OHVIRA Revised Feb 8th 2014_deLAnge (1)

Case Report

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Hysterectomy and Salpingectomy on Non Classical Obstructed Hemivagina and Ipsilateral Renal Agenesis (OHVIRA) Syndrome

Urogynaecologist managing the case, Manuscript Author: 1. BI Santoso: Urogyna


writing Affiliations: - Indonesian Urogyna Urogynaecology Association.


- Obstetrics and GynecologyGynaecology GynecologyGynaecologyDepartementDepartment DepartementDepartment , Faculty

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of Medicine University of Indonesia – Cipto Cipto Mangunkusumo Hospital,


Jakarta – Indonesia. Indonesia.


FINANCIAL DISCLAIMER/ CONFLICT OF INTEREST: NONE

2. Gita Nurul Hidayah: Obstetrics and GynecologyGynaecology


Residence assist assist in managing the case, Manuscript Manuscript writing Affiliation: - Obstetrics and GynecologyGynaecology GynecologyGynaecologyDepartementDepartment DepartementDepartment , Faculty

Formatted: English Formatted: English (Indonesia) Formatted: English Formatted: English (Indonesia)

of Medicine University of Indonesia – Cipto Cipto Mangunkusumo Hospital,


Jakarta – Indonesia. Indonesia.


FINANCIAL DISCLAIMER/ CONFLICT OF INTEREST: NONE 1

Corresponding address: [email protected], +628568997088

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Word count: 993

Abstract

The OHVIRA (Obstructed Hemivagina and Ipsilateral Renal Agenesis) syndrome is a rare congenital malformation of the urogenital tract. An 18 year old womanMiss 18 years old with recurrent dysmenorrheal dysmenorrhea was being referred to us. Two years ago before referral, she came presented to

another consultant gynaecologist with the same symptoms and at that time to a

gynecologist with dysmenorrheal, and underwenta laparotomy was performed because of a

a

laparotomy due to suspected endometriosisendometrioma cyst. During surgery, the gynecologist found a didelphys uterus didelphys with a right hematometra was found and evacuated the hematometra was evacuated. Recently, the dysmenorrhea symptoms d ysmenorrheal recurred and the patient was presented to us. On physical examination we found an uterine enlargement with no vaginal bulging. Our UltrasoundOn ultrasound founda Didelphys uterus didelphys with a normal left uterine cavity and cervical canal was seen next to a hematometra and ; right hematometra and right hematosalpinx on the right side. Intravenous pyelography revealed absence of the Rr ight kidney. was nonvisualized in intravenous pyelography. We performed a We did laparotomy right salpingectomy and right hysterectomy through laparotomy. On six months follow up, no recurrent pathology was found. The first initial diagnosis was hard duechallenging due to the rareness of the syndrome and the non-clasical manifestation. For In such a case, with history of conservative surgical treatment, we suggest a hysterectomy and salpingectomy.

Keywords

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OHVIRA. Herlyn-Werner-Wunderlich. Didelphys.

Introduction Prevalence of The obstructive Müllerian anomalies prevalence is 0.1% and 3.8.1 The Obstructed

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Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) syndrome, also known as Herlyn-WernerWunderlich syndrome, is caused by a disorder in the lateral and vertical fusion on of the? Müllerian

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mulerrian duct. It is defined as a didelphys uterus withdidelphys with unilateral obstructed


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hemivagina and ipsilateral renal agenesis.


The rareness of thise case syndrome makes the diagnosis difficult/challenging. The classical signs and symptoms are a post menarche girl with pelvic pain and vaginal bulging4 despite regular menstrual bleeding combined with, and the ipsilateral renal agenesis found in on imaging. Other s describes the

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paravaginal cystic mass containing old blood with ipsilateral renal agenesis in a patient whose worsening dysmenorrhea dysmenorrheal since menarche as the pathognomonic.5 However, OHVIRA could present with various and nonspecific clinical maniffestationmanifestations . Our case was a non


classical OHVIRA syndrome and which was misdiagnosed at initial presentation. once got misdiagnosed.

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uterus didelphys. (Figure 1). No right kidney was visualised on ultrasound examination. Absence of


the right kidney, was confirmed by non visualized right kidney on intravenous pyelography.



Figure 1. No connection of the right uterus to the left uterus. No right cervix.

A laparotomy was performed on which we found a Then we did laparotomy an found didelphys


uterus didelphys with a right hematosalpingx ( size 15 x 8 x 6 cm) and an enlarged right uterus


enlarged ( size 20 x 12 x 10 cm). The Lleft corpus of the uterus, left tube and both ovaries were normal. On the right uterus we found no cervical part and no connection to the left uterineuterus. We

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did performed a right salpingectomy and right hysterectomy. (On further exploration, we found appendicitis after which was decided to perform appendectomy  relevance? and without clinical signs how could this be diagnosed, my suggestion would be to leave this out).

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The histopathologic histopathology results for the takenof the right uterus and right salphing was


chronic endometritis and adenomyosis. The right salping showed, chronic salphingitis and hydrosalphinxing with external endometriosis. ( For the appendices, the histopathologic result was


chronic appendicitis, external endometriosis not yet could be excluded, no sign of malignancy. ?) The histopathologic results also confirmed that macroscopically, there was no cervical part or vaginal part of the right uterus; and microscopically there were no endocervics, ectocervics nor vaginal epithelial

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tissue.

The Ppatient had an uncomplicated post operative recovery and went homewas discharged in good condition two days after surgery in good condition. On six months post operative evaluation, there

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was no pathology found in clinical exam or in ultrasound exam. What about symptoms?

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Discussion

Our case has some variation compared to the classical OHVIRA syndrome. There was no right cervical part, no right vaginal part, and no longitudinal vaginal septum. The right uterine uterus was


completely separated from the left uterine uterus by a well established uterine wall (? Uterine wall?


myometrium). There was no connection from the right uterus to the left cervix or to the vagina, as we


did not find vaginal bulging on physical exam, nor onthe ultrasound imaging (Figure 2). The


supporting diagnostic examination used in our case was ultrasound imaging and intravenous


pyelography. Other recommends Magnetic Resonance Imaging and lLaparoscopy for supporting establishing the diagnosis.2


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Figure 2. Schematic Illustration of our OHVIRA case

The primary management of OHVIRA syndrome is vaginoplasty to relieve the obstruction and pelvic pain,1,3,4 and also formaintain fertility sparing.5 But Since as we have knownwe were preoperatively informed preoperatively from through the physical and ultrasound examination about the absence of a connection between the right and left uterus as well as an absence of a right cervicovaginal area, we


knew vaginoplasty was not a suitable option in this patient. and ultrasound imaging, our case had no connection of the right uterine to the left uterine nor to the cervicovaginal area so that the


vaginoplasty was not suitable. Uterine septum excision was also not suitable since the uterus was completely separated by well established myometrium as previously shownestablished from on


ultrasound imaging. Considering the recurrence of symptoms after her first surgery and the pathology found, we decided to do perform a right salpingectomy and right hysterectomy. Our histopathologic exam confirmed that macroscopically and microscopically there was no cervical part, or vaginal part of the right uterus.

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However, another report suggest not to do perform a hemihsysterectomy with or without 2

salpingoophorectomy in view of possible detrimental effects on fertility potentialto improve fertility.

(but you did not perform nor consider an oophorectomy so might want to put salpinectomy here and

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not salpingoophorectomy) However , suchT his report was however described amade on a classic OHVIRA case with hemi-hematocolpos, with no hematometra, no hematosalpinx, and no history of failed conservative surgical management. The more aggressive surgical treatment in our case was also


supported by the foreseen difficulty of long term follow up due to the financial situation and geographic location of our patient. One of the challenges of working in our country consisting of more than 17 thousand islands. also preferred for our patient since long term follow up would be


difficult. This is due to the financial problem of our patient, and the follow up visit would be difficult as our country consists of more than 17 thousands islands.

Conclusion

Our case was a non classical OHVIRA syndrome. The absent of vaginal buldingbuldging shall does not exclude the OHVIRA diagnosis. As we found no pathology on six month follow up, we suggest


to doto consider performing a the unilateral hysterectomy and salpingectomy for OHVIRA syndrome


with rudimentary unilateral uterus and reccurencerecurrence of complains symptoms despite of


previous conservative surgical treatment.

Consent

Written informed consent was obtained from the patient for publication of this case report and any accompanying images.

Awacknowledgement

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Thank you for Mr. Jan-Paul W.R. Roovers, M.D., PhD, uro-gynaecologist

who has reviewed this


manuscript; and dr. Aria Wibawa, OBGYN, consultant of fetomaternal for the excellent ultrasound imaging.

Conflict of Interest

None

References

1. V DaCosta, L Christie, S Wynter, J Harriott, J Frederick. Uterus Bicornis Bicollis, Imperforate Hemivagina and Ipsilateral Renal Agenesis Case Report and Literature Review. West Indian Med J 2009; 58 (4): 379. 2. Andujar MTA, Galan EVA, Casas CC. Acute Urinary Retention Caused by OHVIRA Syndrome. Int Urogynecol J. DOI 10.1007/s00192-013-2201-0. Published online August 20th 2013. 3. Han B, Herndon CN, Rosen MP, Wang ZJ, Daldrup-Link H. Uterine didelphys associated with obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome. Radiology Case Reports. [Online] 2010;5:327. 4. DK Shah, MR Laufer. Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome with a single uterus.Fertility and Sterility Vol. 96, No. 1, July 2011 5. C Hoeffel, M Olivier, C Scheffler, C Chelle, JC Hoeffel. Uterus Didelphys, Obstructed Hemivagina and Ipsilateral Renal Agenesis. European Journal of Radiology 25 (1997) 246-248.

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OHVIRA Revised Feb 8th 2014_deLAnge (1)

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