Vaginal agenesis aka Mayer-Rokitansky-Kuster-Hauser Syndrome: Life with an incomplete vagina and uterus!

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What is Vaginal Agenesis?

Vaginal agenesis is a rare disorder that occurs when the vagina doesn’t develop. And the uterus may only develop partially or not at all. This condition is present before birth, and may also be associated with kidney, heart or skeletal abnormalities. Some girls may have a shorter vagina, a remnant of one, or lack one altogether. It is not uncommon to have other issues in the reproductive tract.

Symptoms

The symptoms of vaginal agenesis relates to a condition called “amenorrhea.” Amenorrhea is the lack of menstrual period after puberty has started. Girls with amenorrhea because of vaginal agenesis can grow normally. However, they may experience the following:

  • “Painful amenorrhea.” This condition happens to girls with a uterus.
  • “Painless amenorrhea.” This condition happens to girls who do not have a uterus and do not have a menstrual cycle.
  • Monthly cramping and abdominal pain. The pain is due to buildup of menstrual flow from the obstruction caused by the missing vagina.

Causes

The cause of vaginal agenesis is not entirely known. Scientists think that at some point during the first 20 weeks of pregnancy, tubes called the mullerian ducts don’t develop properly. Normally, one of these ducts develops into the uterus and vagina, and the other grows into fallopian tubes.

Diagnosis

Because the outer sex organs appear normal, it is often not found until around age 15, when a young girl notes that she has not had her period and seeks medical care. The diagnosis is by physical exam and imaging. An ultrasound may use to check the womb and ovaries. If needed, MRI can show a fuller picture of the reproductive tract.

Treatment

Most girls start treatment in their teens, but some may want to wait until they are ready to become sexually active.

Self-Dilation

Some young women can have a vagina made without having surgery. A very small tube, called a dilator, is pressed against the skin where the vagina should be for about 15 to 20 minutes a day. This is easier after a bath because the skin is soft and stretches well. This works best for girls who have a dimple in the area.

Vaginoplasty

Most young women will need surgery, and how this is can vary. The vagina is made with a graft of skin or buccal mucosa (inner lining of the cheek), or with part of the large bowel.

Skin Graft Method

The surgeon takes a thin piece of skin from the patient’s buttocks and places it over a mold to make a vagina. Artificial skin has also recently been recommended. The surgeon then makes a small cut where a vagina would normally be,and places the mold so the graft will attach to make the inside of a vagina. After surgery, you are likely on bed rest for a week. A catheter is placed into the bladder so urine can drain. The mold is removed after 7 days.

Bowel Vaginoplasty

This method is more complex. The night before surgery, you must empty your bowels to remove stool and bacteria. During surgery, part of the lower colon removes through a cut in the belly. One end of the bowel is then close while the other stays open. The colon is sewn onto the vaginal remnant, acting as a vaginal opening. After the surgery, a mold will place in the new vagina for 3 days. A catheter will place in the bladder through the urethra so that urine can drain.

After treatment

Patients treated with a skin graft most often wear a vaginal dilator for 3 months after surgery. It is removable for voiding, bowel movements, showering and sex. After 3 months, you mostly wear the dilator only at night for about 6 months. Vaginal stenosis, or a tightening of the vagina, is the major drawback of this method. Needs only one operation with bowel vaginoplasty. You will have to consult the doctor 3 weeks after surgery and again in 3 months. You may have a tightening of the vagina.

When can I have sex? Can I have a normal sex life and children?

Though you should talk to your health care provider before having sex, it is often ok to start 4 to 6 weeks after treatment. Lubrication will likely be needed since the skin will not make the same substances as normal vaginal tissue. Since much of sexual pleasure comes from stimulation of the clitoris, and not the vagina, you should enjoy normal sensations and a good sex life. Since the changes are internal, no one will be able to tell that you have had treatment.

Your anatomy will be the biggest factor in whether you will be able to have children. It is very likely that you will be able to become pregnant if your uterus, ovaries and fallopian tubes are normal. It is unlikely that you will be able to go through pregnancy by yourself if your uterus is tiny or absent. But if your ovaries are healthy, you can work with fertility specialists on in vitro fertilization using a surrogate mother.

References:

a) https://www.urologyhealth.org/urologic-conditions/vaginal-abnormalities-vaginal-agenesis

b) https://www.mayoclinic.org/diseases-conditions/vaginal-agenesis/symptoms-causes/syc-20355737

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