There is a little-known condition that occurs during the development of a female fetus inside her mother’s womb. It leaves her without a uterus or the upper third of her vagina. It is common enough that we’ve all likely met someone with the condition. It’s also rare enough—and has a stigma around it—that most of us have never heard of it.
One in every 5,000 women around the world suffer from this congenital disease in which they are born without a uterus. Present at birth, Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a condition in which these women, who are genetically female with normal chromosomes, ovaries, eggs, and female sex hormones, cannot get pregnant and very often have painful intercourse due to a lack of cervix and uterus. Very often, the vagina of MRKH syndrome patients is too small for intercourse and need dilation treatment to expand the length of the vaginal canal. In some cases, women have to undergo surgery to create a vagina that allows for intercourse. In addition, due to the lack of a womb, women are unable to carry their own children, although their ovaries and eggs are intact.
Unfortunately, the cause of MRKH is unknown and little research has been done on this condition. However, the abnormalities come from the Mullerian ducts unable to complete full development. The Mullerian ducts are two ducts in the embryo that become the uterine tubes, uterus, cervix, and a part of the vagina. In males, the duct is unable to grow due to the presence of anti-Mullerian hormone. In cases of MRKH, it is thought that genetic and environmental factors play a role in the underdevelopment of the Mullerian duct.
MRKH syndrome first manifests itself by the lack of periods. As many women who have the condition do not have a uterus, and therefore no endometrium lining to shed, MRKH is usually noticed in the patient’s late teen years. A doctor may then confirm the diagnosis by conducting an ultrasound to measure the length of the vagina as well as determine if a uterus is present or not.
Not only is the cause unknown, there is also no treatment for this condition. However, due to the most recent cases of successful uterus transplants, there may be a possibility that these women can carry their child.
If you or a loved one would like to learn more about MRKH syndrome and treatment options, please contact our office (or your personal gynecologist) for a referral to the closets specialist with expertise with this condition.
El Camino Women’s Medical Group offers the latest Minimally Invasive Solutions for gynecologic problems. Drs. Amy Teng, Erika Balassiano, and Pooja Gupta, all members of AAGL (American Association of Gynecologic Laparoscopy) are highly trained and experienced in the field of Minimally Invasive Gynecgologic Surgery. Dr. Erika Balassiano is also completed a Minimally Invasive Gynecologic Surgery Fellowship, under the supervision of world-renowned Dr. Camran Nezhat.
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