MRKH syndrome also known as Mayer-Rokintansky-Kuster-Hauser syndrome is a rare disorder. It only affects women because this syndrome is when a woman is born without a uterus or a vagina or the uterus and vagina are underdeveloped. Some people call this no wombs syndrome. One in every five thousand women have this syndrome. The cause of this syndrome is unknown, but studies are showing that it may be a genetic disorder.
It usually takes until a woman is between the ages of 15 and 18 to be diagnosed with MRKH syndrome. This is because the first sign of this condition is a lack of a period. Even with a missing or underdeveloped uterus or vagina the outward anatomy looks normal. The only difference is that the vagina opening may be tinier. Some cases have been noticed when a woman goes to have sexual intercourse and it deems to be difficult. You can not diagnose MRKH syndrome on your own a medical professional will have to help you diagnose. If you have a lack of a menstrual cycle and you go see your doctor after a physical examination they may want to do an MRI, or ultrasound to look for signs of an underdeveloped uterus or missing one. The thing with MRKH syndrome of women who have this syndrome still usually have working ovaries. So they are still producing eggs. With the lack of a uterus though it causes them to be unable to bear their own children.
There are two main types of MRKH syndrome. Type one is the most well known. It is when the body lacks a uterus or vagina. While type two can also show damage to the kidneys and musculoskeletal system. About half the women that have MRKH syndrome have type two. It has also been shown that women with MRKH syndrome also have hearing problems as well.
This syndrome is chronic. You will live with it for your whole life if diagnosed with it. There are some ways to make your life mostly normal though. If you suffer from a smaller vaginal opening there are some surgical and nonsurgical options that you have. There are vaginal dilators that you can use to help expand your opening. Using these consistently will help allow the vagina opening to become larger and stay so as long as you keep using the dilator. Another option for this that is surgical is to do surgical vaginal traction. Where strings are attached to the opening and with pull the strings increases the size of the opening. Once the appropriate size of vagina is acquired the traction device will be removed. Another surgical option is a vaginoplasty. This is when a doctor will create a vaginal cavity, dilators will need to be used after the surgery though to continue the opening that was created in the vaginoplasty.
Since women with MRKH cannot conceive and carry their own children most of them have working ovaries. You can have eggs extracted then with the use of IVF have a surrogate carry your biological child. There is also always adoption. A more complex and dangerous option is a uterine transplant.
MRKH syndrome is life changing when discovered some people need therapy to help cope or to find a support group. The main question is are you still a woman if you have MRKH syndrome. Yes, because you have the genetic makeup and the amount of chromosomes to make you a woman even if you end up with this syndrome. There are lots of ways to help you make your life as close to normal as possible with this syndrome. With your doctors help you will be able to go about your life as normal as possible.
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