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A woman with complete vaginal agenesis may have this abnormality based on the fact that the vagina did not grow during embryologic development and this is called Mayer-von Rokitansky-Kuster-Hauser's syndrome. There are many variations to this syndrome; a woman may have no vagina and no uterus, (in which case she would however have normal ovaries) alternatively, she may have no vagina and may have a single midline uterus and no cervix. If this is the case she will not have periods that will allow flow of blood out of her body as she has no cervix and no vagina. With menstruation and shedding of the endometrial lining, the blood would go in a retrograde fashion.

Women with a midline uterus and vaginal agenesis have options for correction of this problem which include: suppression of retrograde menses with the utilization of a continuous oral contraceptive pill with preservation of the uterus in the midline so that she could potentially carry a pregnancy with the utilization of assisted reproductive technologies and a planned abdominal delivery (cesarean section).

Alternatively, surgical procedures have been described to create a communication between a vagina which is created and the upper uterus. There have been cases where this has been successful, however, there have been numerous cases where this has resulted in an infection and the need for a hysterectomy, and there have been four reported deaths from this procedure.

Thus, at Children's Hospital Boston it is recommended that a woman with vaginal agenesis with a midline uterus and no cervix go through a procedure for creating a functional vagina for sexual relations and would maintain her uterus with the utilization of continuous oral contraceptive pills to suppress retrograde menses and the risk of endometriosis. She would thus be a candidate for reproductive technologies with the utilization of GIFT for the placement of the eggs and sperm within the fallopian tubes for possible pregnancy. The birth of the child would require a cesarean section.

Women with vaginal agenesis can also have small rudimentary uterine horns which are lateral to the midline. These uteri can also function, if they contain an endometrial stripe women with rudimentary uterine horns can also be managed in a similar fashion to what is described above, for women with a single midline uterus.

Vaginal agenesis can be diagnosed on physical examination with additional information gathered from ultrasound or MRI. The differential diagnosis includes androgen insensitivity, which is described above. Women with vaginal agenesis from Mayer-von Rokitansky-K|ster-Hauser's syndrome have a karyotype of 46, XX which is the most common for women. Thus obtaining a karyotype can also be helpful in making a definitive diagnosis; additionally a testosterone level can also be helpful in making a diagnosis. Women with Mayer-von Rokitansky-K|ster-Hauser's syndrome will have a testosterone level in the "normal female range," and women with androgen insensitivity will have a testosterone level in the "normal male range."

Women with vaginal agenesis will need to create a vagina to have normal sexual function. There are numerous options for creation of a vagina. It is our recommendation that we initially start with the utilization of vaginal dilators to create a functional vagina. The dilator is pushed against the area where the vagina should be located, and with constant pressure on a daily basis, a woman can create a functional vagina. It should be noted that a woman with vaginal agenesis can have normal orgasmic function as the clitoris and the external genitalia are formed normally. The vagina should have normal vaginal lubrication if the vagina is created with the utilization of vaginal dilators. The process of vaginal dilatation with the utilization of dilators can take between six months and two years, depending on the frequency of the utilization of the dilators.

Alternatively, a vagina can be created with the utilization of a skin graft and this procedure is called a McIndoe procedure. A split thickness skin graft is taken from the buttock and a space is created for the placement of vaginal mold with the skin graft affixed to it. This is a surgical procedure and the woman needs to remain at bed rest in the hospital for approximately seven days, in order for the skin graft to "take." At the end of the seven-day period, the woman is taken back to the operating room for removal of the mold which is used to create the vagina. Once the mold is removed, then the woman is asked to use a vaginal dilator on a continuous basis to avoid stricture of the skin graft and the newly created vagina. The utilization of a skin graft can thus result in a normal, functional vagina. Some women find that they need to utilize water-based lubricants for sexual activity, as the skin of the newly created vagina may be dry.