Born Without a Uterus, Not So Rare

women born without a uterus
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As news reports circulate that Derya Sert, the 22-year-old Turkish woman born without a uterus and who received a uterus transplant from a cadaver, may be pregnant, questions arise about this medical condition. Women who are born without a uterus have a little known but not so rare condition called Mayer-Rokitansky-Kuster-Hauser syndrome, or MRKH, and here’s what you should know about this disease.

How many women are born without a uterus?

In the United States, approximately 75,000 women are affected by MRKH, a congenital disease that is found in about 1 in 4,500 females. The disease is also known as vaginal agenesis/atresia or mullerian agenesis.

Women who have MRKH have fully functional ovaries, even though they may be located in unusual places in the body cavity. However, for reasons unknown, they are born without a fully functional uterus, cervix, and upper vaginal canal. All other female characteristics, including external genitalia and breast development, are normal, and genetically they have two X chromosomes, characteristic of the female sex.

Typically, females do not discover they have MRKH until they begin seeking help from their family doctor or gynecologist because they do not get their period by the time they are 16 or 18 years old. Unfortunately, many doctors are not familiar with MRKH, and so females may see several physicians, including a gynecologist, endocrinologist, and sometimes a urologist before a diagnosis is made.

A diagnosis of MRKH is usually made after women have undergone imaging, such as ultrasound or magnetic resonance imaging (MRI), or laproscopic surgery. Once a diagnosis is established, women are usually referred to a clinical psychologist who specializes in reproductive issues or a geneticist who can help them cope with the news of their condition.

Women born without a uterus may also have problems with their kidneys, including abnormally formed kidneys or those that are located in different places, or a single kidney. Some other symptoms may include heart defects, ringing in the ears (tinnitus) and vertebrae abnormalities.

What causes MRKH?
Scientists have not determined the exact cause of MRKH. Some researchers suggest it is caused by mutations in one or more specific genes. Specifically, mutations of the genes WT1, PAX2, HOXA7-HOXA13, PBX1, and WNT4 have been named as possible suspects in the development of MRKH.

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A new study appearing in ISRN Obstetrics and Gynecology (February 2013) notes that MRKH may be caused by “the lack of development of the Mullerian ducts between the fifth and the sixth weeks of gestation.” The Mullerian ducts are the embryonic tubes that become the uterus and part of the vagina in the female.

Treatment of MRKH
Women with MRKH have several treatment options. One is to use vaginal dilators, also known as the Frank method. This is a nonsurgical approach and is recommended by the American College of Obstetricians and Gynecologists as a way to create a vagina.

Basically, teen girls or women insert a series of dilators (which look like tampons), over time, into the vaginal area to stretch the available tissue. This method can be very effective for some women.

Two other options include surgery. One is a vaginoplasty, which involves creating a vagina using a skin graft from another part of the body, such as the buttocks or bowel. The other is the Vecchetti procedure, which involves combining the Frank method and surgery. The most dramatic of the treatment options is a uterus transplant.

Support for women born without a uterus
A foundation called Beautiful You MRKH Foundation offers information and support for women who are born without a uterus and their loved ones. The spokeswoman for the foundation, 24-year-old Jaclyn Schultz, is Miss Michigan 2013, who was diagnosed with MRKH at age 15.

In a recent WXYZ news report, Schultz explained that “I feel like there is a reason that I was born not to be able to have my own children, so…I want to adopt and save a child that maybe can’t have a loving family.”

Women who want more information about MRKH can visit the Beautiful You MRKH Foundation website, which provides links to many other helpful resources. Being born without a uterus is not so rare, and there is help for women who have this disease.

SOURCES:
Beautiful You MRKH Foundation
Emedicine
WXYZ news report

Updated 9/7/2015

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Comments

Please help me my sister born without utrus
My beautiful 30 year old daughter is exploring possibilities for children as well as her own health dealing with her condition. She was born without a uterus....anything I can do to help her ? feeling sad, MJ.
Mary Jo: This article offers several options for women like your daughter. Probably the best thing you can do to help her is to assist her exploration of each option and support her choice.
I found out when I was 17 I was born without an uterus, and a shortened vagina. I did have surgery at the time of discovery to lengthen the vagina for such time I chose to become sexually active. As to the missing uterus: To be honest, it didn't really affect me much. I was happy I would never have to deal with a monthly menstral cycle, and figured if I wanted children, I could adopt. There are many children who need loving homes and parents. Other than this, I have been blessed with very good health, and a knowing I can handle positively anything that comes my way. When I met my future to be husband, I knew we were on the way to becoming serious, I told him about my condition (knowing that for some men, not being able to have their own children could become an issue) so he could chose what path he wanted to take. I knew if he chose to move on, I understood his choice, and that life would bring to me what I needed. However, I was quite fortunate that he chose to stay. We have been together 43 years, and life has been a ball for us. Adoption did not work out for us the times we tried, due to natural parents changing their minds - but we grasped onto our future, and enjoy all children who have touched our lives - and know that God's plan for us moved in a different direction. I pray you too will have a happy life, and take each blessing as they come.
I found out I was 1 and million girls born with out a uterus I don't know why or how but im 34 now and would trade life to give life nobody understands me but how I would trade life it self to give life I want to have kids and have a family I call my own
I'm so sorry you feel that way!. I always wanted to children of my own! God didn't have that plan for me! I too was born with out a uterus! And to top it off I have a sister with the same condition! I have come to terms with my condition! I met my husband in high school! Let's talk about unconditional love!! He loves me! For me! Not for what he can get out of me. Or what I can give him! I am 40 years old and happily married almost 19 years! And I will be getting married in a Catholic Church soon. Yes I am cotholic! And that's what has kept us together! My husband and I! Our faith has a lot to do with it. As for my sister she has also met a unselfish great man who accepts her! And loves her for her! And not for what she can give him! The first thing we decided to do is to love ourselves for God made us this way! God makes no mistakes! Best of luck to you! And best wishes.
I am happy that at least there are friends around who knows the pain that i go through. Yes I was born without uterus and even without a breast, I don't know how to call myself, it was diagnosed when i was 19 and I still don't know how this condition is so severe and worst for me.
I have the same problem I was wondering if you can do surrogate mother? But I don't have a Virgina so how would they get my eggs out?
I am also born with a small uterus. I actually have a uterus but it is 4cm. I got to know that i'm having MRKH a year ago. I was 19. I am so disappointed and I hope that I can be a mother. I don't have a vagina but I have a small uterus. Please could someone help me.
I have same problem. I don't have a utuers and doc suggest me lepnoscope.tell me plz tell me its helpful for me?
i m born without uterus.bt my overies are normally.i just want to that is treatment possible.nd will i am able to be a mother
I am also mrkh problem but I want marry with my boyfriend he knows all my disability but can I give him full satisfied sex life and happy married life ....we adopt baby but can I give him better sex life pls pls tell me .....
hi sonali nikam, Am also with the problem like you... but I got married... now am living so happily with normal sexual activities... so you can marry dear... All the best for your beautiful life
I was born without a uterus. I'm 31 now and I was wondering if you still have both ovaries can you go into early menopause? Or will I have menopause at all? I really can't find out any info on this.
I was born without a uterus and did not find out until I was 18 I'm 53 and at the time I found out not a lot was known about the disease. I knew I would never carry a child but was never told about the other issues that go along with it such as shorter vaginal canal or ovaries being out of place so I suffered with horrible menstrual cramps of course without a period. When I began having intercourse it was always painful and unpleasant because of the shortage of the canal. I did have my eggs frozen and 4 years ago used a surrogate surrogate to have my only child. It took a very long time but I was determined to be a mom. I have suffered in silence my whole life with painful intercourse not tellng my husband and just well living with it. As for early menapause I did not start to early at 40 I began. Perimenapuase but now at 53 I'm full blown because my ovaries have dried up. I do biodenical treatments which help but because of that sex is really painful due to dryness. I have started looking into vaginalplasty recently that is now available to lengthen the vaginal canal to have a more enjoyable sex life. I hope this helps.
was also born with the same problem tho some reports says no uterus and others states that is vry small measuring 2.16 kindly advice what to do to have biological kid 33yrs frm kenya
I was diagnosed with MRHS at age age 18. This was in the 80's and nobody really talked about it. I told my husband about 2 weeks into our relationship.and he was very understanding. We have been married 35 years. We have adopted 3 children. Two of our children are mildly special needs. We are raising our grandson now. We adopted him when he was 4 months old ,and we were in our 50's. He is 5 years old now. The odd thing was in my early 30's I started having a lot of spinal issues. I have had to have several spinal surgeries. After doing some reading recently I came across some information that finally answered a lot of questions for me. There are two types of MRHS and type 2 does involve spinal issues. So if you are having a lot of back pain it would be good to check this out, and mention it to your MD. Also I did go through menapause at about age 45. It lasted on and off for about 7 years.
My daughter has been diagnosed with mrkh. She doesn't have a uterus and ovaries. MRI report will reveal if she has ovaries or not. I want to know how painful is vaginoplasty and how long it takes to recover.