Obstructed hemivagina with ipsilateral renal and urinary tract anomaly presenting as an unusual cause of acute abdomen: a radiologic perspective

  1. Kim Ivan R Mendoza and
  2. Dan Philippe C Nicer
  1. Department of Radiology, Philippine General Hospital, Manila, Philippines
  1. Correspondence to Dr Kim Ivan R Mendoza; krmendoza4@up.edu.ph

Publication history

Accepted:08 Jun 2022
First published:27 Jun 2022
Online issue publication:27 Jun 2022

Case reports

Case reports are not necessarily evidence-based in the same way that the other content on BMJ Best Practice is. They should not be relied on to guide clinical practice. Please check the date of publication.

Abstract

Obstructed hemivagina with ipsilateral renal anomaly (OHVIRA) is a rare congenital genitourinary defect with a triad of unilateral vaginal obstruction, uterine anomaly and ipsilateral renal agenesis. This paper reports an unusual presentation of OHVIRA, with our patient experiencing severe abdominal pain from a left tubo-ovarian abscess that is contralateral to the side of the vaginal outflow obstruction. Another reportable finding is our patient’s rare association of a trifid ureter that fuses distally before inserting ectopically in her vaginal canal. Lastly, this case report also emphasises the importance of radiologists’ expertise in suspecting the diagnosis early on and in contributing to the preoperative evaluation of patients with OHVIRA, thereby providing adequate management for these patients.

Background

The combination of an obstructed hemivagina with a didelphic uterus was first reported in 1922,1 but its actual triad with ipsilateral renal agenesis, termed Herlyn-Werner-Wunderlich syndrome (HWWS), was only published in 1950.2 Because other uterine and renal anomalies can also arise from the pathological development of the Mullerian and Wolffian structures, the acronym OHVIRA (obstructed hemivagina with ipsilateral renal anomaly) is better used in describing these patients compared with HWWS. The incidence of OHVIRA syndrome is believed to be between 0.5% and 5%.3 Mullerian duct anomalies (MDAs) occur in approximately 7% of reproductive-age women,4 with 40% of them having an associated congenital renal anomaly. Renal agenesis is the most common renal anomaly associated with uterine duplication, having an incidence of 63%–81%. Furthermore, in patients with MDAs, an obstructed hemivagina is also a common association,5 with at least 65% occurring on the right side.6

In the Philippines, there are no studies establishing the incidence of OHVIRA syndrome. As of this writing, there are at least 10 locally published reports about this condition,7–9 but none offers an extensive imaging perspective regarding patients with OHVIRA. The purpose of this case report is to describe the imaging findings in patients with OHVIRA syndrome and to emphasise the role of radiologists in its early detection and preoperative evaluation. Furthermore, our patient has two main reportable points from those that have been published locally and internationally (see online supplemental appendix 1 for the reviewed case reports). First is that this patient has a trifid ureter with an ectopic insertion into her vagina resulting in the later onset of urinary incontinence post-treatment of the obstructed hemivagina. The second is that this patient has a right-sided urinary tract anomaly with a longitudinal vaginal septum obstructing the same outflow tract, but presented with an acute abdomen in the emergency department due to a left tubo-ovarian abscess.

Supplementary data

[bcr-2022-249959supp001.pdf]

Case presentation

This is a case of a nulligravid in her 20s who presented with a history of progressive dysmenorrhoea 5 years after the onset of menarche. She then experienced sudden-onset left flank pain associated with fever and vaginal discharge, prompting a consult to the emergency department. On initial examination, the cervix was unappreciated due to a palpable cystic mass approximately measuring 4×4 cm in size. Acute abdomen secondary to a pelvic outflow tract obstruction was considered at this point.

Investigations

Initial examination using a contrast-enhanced abdominal CT scan (as shown in figure 1) revealed a dilated uterine and right cervical canal (figure 1A), a small right kidney with trifid renal pelvis exhibiting moderate hydronephrosis, and a multicystic lesion with wall enhancement located superolateral to the uterus (figure 1B) favouring a tubo-ovarian abscess. The patient was surgically treated at this point, confirming a didelphic uterus and left tubo-ovarian abscess intraoperatively.

Figure 1

Abdominal CT scan with contrast done using GE Healthcare Discovery ST showing the pathologies related to the patient’s initial acute abdomen presentation at the emergency room. (A) Axial image (316 mA, 100 kV, 80 mL of Ultravist contrast material) showing a dilated uterine cavity and hemivagina compatible with haematometra and haematocolpos, respectively. (B) Axial image (316 mA, 100 kV, 80 mL of Ultravist contrast material) showing a multicystic lesion with peripherally enhancing walls compatible with a tubo-ovarian abscess.

Other investigations also include vaginoscopy, which was done when the patient presented with another episode of pelvic pain associated with foul-smelling discharge a year after the above-mentioned surgery. Results revealed a longitudinal vaginal septum obstructing the right outflow tract and an unremarkable left outflow tract. Surgical removal of the vaginal septum was done but no pathological analysis of the septum performed.

Transvaginal ultrasound, CT urogram and plain MRI of the abdomen were also done as work-up for the patient when she consulted for urinary incontinence 6 months after the removal of the vaginal septum. These findings will be later on discussed in the paper.

Treatment

Initial treatment of the patient done in another institution was an emergency laparotomy which revealed a uterine didelphys with dilated right cervical canal and a cystic mass posteroinferior to the uterine corpus. This cystic mass is purulent fluid on intraoperative aspiration and a left salpingo-oophorectomy was done to treat this left tubo-ovarian abscess. At this point, the hemivaginal septum was not yet detected and was only suspected on consult with our institution. Surgical excision of the obstructing longitudinal vaginal septum was then performed to relieve the patient of her second episode of severe pelvic pain. At this point, a diagnosis of OHVIRA was given due to its association with an ipsilateral renal anomaly. As for the treatment of the trifid ureter, examination under anaesthesia, retrograde pyelography and laparoscopic right nephrectomy were done. Intraoperative findings showed a hypoplastic right kidney and an ectopic ureteral orifice noted immediately posterior to the urethral meatus that is connected to a dilated ureter, which splits into three distinct ureters draining into three separate calyces.

Outcome and follow-up

After the initial surgery for the left tubo-ovarian abscess, the patient allegedly experienced no symptoms until 2 years after when she then presented with an episode of severe pelvic pain associated with foul vaginal discharge, prompting consult at a tertiary hospital. After removal of the obstructing vaginal septum, she no longer reported recurrence of dysmenorrhoea; however, she noticed an increased urine output per her vagina, prompting the use of diapers to compensate for urinary incontinence. Status post laparoscopic nephrectomy, the patient had good postoperative outcome and no longer experienced urinary incontinence.

Radiological imaging findings and discussion

One of the main components of OHVIRA is the MDA. The Mullerian (paramesonephric) ducts are paired urogenital structures from which the fallopian tubes, uterus and the upper two-thirds of the vagina develop.10 In theory, embryological disruption in the eighth to ninth week of gestation can simultaneously affect the paramesonephric and mesonephric ducts, which could result in uterine and vaginal abnormalities with an ipsilateral renal anomaly. This was proven by Gruenwald11 in his experiment which showed that non-fusion of the Mullerian ducts results in a didelphic uterus if the caudal portion of the mesonephros is destroyed. Uterine didelphys accounts for 11% of MDAs, and aplasia or hypoplasia of the uterus and proximal vagina makes up 5%–10%.12 The American Society for Reproductive Medicine provides the most widely accepted classification of MDAs, which groups the uterine anomalies as shown in table 1.

Table 1

Summary of Mullerian duct anomalies23

Classification Embryological pathology Anomaly
Mullerian agenesis Failure of development of both paramesonephric ducts.

  • Mullerian agenesis without or with right/left uterine remnant with functional endometrium.
Cervical agenesis Likely from Wnt and homeobox gene families abnormality.

  • Cervical agenesis.

  • Distal cervical agenesis.
Unicornuate uterus Failure of development of one paramesonephric duct. R/L unicornuate uterus with or without the following:

  • R/L atrophic uterine remnant.

  • R/L distal atrophic uterine remnant (with or without functional endometrium).

  • R/L uterine horn communicating at the level of the cervix.
Uterus didelphys Complete lack of lateral fusion of the two paramesonephric ducts.

  • Uterus didelphys and longitudinal septum.

  • Uterus didelphys ± longitudinal septum of variable length.

  • Uterus didelphys and obstructed R/L hemivagina.
Bicornuate uterus Incomplete lack of lateral fusion of the two paramesonephric ducts.

  • Bicornuate uterus (serosal indentation >1 cm) with or without R/L communicating tract.

  • Uterus bicornuate bicollis.

  • Combined bicornuate septate uterus.
Septate uterus Failure of resorption of the midline septum between the fused paramesonephric ducts.

  • Partial septate uterus (septum length >1 cm and septum angle <90°).

  • Normal/arcuate uterus (normal length ≤1 cm and angle >90°).

  • Robert’s uterus.

  • Complete septate uterus with duplicated cervices and longitudinal vaginal septum.

  • Complete septate uterus with septate cervix and longitudinal vaginal septum.

  • Complete septate uterus, duplicated cervices and obstructed R/L hemivagina.
Transverse vaginal septum Mullerian ducts did not fuse with the urogenital sinus.

  • Mid-vaginal septum.

  • Distal vaginal septum.
Longitudinal vaginal septum Failure of complete canalisation of the vagina. Longitudinal vaginal septum of variable length without or with the following association:

  • Uterine didelphys.

  • Complete septate uterus and duplicated cervices.


Any of these may also be associated with an obstructed R/L hemivagina.
Complex anomalies Many proposed aetiologies all involving abnormalities in the development of the paramesonephric and mesonephric ducts. Some reported anomalies are also linked to diethylstilbestrol exposure.

  • Bicornuate uterus with bilateral obstructed endometrial cavities.

  • Uterus didelphys with communicating hemiuteri and unilateral R/L cervicovaginal atresia.

  • Obstructed R/L hemivagina, hemiuterus and single cervix with separate contralateral R/L patent hemiuterus, cervix and vagina.

  • Bicornuate uterus with R/L communicating tract and transverse vaginal septum.

  • Uterus isthmus agenesis.

  • R/L, right/left.

Ultrasound is the first-line modality in assessing the female pelvic organs due to its availability; however, MRI remains the imaging of choice for the diagnosis of MDAs due to its high soft tissue contrast. On transvaginal ultrasound of our patient (as shown in figure 2), the uterus was noted to have two divergent uterine horns separated by a fundal cleft (figure 2A). The endometrial cavities were completely separated with no evidence of communication. Each of the uterine horns led towards a separate cervix (figure 2B). The right uterine horn (figure 2C) measured 3.4×3.8×2.9 cm (length x width x anteroposterior dimension), while the right cervix measured 2.5×1.3×1.8 cm. The left uterine horn (figure 2D) measured 4.4×3.7×3.1 cm, while the left cervix measured 2.3×1.2×1.5 cm. The endometrial stripe appeared thin and homogeneously echogenic in both uterine horns, with a thickness of 3.5 mm on the right and 4.6 mm on the left.

Figure 2

Ultrasound features suggestive of uterine didelphys done using an intracavitary probe (frequency of 3–1 MHz) of Toshiba Aplio 500 Platinum series. (A) Transverse view showing two uterine horns separated by a fundal cleft (arrow). (B) Transverse view showing two distinct cervical canals (arrows) each leading to a separate uterine cavity. (C) Longitudinal and transverse views of the right uterine cavity (measured by dotted lines labelled A–D) showing a white echogenic line representing the endometrium (measured by the dotted line labelled E). (D) Longitudinal and transverse views of the left uterine cavity also showing a white echogenic line representing the endometrium.

On plain MRI of the pelvis (as shown in figure 3), the T2-weighted image (T2WI) (figure 3A) showed complete duplication of two separate uteri with divergent apices identified within a large fundal cleft. The endometrial cavities were uniformly separate with no evidence of communication, exhibiting preserved zonal anatomy and minimal T2WI-fluid endometrial cavity signals. Two separate cervices were also documented (figure 3B) and there was a single vaginal canal (figure 3C). T2-weighted is the primary MRI sequence that is useful in diagnosing MDAs due to its accurate uterine anatomical detail. T1-weighted sequences are not useful in demonstrating uterine didelphys and other MDAs since it lacks contrast of the uterine layers; however, this sequence is still important in confirming the presence of haematocolpos and haematometra.

Figure 3

MRI features suggestive of uterine didelphys done using 1.5 Tesla GE Healthcare SIGNA Artist. (A) Axial T2W imaging (fat saturation 1.5, repetition time 2002, time to echo 84.8) shows two separate uterine cavities separated by a large fundal cleft. (B) Axial T2W imaging (same parameter as above) shows two separate cervical canals each connected to the above-described completely separated uterine cavities. (C) Axial T2W imaging (same parameter as above) shows fusion of the two cervical canals into a single vaginal canal. No vaginal septum was identified since this imaging was done after excision of the longitudinal vaginal septum during work-up for the patient’s urinary incontinence.

Since our patient has definite evidence for MDA, the other component which is the ipsilateral renal anomaly, must be satisfied in order to diagnose OHVIRA. During embryogenesis, the development of the definitive kidney is induced by the ureteric bud which arises from the Wolffian duct. Disruptions in the development of the Wolffian duct result in lateral displacement of the Mullerian duct, which leads to failure of adequate fusion with urogenital sinus, resulting in the formation of a blind sac, imperforate vagina or an obstructed hemivagina.13 Furthermore, failure of fusion of the two fused paramesonephric ducts and the ascending sinovaginal bulb leads to a persistent vaginal septum or even vaginal atresia. The most common location of the septum is in the upper vagina (46%) and other locations include the mid-vaginal area (40%) and the lower vaginal region (16%).14 Because of this embryological relationship, aside from renal agenesis, other renal anomalies such as horseshoe kidney, pelvic kidney, cystic renal dysplasia, duplication of the collecting system and ectopic ureters have all been described to be associated with OHVIRA syndrome.15 In the majority of cases, the side of renal agenesis is predictive of the location of the obstructive hemivagina more than 50% of the time. In the case of our patient, she satisfies the second component of OHVIRA, which is an obstructed hemivagina, proven on vaginoscopy and seen on the initial contrast-enhanced abdominal CT scan.

For confirmation of the associated ipsilateral renal anomaly, which is the third component to diagnose OHVIRA, CT urogram and renal scintigraphy (as shown in figures 4 and 5, respectively) were done. On CT urogram, the right kidney was small (figure 4A), measuring 7.6×3.1×2.5 cm (craniocaudal x width x anteroposterior dimension), and there was marked dilatation of the right calyceal system with associated blunting of the calyces and pronounced thinning of the right renal parenchyma (figure 4B). Furthermore, pooling of contrast material in the delayed phases was observed on the right (figure 4C) after 25 min of contrast injection. On the other hand, the left kidney was normal in configuration with intact parenchymal thickness and measured 12.3×5.3×5.9 cm (craniocaudal x width x anteroposterior dimension). The left pelvicalyceal system and the ureter were not dilated. No calculi or definite masses were seen in both kidneys.

Figure 4

CT urogram done using GE Healthcare Discovery ST 16 showing renal hypoplasia ipsilateral to the obstructed hemivagina. (A) Coronal image taken during the corticomedullary phase (339 mA, 100 kV, 85–90 mL of Ultravist contrast material) showing hypoplastic right kidney and normal left kidney exhibiting good corticomedullary differentiation. (B) Coronal image taken during the nephrogenic phase (339 mA, 100 kV, 85–90 mL of Ultravist contrast material) showing the same findings as in A. (C) Coronal image taken during the 25 min delayed phase (428 mA, 100 kV, 85–90 mL of Ultravist contrast material) showing pooling of contrast material within the dilated trifid ureters on the right hypoplastic kidney and good excretory function of the left kidney.

Figure 5

Renal scintigraphy done using Siemens Symbia TruePoint SPECT CT, 8.6 mCi of Technicium-99m diethylene-triamine-pentaacetate(Techscan of Global Medical Solution). Scintigram shows a series of coronal images showing flow and renal phases on injection of technetium-99-labelled diethylenetriamine pentaacetate. There is adequate perfusion of the left kidney and severely reduced perfusion of the right kidney during the initial bolus of tracers, as demonstrated on the images marked by the single-arrowed line. Late and progressive accumulation of Tc-99 DTPA on the inferior portion of the right kidney is observed (curved line), while the left kidney shows good excretion of tracers (straight line).

Renal function of the right kidney was also assessed using renal scintigraphy since it is not entirely absent based on the CT urogram. Figure 5 shows sequential images of the kidneys that were obtained after injection of 8.6 mCi of Technicium-99m diethylene-triamine-pentaacetate (DTPA). The initial bolus phase showed severely reduced perfusion of the right kidney and adequate perfusion of the left kidney (marked by the double-arrowed line). The right kidney was very faintly labelled, with late and progressive accumulation in what appeared to be the inferior aspect of the renal cortex (marked by the single-arrowed line and curved arrow in the later phases). The left kidney on the other hand showed good elimination with no associated focal labelling defect seen. The calculated glomerular filtration rate value for the right kidney was 2.2 mL/min and for the left kidney 89.0 mL/min. Although DTPA scan is not an entirely adequate study to diagnose renal agenesis since it will only measure renal function, this information is still useful in confirming the ipsilateral renal anomaly. The better nuclear scintigraphy study to assess renal agenesis is a Dimercaptosuccinic acid (DMSA) radionuclide scan since it provides an assessment of the renal cortex and evaluation of renal structure and morphology.16

Although it is apparent that this patient does not have right renal agenesis, the CT urogram and renal scintigraphy findings are supportive of a right kidney that is severely hypoplastic and has a negligible renal function. With this, there is a need for us to determine if this small hypoplastic right kidney is congenital or acquired in origin in order to commit to a diagnosis of OHVIRA. The three most common causes of a small kidney are renal artery stenosis, chronic pyelonephritis and congenital hypoplasia. A summary of radiological differentiation is noted in table 2, and based on this the most likely origin of our patient’s small kidney is a type 1 congenital hypoplastic kidney instead of its closest differential, chronic pyelonephritis. The congenital origin is more favoured because the renal outline of our patient was smooth and regular in contrast to the ill-defined with loculated depression outline seen in an inflammatory origin.17 Furthermore, despite vesicoureteral changes of the markedly dilated calyceal system, our patient did not present with hypertension clinically. Furthermore, our patient has a thin right renal artery (as shown in figure 6A,B) and two left renal arteries (figure 6A), supportive of a possible congenital origin. Another supportive point for congenital renal hypoplasia is stated in the study of Braaseh and Emmet [17], where a diagnostic consideration of congenital hypoplasia is favoured instead of atrophic pyelonephritis if (1) the excretory urogram is much poorer, often absent; (2) the ureter is diminished in size and calibre; (3) no cicatricial deformity is demonstrated; (4) minor calyces are/may not be radiographically apparent; (5) calyces are negligible in size in keeping with the size of the kidney; (6) there is markedly decreased renal pelvis volume often less than 1 mL (never pyelectasis); and (7) there is absent renal parenchymal displacement. These findings are all present in our patient except for the diminished ureter size and calibre, which can be explained by the distally inserted trifid ureter, resulting in reflux hydronephrosis. The only way to accurately confirm the congenital origin of our patient’s renal anomaly is imaging documentation during childhood or pathological analysis of the remaining right renal parenchyma. With all the imaging and clinical evidence stated, the authors are highly convinced that our patient has ipsilateral renal hypoplasia, fulfilling all the criteria for diagnosing OHVIRA.

Table 2

Radiological differentiation of the three most common origins of small kidneys17

Radiological parameter Renal artery stenosis Chronic pyelonephritis Congenital hypoplastic kidney
Size Small (3–6.2 cm), in normal position. May show increase (if changes are infiltrative nature) or decrease (stage of contraction) in size. Small, may be infantile in type; medullary and cortical substance may be present.
Outline Smooth and regular in spite of marked decrease in renal parenchyma. Ill-defined with loculated depression and hardly discernible due to localised shrinkage. Smooth and regular in all cases.
Excretion time Majority are delayed to no excretion, with few reported to be normal. No excretion or very delayed in all small kidneys. Normal or no excretion especially if with overlapping chronic pyelonephritis.
Contrast density Predominantly reduced contrast of the affected side. Reduction of the glomerular filtration rate and concentrating ability. Normal in cases with excretion.
Changes in vesicoureteral system Normal ureter and is usually thin. Hypotonia of the ureter presenting as ureteric dilatation+presence of hypertension is a specific radiological/clinical sign. Type 1: markedly elongated and bizarre calyces, renal pelvis small, ureter thin (except if with distal obstruction, it can be dilated).
Type 2: miniature kidney with proportional normal morphology calycine system.
Renal vessels Usually bilateral involvement of renal artery (stenosis). Normal Mostly normal, may be smaller in some, with associated decrease in blood flow and opacification.
Figure 6

CT urogram done using GE Healthcare Discovery ST 16 demonstrating the renal arteries. (A) Coronal image taken during the arterial phase (339 mA, 100 kV, 85–90 mL of Ultravist contrast material) demonstrating one right renal artery showing a decrease in calibre (1.9 mm) relative to the two left renal arteries, which measure 5.0 mm and 6.2 mm, respectively. (B) Axial image taken during the arterial phase (339 mA, 100 kV, 85–90 mL of Ultravist contrast material) showing the tract of the hypoplastic right renal artery (measuring 1.8 mm) from its take-off from the aorta.

Another peculiar finding in this case report is the presence of a trifid ureter with ectopic insertion in the lower vaginal canal. In the review of published case reports in PubMed (a total of 34 reports reviewed as summarised in online supplemental appendix), none of these reported a trifid ureter associated with OHVIRA. CT urogram or magnetic resonance (MR) urography is the imaging modality option in assessing the renal collecting system, but due to the lack of MR urography protocol at our institution only CT urogram and plain abdominopelvic MRI (as shown in figure 7) were done. CT urogram showed that the right calyceal system did not appear to meet and form the right renal pelvis. Instead, three urinary collecting systems were seen directly arising from the major calyces (figure 7A: superior, middle and inferior). Three dilated ureters were present: (1) the ureter arising from the right superior major calyx was noted to course along the lateral aspect of the right psoas muscle, abutting the inferior vena cava; and (2) the other two ureters arising from the middle and inferior major calyx course inferiorly, converging at the level of the L5 vertebra. The triplicated right ureters then converged at the level of S3 (figure 7B), then the fusion of these ureters went on as a single dilated urinary tract that terminates and communicates at the 11 o’clock to 12 o’clock position of the lower third of the vagina (figure 7C). No communication of the right ureters with the urinary bladder was demonstrated. According to Smith’s classification,18 triplication of the ureters is anatomically divided into (1) complete triplication (35%); (2) incomplete triplication, where two out of three ureters join before inserting into the bladder and thus two orifices are present in the bladder (21%); (3) trifid ureters, which unite and drain through a single orifice (31%); and (4) double ureters, with one bifurcation as an inverted Y draining into three orifices (9%). In the case of our patient, Smith’s classification type 3 is present (trifid ureter) and it is ectopically inserted into the lower vagina. The loss of its normal physiological sphincter is the cause of its reflux dilatation as compared with a normally inserted ureter. In some cases, complete obstruction can also occur if the ectopic site inserts as a ureterocele.19

Figure 7

CT urogram done using GE Healthcare Discovery ST 16 demonstrating trifid ureter and plain pelvic MRI showing fusion of the trifid ureter before inserting ectopically into the vagina. (A) Coronal image taken during the arterial phase (339 mA, 100 kV, 85–90 mL of Ultravist contrast material) showing the dilated trifid right ureter. (B) Sagittal T2W imaging (FS 1.5, TR 6612, TE 1037) showing the convergence of the trifid ureters at the level of the S3 vertebra. (C) Sagittal T2W PROPELLER image (FS 1.5, TR 6612, TE 103.7) showing the dilated distal right ureter inserted into the vaginal region. T2W, T2-weighted.

This trifid ureter with distal vaginal ectopic insertion is a very important association that was overlooked in the management and was only completely evaluated after the resection of the vaginal septum. On the initial contrast-enhanced abdominal CT scan done in another institution, the distal ectopic insertion was suspected and a CT urogram was suggested for further evaluation. The patient was then lost to follow-up after the initial management of the left tubo-ovarian abscess and she only consulted at our institution during the second instance of obstructed hemivagina, prompting emergency surgical excision of the obstructing vaginal septum. The patient was then referred to the urology service for the work-up and management of the trifid ureter anomaly. Because of these factors, the initial delay in recognition and management of the collecting system anomaly resulted in the patient’s urinary incontinence postremoval of the obstructing septum. This stresses the importance of adequate preoperative evaluation prior to definitive removal of the vaginal septum in patients with OHVIRA syndrome.

OHVIRA syndrome is commonly undiagnosed and is asymptomatic during early childhood due to the presence of normal external genitalia that often masks the internal urogenital anomalies. There is only one published case of an early manifestation in the newborn, which presented as a persistent prolapsed mass in the vaginal introitus.20 In the majority of patients, medical consult for clinical symptoms only happens after the onset of menarche, with the most common being progressive dysmenorrhoea. In a 10-year review of this anomaly, most patients present with dysmenorrhoea (73%), a pelvic or paravaginal mass (71%), and an affected right uterus and vagina ipsilateral to the renal anomaly (63.5%).21 The main pathology of this syndrome is the obstructed hemivagina, which results in blood accumulation in the uterus and retrograde bleeding in the abdomen through the fallopian tube, causing pelvic pain, haematometra, haematocolpos and finally endometriosis. Pyocolpos and tubo-ovarian abscess can also occur in these patients due to secondary infection of retained menstrual blood in the obstructed hemivagina, and these disease entities often present in the emergency department as an acute abdomen. In the case of our patient, the tubo-ovarian abscess that causes the acute abdomen was located contralateral to the obstructed hemivagina. No case reports have been published with such clinical presentation, which makes our patient’s case more interesting. The classification of HWWS based on vaginal morphology as shown in table 3 provides the most plausible explanation for a contralateral tubo-ovarian abscess formation. Partial reabsorption of the vaginal septum could lead to small communication between the two hemivaginas, which often presents with purulent vaginal discharge due to ascending genital tract infections, more so if there is communication also with the uteri. If the vaginal septum is very thin, the pressure building up from the distended hemivagina may result in microperforation along the septum, thereby allowing the flow of menses and/or vaginal discharge onto the normal outflow tract. Although a majority of published articles document the infection ipsilateral to the obstructed side, we theorised that this can also happen to the contralateral side if the infected fluid passes through this communication and ascends to the contralateral uterus, adnexa or other regions within the pelvis, for example in the cul-de-sac.

Table 3

Classification of OHVIRA syndrome based on vaginal morphology24

Classification Subclass and nomenclature Findings Clinical features
Class 1: completely obstructed hemivagina 1.1 Blind hemivagina.

  • Completely obstructed hemivagina.

  • The uterus behind the septum is completely isolated from the contralateral uterus with no communication between the duplicated uterus and vagina.

  • Haematocolpos, haematometra, haemoperitoneum and endometriosis; secondary pelvic adhesions, pyosalpinx and pyocolpos.
1.2 Cervicovaginal atresia without communicating uteri.

  • Completely obstructed hemivagina.

  • The cervix behind the septum is maldeveloped or atretic.

  • Same as 1.1.
Class 2: incompletely obstructed hemivagina 2.1 Partial reabsorption of the vaginal septum.

  • Small communication exists between the two vaginas.

  • The uterus behind the septum is completely isolated from the contralateral uterus.

  • Later onset of symptoms presenting years after menarche, purulent of bloody vaginal discharge, ascending genital infections.
2.2 With communicating uteri.

  • Small communication exists between the duplicated cervices.

  • Menstrual blood drainage is still impeded.

  • OHVIRA, obstructed hemivagina with ipsilateral renal anomaly.

Diagnostic imaging plays a very important role in diagnosing this rare developmental anomaly of the genitourinary system in females. MRI is the modality of choice for diagnosis, characterisation and preoperative evaluation in patients suspected of having MDAs due to its multiplanar capability, inherent high soft tissue contrast and better characterisation of the pelvic anatomy. However, MRI is not widely available and thus sonography can be used as the initial modality for work-up of these patients. OHVIRA should be highly suspected in patients with a renal anomaly in the presence of a uterine abnormality complicated by findings suggestive of haematometra, haematocolpos or haemoperitoneum. However, on imaging, when there is a large fluid collection, visualisation of the vaginal septum and diagnosis of a didelphic uterus may be difficult on ultrasound and can lead to an inaccurate diagnosis. CT scan of the abdomen may also be helpful especially in the setting of acute abdomen since it will be able to detect complications such as tubo-ovarian abscess and rule out other causes of abdominal pain. Laparoscopy remains the gold standard of diagnosis, but may not be required prior to management, especially if the patient presents with imaging findings compatible with OHVIRA syndrome. Early detection of this syndrome is important because surgical resection of the obstructing vaginal septum is the mainstay of management for providing pain relief and preventing further complications. This is further emphasised in the case series study of Tan et al 22 in 2019, where screening for HWWS syndrome using ultrasound in female babies with renal agenesis or multicystic dysplastic kidney (a non-heritable paediatric cystic renal disease formed in utero) is done in order to do presymptomatic elective surgery in patients with possible OHVIRA. This is helpful in preventing the obstructive hemivagina component as well as other urogynaecological anomalies in patients with OHVIRA that may cause infertility and renal impairment later on.

Patient’s perspective

Getting diagnosed with this rare disease really change my life, most especially the latter treatment where I was diagnosed with ectopic ureter insertion in my vagina. I am very relieved that I need not anymore to wear diapers every day to compensate for my previous urinary incontinence. I am also now free of pelvic pain, I am able to sleep well knowing that I will not anymore experience pain because I was already completely treated. My advice to those people who have or will have the same condition as me, stay strong and someday God will find a way for us to be treated. Most especially through the help of doctors who endlessly have the passion to learn and share rare cases like us, so that the world will know that this disease exist so that they can be better helped by future doctors. This experience has really changed my life and I am very grateful for this opportunity.

Learning points

  • Prompt and accurate diagnosis of obstructed hemivagina with ipsilateral renal anomaly is essential for early relief of symptoms, preservation of fertility and prevention of further complications.

  • This should always be suspected in patients presenting with pelvic pain showing an obstructed vagina, ipsilateral renal anomaly and Mullerian duct abnormalities on work-up imaging.

  • Radiologists’ expertise plays an important role in assessing the extent of the genitourinary anomaly and in detecting life-threatening complications such as a tubo-ovarian abscess.

  • Proper preoperative evaluation using sonography, MRI with magnetic resonance urography or CT urogram is suggested so that surgeons will have proper planning before executing definitive treatment in these patients in order to prevent later complications.

  • Presymptomatic surgery may also be contemplated in these patients if detected early.

Ethics statements

Patient consent for publication

Footnotes

  • Contributors KIRM: submitting author, writing of the draft and final paper, gathering of data and obtaining consent from the patient, accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated, and revision of the paper in accordance with the reviewers’ comments and suggestion. DPCN: case report adviser, revising the paper critically for important intellectual content, approval of the version to be published and checking of revisions.

  • 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.

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