July 08, 2009

Vaginal Agenesis

Vaginal agenesis is a congenital disorder of the reproductive system affecting one in 5,000 females. It occurs when the vagina, the muscular canal connecting the cervix of the uterus to the vulva, stops developing because the vaginal plate fails to form the channel.

Some patients may have a shorter vagina, a remnant of one or lack of one all together (Mayer-von Rokitansky-Kuster-Hauser's syndrome). With vaginal agenesis, it is not uncommon to have other malformations in the reproductive tract, such as an absent or small uterus.

In addition, 30 percent of patients with vaginal agenesis will have kidney abnormalities, the most common of which is the absence of one kidney or the dislocation of one or both organs. The two kidneys may also be fused together, forming a horseshoe-like shape. Approximately 12 percent of patients also have skeletal abnormalities, with two-thirds of this group experiencing problems affecting the spine, ribs or limbs.

How is vaginal agenesis diagnosed?

Because external genitalia appear normal, vaginal agenesis is typically not diagnosed until puberty (around age 15), when a young girl notices that she has not had her menstrual period and seeks medical attention. The diagnosis is made by physical examination and a series of X-ray tests. They may include an ultrasound to check if the uterus and ovaries are both present and entirely intact. The patient may even be asked to undergo a MRI that will show a more detailed picture of her reproductive tract.

While most vaginal agenesis sufferers are not aware of their condition until their teen years, a subgroup of these patients will be diagnosed during infancy. In this case, the abnormality is usually detected during an examination or test for unrelated problems.

How is vaginal agenesis treated?

Self-dilation: Some women can have their vagina reconstructed without having an operation. Pressure is applied over the area where the vagina should be with a very small tube, called a dilator. The dilator is held against the skin and pressure is applied for about 15 to 20 minutes a day. Usually, this is more comfortable after the patient has taken a bath because the skin is soft and stretches more easily.

Vaginoplasty: Most young women, however, will require surgical reconstruction. Techniques vary widely, but sufferers usually undergo either a skin graft or bowel operation. In the first procedure, the surgeon creates a vagina by cutting a thin piece of skin from the patient's buttocks and placing it over a mold to create a vagina. He or she then makes a small incision where a normal vagina would be located and inserts the mold so the graft will attach naturally to make the inside of a vagina. After the surgery, the patient is usually on bed rest for a week, during which time a catheter is placed into the bladder for drainage. The mold is removed after seven days. With a bowel vaginoplasty, a portion of the lower colon is removed through an abdominal incision. One end of the bowel is then closed, while the other remains open, functioning as a vaginal opening. The colon is sewn into the vaginal remnant. The night before surgery, patients undergoing this vaginoplasty must empty their bowels to remove stool and bacteria. Following the surgery, a mold will be inserted into the new vagina for three days. During this period a catheter is placed into the bladder through the urethra so that urine can drain.

What can be expected after treatment for vaginal agenesis?
Patients undergoing a skin graft usually wear a vaginal dilator for three months after surgery. It is removed for urination, bowel movements, showering and sexual intercourse. After three months, the patient usually wears the dilator only at night for approximately six months. Vaginal stenosis, or a tightening of the vagina, is the major complication of this procedure.

Only one operation is needed with bowel vaginoplasty. The patient will be seen three weeks after the surgery and again in three months. Some women will experience a tightening of the vagina. If this occurs, dilation will be performed under anesthesia. Home dilation is not necessary.

Frequently asked questions:

At what age should my daughter consider having a vagina created?

When she starts is up to her. Most girls begin the process in their teens, but she may want to wait until some time in the future when she is ready to become sexually active.

After surgery, when can she begin to have sexual intercourse?

Although patients should consult their physician before having sexual intercourse, it is usually acceptable to begin four to six weeks after the procedure. The patient will probably need lubrication since the skin will not produce the same substances as normal vaginal tissue. Lubrication after a bowel vaginoplasty is less of a problem.

Will she be able to lead a normal sex life?

Since much of sexual pleasure comes from stimulation of the clitoris, the female erectile structure, and not the vagina, she should enjoy normal sensations and a good sex life. Since reconstruction is internal, no one will be able to tell that a patient has undergone the procedure.

Will my daughter be able to have children?

Your daughter's individual anatomy will be the biggest factor in whether or not she will be able to have children. It is very likely she will be able to become pregnant if her uterus, ovaries and fallopian tubes are normal. It is unlikely that she will be capable of going through pregnancy by herself if her uterus is tiny or absent. However, since the ovaries usually remain normal, fertility specialists will be able to harvest an egg, fertilize it with her partner's sperm, and implant it into a surrogate mother who would carry it to term. Adoption is another option.

Do doctors know the cause of vaginal agenesis?

There are no known risk factors for vaginal agenesis.

Adapted from:American Urological Association