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
Hospital, ) Mumbai, India
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.
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%.  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
in 1925.  Wilson
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
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. It is a feasible, convenient, and effective tool in the management of such cases.
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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