MRKH:
A Guide for Teens
 |
 MRKH FAQ's |
| Vaginal Dilator Instructions |
| McIndoe Surgical Procedure |
| MRKH Guide for Parents |
You may have just learned that you have MRKH (Mayer Rokitansky Kuster Hauser Syndrome). You’re probably thinking, “Why is the name so long?” It’s extra long because this condition is named after all of the doctors who discovered it. Aside from being overwhelmed with the name of this condition, it is also normal to feel confused, scared, and sad about having MRKH. Most likely you and your parents have a lot of questions. We hope that this guide will help answer your concerns. We also have a special guide for your parents.
What is MRKH?
MRKH is a congenital disorder that affects the female reproductive tract. Congenital means that it is there at birth. About 1 in every 5,000-10,000 female babies has this condition. MRKH is a syndrome (group of symptoms). We do not know the cause of this syndrome but we do know that when a baby grows in the mother’s uterus (womb), systems develop. One of the systems is called the reproductive system, which includes the uterus, vagina, fallopian tubes, and ovaries. The reproductive system is formed during the first few months of “fetal” life (while a baby is still in her mother’s womb). With MRKH, the reproductive system starts to develop but doesn’t completely finish.
Girls with MRKH have normal ovaries and fallopian tubes. Most often the uterus is absent or tiny. The vagina is typically shorter and narrower than usual or it may be absent. Sometimes, there may be one kidney instead of two. About 3% of girls will have a minor hearing loss and some may have spinal problems such as scoliosis (curvature of the spine).
When is a girl likely to find out she has MRKH?
The most common age for MRKH to be diagnosed is when a young woman is between 15 and 18 years old. That’s when a young woman is likely to see her health care provider because she hasn’t started her period. Some girls may find out at an earlier age or when they are older.
What will happen at my doctor’s appointment?
Your doctor will probably ask you questions such as: “When did you notice that your body was changing…going through puberty?” Next, he/she may want to take a look at your outer female organs and also check to see how long your vagina is. Your doctor will gently put a Q-tip or gloved pinky finger at the opening of your vagina and then very slowly and carefully put it into the vagina to see how deep your vagina is. If your doctor thinks you might have MRKH, he/she will probably order a test called an ultrasound or an MRI (magnetic resonance imaging). These tests do not hurt and are similar to having an x-ray. Usually your doctor will have you see a specialist who has experience taking care of young women with MRKH. A pediatric and adolescent gynecologist is a doctor with special training in young women's reproductive health.
What can a pelvic ultrasound or MRI show?
A pelvic ultrasound is usually the first test to check to see if a uterus or womb is present. This test can also confirm that you have two ovaries and two kidneys. Sometimes a very tiny uterus can be seen. A tiny uterus is called a “uterine horn or remnant”. You may need to have an MRI so that your doctor can see your female organs in more detail.
If I have an incomplete vagina, what are my options?
If you have been told that you have MRKH and your vagina is incomplete or absent, you have some options.
Creating a vagina with dilators
Most of the time girls with MRKH choose to make a vagina by using vaginal dilators. This treatment is very effective and “noninvasive” (does not have the risks of an operation). There are different kinds of dilators available and they come in different sizes. The most commonly used dilators look like a think plastic tube similar to a slender tampon. Starting with the smallest dilator you will learn how to hold the dilator and apply pressure to stretch your vagina. In the beginning, most of the dilator will be on the “outside” and used as a “handle” (as it can only go in so far). You will use the dilator for about 15-20 minutes, twice a day. Over time, you will begin to notice that the dilator is able to go into your vagina a bit further. This means you have made progress! As your vagina stretches, your doctor will know when to give you the next size. The next size will be slightly wider.
Surgery to create a vagina
McIndoe procedure- This is the most common surgical procedure to make a vagina. A vagina is created with a skin graft usually from your buttocks (bottom) or with a special skin-like material and a vaginal mold. Young women who have this procedure must stay in bed, in the hospital for about a week so that the newly created vagina will heal. A soft dilator must be worn all the time for a while, taking it out only to pass urine or poop. Even though a vagina is created faster with surgery, it is still necessary to use a dilator. Surgery to create a vagina should not be thought of as a “quick fix solution”.
Bowel vagina- This is a major operation which involves making a vagina using a section of the bowel. The advantage of this procedure over the McIndoe operation is that you don’t have to stay on bed rest for a week. The disadvantage is that girls who opt for this procedure will likely have chronic vaginal discharge.
Other surgical procedures to create a vagina include:
Waiting or doing nothing
You are the one who should be in control of your body. Deciding to make or not make a vagina should be your decision and if you decide you want to, when should be your decision too! If you are not planning to have vaginal intercourse, it is not something you need to do. If you plan to have vaginal intercourse now or in the future, making a vagina is something you might want to do.
How often do I need to see my doctor?
It is VERY important to keep appointments with your doctor so he/she can check your progress (whether you have decided to use dilators or have surgery). If you decide to use dilators, your doctor will need to check that you are applying pressure in the right place. As your vagina stretches, your doctor will give you the next (slightly bigger) dilator.
How long will it take to create a vagina with dilators?
The average amount of time it takes to create a vagina (if the dilator is used at least 15-20 minutes, twice a day) is about 3-14 months. It can take less time for some young women or more time for others. If the dilators are not used every day, it can take a year or more. For more information, read the following guide: Instructions on the Use of Vaginal Dilators.
A recent study done at Children’s Hospital Boston found that 88% of girls who chose to use dilators consistently to make a vagina, were able to do so within a year and a half.
Do the dilators hurt?
Using dilators to create a vagina shouldn’t hurt. Remember, you have control over the amount of pressure that you apply. You should get used to applying enough pressure so the skin will stretch but you should not be in pain.
When should I start to create a vagina?
There is no right or wrong time to create a vagina. The decision to start or not to start should be yours. You may be ready to start using dilators when you are in high school or you may decide to wait until you are older. Some questions to ask yourself: Do I have time in my day to use the dilator?”; “Do I have privacy?”; “Am I comfortable touching myself?”, “When do I plan to become sexually active?”
What if the dilators don't work or I just can't use them?
Most girls can learn to use a dilator and have success in creating a vagina. Sometimes, it can take a while until you feel comfortable using it. If you have any questions or wonder if you are using the dilator correctly, be sure to ask your doctor or nurse specialist. In some cases, if your doctor feels that little progress has been made, he or she may suggest that you take a break for a while and try again later. Dilator treatment is the standard, most efficient and nonsurgical treatment for MRKH; it is recommended by the American College of Obstetricians and Gynecologists (ACOG) as the first choice of treatment to create a vagina for girls with MRKH. Surgery should only be considered if dilators have been tried (under the supervision of a gynecologist who specializes in treating young women with MRKH).
Why might I have pelvic (belly) pain each month?
Some women with MRKH may have a tiny uterus called a “uteri or uterine remnant”. The uterus or womb is not big enough to carry a baby but it can cause pelvic pain if blood from this small uterus goes into the belly. Your doctor will be able to tell if you have a small uterus and if it needs to be taken out. If you have belly pain, it is important to tell your doctor.
Can anyone tell that I have MRKH?
Some young women wonder if anyone can tell if they have MRKH. The answer is no. No one, except you and your doctor, can tell that you were born with an incomplete vagina and following treatment, with dilators or surgery, your sexual partner will not be able to feel any difference.
Will I be able to have children?
If you were born with an incomplete vagina but have a normal size uterus, it is likely that you will be able to become pregnant and deliver a baby.
If you were born without a uterus or if your uterus is tiny, you will not be able to "carry" a pregnancy. Since your ovaries are normal and make eggs, an egg can be fertilized with your partners' sperm. Someone else such as; your sister, friend, or another person you choose, could be the surrogate mother. Surrogate mothers are women who agree to carry a pregnancy for a couple. You and the baby's father would be the biological parents of this child. Adoption is another choice for some couples. Fertility options are improving every day. By the time you are ready to have children, there may be more options available to you.
Is there anyone else I can talk to about having MRKH?
Some young women find it helpful to talk with a parent(s), while other girls prefer to talk with a counselor or a close friend. We know that it can be very helpful to talk with someone your own age that has MRKH. The Center for Young Women’s Health at Children's Hospital Boston offers free monthly chats for young women with MRKH.
If you have been diagnosed with MRKH or you would like an evaluation or second opinion, you may contact the Gynecology Program at Children's Hospital Boston at: (617) 355-7648. |
Next: MRKH FAQ's |
Updated: 3/26/2009
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