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CASE REPORT
Year : 2017  |  Volume : 3  |  Issue : 1  |  Page : 37-39

Uterus Didelphys with an Obstructed Unilateral Vagina by a Transverse Vaginal Septum Associated with Ipsilateral Renal Agenesis: OHVIRA Syndrome − An Unusual Case Report and Review of Literature


Department of Obstetrics and Gynaecology, Maulana Azad Medical College, New Delhi, India

Date of Web Publication1-Mar-2017

Correspondence Address:
Asmita Rathore
Department of Obstetrics and Gynaecology, Maulana Azad Medical College, New Delhi - 110 002
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2394-7438.201101

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  Abstract 

The triad of uterine didelphys, obstructed hemivagina, and unilateral renal agenesis, called as OHVIRA syndrome is the least common of all Mullerian malformations. We report a case of a 16-year-old girl who presented with lower abdominal pain and amenorrhea. Radiological investigations led us to the diagnosis of OHVIRA syndrome. She was hospitalized and operated upon. OHVIRA syndrome, though a rare Mullerian anomaly, should be kept as a differential diagnosis in a young girl presenting with cyclical pain and amenorrhea with renal agenesis so that surgical management can be performed before long-term complications set in.

Keywords: Mullerian, obstructed hemivagina, OHVIRA, renal agenesis, uterine didelphys


How to cite this article:
Chhikara U, Sahu L, Rathore A. Uterus Didelphys with an Obstructed Unilateral Vagina by a Transverse Vaginal Septum Associated with Ipsilateral Renal Agenesis: OHVIRA Syndrome − An Unusual Case Report and Review of Literature. MAMC J Med Sci 2017;3:37-9

How to cite this URL:
Chhikara U, Sahu L, Rathore A. Uterus Didelphys with an Obstructed Unilateral Vagina by a Transverse Vaginal Septum Associated with Ipsilateral Renal Agenesis: OHVIRA Syndrome − An Unusual Case Report and Review of Literature. MAMC J Med Sci [serial online] 2017 [cited 2019 Oct 18];3:37-9. Available from: http://www.mamcjms.in/text.asp?2017/3/1/37/201101


  Introduction Top


Triad of uterine didelphys, obstructed hemivagina, and unilateral renal agenesis is called OHVIRA syndrome or Herlyn-Werner-Wunderlich syndrome. True incidence of uterine anomalies is difficult to determine. Mullerian malformations have an estimated incidence of 1.1%–3.5%.[1] The least common of these malformations is OHVIRA syndrome.


  Case Report Top


A 16-year-old unmarried girl presented with spasmodic and periodic lower abdominal pain with amenorrhea for 4 months. Her age of attaining menarche was 14 years and she had regular cycles previously, lasting for 3–4 days and occurring at 30-day interval. Physical examination revealed 14-week size abdominopelvic mass with mild abdominal tenderness. External genital inspection showed normal external genitalia with torn hymen.

Magnetic resonance imaging pelvis revealed didelphys uterus with obstruction in the right hemivagina resulting in hematometra and hematocolpos associated with ipsilateral renal agenesis [Figure 1] and [Figure 2]. Both ovaries were normal. Contrast-enhanced computed tomography revealed normal left uterine moiety with absence of right kidney with enlarged left kidney with mild hydroureteronephrosis (obstructive uropathy).
Figure 1: Magnetic resonance image showing ipsilateral right-sided renal agenesis with hematometra with mild left hydroureteronephrosis secondary to obstruction

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Figure 2: Magnetic resonance image showing uterine didelphys with right-sided hematometra, hematocolpos, and hematosalpinx

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Vaginal septum resection and drainage of hematometra were performed. Under general anesthesia, pelvic examination was performed. On per-speculum examination, visible bulge was seen anteriorly [Figure 3], 4–5 cm on the right side of vagina. On per-vaginal examination, a mass of 20-week size was palpable. Chocolate-colored altered collected blood about 300 ml was drained. Thick right-sided vaginal septum was seen [Figure 4]. Septum resection was performed with marsupialization of the cut edges of the septum with adjacent vaginal mucosa. Bilateral cervices were identified. Mould prepared with amnion and foam was placed over the right side of vagina to prevent reobstruction due to circumferential scarring and vaginal stenosis.
Figure 3: Intraoperative image showing visible bulge anteriorly

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Figure 4: Intraoperative image showing thick right-sided vaginal septum after incision and drainage of hematometra

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Mould removal was performed after 48 h followed by ceramic mould placement. It was kept in place for 3 weeks. Serial digital dilatations were performed. The patient resumed normal regular menses after 40 days. On postsurgery, ultrasound pelvis revealed didelphys uterus with no intrauterine collection with endometrial thickness of 3 mm and 4 mm of both endometrial cavities [Figure 5].
Figure 5: Postoperative ultrasound image after 6 months showing empty uterine cavity of uterine didelphys with endometrial thickness of 3 mm and 4 mm of right and left sides, respectively

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  Discussion Top


Mullerian duct defects are classified by the American Fertility Society as segmental, unicornuate, didelphys, bicornuate, septate, arcuate, or diethylstilbestrol related.[2] OHVIRA syndrome has an incidence of 0.16%–3.8% of these Mullerian anomalies.[3] The combination of obstructed hemivagina and uterus didelphys was first reported in 1922,[4] but the triad including unilateral renal agenesis was initially reported in 1950.[5] Some reports have shown association of OHVIRA syndrome with high-riding aortic bifurcation, inferior vena-cava duplication, intestinal malrotation, and ovarian malposition.[6]

Patients with this syndrome may present with a wide variety of symptoms such as secondary amenorrhea, dysmenorrhea, chronic pelvic pain which may be cyclical in nature, or palpable pelvic swelling due to collection in obstructed hemivagina.[7],[8] Sometimes, continuous menstrual flow from the unobstructed hemivagina may be mistaken for normal menstruation delaying the diagnosis. If it is not diagnosed and managed early, reversed menstruation through  Fallopian tube More Details may occur which may lead to sequelae such as endometriosis, pelvic adhesions, infertility, and pelvic inflammatory disease (PID).[8] Didelphys uterus is associated with reproductive issues such as miscarriages, preterm labor, and placental dysfunction in the future. Some authors have shown association of urinary tract anomalies such as renal agenesis, ectopic kidney, double renal pelvis, and horseshoe kidney with Mullerian anomalies. Most commonly associated anomaly with this syndrome is renal agenesis.[9],[10]


  Conclusion Top


OHVIRA syndrome is a rare Mullerian anomaly with potential short- and long-term complications. It can be suspected if normal menstruating girl presents with cyclical pain with mass and renal agenesis. Surgery for it should be performed before serious complications such as endometriosis, PID, and obstructive uropathy arise in these women. Awareness of such anomalies helps in early and prompt diagnosis.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Strassmann EO. Fertility and unification of double uterus. Fertil Steril 1966;17:165-76.  Back to cited text no. 1
    
2.
The American Fertility Society classifications of adnexal adhesions, distal tubal occlusion, tubal occlusion secondary to tubal ligation, tubal pregnancies, müllerian anomalies and intrauterine adhesions. Fertil Steril 1988;49:944-55.  Back to cited text no. 2
    
3.
Smith NA, Laufer MR. Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome: Management and follow-up. Fertil Steril 2007;87:918-22.  Back to cited text no. 3
    
4.
Purslow CE. A case of unilateral hematocolpos, hematometra and hematosalpinx. J Obstet Gynaecol Br Emp 1922;29:643.  Back to cited text no. 4
    
5.
Embrey MP. A case of uterus didelphys with unilateral gynatresia. Br Med J 1950;1:820-1.  Back to cited text no. 5
    
6.
Coskun A, Okur N, Ozdemir O, Kiran G, Arýkan DC. Uterus didelphys with an obstructed unilateral vagina by a transverse vaginal septum associated with ipsilateral renal agenesis, duplication of inferior vena cava, high-riding aortic bifurcation, and intestinal malrotation: A case report. Fertil Steril 2008;90:2006.e9-11.  Back to cited text no. 6
    
7.
Vercellini P, Daguati R, Somigliana E, Vigano P, Lanzani A, Fedele L. Asymmetrical lateral distribution of obstructed hemivagina and renal agenesis in women with uterus didelphys: Institutional case series and a systematic literature review. Fertil Steril 2007;87:719-24.  Back to cited text no. 7
    
8.
Jindal G, Kachhawa S, Meena GL, Dhakar G. Uterus didelphys with unilateral obstructed hemivagina with hematometrocolpos and hematosalpinx with ipsilateral renal agenesis. J Hum Reprod Sci 2009;2:87-9.  Back to cited text no. 8
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9.
Speroff L, Glass RH, Kase NG. Development of the mullerian system. In: Mitchell C, editor. Clinical Gynecologic Endocrinology and Infertility. 6th ed. Baltimore, MD: Lippincott, Williams and Wilkins; 1998. p. 124.  Back to cited text no. 9
    
10.
Fedele L, Bianchi S, Agnoli B, Tozzi L, Vignali M. Urinary tract anomalies associated with unicornuate uterus. J Urol 1996;155:847-8.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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