Vaginal agenesis at a glance
- Vaginal agenesis is a birth defect in which a woman is born without a vagina.
- Women typically discover the condition when their menstrual cycle does not begin during puberty.
- Non-surgical and surgical treatments are available to correct the condition.
- Women with vaginal agenesis may regain full fertility after treatment, but in some cases may only be able to get pregnant through assisted reproductive treatments.
Causes of vaginal agenesis
During pregnancy, a baby’s reproductive system may not finish developing in the mother’s uterus. This can cause the baby to be born without a vagina, or have a shorter vagina or a remnant of one. This condition is called vaginal agenesis.
Vaginal agenesis is a birth defect that affects 1 out of 5,000 to 7,000 female infants. It also may be accompanied by other malformations in the reproductive tract, such as an absent or small uterus. The cause for vaginal agenesis is unknown.
Vaginal agenesis and infertility
Without correction, the condition can make sexual intercourse and childbearing impossible.
Mayer-von Rokitansky-Küster-Hauser syndrome (MRKH) is the most common form of vaginal agenesis. All women with MRKH have functional ovaries, develop normally, and produce the normal level of hormones.
Women who have vaginal agenesis but do have a complete uterus may be able to have genetic offspring through in vitro fertilization (IVF). If there is no uterus, women with MRKH cannot carry a pregnancy, but genetic children may still be possible through surrogacy.
Symptoms of vaginal agenesis
Women with vaginal agenesis typically discover the condition when their menstrual cycle does not begin during puberty. Some find out earlier through surgeries for other conditions, such as a hernia.
The condition also may be recognized if there is a small pouch or dimple in place of the vaginal opening. Lower abdominal pain may also occur if a uterus is present but lacks a connection to a vaginal canal.
Women with MRKH may also be missing a kidney, or suffer hearing loss and curvature of the spine.
Vaginal agenesis treatment
Non-surgical vaginal dilation in adolescence is generally the first line of vaginal agenesis treatment.
Intermittent self dilation
Many women with vaginal agenesis are able to form a vagina using dilators. This is an effective and non-invasive method where the patient uses a dilator to stretch the skin where the vagina belongs. By applying pressure for 15 to 20 minutes twice a day over several months, this method can create a functional vagina.
Continuous dilation (Vecchietti procedure)
Another method to create a vaginal passageway uses an olive-shaped device that is placed at the vaginal opening. This device connects laparoscopically to a traction device placed on the lower abdomen. The traction device is tightened daily, gradually pulling the olive-shaped device inward to create a vagina in a period of about one week.
The continuous dilation technique is minimally invasive and achieves the same results as self-dilation in a faster time frame. Regular manual dilation and/or sexual intercourse will help to maintain a functioning vagina.
Surgical treatments include vaginoplasty or grafts to create a vagina using skin, bowel, or peritoneum.
Skin graft vaginoplasty (McIndoe procedure)
Skin graft vaginoplasty involves taking a skin graft from the buttocks and placing it on a mold. Other materials that act like skin can also be used. The mold is inserted in a surgically-created space where the vagina should be. This is an in-patient procedure where the patient must be hospitalized on bed rest for seven days with the mold in place to allow for healing. A dilator must then be worn for approximately three months after the surgery.
The new vagina will be functional after approximately two months. Ongoing manual dilation is required to maintain the vagina.
Bowel graft vaginoplasty
Bowl graft vaginoplasty inolves transplanting a portion of the bowel to a space where the vagina is normally located. This is a major operation requiring abdominal surgery, and many women will have chronic vaginal discharge after this procedure, requiring them to wear a pad every day. However, bowel graft vaginoplasty typically does not require daily manual dilation following the procedure.
Laparoscopic assisted peritoneal neovagina (Davydov)
A laparoscopic procedure can also correct the absent or undeveloped vagina by surgically dissecting the space where the vagina should be located and laparoscopically mobilizing the inner wall of the abdomen to fill that space. The new vagina must be maintained with intermittent manual dilation and is functional after six weeks.