Rokitansky Syndrome Affects 1 In 5,000 Women - But You've Probably Never Heard Of It

Talking about medical conditions surrounding the female reproduction system has been seen as taboo for too long. Thankfully, the discourse concerning reproductive health is becoming less off-limits, even if it is a very slow change. We are able to talk more and more about the female body and the reproduction system within it in an open manner, and people with vaginas are able to take more control over their health and learn about potential health issues, such as Rokitansky Syndrome.

1 in 5,000 women suffer from Mayer-Rokitansky-Küster-Hauser Syndrome, a condition that causes the vagina and/or uterus to be underdeveloped or completely absent. Though rare, this condition can be treated. A vagina can be created with the help of surgery and dilators, and women have successfully given live birth with transplanted uteruses. You'd never know a woman had it, though; there are no external effects, as women who have MRKH otherwise develop normally through puberty. Here is everything you need to know about MRKH syndrome, and what to do if you suspect you have it. 

Photo: Chapendra / flickr / CC-BY-NC 2.0

  • Rokitansky Syndrome Occurs In The Womb

    The good news is that if you're reading this and currently have a vagina, you do not have Rokitansky syndrome, nor will you get it later in life. The genetic abnormality occurs in the womb, possibly around 20 weeks. While the female fetus develops, the reproductive system develops out of the Mullerian ducts. One of these ducts is in charge of creating the vagina and uterus, while the other develops into the fallopian tubes. Scientists think that these ducts don't develop properly, thus causing Rokitansky syndrome.

  • What Causes Rokitansky Syndrome?

    There's no confirmed cause of Rokitansky syndrome, and no one has found an environmental link to the development of MKRH. And though researchers know that MKRH affects genes, they also know that different genes can cause MKRH - it's not the same gene affected each time.  At this point, it's not known why some women and have it and others don't. It may be random. However, it's believed that it can be passed down through families.

  • You May Or May Not Have A Vagina And Uterus

    Some women with MRKH have no vaginal opening at all; others have a very small vaginal opening. Some women have a uterus; others do not. Some may have a small or undeveloped uterus; unfortunately, these women still cannot carry a child. There is no "one-size-fits-all" when it comes to the reproductive systems of people with MRKH.

  • How Do You Know If You Have MKRH?

    Most young women with MKRH don't realize it until they don't start their period as teens, and this is typically the point when MKRH is diagnosed by a doctor. Not all women with MKRH are completely missing a vagina, though - it may just be underdeveloped. Some with an undeveloped vagina will experience difficulty or pain with sexual intercourse, another possible first indicator of MKRH. 

  • There Are Two Types Of MKRH

    MKRH has two forms: Type 1 and Type 2. Type 1 is pretty straightforward; the vagina and/or uterus are underdeveloped or not developed at all. Otherwise, women with MKRH Type 1 don't have any other associated health problems.

    Type 2, however, involves organs outside the reproductive system. MKRH Type 2 is characterized by kidney and skeletal issues. Sometimes a kidney is missing entirely, deformed, or placed incorrectly in the body. When your renal system is affected, you can experience not only UTIs and kidney stones, but also growth problems. Aside from possible skeletal issues in the spine, 10-25% of those afflicted also experience hearing problems. 

    The good news is that Type 2 is far less common that Type 1, occurring in 1-9 out of 100,000 live births. 

  • No Vagina? You Have Options

    If you don't have a vagina, fear not. Doctors can create a vagina with a two-hour surgery, allowing women with Rokitansky syndrome to have a normal sex life. 

    For those who have an underdeveloped vagina, one can be created by using dilators, which stretch the vagina. Dilators of increasing size are inserted into the vagina for anywhere from half an hour to two hours. Even with surgery, dilators must be used. 

    It's also possible to elect not to create a vagina, though this negates the possibility of what is seen as a traditional vaginal sex life.