Skip to main content

A rare case report of urogenital anomaly in a teenage girl: Herlyn–Werner–Wunderlich syndrome/OHVIRA syndrome (Obstructed Hemivagina and Ipsilateral Renal Anomaly)

Abstract

Background

Herlyn–Werner–Wunderlich syndrome also known as OHVIRA syndrome is a rare complex congenital developmental anomaly characterized by the triad of uterine didelphys, obstructed hemivagina causing hematometrocolpos and ipsilateral renal anomaly.

Case presentation

Here we report a case of a 14-year-old girl who presented with acute onset lower abdominal pain. Ultrasound and magnetic resonance imaging showed uterus didelphys, hematometrocolpos, obstructed hemivagina and right renal agenesis. Patient underwent hematocolpos drainage.

Conclusions

OHVIRA syndrome is an uncommon congenital anomaly. Imaging plays a major role in diagnosis. Surgery is the treatment of choice to resect the septum and relieve the obstruction. An early correct diagnosis is the goal to relieve the symptoms and prevent complications, caused by retrograde menstruation which may result in endometriosis and, also, preserve sexual and conception abilities.

Background

OHVIRA syndrome is characterized by uterine didelphys, obstructed hemivagina causing hematometrocolpos and ipsilateral renal anomaly. This triad is known as “Herlyn–Werner–Wunderlich syndrome,” and it was first described by Purslow [1]. It is a rare obstructed Mullerian duct anomaly. The incidence of Mullerian duct anomalies ranges from 0.8 to 4%, and the incidence of OHVIRA syndrome is estimated to be 1/20,0000 in the general population [2, 3]. Review of literature in 15 reported studies consisting of 1771 patients during 1965–1996 period revealed mean incidence of uterine didelphys to be 11.1% [4]. Patients with didelphic uterus, 29.1% had associated renal anomalies with 23.6% diagnosed to have renal agenesis and obstructed hemivagina [5]. It usually presents in a post-pubertal adolescent female after menarche with nonspecific symptoms like lower abdominal pain, urinary retention, pelvic pain and a mass.

In this study, we report a case of a 14-year-old girl from India who presented with acute onset lower abdominal pain. Abdominal ultrasound (US), magnetic resonance imaging (MRI) and intraoperative findings are reported in this study.

Case presentation

A 14-year-old female presented with abdominal pain since 5 days, which was sudden in onset, gradually progressive, non-radiating, intermittent and relieved on medication. The patient attained menarche at 12 years of age. Menstrual history: 5/30, regular, normal flow with presence of clots and dysmenorrhea, 3 pads/day. General examination was unremarkable. Secondary sexual characters were attained. On examination per abdomen was soft and non-tender; local examination showed no bulge. On per rectal examination posterior vaginal wall bulge was felt.

Ultrasound abdomen and pelvis showed absence of right kidney in right renal fossa (Fig. 1a) and compensatory hypertrophy of contralateral left kidney measuring 133 × 55 mm (Fig. 1b). Evidence of two uterine horns (Fig. 2a) and two uterine cavities noted measuring 57 × 25 mm on the right side (Fig. 2b) and 64 × 22 mm on the left side (Fig. 2c)—uterine didelphys. The right horn of uterus and cervical canal are seen extending into a large well-defined hypoechoic collection with internal echoes measuring approximately 100 × 75 mm suggestive of hematometrocolpos (Fig. 3). The left horn of uterus and cervical canal appear collapsed. No gross adnexal pathology is seen. Both ovaries are visualized normally (Fig. 4).

Fig. 1
figure 1

a and b USG abdomen and pelvis of the patient showing absent right kidney in the right renal fossa (arrow in a) and compensatory hypertrophy of the contralateral left kidney (arrow in b)

Fig. 2
figure 2

ac USG abdomen and pelvis showing two uterine horns (arrows in a). Right uterine cavity (star in b) and left uterine cavity (star in c)

Fig. 3
figure 3

USG abdomen and pelvis. The right uterine horn and cervical canal are seen extending into a large well-defined hypoechoic collection with internal echoes (star) measuring approximately 100 × 75—s/o hematometrocolpos

Fig. 4
figure 4

a and b. USG abdomen and pelvis showing normal sized right ovary (arrow in a) and normal sized left ovary (arrow in b)

MRI pelvis was done which revealed complete duplication of uterine horns as well as duplication of the cervix and vagina (Fig. 5). The right hemivagina was distended by T1 hyper intense, T2 hypo intense collection measuring 74 × 74 × 95 mm with low-lying debris displacing the left hemivagina, rectum to the left and compression of the urinary bladder anteriorly—likely obstructed right hemivagina with subacute blood secondary to longitudinal vaginal septum (Fig. 6). The left hemivagina appeared collapsed. No communication between the duplicated uteri and vagina could be established. Bilateral ovaries were normal. Right kidney is absent in right renal fossa. Left kidney showed compensatory hypertrophy (Fig. 7). Additionally, a T1 hyperintense cystic structure with T2 shading was noted in the right adnexa inserting into the upper vagina—likely a blind right ectopic ureter with hematic reflux from hematocolpos (Fig. 8). All these features are suggestive of OHVIRA syndrome.

Fig. 5
figure 5

MRI pelvis T2-weighted axial images showing two uterine cavities (arrows)

Fig. 6
figure 6

ac MRI axial T2W axial, T1W axial and T2W sagittal images. The right hemivagina is distended by T1 hyperintense (star in b), T2 hypointense (stars in a and c) collection measuring 7.4 × 7.4 × 9.5 cm and low-lying debris displacing the left hemivagina, rectum to the left and compression of the urinary bladder anteriorly (arrow in c)—likely obstructed right hemivagina with subacute blood secondary to longitudinal vaginal septum

Fig. 7
figure 7

MRI T2-weighted coronal images. Absent right kidney in right renal fossa (thin arrow) with left kidney showing compensatory hypertrophy (thick arrow)

Fig. 8
figure 8

MRI pelvis T1 axial images. A T1 hyperintense cystic structure (star) in the right adnexa inserting into the upper vagina—likely a blind right ectopic ureter with hematic reflux from hematocolpos

The patient underwent drainage of the hematocolpos. Around 150 ml of chocolate colored fluid was drained. The Intraoperative findings are shown in Fig. 9. Postdrainage ultrasound 2 months later showed minimal fluid in the lower uterine cavity (Fig. 10).

Fig. 9
figure 9

a and b Intraoperative photograph showing smooth bulge (arrow in a) caused by right hematocolpos. Intraoperative photograph of septoplasty procedure showing drainage (around 150 ml) of chocolate colored fluid (arrow in b)

Fig. 10
figure 10

USG abdomen and pelvis postdrainage showing minimal fluid in the lower uterine cavity of the right uterus (arrow)

Discussion

OHVIRA syndrome is often misdiagnosed. The patient generally presents at puberty with pelvic pain. In the adulthood, they present with primary infertility, hydrometrocolpos, ischiorectal swelling and urinary obstruction. There can be a delay in diagnosis due to normal onset of puberty and menstruation [6].

Herlyn–Werner–Wunderlich syndrome (HWWS) is a combination of type III Mullerian anomaly with mesonephric duct anomaly with vaginal septum. Renal anomalies can occur in 50% of patients with Mullerian anomalies. The classic renal manifestation of OHVIRA syndrome is ipsilateral renal agenesis, but cases of dysplastic kidneys, duplicated kidneys, recto vesical bands, or crossed fused ectopia and ectopic ureters [7] should be looked for. Contralateral renal anomalies have been reported in up to 50% of the cases [8]

The incidence of uterus didelphys, related to HWW, is approximately 1/2,000 to 1/28,000, and it is accompanied by unilateral renal agenesis in 43% of cases [9].

Embryology of uterovaginal development is essential to understand the pathogenesis of Mullerian anomalies. Two theories have been described, the classic theory and acien theory. The classic theory described that the vagina is divided into upper and lower parts, where upper part of vagina is developed by Mullerian ducts and lower part is developed by sinovaginal bulbs of the urogenital sinus. This theory talks about Mullerian duct anomalies, but not the development of anomalies such as OHVIRA and the association of Mullerian duct anomalies with kidney pathology [10]. Acien theory explains that there are two ducts—Mullerian and paramesonephric ducts. Another duct is Wolffian duct, also known as mesonephric duct. Uterus and cervix are formed from joined Mullerian ducts, and vagina is formed from Wolffian duct. The vaginal lining consists of paramesonephric cells, derived from the paramesonephric tubercle, but the entire vagina is not developed from paramesonephric ducts. And since vaginal and renal development occurs from the Wolffian duct, if there is any error in derivation from one side of the Wolffian duct, it will affect the same side of the kidney and vagina or both. This theory helps in understanding complex anomalies such as OHVIRA [11, 12].

Near fifth week of gestation, Wolffian duct gives rise to the metanephric diverticulum. If this does not form, it will lead to the agenesis of the same side of the kidney and hemivagina. Mullerian development is lateral to the Wolffian duct at around nine weeks. After crossing the Wolffian duct, they come in midline and fuse to form the cervix and uterus. If they don’t fuse, it results in uterine didelphys. Hence anomalies in the Wolffian duct and the fusion of two Mullerian ducts will give rise to OHVIRA.

The diagnosis of such a syndrome requires multimodality approach, including detailed history, examination and appropriate imaging studies. Ultrasound and MRI or the imaging modalities are used for diagnosing such a condition. 3D ultrasound has 93% sensitivity and 100% specificity in the assessment of Mullerian duct anomaly. However, it requires the expertise of the radiologist due to small size of the uterus, non-reactive endometrium and distended vagina in prepubertal and pubertal girls [13]. The Gold standard investigation is MRI which can confirm the diagnosis and provide detailed information about the internal and external uterine anatomy and can diagnose associated extragenital anomalies. It has 100% diagnostic accuracy [14].

In our patient, there was a suspicion of a dilated ectopic ureter inserting into the obstructed right hemivagina. However, this finding requires further investigation like MR urography to confirm its presence, which was not done in our case. The management of ectopic ureter in such a case would be transvaginal ligation of the ureter or laparoscopic transperitoneal nephrectomy [15].

The absence of ipsilateral absent of kidney, however, doesn’t fully exclude the possibility of a small atrophic kidney. Additional investigations like intravenous urography, Technetium-99 m dimercaptosuccinic acid (DMSA) renal scan and contrast vaginography can be done to look for small atrophic kidney. However, these investigations were not performed in our case.

Primary management is vaginoplasty. It is done to relieve the obstruction. Before two-stage procedure used to be done, with the initial surgery to relieve the obstruction and the second to resect the septum. Currently, a single-stage vaginoplasty is done in which complete resection of the septum in a single procedure is carried out [16]. Surgery helps in relief of pain due to obstruction. It also reduces of the chance of pelvic endometriosis caused by retrograde menstrual seeding which can lead to infertility and chronic pelvic pain. Patients can have normal sexual life and can conceive and carry pregnancy to term. In a study, Altchek and Paciuc have reported pregnancy occurring twice in a previously obstructed didelphys uterus after surgical correction [17]. Therefore, effort must be made to preserve the obstructed uterus.

Conclusions

In conclusion, OHVIRA syndrome is an uncommon congenital anomaly. Imaging plays an important role in diagnosis. Surgery is the treatment of choice to resect the septum and relieve the obstruction. Early diagnosis helps to relieve the symptoms and prevent complications, which are caused by retrograde menstruation resulting in endometriosis and, also, preserve sexual and conception abilities [18].

Availability of data and material

The data are taken solely from our institution.

Abbreviations

HWWS:

Herlyn–Werner–Wunderlich syndrome

OHVIRA:

Obstructed hemivagina, ipsilateral renal agenesis

References

  1. Purslow C (1922) A case of unilateral haematocolpos, haematometra and haematosalpinx. J Obstet Gynaecol Br Emp 29:643. https://doi.org/10.1111/j.1471-0528.1922.tb16100.x

    Article  Google Scholar 

  2. Gidwani G, Falcone T (1999) Congenital malformations of the female genital tract. Diagnosis and management. Lippincott Williams & Wilkins, Philadelphia, p 145–168. https://doi.org/10.1007/978-1-4471-5146-3

  3. Aveiro AC, Miranda V, Cabral AJ, Nunes S, Paulo F, Freitas C (2011) Herlyn–Werner–Wunderlich syndrome: a rare cause of pelvic pain in adolescent girls. BMJ Case Rep 2011:bcr0420114147. https://doi.org/10.1136/bcr.04.2011.4147

    Article  PubMed  PubMed Central  Google Scholar 

  4. Acién P (1997) Incidence of Müllerian defects in fertile and infertile women. Hum Reprod 12(7):1372–1376. https://doi.org/10.1093/oxfordjournals.humrep.a019588

    Article  PubMed  Google Scholar 

  5. Pinsonneault O, Goldstein DP (1985) Obstructing malformations of the uterus and vagina. Fertil Steril 44(2):241–247. https://doi.org/10.1016/S0015-0282(16)48745-5

    Article  CAS  PubMed  Google Scholar 

  6. Haghgoo A, Faegh A, Nasiri S, Akhbari F (2024) Vaginoscopic resection of hemivagina, in a 20-year-old virgin female with prior misdiagnosis of OHVIRA syndrome as a bicornuate uterus: a case report. Clin Case Rep 12:e8661. https://doi.org/10.1002/ccr3.8661

    Article  PubMed  PubMed Central  Google Scholar 

  7. Tanaka YO, Kurosaki Y, Kobayashi T, Eguchi N, Mori K, Satoh Y, Nishida M, Kubo T, Itai Y (1998) Uterus didelphys associated with obstructed hemivagina and ipsilateral renal agenesis: MR findings in seven cases. Abdom Imaging 23(4):437–441. https://doi.org/10.1007/s002619900375

    Article  CAS  PubMed  Google Scholar 

  8. Fedele L, Motta F, Frontino G, Restelli E, Bianchi S (2013) Double uterus with obstructed hemivagina and ipsilateral renal agenesis: pelvic anatomic variants in 87 cases. Hum Reprod 28(6):1580–1583. https://doi.org/10.1093/humrep/det081

    Article  CAS  PubMed  Google Scholar 

  9. Del Vescovo R, Battisti S, Di Paola V, Piccolo CL, Cazzato RL, Sansoni I, Grasso RF, Zobel BB (2012) Herlyn–Werner–Wunderlich syndrome: MRI findings, radiological guide (two cases and literature review), and differential diagnosis. BMC Med Imaging 9(12):4. https://doi.org/10.1186/1471-2342-12-4

    Article  Google Scholar 

  10. Li S, Qayyum A, Coakley FV, Hricak H (2000) Association of renal agenesis and mullerian duct anomalies. J Comput Assist Tomogr 24(6):829–834. https://doi.org/10.1097/00004728-200011000-00001

    Article  CAS  PubMed  Google Scholar 

  11. Acién P (1992) Embryological observations on the female genital tract. Hum Reprod 7(4):437–445. https://doi.org/10.1093/oxfordjournals.humrep.a137666

    Article  PubMed  Google Scholar 

  12. Sánchez-Ferrer ML, Acién MI, Sánchez del Campo F, Mayol-Belda MJ, Acién P (2006) Experimental contributions to the study of the embryology of the vagina. Hum Reprod 21(6):1623–1628. https://doi.org/10.1093/humrep/del031

    Article  PubMed  Google Scholar 

  13. Kupesic S, Kurjak A (2000) Ultrasound and doppler assessment of uterine anomalies. In: Kupesic S, de Ziegler D (eds) Ultrasound and infertility. Parthenon, Pearl River, pp 147–153

    Google Scholar 

  14. Carrington BM, Hricak H, Nuruddin RN et al (1990) Mullerian duct anomalies: MR imaging evaluation. Radiology 176:715–720. https://doi.org/10.1148/radiology.176.3.2202012

    Article  CAS  PubMed  Google Scholar 

  15. Smith NA, Laufer MR (2007) Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome: management and follow-up. Fertil Steril 87(4):918–922. https://doi.org/10.1016/j.fertnstert.2006.11.015

    Article  PubMed  Google Scholar 

  16. Jaidane M, Slama A, Bibi M (2009) A tumor of an ectopic ureter mimicking uterine cervix adenocarcinoma: case report and brief review. Int Urogynecol J 20(11):1393–1395. https://doi.org/10.1007/s00192-009-0893-y

    Article  Google Scholar 

  17. Altchek A, Paciuc J (2009) Successful pregnancy following surgery in the obstructed uterus in a uterus didelphys with unilateral distal vaginal agenesis and ipsilateral renal agenesis: case report and literature review. J Pediatr Adolesc Gynecol 22:e159–e162

    Article  PubMed  Google Scholar 

  18. Lecka-Ambroziak A, Skobejko-Włodarska L, Ruta H (2023) The need for earlier diagnosis of obstructed hemivagina and ipsilateral renal agenesis/anomaly (OHVIRA) syndrome in case of renal agenesis in girls—case report and review of the literature. J Clin Med 12(23):7284. https://doi.org/10.3390/jcm12237284

    Article  PubMed  PubMed Central  Google Scholar 

Download references

Acknowledgements

The author would like to thank his great and beloved teachers, his closest friends and his mentors for their aids in making her complete this article.

Funding

This article did not receive any specific grant from funding agencies in the public, commercial or not-for-profit sectors.

Author information

Authors and Affiliations

Authors

Contributions

DJ did the major write up of this review article. The case in this review article was diagnosed and followed up by BB. The work was carried under the guidance BB and BV provided us the insight and knowledge to diagnose the syndrome. All the authors read the rough draft and provided valuable suggestions for the final draft. BB reviewed this article for corrections and final draft.

Corresponding author

Correspondence to Govardhana Das Joel.

Ethics declarations

Ethics approval and consent to participate

Not applicable.

Consent for publication

An informed consent was obtained from the patient to use the necessary data for publication.

Competing interests

The author claims that there is no competing interest.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/.

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Joel, G.D., Basetti, B. & Mallula, B.V. A rare case report of urogenital anomaly in a teenage girl: Herlyn–Werner–Wunderlich syndrome/OHVIRA syndrome (Obstructed Hemivagina and Ipsilateral Renal Anomaly). Egypt J Radiol Nucl Med 55, 177 (2024). https://doi.org/10.1186/s43055-024-01312-0

Download citation

  • Received:

  • Accepted:

  • Published:

  • DOI: https://doi.org/10.1186/s43055-024-01312-0

Keywords