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Mayer-von Rokitansky-Kuster-Hauser syndrome, or vaginal agenesis, is the
condition of being born without a vagina. One girl in between 4 and 5 thousand
will be affected by this condition. Some girls with vaginal agenesis have
normal, fully functional uteri, and some have only a small "remnant"
uterus. The presence and size, or absence of a uterus is determined by use of
ultrasound imaging and MRI.
Since all girls with vaginal agenesis have functional ovaries they develop
normally through childhood and into puberty, growing breasts and pubic hair at
the right times. They (or their parents) are usually alerted to there being a
problem when menstrual periods fail to appear.
Examination by their doctor will reveal that the affected girl's external
genitalia is normal, but that where the vaginal opening should be there is only
a shallow indentation, or no indentation at all
Women with functional uteri can have a problem with vaginal agenesis in that
while their ovaries and uterus have regular menstrual cycles, there is nowhere
for the endometrium to go during menses. The endometrium, instead of flowing
out through the vagina, travels in a retrograde fashion, making endometriosis more
likely than in normally developed women.
Women in this situation can choose to take a continuous hormonal
contraceptive pill which will suppress menses while preserving the functionality
of their uterus, or may choose to have a hysterectomy.
Since the condition is usually discovered between the ages of 15 and 19,
hysterectomy is rarely the chosen option.
Corrective procedures.
The safest and most effective way of creating a vagina is done by the girl
herself. Her gynecologist will provide her with dilators which she must apply
with continuous pressure to the place where her vagina should be for about 20
minutes a day after a warm bath when her skin is most relaxed and pliable. If
this process is carried out faithfully she will have a functional vagina in
between 3 and six months. Many women feel ready to begin this treatment at about
16 years of age, although some decide to wait until they are ready for a sexual
relationship.
Vaginal agenesis can also be corrected surgically. A skin graft from the
buttock or bowel is placed over a vaginal mould which is surgically placed
inside the woman. She remains in hospital and in bed for at least seven days.
After the seventh day, the vaginal mould is removed.
Women who use this procedure
need to wear a vaginal dilator constantly to avoid stricture of the skin graft and the newly created vagina.
Vaginas created with buttock skin tend to be rather dry, and those created
with bowel tissue tend to produce mucus all the time, sometimes necessitating
the woman's wearing a sanitary napkin at all times.
Concerns exist regarding the utilization of bowel for the creation of a vagina due to the risk of sexually transmitted diseases and the fact that bowel is a poor protective barrier against these diseases when compared with skin.
Rarely, doctors attempt to create a cervix to join the surgically created
vagina to the existing uterus. This procedure is sometimes successful, but often
infections necessitating hysterectomy set in. There have been four deaths
reported as being complications of this procedure.
Sexuality after vagina creation
Almost all women who have created vaginas have just as successful sex lives
as women born with vaginas. As women with vaginal agenesis have clitorises and
labia identical with women who have vaginas from birth, they are fully capable
of sexual pleasure and orgasm, and their partners will not be able to tell they
were born without a vagina, although some women with surgically created vaginas
need more artificial lubricant than most women with natural vaginas.
Dilator created vaginas have the same lubricative properties as natural
vaginas.
Fertility
Women born without a vagina, but with normal sized uteri are usually able to
become pregnant and be delivered of a baby with help from such reproductive technologies
as IVF and caesarian deliveries.
Those with remnant uteri are limited to surrogate pregnancies using their own
eggs. | 
