1. Introduction

1. INTRODUCTION

Vulvovaginitis is defined as an inflammation of the vagina and the vulva. It is a common problem observed in girls within the pediatric and adolescent age groups. Depending on the age of the patient, the cause, pathogenesis, and treatment vary. Symptoms include abnormal vaginal discharge, itching, odor, discomfort, burning sensations, and dyspareunia. Some patients also complain of genitourinary symptoms, such as urinary frequency and urgency. Depending on the causes, there are infectious and non-infectious cases of vulvovaginitis observed in adolescent girls. Infectious causes of vulvovaginitis are more common and include bacterial vaginosis, vulvovaginal candidiasis, and Trichomonas vaginalis(1). Bacterial vaginosis is the most common cause in the infectious group. Approximately 23% adolescent girls, between the ages of 14 and 19 years, experience bacterial vaginosis(2). Although the frequency of non-infectious vulvovaginitis is less common than that of the infectious group, the causes of non-infectious vulvovaginitis are also considered important. These include vulvar inflammation and irritation caused by various chemical irritants, retained foreign bodies, trauma, poor hygiene, and Müllerian anomalies associated with partial outflow tract obstruction. To determine the causes of vulvovaginitis, a detailed medical history should include the age of the patient, last menstrual period, recent antibiotic use, and tampon use. Sexual history must be recorded to accurately diagnose STIs (sexually transmitted infections) and sexual abuse.

We present a rare case of a 16-year-old adolescent girl diagnosed with obstructed hemivagina and ipsilateral renal anomalies (OHVIRA) syndrome due to persistent symptoms of recurrent vulvovaginitis over the course of one year.

2. Case Report

2. CASE REPORT

A 16-year-old, sexually inactive girl visited the gynecological department with symptoms of persistent purulent vaginal discharge and recurrent vulvovaginitis that persisted over a year. The vaginal discharge was yellowish and had a foul odor. Although the girl was receiving treatment at other hospitals, it was ineffective and the symptoms continued to recur. Menarche occurred in the girl at the age of 12 years with a regular menstrual cycle of 30 days, and there was no dysmenorrhea that required medication. The last menstrual period occurred a week prior to the hospital visit. The young girl had no previous medical and surgical history. During the physical examination, the secondary sexual characters were in the normal range with Tanner stage III for breast development and pubic hair. The vulva was hyperemic and excoriated due to inflammation, and we observed yellowish discharge during the gynecological examination. To examine vaginal discharge and vulvar inflammation, we performed routine cultures and a test for 12 types of sexually transmitted diseases (STDs). The results were positive for Escherichia coli (E. coli) infection. The young girl was treated for one week with antibiotics specific to E. coli. An imperforated hymen excluded the possibility of penetration of the hymen. A transrectal ultrasound showed mixed echoic lesion below the uterus and no intrauterine fluid collection (Fig. 1). Magnetic resonance imaging (MRI) showed two separate uteruses and two separate cervixes (Fig. 2(A) and Fig. 2(B)). The upper vagina was distended and filled with T2 heterogeneous hyperintense content (Fig. 2(C) and Fig.2(D)). Uterine didelphys with upper transverse vaginal septum was diagnosed in the sagittal view (Fig. 2(E)). The proximal and midportion of the vagina were normal in appearance. When we performed pelvic-enhanced computed tomography (pelvic-ECT) to investigate other associated malformations, we diagnosed left ovarian agenesis (Fig. 3). The young girl was diagnosed with OHVIRA syndrome.

After the patient was placed in the lithotomy position under general anesthesia, hysteroscopy was used to penetrate the vagina and to identify the location of the septum. After blocking the vaginal opening with both hands so that the hysteroscopic media could be filled into the vagina, a 5-mm-office hysteroscopy was introduced into the vagina. A hysteroscopy was pressed on the suspected area of the septum to confirm the location of the septum, since the hole of the septum was not visible (Fig. 4). We identified the location of the septum by checking the location where pus-like discharge drained out. Although we performed hysteroscopic septoplasty, it failed. After a small sized speculum was used to dilate, the septum was targeted using an elliptical forcep, and we performed wide excision of the septum. We identified the left side of the uterine cervix under the septum. Vicryl 3-0 was used for circular suturing to prevent re-adhesion and bleeding.

The patient recovered well without any complications or discomfort and was discharged on the third day following surgery. The patient displayed good post-surgical recovery. There was no evidence of recurrence and stricture formation in a pelvic-MRI performed one year following the procedure (Fig. 5). In the year following the surgery, the patient did not experience any symptoms of vulvovaginitis.

3. Discussion

3. DISCUSSION

Obstructed hemivagina and ipsilateral renal anomalies (OHVIRA) syndrome, known as Herlyn-Werner-Wunderlich (HWW) syndrome, was first described by Purslow in 1922 and is a rare disorder with an estimated incidence of 0.1%–3.8%(3). It is composed of a triad of double uterus (didelphys, bicorcuate, or septate uterus), obstructed hemivagina, and ipsilateral renal anomalies (renal agenesis/anomaly). These malformations are a result of an anomalous development of Müllerian ducts around the eighth week of gestation. At 4 to 6 weeks of gestation, the fetal urogenital system begins to develop. At this stage, both male and female embryos possess Wolffian ducts and Müllerian ducts. Between 6 and 9 weeks of gestation, the Müllerian ducts develop while the Wolffian ducts regress under the influence of estrogen and in the absence of testosterone and the anti-Müllerian hormone (AMH) in female embryos. The Müllerian ducts initially form separately on each side of the uterus and gradually move toward the midline, where they fuse. By 9 to 12 weeks of gestation, the fused portion forms the uterovaginal canal that subsequently gives rise to the uterus, uterine cervix, and the upper two-third section of the vagina. Following fusion, the septum formed at the fusion line should disappear to establish a normal uterine cavity. It is believed that any disruption in this process can lead to the development of Müllerian anomalies. In the OHVIRA syndrome, failure in the fusion stage leads to the formation of uterine didelphys, and failure in the regression stage results in the development of a vaginal septum.

The clinical presentations of the OHVIRA syndrome are diverse on the basis of anatomical abnormalities and the timing of onset. Patients could present with various symptoms, such as dysmenorrhea, abdominal or pelvic pain, pelvic masses, and menstrual irregularities. Urological symptoms, such as urinary retention or incontinence may also occur. Among these, abdominal pain and dysmenorrhea were the most common symptoms(4). Although it can present with a variety of these symptoms, many cases are asymptomatic. It is rare for a patient to be diagnosed with recurrent vulvovaginitis, as in our case study. The cause of these symptoms can be attributed to the gradual accumulation of menstrual fluid caused by the vaginal septum, leading to the formation of hematocolpos that became infected and formed pyocolpos. This pus-like discharge then leaked through the opening of the penetration. The appearance of these various symptoms is mainly determined by the location of the vaginal septum and the fenestration of the septum. Transverse vaginal septum is reported in approximately 1 in 2100 and 1 in 72 000 females(5). Vaginal septums are located in 6% to 46% of the upper vaginal area, 22% to 40% of the middle section, and 15% to 72% of the lower vagina(6). The septa are generally less than 1 centimeter in thickness and may have a small central or eccentric perforation.

Although this syndrome is rarely diagnosed during the prenatal period, it is generally diagnosed after menarche, and the average age at diagnosis is 14. In such cases, the importance of early diagnosis is emphasized. A delayed diagnosis due to various reasons can lead to complications, such as pelvic inflammatory disease and endometriosis, which can affect fertility and the quality of life. Furthermore, it is necessary to conduct an early evaluation of other conditions because they can also be present. In addition to urogenital anomalies, such as ipsilateral renal agenesis, ipsilateral renal dysplastic/multicystic kidney, and compensatory contralateral kidney hypertrophy, concomitant malformations have been reported in 15.1% of cases in the skeletal system and 0.4% in the cardiovascular system(7).

Ultrasound and MRI are used to diagnose OHVIRA syndrome. Ultrasound is the most commonly employed imaging method because it is simple and easy to perform. The most frequent diagnostic findings are hematometra and hematocolpos. Vulvovaginitis may also present as conditions, such as hematosalpinx/piosalpinge, tubo-ovarian abscess, and pyometra. MRI is considered the gold standard to diagnose this condition and is also known to have advantages in diagnosing various Müllerian anomalies and the continuity of the vagina.

There are various treatment options, including vaginal septum incision, vaginal septum resection, hysteroscopy resection, laparoscopy, and laparotomy. The most important of these treatments is the removal of the vaginal septum. In this process, it is important to remove the entire vaginal septum to form a permanent opening for the menstrual flow. The widely used hysteroscopic incision of the vaginal septum was recently introduced(8). This vaginoscopic surgery may be a good option for adolescent girls with a narrow and immature vagina. The location and the thickness of the vaginal septum are important factors that can affect the surgical treatment method. In cases where the thickness of the vaginal septum is more than 2 cm, the abdominoperineal approach methods are recommended because it is difficult to completely remove the septum and this can create a defect between the proximal and the distal vagina following the operation(6). Laparoscopy was not routinely applied for diagnosis and treatment. A hemihysterectomy using laparoscopy or laparotomy may be necessary in cases with an extrinsic proximal vaginal septum or a serious infectious complication.

4. Conclusions

4. Conclusions

In the OHVIRA syndrome, it is crucial to diagnose and treat girls at an early age. A delayed diagnosis can lead to various complications that could impact the quality of life and fertility. It is rare for adolescent girls to present with recurrent vulvovaginitis, as observed in our case. Müllerian anomalies should be considered in cases of recurrent vulvovaginitis in adolescent girls that are unresponsive to conventional treatment.