Mayer Rokitansky Kuster Hauser Syndrome

What is MRKH or Mayer-Rokitansky-Kuster-Hauser Syndrome?

This condition is defined as “an uncommon variation in the prenatal development of the female genital tract. Its features include an absent or very short vagina and a uterus that can be absent or immaturely formed. Females with Mayer- Rokitansky-Kuster-Hauser syndrome have functioning ovaries, normal external genitalia and the typical, 46, XX, female chromosome pattern. Breast development and growth of pubic hair are also normal. Associated renal and/or skeletal abnormalities are common.” It has been determined that the absence of a vagina and uterus is a result of the Mullerian ducts failing to form properly early in embryonic development, its underlying cause is unknown.

How common is MRKH syndrome?

The commonality of MRKH syndrome varies. Originally it was said that this syndrome occurs in 1/4,000-5,000 births but recent studies have claimed it is even more rare at 1/10,000 births.

When is MRKH syndrome diagnosed?

This syndrome is usually diagnosed as a teenager after the teen fails to begin her first period. Once taken to the gynecologist they will attempt to perform a pelvic exam. Sometimes these can be too much for those with MRKH syndrome. Instead an external ultrasound will be performed to see if the fully formed uterus is present. If not then the teen will be diagnosed with MRKH syndrome. Blood work is also to be expected. The typical age range of those diagnosed is between 15 and 18 years old.

Can MRKH syndrome be treated or cured?

Ultimately no, you cannot change the fact of having MRKH syndrome or the fact that you cannot physically have your own children. BUT you have ovaries and produce eggs so you can do surrogacy. HOWEVER you can create the internal aspect of your vagina if you don’t have even a short internal vagina. There are various surgical routes you can take to ensure comfort during intercourse, but there are no successful forms of uterus transplants yet.There are also non-surgical methods to aid in this as well with the use of dilators. Or you could even opt to do nothing at all.

To read more on the treatment methods go here: http://www.youngwomenshealth.org/mrkh_treatment_options.html

What are the negatives?

For some finding out that you can never conceive your own children can be devastating. As I have been through this myself I recommend having the teen find out as soon as possible. I feel that knowing for so long has given me a kind of peace with it even though I didn’t find out until I was eighteen. I think that if I would have known even earlier it would have been even better. Of course if you decide to go with surgical methods to help the teen have a normal sexual life then she will have to recover. MRKH syndrome can also cause mental disorders such as depression from the shock. Which is completely understandable. Please support the teen, tell her there is no shame in seeking therapy, because it is a lot to deal with. Also depending on who the teen reveals her condition to she may face some harsh opinions. Some claim that because the teen lacks a uterus she is not a woman. This is ridiculous, genetically she is a girl, and last I checked men don’t have breasts and ovaries. The teen may have to develop a thick skin if she chooses to share. I find that the more casual you are about it the more open and understanding people are about it.

What are the positives?

Ultimately no, you cannot change the fact of having MRKH syndrome or the fact that you cannot physically have your own children. BUT you have ovaries and produce eggs so you can do surrogacy. HOWEVER you can create the internal aspect of your vagina if you don’t have even a short internal vagina. There are various surgical routes you can take to ensure comfort during intercourse, but there are no successful forms of uterus transplants yet. There are also non-surgical methods to aid in this as well with the use of dilators. Or you could even opt to do nothing at all.

Personal Experience:

I was diagnosed when I was 18 years old. I started going to the gynecologist when I was 16-17 years old. Instead of giving me an exam they decided to just get me blood work and start me on birth control in an attempt to jump start my period. However, it didn’t work a year went by and no period. I went back to the doctor and she attempted to give me a pelvic exam. It was so incredibly painful that she stopped. She ordered for me to have an external ultrasound. I went in for the ultrasound and they found my ovaries but could not locate a full uterus. They found that I had a very under formed uterus. Thankfully I have enough of one to not need surgery or dilators. I chose to skip those methods and do nothing. I did however eventually decide to see a therapist a few times to ensure that I would be okay and I was.

Honestly. I am completely thankful that MRKH syndrome happened to me and not to someone else. I have always wanted to adopt a child even as a kid. While I am able to have a child on my own I feel like it is a good thing to give a child a good life one day. So if MRKH syndrome was to happen to someone I am glad it happened to me. I am very confident and own my syndrome I am not ashamed or afraid that I have it. Some people do not totally understand and claim women without uteruses are not women, but they are wrong. We have the female genetic makeup; we just didn’t develop enough in our uterus during the prenatal stage of our development. So I have just learned to live with it. Honestly I hardly ever even think about it anymore. There are just so many other positive things in life than dwelling on what I don’t have. I found a great man and he so understands and is fine with my situation. So I suggest not being afraid, own who you are, and if you have health concerns go to your doctor ASAP. Why worry yourself into oblivion? There is no need too; you are perfect the way you were made.