A rare case of OHVIRA (obstructed hemivagina and ipsilateral renal anomaly) syndrome: Case report

Abstract

Obstructed hemivagina and ipsilateral renal anomaly syndrome (OHVIRA) also known as Herlyn-Werner-Wunderlich syndrome is a rare type of Müllerian duct anomaly with concomitant mesonephric duct anomalies. It is characterized by uterus didelphys, obstructed hemi-vagina, and commonly renal agenesis however; other types of mesonephric duct anomalies such as duplicated kidneys, dysplastic kidneys, rectovesical bands, or crossed fused ectopia have also been reported. We present a case report of a 21-year-old female patient experiencing new-onset vaginal bleeding and pelvic pain over the past month. Subsequent multi-sequence MRI revealed characteristic features indicative of OHVIRA syndrome. Clinical manifestations typically encompass pelvic pain and menstrual abnormalities but mainly depend upon the presence of communication between the hemi-uterus. This article aims to discuss the clinical presentation, types, and diagnostic utility of ultrasound and MRI in OHVIRA syndrome. Additionally, we discuss the possible management plans.

Introduction

A 21-year-old nulligravida woman who presented with symptoms of heavy menstrual bleeding, accompanied by a smelly clotted discharge and vague lower abdominal pain. Pelvic MRI showed uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis suggesting OHVIRA syndrome. She was managed with vaginal septum resection and had a smooth post-op course.

Case presentation

A 21-year-old nulligravida woman presented to the gynecologic OPD with heavy menstrual bleeding with associated smelly clotted discharge of 1-month duration with associated vague lower abdominal pain. Before her current complaint, she stated that she had a regular menstrual cycle of 28 days but complained she had moderate abdominal pain with each cycle. Her menarche was at the age of 13. Her vital signs were all in the normal range and her physical examination was unremarkable. Complete blood count was normal with a hemoglobin of 14.8 g/L. For the above complaint, she was investigated with pelvic MRI which revealed an absent right kidney, and uterus didelphys with associated moderate heterogeneous fluid signal intensity collection in the right hemi-uterus and vagina as well as mild collection in the left hemi-uterus.

Imaging findings

Multi-sequential MR images demonstrate 2 separate uterine cavities with widely divergent apices, separated cervix, and upper vagina (Fig. 1). The right uterine cavity has a moderate T2 bright and T1 dark heterogeneous endometrial collection extending to the cervix and upper part of the vagina noted with abrupt termination (Fig. 4). The left uterine cavity has a minimal endometrial collection having similar signal intensity. There is 3 mm measuring defect on the cervical stroma which separates the 2 cervical canals having T2 hyperintense signal intensity suggesting communication of the 2 canals (Fig 2). There are two distinct vaginal cavities, with the right one being fluid-filled and exerting pressure on the left one (Fig. 3). T2 weighted coronal image (Fig. 5) shows empty right renal fossa and a compensatory enlargement of the left kidney measuring 13.5 cm.

Fig. 1.

T2 -weighted axial MR image showing two separated and divergent uterine cavities (green and red arrows).

Fig. 4.

T2-weighted sagittal image, with high-signal intensity heterogeneous endometrial collection extending into the upper 2/3 of the right vaginal canal with abrupt termination distally.

Fig. 2.

T2-weight axial image at the level of the cervix, showing moderate high signal intensity endometrial collection in the right side with associated continuous signal intensity communicating the right and left side (green arrow) and mild collection in the left side.

Fig. 3.

At the level of the vagina, a T2-weighted axial image reveals 2 distinct vaginal cavities, with the right vagina being fluid-filled and pushing the left vagina.

Fig. 5.

T2 weighted coronal abdominal image, showing empty right renal fossa with compensatory enlargement of the left kidney (13.5 cm).

Management

A complete resection of the vaginal septum, extending from the external cervical OS to the lateral vaginal wall, was performed under direct visualization. This was followed by a smooth postoperative course and her normal menstrual cycle returned three weeks postsurgery. She was linked to the psychiatry outpatient department for psychological support to cope with the diagnosis and the implications for reproductive health.

Discussion

The imaging features are consistent with a didelphys uterus with 2 separate uterine cavity, cervix, and vagina. Bicornuate uterus can also be considered in the differential but the presence of 2 separated vaginal cavities would make it unlikely [1].

The cause of the OHVIRA remains unknown. However, it is proposed that the mesonephric duct is needed for the normal development and fusion of Mullerian ducts, therefore failure of development of the mesonephric duct leads to renal agenesis with uterine fusion anomalies [2,3].

The absence of the mesonephric duct on 1 side causes renal agenesis creating a space, leading to the ipsilateral Müllerian duct deviation. This will result in failure of fusion with the contralateral side forming a didelphic uterus. Meanwhile, the contralateral Müllerian duct communicates with the urogenital sinus to form the vagina. However, on the affected side, the failure of connection results in an obstructed hemivagina and a blind end sac [4,5]. In addition, it has been reported that right-sided obstruction with ipsilateral renal agenesis is more common [[6], [7], [8]].

The presentation usually is lower pelvic pain during the menstrual cycle unlike hematometrocolpos from a single uterus with no MDA which present during puberty with primary amenorrhea, OHVIRA syndrome presentation can be delayed due to regular menstrual flow from the unobstructed uterus [3,9]. An abdominal exam frequently detects a suprapubic mass with tenderness to palpation.

As in our case, the presentation may be late until the 2 separate cavities communicate with one another and the blocked system leaks via the patent vaginal canal. Communication could occur at the isthmic, cervical, or vaginal levels, and it could be inherited or acquired [10,11].

After conducting a retrospective study on 70 patients, Zhu et al. categorized OHVIRA syndrome patients into 2 groups based on the presence or absence of communication between the obstructed hemivagina and the patent system [6]. Class 1 denotes complete obstruction, characterized by either a blind hemivagina or cervical atresia. This condition results in a substantial accumulation of blood products within the obstructed uterine cavity. Consequently, affected individuals typically present with abdominal distension upon reaching menarche, with the average age of presentation recorded at 12.3 +/- 1.8 years. Class 2 represents a hemivagina completely obstructed with small communication between the duplicated cervices. This communication influences the clinical presentation, with patients presenting with a mean age of 21 as evidenced by the study as well as in our case. Moreover, they usually have normal menstrual cycles, but longer menstrual periods, and illness attacks years after menarche. However, they could also experience dysmenorrhea, irregular vaginal hemorrhage, and intermittent mucopurulent discharge due to recurrent ascending genital infections.

Ultrasound and MRI are established imaging modalities for diagnosing this condition, with ultrasound being the preferred first-line option. Ultrasonography (US) is the first line of imaging for Müllerian duct anomalies (MDAs), especially the presence of hematometrocolpos significantly facilitates the diagnosis of MDA [12]. However, to obtain an accurate MDA classification, it is the gold standard MRI to confirm the diagnosis, providing detailed information of both internal and external uterine anatomy, and to diagnose associated extra genital anomalies. It has 100% accuracy in detecting müllerian duct anomalies [6,13,14].

Treatment for this condition typically involves surgical intervention aimed at removing the vaginal septum, offering symptomatic relief, and preserving fertility. However, in instances where the obstruction is from cervical agenesis, surgical correction becomes challenging. In such cases, the recommended approach involves laparoscopic or transabdominal resection for the surgical removal of the atretic uterus [6].

In conclusion, our case unveils a rare presentation of OHVIRA syndrome characterized by a didelphys uterus accompanied by an obstructed hemivagina and ipsilateral renal agenesis. We emphasize that the clinical manifestation hinges on the presence or absence of communication with the patent contralateral Müllerian system. MRI is the gold standard modality for identifying Müllerian duct anomalies, and can determine whether communication exists between the 2 Mullerian systems. Treatment varies depending on the kind of obstruction. However, simple hemivaginal septum obstruction can be resolved with septum excision; cases involving cervical agenesis necessitate surgical removal of the ipsilateral blocked uterus.

Patient consent

Written informed consent was obtained from the patient's parents for anonymized patient information to be published in this article.