What is MRKH?

 

MRKH is a congenital disorder that affects the female reproductive tract. Congenital means that it’s acquired during development and present at birth. About 1 in every 5,000 female babies has this condition. MRKH is a syndrome (group of symptoms). We don’t know the cause of this syndrome, but we do know that when a baby grows in their mother’s uterus (womb), organs and systems develop. One of the systems is called the reproductive system, which in female babies includes the uterus, cervix, 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 grow but doesn’t completely develop.

 

Girls with MRKH have normal ovaries and fallopian tubes. Most often the uterus is absent or tiny. The vaginal canal 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 diagnosed with MRKH will have a minor hearing loss and some may have spinal problems such as scoliosis (curvature of the spine). Girls with MRKH have normal external genitalia, which means everything on the outside of the vagina is not affected. This part of your body is called the vulva and includes what you can see – clitoris, urethra, labia, vaginal opening, hymen and anus. (Source: Boston Children’s Hospital)

TREATMENT OPTIONS

If you’ve been told that you have MRKH and your vagina is incomplete or absent, you have the following options:

- You can do nothing

- Create a vagina using dilators that expand and stretch your existing vagina over time, using intermittent pressure

- Have a surgical procedure, or “vaginoplasty”, or a combination of surgery and dilation

 

Remember YOU are in control of your own body. Deciding to create or not create a vagina is your decision, and if you decide you want to, when you do so is also your decision! If you’re not planning to have sex, it’s not something you need to do. If you do plan to, creating or expanding your vagina is something you may want to consider.

 

If you’re thinking about treatment, the medical recommendation is that you consider using dilators first. All of the surgical options require general anesthesia and the use of dilators after the operation, therefore, surgery is not a quick fix, or a way to avoid the need for using dilators.

 

DILATION

Starting with the smallest dilator, a young woman learns how to hold it and apply pressure to stretch her vagina. You should make sure that you work with a trained clinician who will teach you how to use the dilators properly and monitor your progress. You will take one home with you and should use it once or twice daily for 15- 20 minutes. As your vagina stretches, your gynecologist will give you the next (slightly bigger) dilator until full length and width is achieved. Once done, the vagina will need to be maintained by either intercourse or dilation once/week.

I chose dilation when I was ready to create my vagina. I didn’t have a partner when I started, so I felt like I could take my time with it, and I was glad that I had control over the process. At first, I did find it difficult to get used to the discomfort that comes with dilating, but with time I became used to it. The best way for me to get through a dilation session was to have something to distract me, whether it was a TV show or something to read or listen to. Something I didn’t expect about dilating was that there were periods when I progressed faster than others. There was a period of time when I moved up a dilator size every few weeks, then there was a time when I was on the same size for a couple months. Dilation could be frustrating at times, especially with the time commitment that it required, but in the end I was happy with the result and proud of the work I had put into it. - Anonymous

 

SURGERY

Surgery to create a vagina, also known as vaginoplasty, involves using tissue or skin from another area of your body to create a vagina.

 

McIndoe procedure: 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 after the procedure for about a week so that the newly created vagina will heal. After the operation, a soft dilator must be worn all the time for about 3-6 months, taking it out only to use the bathroom. Even though a vagina is created faster with surgery, it’s still necessary to use a dilator afterwards.

 

"I had the McIndoe technique at 16 years old because it was presented to me as the only viable option in 1984. Recovering from the McIndoe technique required healing in two areas of my body - first, my newly created vagina - where the incision and mold were placed; and second, the skin graft area on my backside. Once the mold is removed, dilation is to begin right away. There was some discomfort associated with dilating, so I wasn’t very consistent with it. As a result, I lost some depth in my vagina. Dilation is necessary on an ongoing basis. Though I wasn’t sexually active for many years after the surgery, having had the procedure allowed me to feel I had the option to be sexually active, if I chose to. When I eventually became sexually active, I was able to have sex comfortably. I would choose to have the McIndoe technique again, but with a different awareness from having learned dilating is an important part of ongoing self-care to maintain the results of the surgery and to be your own medical advocate – get second opinions, select a medical professional who is compassionate and understanding, and take your time making the right treatment decision for you." - Anonymous

Williams procedure: This procedure involves the creation of a vaginal “pouch”. It’s sometimes used when other surgical procedures have failed. Dilators are necessary following the procedure, but for only about 3-4 weeks, instead of up to 6 months (which is more typical with the McIndoe procedure). The down side with this procedure is that the angle of the newly created vagina can be awkward and the cosmetic appearance may be poor.

 

Bowel vagina: This is a major operation which involves creating a vagina using a section of the bowel. The recovery involves 4-6 weeks of healing from major surgery and dilators will need to be used afterwards. Young women who have this procedure usually experience chronic vaginal discharge requiring the need to wear a pad.

 

Laparoscopy-Vecchietti procedure: This technique involves securing a traction device to the skin outside of the abdomen. This is done during a laparoscopy while the patient is under general anesthesia. At the same time, a plastic bead (about the size of an olive) is placed in the vaginal space and held with string that’s threaded up through the vagina, into the abdominal cavity, and out to the traction device. The vaginal length is achived by using continuous pressure and turning the “crank” on the outside of the abdomin, which pulls the plastic bead upwards. The vagina can be created in about 7-10 days, but requires a long hospital stay to complete the process. Afterwards, a second procedure with anesthesia is necessary to remove the equipment. This procedure also requires the need to use vaginal dilators. 

 

Davydov procedure: The Davydov operation creates a vagina using a patient’s own peritoneal lining: a membrane that lines the walls of the abdominal and pelvic cavities. Under anesthesia, one incision is made where the vaginal opening should be and another incision is made near the belly button to allow a thin instrument to be inserted into the peritoneal cavity. The peritoneal lining is pulled down and stitched in place at the vaginal opening. The top of the newly created vaginal canal is sewn closed and the vaginal space is packed with gauze. The gauze is removed about two days after the operation. Then the patient must use various size vaginal dilators a few times a day for several months or until she becomes sexually active. Most women who have this procedure stay in the hospital overnight for observation and then return for follow-up visits 7-10 days after surgery.

"Having been diagnosed back in 2014, I pushed off any treatment options until I was fully ready to accept this condition. Doing any sort of treatment is a big decision for anyone, you must be both mentally and physically ready or it will not be successful. Dilation is typically recommended as the first treatment option you should try. My specialist was able to show me how the process worked. She said it would take a lot of effort and it would be important to stick with it. I started out with 1cm and by doing dilation on and off for 6 months, I managed to get to around 4 cm (which is amazing). Eventually dilation stopped working for me and I couldn’t get past that 4 cm mark. This sucked because I saw so many people complete the process with no problems. With the many online support pages I was in, I found there were also surgery options out there. I did my research and found the perfect fit for me. Dr. Miklos and Moore out of Atlanta, Georgia offer a surgery called: The Laparoscopic Davydov Neovagina. The procedure creates a full-length vagina immediately using the patient’s own peritoneum or lining of the pelvis. They are very knowledgeable with MRKH patients and were there for me every step of the way. After about three weeks of recovery, I was back to normal. If you are looking to go down the surgery route, I would strongly recommend visiting their website and seeing if this is a good fit for you." https://www.miklosandmoore.com/mrkh-atlanta/ 

- Tori

(Source: Boston Children’s Hospital)

FERTILITY OPTIONS

Having MRKH does NOT mean you cannot have children. Having MRKH means you will not be able to carry your own children. Lucky for us, there are other ways in today's world to become a parent. 

IN VITRO-FERTILIZATION (IVF)

In vitro fertilization (IVF) refers to the retrieval and fertilization of an egg and sperm performed in a lab leading to embryos which are then transferred into a womb in hopes that a pregnancy will occur and result in the birth of a child. 

Stages Of An In Vitro Fertilization Cycle 

(Source, Ottawa Fertility Centre)

1. Ovarian stimulation

Injections of fertility medications called gonadotropins are taken daily by the woman, which causes the growth of multiple follicles. At the same time, additional injections of medications called GnRH agonists or antagonists are also taken daily to prevent ovulation. During ovarian stimulation, progress is monitored with a series of blood tests and vaginal ultrasounds. Lastly, an injection of a hormone called ovidrel or human chorionic gonadotropin (hCG) is taken to cause final maturation of the eggs in preparation for the egg retrieval procedure. The timing of this “trigger” injection is determined by egg follicle sizes.

2. Egg retrieval and sperm collection

Approximately 36 hours following the “trigger” injection, patients will have their egg retrieval procedure. An ultrasound probe is placed in the vagina and a needle is passed through it, into the ovary. The eggs are collected from the follicles. Although there can be some discomfort, medication is given to minimize pain and the egg retrieval is generally well-tolerated. Most commonly, men will provide an ejaculated sperm sample for use, just before the egg retrieval procedure. In other situations, previously cryopreserved sperm, donor sperm or surgically retrieved sperm will be used for fertilization.

3. Fertilization and embryo culture

After a period of incubation in the lab, each egg is placed in a dish with a large number of moving sperm and fertilization is allowed to occur. If the sperm are low in number or movement, a single sperm can be selected and injected into each egg using a technique called intracytoplasmic sperm injection (ICSI). After eggs and sperm are combined, fertilization may occur. The resulting embryos are allowed to grow in the lab until ready for transfer into the uterus.

4. Embryo transfer

One or more embryos are placed into the uterus using a soft, flexible catheter or tube that is guided through the cervix by ultrasound. The discomfort of an embryo transfer procedure is similar to that of having a pap test, but with a full bladder. After the embryo transfer, following 15 minutes of rest, patients return home and are advised to rest for that day. Any good quality embryos remaining after the transfer may be cryopreserved for future use.

5. Progesterone supplementation and pregnancy test

Starting the day of the egg retrieval procedure, progesterone tablets are placed in the vagina in order to support a developing pregnancy. Occasionally, progesterone is given as an injection. A pregnancy test is performed approximately 17 days after egg retrieval. If positive, an ultrasound is performed to assess the health of the pregnancy, which is usually done two to three weeks after a positive pregnancy test.

ADOPTION/FOSTER CARE

(INFO WILL BE HERE SOON)