Obstructed Hemi-Vagina with Ipsilateral Renal Agenesis - OHVIRA

OHVIRA is a complex condition in which a girl is born with:

• a double uterus;
• a double vagina, with one side obstructed by a wall of tissue that may block menstrual blood from flowing out of the body;
• a missing kidney.  

There may be other symptoms in addition to these three.

OHVIRA stands for "obstructed hemi-vagina with ipsilateral renal agenesis."

Patients can be seen by Texas Children's experts in Pediatric and Adolescent Gynecology.

Causes & Risk Factors

The condition develops in utero, when the baby’s reproductive system and other organs fail to develop properly.

The cause of this abnormal fetal development is not yet known.

Symptoms & Types

Symptoms may include:

• Pelvic pain
• Painful periods (dysmenorrhea)
• Vaginal or pelvic mass
• Irregular bleeding
• Unusual vaginal discharge
• Vomiting
• Constipation
• Fever

Diagnosis & Tests

In most cases, OHVIRA isn't diagnosed until a girl reaches puberty and experiences menstrual problems or other symptoms.

Diagnosis starts with a thorough medical history and physical exam, including a pelvic exam.

Additional testing may include ultrasound or MRI (magnetic resonance imaging) to view the internal reproductive organs.

Treatment & Care

Treatment depends on the individual patient and what symptoms are present.

Treatment strategies typically include surgery to remove the wall of tissue that is blocking the vagina.  This improves menstrual flow and reduces complications with fertility and pregnancy.