Diagnosis of congenital oblique vaginal septum syndrome: A case report and literature review.

The authors obtained written informed consent from the patient for the publication of this case report.

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GQ G: Data curation, Writing – Original draft preparation. YN J: Writing – Review and Editing. Mengling Li and Xin Hang: Investigation. All authors have read and approved the final manuscript.

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Yina Jiang.

Clinically, for adolescents presenting with cyclic lower abdominal pain, ultrasound examination is crucial for identifying the potential etiology of symptoms . Prompt diagnosis and intervention are crucial to address the underlying condition, which may include disorders such as vaginal oblique septum syndrome (OVSS), among others. The timely use of diagnostic tools, such as ultrasound, can facilitate early identification and subsequent management, potentially preventing the onset of complications and to alleviating existing symptoms.

Congenital vaginal oblique septum syndrome (OVSS) is a known anomaly of the reproductive tract, widely believed to arise from abnormal development of the paramesonephric ducts [ 5 ]. The clinical presentation of OVSS varies with the type of septum, the extent of obstruction, uterine development, and the patient's age. Symptoms typically emerge following menarche during adolescence and may include dysmenorrhea, pelvic pain, vaginal discharge, purulent discharge, and vaginal wall masses [ 6 ]. In 2022, the Obstetrics and Gynecology Branch of the Chinese Medical Association classified OVSS into four types ( Fig. 2 ): Type I (Imperforate Oblique Septum): The lower segment of one vagina is occluded, with a channel between the two uteruses and vaginas, leading to blood accumulation in the upper segment of the vagina and the uterine cavities on both sides of the septum. Type II (Perforated Oblique Septum): The lower segment of one vagina is occluded, but the septum contains a small hole several millimeters in diameter. The uterus behind the septum is isolated from the opposite side, with partial blood accumulation in the upper segment of the vagina and uterine cavity that can flow out through the small hole, albeit inefficiently. Type III (Imperforate Oblique Septum with Cervical Fistula): The lower segment of one vagina is completely occluded, with a small fistula between the two cervical canals or between the post-septal vaginal cavity and the opposite cervical canal, allowing blood to be discharged from the septal side through the fistula, but again, the discharge is not smooth. Type IV (Cervical Occlusion): The cervix on the obstructed side is underdeveloped, and the post-septal vaginal cavity below is narrow, without blood accumulation, and may also be absent. Clinically, Types II and III are more common. OVSS is often not diagnosed until menarche, with Type I patients predominantly presenting with dysmenorrhea, which is typically progressive and severe. Patients with Types II and III may experience poor menstrual blood discharge, prolonged menstrual cycles, continuous spotting, or vaginal discharge, primarily purulent ( Fig. 3 ). Fig. 3 The different types of Oblique Vaginal Septum Syndrome. Fig. 3 The different types of Oblique Vaginal Septum Syndrome. This case report concerns a patient with Type I OVSS, primarily exhibiting progressive dysmenorrhea, which aligns with the findings of Zhu et al. [ 7 ]. The patients with complete vaginal obstruction present with characteristic clinical symptoms at a significantly younger age compared to those with incomplete obstruction. Patients with OVSS who have one patent vagina are capable of normal pregnancies, and in cases of Type II or III septum, pregnancy can occur in both uterine cavities. Cozzolino and Albulescuhave each reported favorable pregnancy outcomes in cases of women with a double uterus [ 8 , 9 ]. Candiani et al. conducted a long-term follow-up of reproductive performance in 51 OVSS cases, totaling 22 pregnancies, of which 36 % (8/22) resulted in full-term deliveries, indicating a generally positive pregnancy experience [ 10 ]. Surgical correction is considered the optimal therapeutic approach for addressing congenital vaginal oblique septum syndrome [ 11 ].Surgical correction of the vaginal oblique septum should be performed promptly following diagnosis to alleviate symptoms quickly, prevent long-term complications related to retrograde menstruation, such as hematocolpos, infectious pyometra, endometriosis, and pelvic adhesions, and to protect the patient's fertility and reduce the risk of reproductive tract infections. Therefore, early diagnosis and treatment are of utmost importance. Ultrasound examination is a simple, radiation-free, cost-effective, highly reproducible, and accurate diagnostic method, making it the preferred and reliable choice for diagnosing OVSS. However, the diverse clinical manifestations of this congenital anomaly can lead to delayed diagnosis and treatment if there is a lack of awareness.

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The occurrence of Oblique Vaginal Septum Syndrome (OVSS), a rare anomaly of the urogenital tract, is estimated between 0.1 % and 3.8 % of cases [ 1 ]. This congenital condition encompasses a double uterus (occasionally fully septate), double cervix, and an oblique vaginal septum. It commonly coexists with urinary system anomalies on the same side as the septum, with ipsilateral renal agenesis being the most prevalent [ 2 , 3 ]. The syndrome involves a vaginal membrane originating between the two cervical canals, obliquely adhering to one side of the vaginal wall. This membrane obstructs the vaginal canal on that side, creating a post-septal vaginal cavity between the septum and the cervix. This case report explores the application of ultrasound in diagnosing and surgically treating OVSS. We received the informed consent to publish this case report from the patient's parents. The case report was in line with the SCARE criteria [ 4 ].

An 11-year-old patient, experiencing her initial menstrual cycle five months prior with moderate flow and mild menstrual discomfort, noted a progressive increase in lower abdominal pain coinciding with her menstrual periods. Her most recent menstrual period was accompanied by severe lower abdominal pain. Laboratory tests revealed normal sex hormone levels and no evidence of malignancy. Color Doppler ultrasound imaging depicted a normal-sized uterus with a widened transverse diameter at the fundus. Two endometrial lines were visible in each uterine cavity, with a hypoechoic structure traversing the cavity and reaching the internal os of the cervix. A 9.7 cm × 6.0 cm anechoic area was identified in the lower segment of the left cervix and vagina, suggestive of hematocolpos ( Fig. 1 ). Renal ultrasound demonstrated normal-sized kidneys without any abnormal findings in the bilateral adnexa. Gynecological examination revealed blood stains on the external genitalia and slight swelling on the left side of the vaginal opening. A palpable mass, approximately 10 cm in diameter with limited mobility, was detected in the vagina and pelvic cavity during rectal examination. Hysteroscopic examination showed a tense bulge in the left vagina and visibility of the right cervix and cervical os. Incision of the septum at the upper end of the right cervix resulted in the expulsion of a large amount of dark red, non-coagulating blood. Ultrasound guidance confirmed a significant reduction in the mass within the left uterine cavity. The left uterine cavity exhibited a nearly normal shape, and the left fallopian tube orifice was clearly visible. The intraoperative diagnosis was confirmed as Type I vaginal oblique septum syndrome (imperforate type). One week following discharge, a follow-up color Doppler ultrasound indicated a uterine anomaly, suggestive of a complete septate uterus ( Fig. 2 ). Fig. 1 Blood accumulation in the left cervical canal extending into the vagina. Fig. 1 Fig. 2 Accumulation of blood in the right uterine body and the left vagina. Fig. 2 Blood accumulation in the left cervical canal extending into the vagina. Accumulation of blood in the right uterine body and the left vagina.

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