Vaginal agenesis is a congenital anomaly (birth defect). It happens when the vagina of a fetus doesn’t develop properly in your uterus. They may be missing all or part of their vagina. Although the condition is present at birth, it might go undiagnosed until later in life. The outer sex organs often appear normal, so women don’t learn that they have the condition until their teenage years. It becomes apparent when they don’t start their monthly period (menstruation).
Vaginal agenesis can occur along with other abnormalities of the reproductive system. Some people may not have a fully developed uterus (the organ in which a fetus grows) or fallopian tubes (which carry eggs to the uterus). Problems with the kidneys or the spine can also occur.
Vaginal agenesis can be treated with medical devices that gradually dilate the vaginal canal. Sometimes, you need surgery to create a vaginal opening. Depending on which reproductive organs the condition affects, it may not be possible to carry a child. But, with treatment, many people with this condition lead healthy lives and enjoy a normal sex life.
This condition affects about 1 of every 5,000 women. About a third of people with vaginal agenesis also have problems with their kidneys. Around 12% of the time, people with this condition also have problems affecting their spine, ribs or bones in the arms and legs.
Healthcare providers don’t know what causes this condition. It happens while a fetus is growing inside your uterus. At some point in the first 20 weeks of fetal development, the female reproductive system doesn’t develop as it should. There may be abnormalities in the vagina, uterus, fallopian tubes or cervix (the lower part of the uterus).
These abnormalities might result from environmental factors, such as exposure to a toxic substance during pregnancy. Or, they might result from genetics. It’s possible to inherit some of the conditions associated with vaginal agenesis.
Vaginal agenesis can be a sign of (or occur along with) several conditions, including various disorders of sex differentiation (DSD). These include:
Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, a rare disorder that causes abnormalities in the reproductive organs. MRKH syndrome is the most common disorder associated with vaginal agenesis. The syndrome causes the vagina and uterus to develop abnormally or not develop at all. Depending on the type and severity of the syndrome, there may also be other abnormalities in the cervix, spine and limbs. Hearing problems and heart and kidney abnormalities can also occur.
Androgen insensitivity syndrome (AIS), an inherited condition in which a baby does not develop a uterus, cervix, fallopian tubes and part of the vagina. In some cases, the vagina may not be present at all.
Signs of this condition include:
Sometimes, providers diagnose this condition during infancy or childhood. But most of the time, doctors diagnose the condition during your teenage years. Many people with the condition discover that they have it when they see their doctor because they haven’t started having a monthly period by age 15.
To diagnose vaginal agenesis, you’re the IWI team will do a:
We may also recommend a blood test to look for chromosomal abnormalities. A DNA blood test can confirm a diagnosis of a genetic disorder, like MRKH syndrome, which is sometimes associated with vaginal agenesis.
Treatments are available to help with this condition. Some people start treatment during childhood. Others wait until puberty or later in life when they start having sex. This choice is very personal. Discussions with the IWI team can help you make the right decision for you.
Treatment for vaginal agenesis includes:
If you have a vaginal opening or a dimple where the opening should be, the IWI team may recommend using a dilator to create a vagina. You use a small tube to make an opening and gradually increase the size every day. This usually takes about 20 minutes a day. The dilation may feel uncomfortable, but it shouldn’t hurt. You may want to increase the size of the dilator after a bath when your skin is soft and more likely to stretch.
Dr. Guerette may recommend a procedure called a vaginoplasty or a neovagina to construct a vaginal canal depending on the specific issue. The technique Dr. Guerette uses will depend on your anatomy and several other factors. Dr. Guerette has developed a specialized technique that does not require taking tissue from other parts of your body (skin grafts) and instead uses collagen grafts. This makes the procedure safer, less painful and simpler for you.
After the surgery, Dr. Guerette places an insert in the vagina to maintain its shape during early healing.
You may need to stay off your feet for about a week. Dr. Guerette may recommend leaving a dilator inside your vagina for a few weeks to 2 months. You can take the dilator out to have sex, go to the bathroom or take a shower.
Dr. Guerette will examine you to determine when you can safely have sex after treatment for vaginal agenesis. Usually, you can start having sex about a month after surgery, but everyone’s recovery time is different. You will need artificial lubrication to make sex comfortable at first.
After treatment, many people have a satisfying sex life. Your partner may not even notice that you had vaginal agenesis or received treatment for the condition.
If your uterus, fallopian tubes and cervix developed normally, you may still be able to have children.
If you don’t have a uterus (or if it’s too small), you may not be able to carry a child (uterine factor infertility). But you may be able to have a child with a surrogate using your own eggs. The IWI team may refer you to a fertility specialist to discuss your options.
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