2. A case of obstructed
hemivagina
with ipsilateral renal
agenesis
(OHVIRA syndrome)
and didelphic uterus
3. OHVIRA syndrome (Obstructed hemivagina with ipsilateral renal
agenesis), also known as Herlyn-Werner-Wunderlich (HWWS), is a rare
Mullerian duct anomaly that results in uterine didelphys, obstructed
vaginal vault, and unilateral renal agenesis.
It may present at birth or soon after the beginning of menses as fluid
collects in the obstructed vaginal vault.
4. The association of uterine anomalies with renal anomalies ipsilateral
to the side of obstruction can be explained by embryologic arrest at
the 8th week of gestation, which simultaneously affects the mullerian
and metanephric ducts. Certain other renal anomalies may also be
associated, such as renal dysplasia, double collecting system and
ectopic ureter
The condition has also been reported to be associated with high-riding
aortic bifurcation, Inferior vena cava (IVC) duplication, intestinal
malrotation and ovarian malposition
5. Case Report
• An 12-year-old girl presented with seven days of abdominal pain.
• Initially she was admitted to surgery dept for concern for … ?acute
appedicitis..? ovarian cyst.
Laboratory examination indicated normal CBC , renal and liver function.
Urinalysis and urine culture were normal .
• CT scan abdomen& pelvis revealed solitary left kidney ( hypertrophied )
,bicornuate uterus,right hematocolpus and normal appendix .
• So patient was refered to gynecologist..
6. Evaluation by Gyn. Doctor ; the patient gave history of menarche
occurred seven months back ,regular menstrual cycle with
dysmenorrhea and her LMP was 11 days back.
At presentation, she was afebrile without back pain, dysuria, chills nor
vaginal discharge.
By inspection there was no imperforate hymen and no bleeding.
8. SO obstructed hemivagina and ipsilateral renal agenesis
was suspected
Magnetic resonance imaging (MRI) confirmed the
diagnosis of right OHVIRA syndrome
She started a course of prophylactic antibiotics and started oral
contraceptive pills to further suppress menstruation.
9. 4 days later & after
taking consent from her
parents she underwent
vaginoscopy under
general anesthesia.
Vaginoscopy identified
the patient’s right hemi-
vaginal bulge
compressing the left
cervix.
10. The hemivaginal bulge was incised (cruciate incision ) by diathermy
allowing drainage of the hematocolpos. This was followed by
insertion of foley’s catheter through the cruciate incision and the
balloon was inflated with 20 ml saline to prevent closure of the
incision and allows drainage of the retained blood .
Her pain has subsequently improved and there is no current plan for
further surgery. She went home after 2 days in good condition
The foley’s catheter was removed after 2 weeks
12. Discussion
The syndrome is a rare entity among females resulting from failure of the
distal Mullerian tubes to fuse normally by the 10th week of gestation.
The triad of obstructed hemivagina and uterus didelphys and ipsilateral
renal anomaly (OHVIRA syndrome) was initially reported in 1950.
13. The incidence of the OHVIRA syndrome is not precisely known, but
according to the literature it is estimated as 0.1–3.5% of all Müllerian
anomalies .
Renal agenesis occurs more frequently in uterus didelphys and can
be found in up to 29.8% of MDAs .
The incidence of müllerian duct anomalies ranges from 0.8% to 4%.
Although its incidence may be as high as 25% in women with recurrent
miscarriages and subfertility .
14. Patients with HWWS are often asymptomatic until menarche when
they present with progressive pain due to hydro- metrocolpos and
hemivaginal obstruction within the first year .
Given the rarity of the syndrome it is frequently misdiagnosed.
Delayed diagnosis can lead to endometriosis.
15. The most common clinical presentation is pelvic pain initiating shortly
after the monarch, associated with a vaginal or pelvic mass and
normal menstrual periods.
Rare presentations may include intermenstrual bleeding, acute
retention of urine, fever, vomiting, and abdominal swelling .
16. Hormonal therapy is used to decrease menstruation and relieve
the dysmenorrhea.
The obstructing vaginal septum seen in this condition is usually
oblique and varies in thickness from very thin to quite thick.
Resection of the vaginal septum is the treatment of choice
Integrity of the hymen represent a major cultural issues in our
community.Hysteroscopic excision of vaginal septum has been
recommended for those patients with good outcome.
17. An obstructed reproductive system is usually drained via external
vaginoplasty.
Hemi- hysterectomy is no longer recommended as pregnancies have
been reported in previously obstructed didelphys uterus after
surgical correction .
Surgeons now make every effort to preserve the obstructed uterus.
18. Postoperative complications of vaginoplasty are uncommon :
in one series of 27 patients , two patients developed vaginal stenosis.
In another series of 43 patients ,one case developed vaginal stricture.
19. We have described a case of OHVIRA syndrome.
Careful consideration must be given in children with renal
malformations to examine the reproductive system given
their common embryology.
Recent advances in the precision and availability of MRI
have allowed the identification and diagnosis of
previously unknown anomalies in OHVIRA syndrome.
In conclusion:
