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.
Disclaimer: This website is primarily for healthcare professionals. The content here does not replace medical advice and should not be used as medical, diagnostic, endorsement, treatment, or prescription advice. Medical science evolves rapidly, and we strive to keep our information current. If you find any discrepancies, please contact us at corrections@medicaldialogues.in. Read our Correction Policy here. Nothing here should be used as a substitute for medical advice, diagnosis, or treatment. We do not endorse any healthcare advice that contradicts a physician's guidance. Use of this site is subject to our Terms of Use, Privacy Policy, and Advertisement Policy. For more details, read our Full Disclaimer here.
NOTE: Join us in combating medical misinformation. If you encounter a questionable health, medical, or medical education claim, email us at factcheck@medicaldialogues.in for evaluation.