhis et al., 2000). The gubernaculum is attached to the uterus forming the utero-ovarian and round ligament. Ectopic or undescended ovaries are characterized by the attachment of the upper pole to an area above the level of the common iliac vessels. Although the term ectopic ovary is commonly used, undescended ovary is probably a more accurate reflection of the underlying pathophysiology. Ovarian maldescent rarely occurs in patients with a normal uterus. On the other hand, the incidence is reported to be 20% when the uterus is absent (Rokitansky-Küstner-Hauser syndrome) and more than 40% in cases of uni-cornuate uterus. (Dabirashrafi et al., 1994 ; Ombelet et al., 2003b). Despite the well-known association of ectopic ovaries and unicornuate uterus, ectopic ovaries are reported only sporadically, suggesting the possibility that many cases go unrecognized We present a case of a unicornuate uterus associated with an ectopic ovary and absent kidney on the contralateral side. A 31-year-old woman was referred to our infertility clinic with a history of primary infertility for more than two years. During the routine infertility exploration a hysterosalpingography (HSG) showed a left sided unicornuate uterus with a patent tube (Fig. 1). On vaginal ultrasound only one ovary could be detected at the normal position, with a normal size and a normal number of antral follicles. On the right hand side no ovary could be visualized. A 3D ultrasound was performed confirming the presence of a unicornuate uterus and …