We report a postpubertal case of obstructed hemivagina and ipsilateral renal agenesis syndrome who presented with dysmenorrhoea and light menstrual flow. Imaging modalities confirmed the diagnosis after which vaginal septal incision and drainage was performed. In addition, we also performed vaginoscopy using hysteroscope, both preincision and postincision and drainage, to visualise the vagina and to exclude any communication between two hemivaginas. Completeness of drainage was ensured by performing intraoperative sonography. In a few instances, communication (fistula or microperforation) between the two hemivaginas would result in spontaneous haematocolpos decompression, mimicking normal menstrual flow and often delaying diagnosis. The role of direct visualisation under hysteroscopy should be emphasised in such cases. We postulate that hysteroscopy and intraoperative sonography may aid in better diagnostic and therapeutic management in such cases.
- Obstetrics and gynaecology
- Reproductive medicine
- Paediatric Surgery
- Urinary and genital tract disorders
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- Obstetrics and gynaecology
- Reproductive medicine
- Paediatric Surgery
- Urinary and genital tract disorders
Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) is a rare female urogenital tract anomaly. The incidence of OHIVIRA ranges from 0.1% to 3.8% in the female population.1 Though the exact aetiology is poorly understood, its pathogenesis involves anomalous development of both paramesonephric (Müllerian) and mesonephric (Wolffian) ducts. Along with giving origin to kidneys, the Wolffian duct induces normal Müllerian duct development. Abnormal development of Wolffian ducts leads to unilateral renal anomalies and obstructed hemivagina.2 Women with this anomaly usually present after menarche with dysmenorrhoea, acute abdominal pain, urinary retention or an abdominopelvic mass and rarely with primary infertility. The medical literature probably does not report OHVIRA syndrome presenting as light menstrual flow associated with dysmenorrhoea. We report a rare case of OHVIRA syndrome presenting as light menstrual flow and dysmenorrhoea.
A girl in her early adolescence presented to our tertiary care institute with lower abdominal pain during menstruation and light menstrual bleeding since menarche. She had attained menarche 1 year ago, and her menstrual cycles were regular, once every 30–34 days, with bleeding of 3 days. She required analgesics for dysmenorrhoea during menstruation. Her medical and surgical history was unremarkable. She had no known medical or surgical diseases. She was Tanner stage IV on physical examination, and abdominal examination was normal. The gynaecological examination was performed after consent as the patient was unmarried. It revealed a single vagina with a pink coloured bulge on the right-side wall. The cervix could not be seen or felt.
Abdominal and pelvic sonography revealed a large well-defined collection of volume 503cc in the vagina suggestive of haematocolpos and atrophic right kidney. MRI revealed uterine didelphys of which the right uterine horn was opening into a blind hemivagina with haematometrocolpos and right haematosalpinx (figure 1A). The left uterine horn, cervix and vagina were normal (figure 1B). Both ovaries were normal (figure 1C). The right kidney was not visualised. A schematic diagram showing the anomalies of our case is depicted for better understanding (figure 2).
Based on the clinico-radiological correlation, findings such as unilateral obstructed hemivagina which resulted in haematometrocolpos, ipsilateral renal agenesis and uterine didelphys suggested the diagnosis to be OHVIRA syndrome.
Conditions which may present with dysmenorrhoea and haematocolpos such as transverse vaginal septum and imperforate hymen were ruled out based on history and clinical examination. These two conditions typically present as progressive cyclical abdominal pain or dysmenorrhoea and absent menstrual bleeding. Contrary to it, our patient had menstrual bleeding along with dysmenorrhoea. As genital examination revealed single vagina, outflow tract obstruction was excluded from clinical diagnosis which was later supported by imaging studies.
The patient was planned for hemivaginal septal incision with drainage of haematometrocolpos. Under spinal anaesthesia and dorsal lithotomy position, vaginoscopy was performed using hysteroscope. The vagina was elongated and a normal cervix was found at the top of it. No communication between the patent hemivagina and the obstructed hemivagina was visualised. Then an incision was made on the bulge on the right lateral wall of the vagina. About 1000 mL of chocolate coloured fluid was drained out. The opening of the right wall of the vagina was enlarged. Haemostatic sutures were taken. Intraoperative transabdominal sonography was done, and the haematocolpos and haematometra were found to be completely drained (figure 3). On repeat vaginoscopy, two separate vaginal cavities could be visualised (figure 4). Hysteroscope was introduced into the newly opened right vagina and normal looking cervix was visualised. A 20 F foley catheter was inserted through the vaginal incision into the right vagina, and its bulb was inflated with 50 mL of normal saline to keep the incisional opening patent. Adequate postoperative care was provided and the intravaginal foley catheter was removed on the sixth postoperative day. She was discharged with good health.
Outcome and follow-up
After 40 days of surgery, the patient was followed up on an outpatient basis. She had normal menstruation without dysmenorrhoea or any procedure related complications. On examination, she had wide communication between two halves of vagina appearing as a single vagina.
OHVIRA syndrome has been increasingly recognised and treated within the spectrum of Müllerian anomalies as a result of access to advanced imaging modalities and gynaecological care. It typically presents with recurrent severe dysmenorrhoea though other uncommon presentations have been reported which includes acute pain abdomen, abdomino-pelvic mass, urinary retention, mucopurulent vaginal discharge and with primary infertility. The diagnosis is typically made after menarche because of gradual increase in severity of dysmenorrhoea secondary to increasing haematometrocolpos due to obstructed hemivagina. This presentation is usually ignored by clinicians as the menstrual flow remains normal through the unobstructed hemivagina and higher prevalence of dysmenorrhoea in this age group.3
Patients usually delay the reporting due to ignorance and unavailability of facility, leading to dysmenorrhoea, pelvic inflammatory disease, endometriosis and infertility.4 We found our case to be unique as our patient complained of light menstrual flow along with dysmenorrhoea.
The pathogenesis of OHVIRA syndrome is related to anomalous development of both paramesonephric (Müllerian) and mesonephric (Wolffian) ducts.2 3 The Wolffian ducts induce the normal development of Müllerian ducts along with giving origin to kidneys. On the side of absent Wolffian duct, the Müllerian duct is displaced laterally and cannot fuse with its contralateral counterpart which results in formation of uterine didelphys with fully developed uterine horns and two cervices. The displaced Müllerian duct cannot come in contact with urogenital sinus leading to imperforate hymen.5
We believe that a correct diagnosis can typically be made from history and clinical examination, in combination with imaging. Usually, sonography is the initial imaging modality in evaluation which can detect haematometrocolpos or haematocolpos and the type of mullerian anomaly. MRI has higher sensitivity in depicting the uterine morphology, associated cervical or vaginal and renal anomalies.6
Irrespective of varied presentation and diagnostic modalities, the treatment goal in OHVIRA syndrome is similar, that is, to relieve the obstruction of hemivagina which can alleviate the symptoms and prevent complications. A systemic review reported vaginal septectomy as the most commonly (85.6%) performed surgery to relieve the obstruction, followed by hemivaginectomy (2.2%).1 More aggressive treatment options such as hemihysterectomy or salpingectomy should be preserved for cases with complex anatomy such as blind and hypoplastic hemivagina.
Role of laparoscopy has been well described in various studies. Laparoscopy can be performed in cases of inconclusive diagnosis on MRI to classify mullerian anomaly and to treat concomitant conditions such as endometriosis which may be due to obstruction leading to retrograde menstruation.7 8 However, the uterine anomaly may not always be accurately depicted under direct visualisation by laparoscopy; the uterine anomaly is usually described as bicornuate with a single cervix by inspection from the external uterine contour. In most cases, the uterine anomaly can be diagnosed with better accuracy by MRI. Further, communication (fistula or micro perforation) between two hemivaginas cannot be diagnosed by laparoscopy; rather can be visualised by vaginoscopy and hysteroscopy as these are present at the level of the vaginal septum or between two cervices. The presence of microperforation can misguide the clinicians by masking the symptoms due to partial periodic evacuation of menstrual blood through it, which can delay the treatment and may result in long-term sequelae. Thus, we first performed vaginoscopy to visualise the vaginal canal to exclude any other anomaly or communication and repeated it after drainage of haematometrocolposalpinx to visualise the new cervical opening. In one reported case, postoperative sonography had been performed, which showed uterine didelphys and complete drainage of blood.3 But we performed intraoperative sonography to exclude any remaining haematometra or haematosalpinx, which obviated the need for any further procedure in the same sitting. We postulate that hysteroscopy and intraoperative sonography may aid in better diagnostic and therapeutic management in such cases.
I was suffering from severe pain abdomen since my menstruation started. Initially I consulted some local doctors but the relief was temporary. Even I went two big hospitals but they referred me here. I was very anxious about my ability of childbearing in the future. The doctors here did some tests and explained me in details about the abnormalities, its treatments and regarding the possibility of pregnancy in future. My surgery is over and I am fine now and I am having normal menstruation.
Detailed history taking and clinical examination is essential while evaluating a post menarche girl with dysmenorrhoea and light menstrual flow.
MRI can appropriately depict the associated uterine and renal anomaly and help in planning the management.
Hemivaginal septal incision and drainage of haematometrocolpos or haematocolpos can effectively alleviate symptoms and restore normal menstrual patterns, which can prevent sequels such as endometriosis and infertility.
Performing vaginoscopy using a hysteroscope and intraoperative sonography can add accuracy in diagnosis and precision in management.
Patient consent for publication
Contributors The following authors were responsible for drafting of the text, sourcing and editing of clinical images, investigation results, drawing original diagrams and algorithms, and critical revision for important intellectual content: AKD, SC, AH and MK. The following author gave final approval of the manuscript: AKD.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.
Competing interests None declared.
Provenance and peer review Not commissioned; externally peer reviewed.