Author
Information
Saxena N*,
Channawar S**, Khadkikar R**, Chauhan AR
(* Third
Year Resident, ** Assistant Professor, *** Additional Professor. Department of Obstetrics
and Gynecology, Seth GS Medical college and KEM
Hospital , Mumbai , India
Abstract
A rare
congenital mullerian anomaly which includes uterus didelphys, obstructed
hemivagina and ipsilateral renal anomaly is a well recognized entity described
as OHVIRA syndrome or Herlyn- Werner-Wunderlich (HWW) syndrome. It is a challenging
diagnosis as patient may have normal menses. We report the management of a 14
year old girl presenting with pain in abdomen and dysmenorrhea diagnosed as
OHVIRA syndrome.
Introduction
Gynecologists are often fascinated by cases of mullerian anomalies.
These include a spectrum of developmental defects ranging from uterine and
vaginal agenesis to duplication of the uterus and vagina to minor uterine
cavity abnormalities. Mullerian anomalies are frequently associated with renal
anomalies. The incidence rate ranges from 0.2 to 7.4%. [1] Didelphic
uterus is a rare mullerian anomaly composed of two separate normal sized uteri
and endocervical canals with cervices that are fused at the lower uterine
segment. Rarer still is the association of didelphic uterus with obstruction of
unilateral vagina; it is frequently associated with ipsilateral renal agenesis.
This is known as OHVIRA (Obstructed
Hemivagina with Ipsilateral
Renal Anomaly) syndrome or Herlyn -Werner-Wunderlich syndrome,
first reported by Wilson
Case Report
A 14 year
old girl presented with complaints of irregular menses, pain in abdomen and
dysmenorrhoea since last 3 months. Patient had attained menarche 9 months ago,
and had regular menses for first 6 months. However since the past 3 months,
bleeding occurred irregularly with increasing dysmenorrhea and lower abdominal
pain even in between the menstrual cycle. She was referred with an
ultrasonography suggestive of bicornuate uterus with hematocolpos of
approximately 8.6 x 4.6 x 3.6 cm, with absence of right kidney in right renal
fossa, probably renal agenesis. On examination, patient was thin built with
normal height for her age. Her general condition was fair, mild pallor was
present, systemic examination was normal; abdomen was soft with no tenderness
and no palpable lump. Her secondary sexual characters were developed as per age
(Tanner stage 2-3). Local examination was normal; detailed internal examination
was deferred at this time.
A repeat
ultrasonography showed bicornuate uterus with both cornua of approximately
equal size, both endometrial cavities normal with endometrial thickness of 5.5
mm, and no fluid collection in the endometrium. There was evidence of a well
defined elongated cystic lesion of size 7.3 x 5.3 x 4.2 cm with echoes within
it seen below the level of uterus in the pelvis in continuity with uterine
cavity on the right side. Another well defined small slightly echogenic soft
tissue seen adjacent to the cystic structure appeared to communicate with
uterine cavity on the right side. Magnetic resonance imaging (MRI) was done
which confirmed didelphic uterus with hematocolpos of right side and absent
right kidney. Hence a final diagnosis of OHVIRA syndrome was made based on the
above clinical picture and findings.
Figure 1:
MRI showing hematocolpos
Examination
under anesthesia with drainage of hematocolpos was planned. Speculum
examination revealed non- intact hymen, roomy vagina of adequate length and
caliber, with a normal cervix seen on left side. High up in the vagina on the
right side, a small dimple was seen over a prominent bulge. On per vaginal
examination, a tense collection of approximately 4 x 5 cm was felt on anterior
and right side. A transverse incision of 1- 1.5 cm was taken over the right
sided bulging area and approximately 50 ml of chocolate colored fluid was
drained suggestive of hematocolpos. After complete drainage, a small normal
cervix was visualized inside the vaginal opening. The vaginal edges were
underrun with simple sutures of polyglactin No 2 -0 and the right vaginal
opening was kept patent by placing a No. 14 Foley catheter with the bulb
inflated with 25 ml normal saline.
A week
later, the Foley catheter was removed under anesthesia and the patient was
re-examined. The right hemivagina was patent and the right normal cervix was
seen. Excision of the edges of the redundant right hemivagina was done with
suturing of edges to the right cervix, to restore the normal anatomy and to
preserve the normal outflow tract. Patient withstood the surgical procedures
well, was discharged on day 4 of second surgery with advice to follow up.
Figure 2:
Left -sided normal cervix
Figure 3:
Intraoperative image - hematocolpos
Figure 4:
Two cervices and patent hemivagina
Discussion
The HWW or OHVIRA syndrome includes didelphic uterus which is
characterized by complete or partial duplication of the vagina, cervix, and
uterus and is included in class III of American Fertility Society (AFS)
classification. The reported incidence in various case series is 0.1-3.8%.
[2, 3] This syndrome is
usually diagnosed at or after puberty with cases presenting as abdominal pain
or dysmenorrhea, due to the associated hematocolpos or hematometra, due to
obstruction to flow of menstrual products which collect in blind hemivagina.
Because of regular menstruation in these patients, diagnosis is often delayed.
Patients can present with complications like endometriosis, hydrosalpinx,
secondary infection, pelvic adhesions causing chronic pelvic pain. Thus if not
diagnosed and treated in time can lead to long term problems like infertility.
The diagnostic modalities which are commonly used include ultrasonography and
MRI. USG can diagnose the collection inside uterus or vagina but cannot
identify the type of mullerian anomaly while MRI has multiplaner and tissue
characterization ability giving precise
information about uterine morphology,
the continuity with each vaginal (obstructed and non-obstructed) lumen. MRI has
more sensitivity in detecting the uterine contour, the shape of cavity,
presence of the septum, as well as presence of the associated pathology such as
endometriosis, pelvic inflammation, and adhesions. [4, 5] Thus MRI
is the gold standard method for accurate diagnosis and allows the most
appropriate treatment option to be used.
Laparoscopy should be reserved when the diagnosis is not confirmed
after imaging or when MRI is not available. Surgical treatment includes
excision of vaginal septum.[6, 7] Hysteroscopic resection of the
septum can be carried out under transabdominal USG guidance, to preserve
hymenal integrity, especially in young females.[8] It is a feasible, convenient, and effective tool in the management of
such cases.
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Citation
Saxena N, Channawar S, Khadkikar R, Chauhan AR. OHVIRA syndrome. JPGO 2014.
Volume 1 Issue 12. Available from: http://www.jpgo.org/2014/12/ohvira-syndrome_1.html