Ohvira syndrome with rare presentations: Case report
OHVIRA syndrome, also known as Herlyn-Werner Wunderlich
syndrome (HWW syndrome), is a Mullerian duct anomaly which is associated with
uterus didelphys, unilateral obstructed hemivagina, and ipsilateral renal
agenesis. OHVIRA syndrome belongs to the group of ORTAs (Obstructive
reproductive tract abnormalities) with incidence varying between 0.1% and 3.8%
in the general female population and 7% in all mullerian anomalies. The patient
presents with varying symptoms with most common symptoms being pelvic pain,
vaginal mass and rarely primary infertility ; and usually presenting after
menarche. The average age of diagnosis ranged from 10- 29 years with 14 years
as the median age and pain being the most common symptom.
A 20-year old unmarried patient who was apparently normal
one month ago when she developed spotting per vagina for one week, changed around
1 pad per day and was associated with white discharge per vagina. It was not
associated with pain or passage of clots. She had no history of similar
complaints in the past. Menarche attained at 11 years of age with past cycles
of regular length and no menstrual complaints. Patient had consulted on OPD
basis for the same for which USG abdomen and pelvis was done and it showed a
bicornuate uterus with early PCOS changes and right lateral wall vaginal cyst.
Patient was a known case of unilateral renal agensis since birth. To rule out
anomalies, MRI pelvis was done which showed OHVIRA syndrome with pyometra.
Other haematological parameters were within normal limits.
Purslow, in 1922, first reported this syndrome of obstructed
hemivagina and ipsilateral renal anomaly. Subsequently, in 1983, Herlyn and
Werner recognised similar cases analogous to the anomaly and since then the
anomaly has been termed as “Herlyn – Werner – Wunderlich” syndrome. To aid in
easy communication of the syndrome, in 2007, Smith and Laufer proposed the
acronym of OHVIRA.
High level of clinical acumen is required for the early
diagnosis of this syndrome and prompt correction of the abnormality to preserve
the fertility of female. The modalities available for diagnosis and surgical planning
include Ultrasound and MRI. Even though USG can help in diagnosis, MRI is
superior to USG in that it aids in better characterization of uterine shape and
relationship of adjacent organs with the uterus due to wider field of view and
multiplanar images. It also indicates the presence of pyometra / hematosalphinx
which are uncommon presentations associated with the syndrome.
The preferred method of treating obstructed hemivagina is
resection of the vaginal septum. A limited resection marsupialization and the
insertion of a Foley’s catheter may be carried out during an initial surgical
procedure in situations when the obstructed hemivagina reaches the hymeneal
ring, enabling the remaining vaginal septum to be removed later. In addition,
particularly in young girls, hysteroscopic excision of the septum under
transabdominal ultrasound guidance may be performed to preserve hymenal
integrity. Unless tubal disease is suspected, diagnostic laparoscopy is not
often advised.
Early diagnosis and surgical correction of the hemivagina is
essential for preserving the reproductive potential of the patients. With the
development of high tech diagnostic tools, such rare syndromes can be diagnosed
early and defects can be promptly corrected for the patient to have a normal
reproductive life.
Source: Sujatha M. S et al. / Indian Journal of Obstetrics
and Gynecology Research 2024;11(1):100–104; https://doi.org/10.18231/j.ijogr.2024.019
