Understanding OHVIRA Syndrome: Causes, Symptoms, and Management

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OHVIRA Syndrome

by Alike Medical Team ∙ Updated on June 13, 2023

Additional names

This group contains additional names: - Herlyn Werner Wunderlich syndrome - Obstructed Hemivagina And Ipsilateral Renal Anomaly

General

OHVIRA is an extremely rare syndrome characterized by a congenital birth defect of the lower abdominal and pelvic organs. It is a type of abnormality of the Mullerian ducts. In most cases, OHVIRA presents as a double uterus with unilateral obstructed (or blind) hemivagina and ipsilateral renal agenesis. It can also affect the urethra, urethral sphincter, ureters, bladder and spleen. Although the true incidence is unknown, it has been reported to be between 0.1% and 3%.

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Signs & symptoms

Although there are no specific symptoms for this condition, common complains include progressively increasing pelvic pain during menstruation and hematocolpos due to the buildup of blood in the body. Other symptoms may include swelling of the abdomen, nausea and vomiting during menstruation, and pelvic pain. Fertility may also be affected. The condition often leads to an increased need for C-sections due to the smaller uteruses.

Diagnosis

The condition is often diagnosed through an MRI or ultrasound. Consulting a specialist (in this case a gynecologist) is recommended.

Treatment

Symptoms can be ameliorated with medication and surgery. In most cases, the blind hemi-vagina is opened, and the fluid drained. In adolescents particularly, vaginoscopic incision of the oblique vaginal septum is a viable option. Pregnancies in women with OHVIRA are categorized as high-risk due to the size and shape of the uteri and cervices as well as the reduced kidney function. In addition, women with undiagnosed OHVIRA can experience genital bleeding during pregnancy and may require hospitalization.mExpectant mothers are often managed with cervical sutures and C-sections to prevent fetal distress during labour.

Note

☝ We provide information on prescription and over-the-counter medicines, diagnosis, procedures and lab tests. This material is provided for educational purposes only and is not medical advice, diagnosis or treatment.

National Institutes of Health ∙ World Health Organization ∙ MedlinePluse ∙ Centers for Disease Control and Prevention

☝ All information has been reviewed by certified physicians from Alike

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