You are on page 1of 8

Case Report

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

Author: 1. BI Santoso: Urogynecologist managing the case, Manuscript writing Affiliations: - Indonesian Urogynecology Association. - Obstetrics and Gynecology Departement, Faculty of Medicine University of Indonesia Cipto Mangunkusumo Hospital, Jakarta Indonesia. FINANCIAL DISCLAIMER/ CONFLICT OF INTEREST: NONE

2. Gita Nurul Hidayah: Obstetrics and Gynecology Residence assist in managing the case, Manuscript writing Affiliation: - Obstetrics and Gynecology Departement, Faculty of Medicine University of Indonesia Cipto Mangunkusumo Hospital, Jakarta Indonesia. FINANCIAL DISCLAIMER/ CONFLICT OF INTEREST: NONE Corresponding address: nh_gita@yahoo.com, +628568997088

Word count: 993 1

Abstract

The OHVIRA (Obstructed Hemivagina and Ipsilateral Renal Agenesis) syndrome is a rare congenital malformation. Miss 18 years old with recurrent dysmenorrheal was being referred to us. Two years ago she came to a gynecologist with dysmenorrheal, and underwent a laparotomy due to suspected endometriosis cyst. During surgery, the gynecologist found a didelphys uterus with right hematometra and evacuated the hematometra. Recently the dysmenorrheal recurred. On physical exam we found uterine enlargement with no vaginal bulging. Our Ultrasound found Didelphys uterus with normal left uterine cavity and cervical canal; right hematometra and right hematosalpinx. Right kidney was nonvisualized in intravenous pyelography. We did laparotomy right salpingectomy and right hysterectomy. On six months follow up, no recurrent pathology found. The first diagnosis was hard due to the rareness and the nonclasical manifestation. For such case with history of conservative surgical treatment, we suggest a hysterectomy and salpingectomy.

Keywords OHVIRA. Herlyn-Werner-Wunderlich. Didelphys.

Introduction
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 disorder in lateral and vertical fusion on mulerrian duct.2 It is defined as a didelphys uterus with unilateral obstructed hemivagina and ipsilateral renal agenesis.3

The rareness of the case makes the diagnosis difficult. The classical sign and symptoms are post menarche girl with pelvic pain and vaginal bulging4 despite regular menstrual bleeding, and the ipsilateral renal agenesis found in imaging. Other describes the paravaginal cystic mass containing old blood with ipsilateral renal agenesis in a patient whose worsening dysmenorrheal since menarche as the pathognomonic.5 However, OHVIRA could present with various and nonspecific clinical maniffestation. Our case was a non classical OHVIRA syndrome and once got misdiagnosed.

Case Illustration A miss 18 years old was being referred to our center due to recurrent progressing dysmenorrheal. She had her menarche on 14 years old. Two years ago she came to a gynecologist with dysmenorrheal especially on right lower quadrant. She was being diagnosed as endometriosis cyst and underwent a laparotomy. During surgery the gynecologist found a didelphys uterus with right hematometra, and he evacuated the hematometra. After surgery she got menstruation suppression injection for about six months followed by oral menstruation suppression therapy for six months. After the menstruation therapy stop, the menstruation recurs followed with the dysmenorrheal. Then the OBGYN sent the patient to our center.

At physical examination we found an abdominal mass, up to 2 fingers bellow navel. Vaginal examination was not done since the patient decline history of intercourse. Our rectal examination

showed no vaginal bulging, and found enlarged uterus as big as 18 weeks of gestational age. From our ultrasound exam we found didelphys uterus; left uterus with normal cavity and cervical canal; while right uterus with hematometra. On the right uterus there were no connection to the left uterus and there were no cervical part seen the right hematometra. So our analysis was the right hematometra and right hematosalpinx correlated with outflow tract obstruction from the right uterus, as in rudimentary type didelphys uterus. (Figure 1). No right kidney on ultrasound, confirmed by non visualized right kidney on intravenous pyelography.

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

Then we did laparotomy an found didelphys uterus with a right hematosalping ( size 15 x 8 x 6 cm) and an enlarged right uterus enlarged ( size 20 x 12 x 10 cm). Left 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

uterine. We did right salpingectomy and right hysterectomy. On further exploration, we found appendicitis after which was decided to perform appendectomy.

The histopathologic result for the taken right uterus and right salphing was chronic endometritis and adenomyosis, chronic salphingitis and hydrosalphing 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 result 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 tissue.

Patient went home two days after surgery in good condition. On six months post operative evaluation, there was no pathology found in clinical exam or in ultrasound exam.

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 was completely separated from the left uterine by a well establish uterine wall. 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 the ultrasound imaging (Figure 2). The supporting exam used in our case was ultrasound imaging and intravenous pyelography. Other recommends Magnetic Resonance Imaging and Laparoscopy for supporting the diagnosis.2

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 for fertility sparing.5 But as we have known preoperatively from the physical exam 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 shown from ultrasound imaging. Considering the recurrence of symptoms after her first surgery and the pathology found, we decided to do 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.

However, other report suggest not to do hemisysterectomy with or without salpingoophorectomy to improve fertility.2 However, such report was made on a classic OHVIRA case with hemihematocolpos, with no hematometra, no hematosalpinx, and no history of failed conservative surgical management. The more aggressive surgical treatment 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 bulding shall not exclude the OHVIRA diagnosis. As we found no pathology on six month follow up, we suggest to do the unilateral hysterectomy and salpingectomy for OHVIRA syndrome with rudimentary unilateral uterus and reccurence of complains 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 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.

You might also like