OHVIRA Syndrome: Causes, Symptoms, and Treatment Options

If you or your loved one has recently been diagnosed with OHVIRA Syndrome (Obstructed Hemivagina with Ipsilateral Renal Anomaly), you might feel anxious, confused, and even overwhelmed.

As a gynecologist who frequently treats young girls and women with rare Müllerian anomalies, I want to simplify this condition for you and help you understand the causes, symptoms, diagnosis, and most importantly, the treatment—so you can make the right decision with confidence.

What is OHVIRA Syndrome?

OHVIRA stands for obstructed hemivagina with ipsilateral renal anomaly. It is also known as Herlyn-Werner-Wunderlich Syndrome. It is one of the rare congenital anomalies that falls under the category of Müllerian anomalies

In simple terms, it means that from birth, the uterus and vagina did not form in the usual way.

Here’s what typically happens:

• The patient has a didelphys uterus (a double uterus).

• One side develops an obstructed hemivagina (a blocked vaginal passage).

• On the same side, the kidney fails to develop (renal agenesis).

Although this sounds complicated, the condition can be effectively treated with modern surgical techniques.

What causes OHVIRA Syndrome?

This syndrome develops due to a disruption in the normal fusion or canalization of the Müllerian ducts during fetal development.

Any disruption in this process can cause one side of the vagina to remain blocked, along with kidney absence on the same side.

Think of it as an “incomplete connection” during the body’s development. It’s important to remember—this is not your fault, nor is it caused by lifestyle, diet, or anything parents did during pregnancy.

What are the symptoms of OHVIRA Syndrome?

Most patients are diagnosed in their teenage years, usually between 16 and 17 years old, when menstrual blood accumulates behind the obstruction, called hematocolpos.

The most common symptoms include:

• Severe lower abdominal or pelvic pain, especially during periods

• Obstruction while urinating/Difficulty or pain during urination

• Recurrent urinary tract infections (UTIs)

• A feeling of pelvic fullness/mass or pressure in the vaginal area 

Because it is so rare, many girls are unfortunately misdiagnosed due to a lack of experience, where the condition is mistaken for an imperforate hymen.

This is a significant issue in countries like India, resulting in many young girls undergoing repeated surgeries without being referred to a specialist.

In fact, the majority of girls treated with OHVIRA syndrome have undergone at least one previous surgery and only come to our unit for the second.

How is OHVIRA Syndrome diagnosed?

Timely and accurate diagnosis is the key to avoiding unnecessary pain and surgeries.

The most effective way to diagnose a patient with OHVIRA syndrome is through a thorough pelvic ultrasound. An MRI of the pelvis with the abdomen is also an option, which will clearly demonstrate the absence of a kidney on the side with an obstruction due to the obstructed hemivagina.

The ultrasound or MRI to be performed does not require any special contrast injection or additional software for accurate disease reporting.

No special contrast or advanced software is required—but the radiologist must be trained and experienced, or else this rare condition can easily be missed.

If you are concerned about symptoms or have been diagnosed with OHVIRA syndrome, we are here to help. Contact our specialist unit at 1800-268-4000 today to schedule a consultation and take the first step toward recovery.

What are the treatment options for OHVIRA Syndrome?

Here’s the part most patients and parents are anxious about—treatment.

For any anatomical anomaly, particularly a Müllerian tract anomaly, surgical management is the sole treatment.  But don’t worry—modern surgical methods are safe, minimally invasive, and fertility-preserving.

As a referral unit for Müllerian anomalies in India, we often perform repeat surgeries due to previous infections. Patients typically develop a scar along the angle of indentation from the obstructed hemivagina.

How is OHVIRA Syndrome treated at our clinic?

• We use a specialized vaginoscopic approach, performed under laparoscopic or robotic guidance.

• The surgery involves removing the obstructed vaginal segment and realigning vaginal tissues.

• A bipolar resectoscope is used for precision during the procedure.

• After surgery, a Ryles tube is kept in place for 15–20 days.

• Follow-ups are recommended every 6 months.

What is the success rate of OHVIRA syndrome treatment?

• Once the surgery is done, the recurrence rate is zero.

• Fertility outcomes are excellent. Unlike other infertility conditions, women with OHVIRA syndrome usually don’t need  in vitro fertilization (IVF) or assisted reproduction because the uterus, ovaries, and fallopian tubes are healthy and functional.

What are the long-term reproductive outcomes for OHVIRA Syndrome patients?

The good news is—once treated correctly, OHVIRA patients can live normal, healthy lives. Fertility is usually preserved, and women can conceive naturally.

This is why choosing the right specialist and the right surgical technique matters so much.

How common is OHVIRA Syndrome?

It is a rare form of the congenital Müllerian anomaly, but not unknown.

In our unit in Mumbai, we operate on 10–15 OHVIRA patients every year, many of whom are referred from across India after failed previous treatments.

What complications can occur if OHVIRA Syndrome is left untreated?

The most significant complication associated with OHVIRA Syndrome is a lack of timely diagnosis. This is often compounded by incorrect surgical management, where only the hemivagina is cut and not resected completely.

This leads to infections and makes repeated surgeries difficult. Therefore, we request all practitioners to refer such complex Müllerian anomalies to a center of excellence like Shree IVF Clinic.

If you or someone you know has been struggling with unexplained pelvic pain, repeated surgeries, or has recently been diagnosed with OHVIRA Syndrome, please don’t delay. Early, correct treatment makes all the difference.

Book a consultation with our Müllerian anomaly specialist, Dr. Jay Mehta, today to discuss the best treatment plan for OHVIRA Syndrome. Your health, fertility, and future matter—and we are here to help.

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