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| Vaginal agenesis by Pharmacytimess |
Overview
Vaginal agenesis (a-JEN-uh-sis) is a rare
disorder in which the vagina doesn't develop, and the womb (uterus) may only
develop partially or not at all. This condition is present before birth and may
also be associated with kidney or skeletal problems.
The condition is also known as mullerian agenesis, mullerian aplasia or Mayer-Rokitansky-Kuster-Hauser syndrome.
Vaginal agenesis is often identified at
puberty when a female does not begin menstruating. Use of a vaginal dilator, a
tubelike device that can stretch the vagina when used over a period of time, is
often successful in creating a vagina. In some cases, surgery may be needed.
Treatment makes it possible to have vaginal intercourse.
Symptoms
Vaginal agenesis often goes unnoticed until
females reach their teens, but don't menstruate (amenorrhea). Other signs of
puberty usually follow typical female development.
Vaginal agenesis may have these features:
·
The genitals look like
a typical female.
·
The vagina may be
shortened without a cervix at the end, or absent and marked only by a slight
indentation where a vaginal opening would typically be located.
·
There may be no uterus
or one that's only partially developed. If there's tissue lining the uterus
(endometrium), monthly cramping or chronic abdominal pain may occur.
·
The ovaries typically
are fully developed and functional, but they may be in an unusual location in
the abdomen. Sometimes the pair of tubes that eggs travel through to get from
the ovaries to the uterus (fallopian tubes) are absent or do not develop
typically.
Vaginal agenesis may also be associated with
other issues, such as:
·
Problems with
development of the kidneys and urinary tract
·
Developmental changes
in the bones of the spine, ribs and wrists
·
Hearing problems
·
Other congenital
conditions that also involve the heart, gastrointestinal tract and limb growth
When to see a doctor
If you haven't had a menstrual period by age
15, see your health care provider.
Causes
It's not clear what causes vaginal agenesis,
but at some point during the first 20 weeks of pregnancy, tubes called the
mullerian ducts don't develop properly.
Typically, the lower portion of these ducts
develops into the uterus and vagina, and the upper portion becomes the
fallopian tubes. The underdevelopment of the mullerian ducts results in an
absent or partially closed vagina, absent or partial uterus, or both.
Complications
Vaginal agenesis may impact your sexual
relationships, but after treatment, your vagina will typically function well
for sexual activity.
Females with a missing or partially developed
uterus can't get pregnant. If you have healthy ovaries, however, it may be
possible to have a baby through in vitro fertilization. The embryo can be implanted
in the uterus of another person to carry the pregnancy (gestational carrier).
Discuss fertility options with your health care provider.
Diagnosis
Your pediatrician or gynecologist will
diagnose vaginal agenesis based on your medical history and a physical exam.
Vaginal agenesis is typically diagnosed during
puberty when your menstrual periods don't start, even after you've developed
breasts and have underarm and pubic hair. Sometimes vaginal agenesis can be
diagnosed at an earlier age during an evaluation for other problems or when
parents or a doctor notice a baby has no vaginal opening.
Your health care provider may recommend
testing, including:
·
Blood
tests. Blood tests to
assess your chromosomes and measure your hormone levels can confirm your diagnosis
and rule out other conditions.
·
Ultrasound. Ultrasound images show your health care
provider whether you have a uterus and ovaries and identify if there are
problems with your kidneys.
·
Magnetic
resonance imaging (MRI). An MRI gives
your health care provider a detailed picture of your reproductive tract and
kidneys.
·
Other
testing. Your health care
provider may also order other tests to examine your hearing, heart and
skeleton.
Treatment
Treatment for vaginal agenesis often occurs in
the late teens or early 20s, but you may wait until you're older and you feel
motivated and ready to participate in treatment.
You and your health care provider can discuss
treatment options. Depending on your individual condition, options may involve
no treatment or creating a vagina by self-dilation or surgery.
Self-dilation
Self-dilation is typically recommended as the
first option. Self-dilation may allow you to create a vagina without surgery.
The goal is to lengthen the vagina to a size comfortable for sexual intercourse.
During self-dilation, you press a small, round
rod (dilator) — similar to a firm tampon — against your skin at your vaginal
opening or inside your existing vagina for 10 to 30 minutes 1 to 3 times a day.
As the weeks go by, you switch to larger dilators. It may take a few months to
get the result you want.
Discuss the process of self-dilation with your
health care provider so that you know what to do and talk about dilator options
to find what works best for you. Using self-dilation at intervals recommended
by your health care provider or having frequent sexual intercourse is needed
over time to maintain the length of your vagina.
Some patients report problems with urinating
and with vaginal bleeding and pain, especially in the beginning. Artificial lubrication
and trying a different type of dilator may be helpful. Your skin stretches more
easily after a warm bath so that may be a good time for dilation.
Vaginal dilation through frequent intercourse
is an option for self-dilation for women who have willing partners. If you'd
like to give this method a try, talk to your health care provider about the
best way to proceed.
Surgery
If self-dilation doesn't work, surgery to
create a functional vagina (vaginoplasty) may be an option. Types of
vaginoplasty surgery include:
·
Using
a tissue graft. Your surgeon may
choose from a variety of grafts using your own tissue to create a vagina.
Possible sources include skin from the outer thigh, buttocks or lower abdomen.
Your surgeon makes an incision to create the vaginal opening,
places the tissue graft over a mold to create the vagina and places it in the
newly formed canal. The mold remains in place about one week.
Generally, after surgery you keep the mold or a vaginal dilator
in place but can remove it when you use the bathroom or have sexual
intercourse. After the initial time recommended by your surgeon, you'll use the
dilator only at night. Sexual intercourse with artificial lubrication and
occasional dilation helps you maintain a functional vagina.
·
Inserting
a medical traction device. Your
surgeon places an olive-shaped device (Vecchietti procedure) or a balloon
device (balloon vaginoplasty) at your vaginal opening. Using a thin, lighted
viewing instrument (laparoscope) as a guide, the surgeon connects the device to
a separate traction device on your lower abdomen or through your navel.
You tighten the traction device every day, gradually pulling the
device inward to create a vaginal canal over about a week. After the device is
removed, you'll use a mold of varying sizes for about three months. After three
months, you may use further self-dilation or have regular sexual intercourse to
maintain a functional vagina. Sexual intercourse will likely require artificial
lubrication.
·
Using
a portion of your colon (bowel vaginoplasty). In a bowel vaginoplasty, the surgeon moves a portion of
your colon to an opening in your genital area, creating a new vagina. Your
surgeon then reconnects your remaining colon. You won't have to use a vaginal
dilator every day after this surgery, and you're less likely to need artificial
lubrication for sexual intercourse.
After surgery, use of a mold, dilation or
frequent sexual intercourse is needed to maintain a functional vagina. Health
care providers usually delay surgical treatments until you feel prepared and
able to handle self-dilation. Without regular dilation, the newly created
vaginal canal can quickly narrow and shorten, so being emotionally mature and
ready to comply with aftercare is critically important.
Talk to your health care provider about the
best surgical option to meet your needs, and the risks and required care after
surgery.
Coping and support
Learning you have vaginal agenesis can be
difficult. That's why your health care provider will recommend that a
psychologist or social worker be part of your treatment team. These mental
health providers can answer your questions and help you deal with some of the
more difficult aspects of having vaginal agenesis, such as possible
infertility.
You may prefer to connect with a support group
of females who are going through the same thing. You may be able to find a
support group online, or you can ask your health care provider if he or she
knows of a group.
Preparing for your
appointment
You'll probably start by discussing your
symptoms with your primary care provider, or your child's pediatrician. He or
she will likely refer you to a doctor who specializes in women's health
(gynecologist).
What you can do
To prepare for your appointment:
·
Make
a list of any signs and symptoms you have, including those that may seem unrelated to the reason for
your appointment.
·
Make
a list of all medications that you take, including prescription and nonprescription drugs,
vitamins, herbal preparations and supplements, and note the doses.
·
Ask
a family member or friend to come with you, if you're comfortable with that. Sometimes it can be
difficult to remember all the information provided during an appointment.
Someone who goes with you may remember something that you missed or forgot.
·
Prepare
questions to ask your health care provider, so you don't forget to cover anything that's important to
you.
Some basic questions to ask include:
·
What's the likely
cause of my condition?
·
Do I need any tests?
·
Is my condition
temporary or long lasting?
·
What treatments are available
and what do you recommend?
·
Are there any
restrictions that I need to follow?
·
Should I see a
specialist?
·
Are there brochures or
other printed materials that I can have? What websites do you recommend?
What to expect from
your doctor
Questions your health care provider may ask
include:
·
What vaginal symptoms
are you experiencing?
·
How long have you
experienced these symptoms?
·
Have you had a
menstrual period?
·
How much distress do
your symptoms cause you?
·
Are you sexually
active?
·
Does the condition limit
your sexual activity?
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