To resolve the stricture, the patient was placed under anesthesia. The vaginal segment was firm and 1-cm thick, full of fibrous, muscular connective tissue, but absent of endocervical and ectocervical components, as confirmed pathologically. An index finger was probed into the hole, and the cervical structure could not be reached; however, a longitudinal septum segmented a cavity into 2 parts. Then we switched to hysteroscopy. There was a 3-cm long longitudinal septum separating the normal endometrium cavity into an asymmetric hemiuterus: the right side was narrower than the left part morphologically, and the tubal ostias could be seen. Laparoscopically, the uterus was single with a round ligament attached bilaterally and enlarged, and the fundal contour was a small, smooth indentation. The speculum examination showed the ovaries and fallopian tubes, which were unremarkable, but the structure of the cervical ligament was unclear (Fig. 2B).
Written informed consent was obtained from the patient.
We described a case of a genital anomaly that has not been previously reported in the literature. The classification systems of the American Fertility Society do not include this condition; however, it can be classified by the European Society of Human Reproduction and Embryology and the European Society for Gynaecological Endoscopy system released in 2013 as U2bC4V4.
The embryology of this complicated spectrum of genital malformations is controversial because of the questionable developmental progress. Based on the classic unidirectional theory, the fusion and canalization of Müllerian ducts progresses in a caudad-to-cranial direction, which makes it impossible to explain how the complete septate uterus developed in the absence of the cervix. However, the bidirectional theory[6,7] can explain the combination of a septate uterus, double cervix, and longitudinal vaginal septum. According to this theory, the fusion and canalization of Müllerian ducts start from the isthmus, and then proceeds in both directions separately. Our case might provide additional evidence to support that the fusion of the upper part of the Müllerian ducts can be complete, but subsequent resorption of the median septum does not occur, resulting in formation of a complete septate corpus. Simultaneously, at the lower part of the Müllerian duct, the fusion progress does not occur or anomalous development results in cervical agenesis and upper vaginal atresia.
The literature, without date limitation, was reviewed in terms of vaginal agenesis or vaginal atresia by searching PubMed for the association with functional uterine anomalies (Table 1). Congenital vaginal atresia occurs as an isolated Müllerian anomaly or as a part of a syndrome.[5,8] Vaginal agenesis is not the same entity as distal vaginal atresia. Only 2% to 7% of patients with partial or total vaginal aplasia have an active endometrium in uterine structures.[8,9] However, in the latter, normal uterine and fallopian tubes exist. Sporadic cases of vaginal atresia combined with a functional uterine anomaly have been reported,[11,12] but no concurrence of septate uterus has been reported (Table 1), although the septate uterus composes approximately 53.7% to 55% of all female genital anomalies.[13,14] In conclusion, such a case is significant for clinical management and emerges as a novel anomaly that supports one embryologic concept. It may be necessary to determine the malformation during sterile operation. Also it is of crucial importance for us to observe the patient well into reproductive and obstetric outcomes in future.
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. The American Fertility Society classifications of adnexal adhesions, distal tubal occlusion, tubal occlusion secondary to tubal ligation, tubal pregnancies, mullerian anomalies and intrauterine adhesions. Fertil Steril 1988;49:944–55.
. Grimbizis GF, Gordts S, Di Spiezio Sardo A, et al. The ESHRE/ESGE consensus on the classification of female genital tract congenital anomalies. Hum Reprod (Oxford, England) 2013;28:2032–44.
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. Growdon WB, Laufer MR. Uterine didelphys with duplicated upper vagina and bilateral lower vaginal agenesis: a novel Mullerian anomaly with options for surgical management. Fertil Steril 2008;89:693–8.
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. Gurlek A, Aslan SS, Firat C, et al. Combined cervical and vaginal reconstruction with prefabricated pudendal thigh flap in a case with cervical and vaginal agenesis (MURCS syndrome): a new and original technique. Ann Plast Surg 2008;61:88–93.
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