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.

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