OHVIRA Revised Feb 8th 2014_deLAnge (1)

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Case Report

Hysterectomy and Salpingectomy on Non Classical

Obstructed Hemivagina and Ipsilateral Renal Agenesis

(OHVIRA) Syndrome

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

writing

Affiliations:

-Indonesian Urogynaecology Association.-Obstetrics and GynecologyGynaecologyDepartementDepartment , Faculty

of Medicine University of Indonesia Cipto Mangunkusumo Hospital,

Jakarta Indonesia.

FINANCIAL DISCLAIMER/ CONFLICT OF INTEREST: NONE

2. Gita Nurul Hidayah: Obstetrics and GynecologyGynaecology

Residence assist in managing the case, Manuscript writing

Affiliation:

-Obstetrics and GynecologyGynaecologyDepartementDepartment , Facultyof Medicine University of Indonesia

Cipto Mangunkusumo Hospital,

Jakarta Indonesia.

FINANCIAL DISCLAIMER/ CONFLICT OF INTEREST: NONE

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Corresponding address:[email protected],+628568997088

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 hematometrawas

evacuated. Recently,the dysmenorrhea symptoms dysmenorrheal recurredand 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 canalwas seen next to a hematometra and ; right hematometra and right hematosalpinxon the

right side. Intravenous pyelography revealed absence of the Rright kidney. was nonvisualized in

intravenous pyelography. We performed a We did laparotomy right salpingectomy and right

hysterectomythrough laparotomy. On six months follow up, no recurrent pathologywasfound. 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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mailto:[email protected]:[email protected]:[email protected]:[email protected]


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OHVIRA. Herlyn-Werner-Wunderlich. Didelphys.

Introduction

Prevalence of The obstructive Mllerian anomalies prevalence is 0.1% and 3.8.1

The Obstructed

Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) syndrome, also known as Herlyn-Werner-

Wunderlich syndrome, is caused by a disorder inthe lateral and vertical fusion on of the? Mllerian

mulerrian duct.2 It is defined as a didelphys uterus withdidelphys with unilateral obstructed

hemivagina and ipsilateral renal agenesis.3

The rareness of thisecase syndrome makes the diagnosis difficult/challenging. The classical signsand

symptoms are apost menarche girl with pelvic pain and vaginal bulging4 despite regular menstrual

bleedingcombined with,and the ipsilateral renal agenesis found in on imaging. Othersdescribesthe

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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uterusdidelphys. (Figure 1). No right kidney was visualised on ultrasoundexamination. Absence of

the right kidney, was confirmed by non visualized right kidney onintravenous 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

did performed a right salpingectomy and right hysterectomy. (On further exploration, we found

appendicitis after which was decided to perform appendectomyrelevance? 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 resultsalso confirmed that macroscopically, there was no cervicalpart or vaginal part

of the right uterus; and microscopically there were no endocervics, ectocervics nor vaginal epithelial

tissue.

The Ppatient had an uncomplicated post operative recovery and went homewas discharged in good

condition two days after surgeryin good condition. On six months post operative evaluation, there

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

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 uterusby a well establisheduterine 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 fertilitysparing.5But Since as we have knownwe were preoperatively

informedpreoperatively from through thephysical and ultrasound examinationabout 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

salpingoophorectomy in view of possible detrimental effects on fertility potentialto improve fertility.2

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

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 vaginalbuldingbuldgingshall does

not exclude the OHVIRA diagnosis. As we found no pathology on six month follow up, we suggest

to doto consider performing a theunilateral 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, ImperforateHemivagina 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 20

th2013.

3. Han B, Herndon CN, Rosen MP, Wang ZJ, Daldrup-Link H. Uterine didelphys associated withobstructed 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, ObstructedHemivagina and Ipsilateral Renal Agenesis.European Journal of Radiology 25 (1997) 246-248.



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