Vaginal Agenesis

What is vaginal agenesis?

Vaginal agenesis is a condition in which a girl is born without a vagina. The external genital organs are formed normally so that from outside her genitalia appear as normal female genitalia. Patients with vaginal agenesis have normal female karyotype (46 XX) and normal female phenotype.

Is vaginal agenesis inheritable?

Some of the conditions associated with vaginal agenesis may be inheritable. Other cases may be caused by exposure to environmental teratogens exposed in utero.

What causes a girl to be born without a uterus?

A girl can be born without a uterus and/or vagina due to some genetic conditions or exposure to environmental teratogens inside the uterus.

What are the Syndromes associated with vaginal agenesis?

There are mainly two conditions associated with vaginal agenesis:

• Mayer-Rokitansky-Küster-Hauser Syndrome (MRKH Syndrome): It is a very rare genetic condition that causes abnormal development of genital organs. In this syndrome the vagina and uterus may either be absent or may have developed abnormally.
• Androgen Insensitivity Syndrome (AIS) It is an inherited condition in which a baby is born without a uterus, fallopian tubes and vagina. In some cases part of the vagina may have been formed.

What are the additional findings in MRKH Syndrome?

In MRKH Syndrome there may be additional abnormalities in the spine and limbs. It is also possible to have heart and kidney anomalies or hearing problems.

What are the symptoms of vaginal agenesis?

• Having no menstrual period by the age of 15
• Cramping and abdominal pain due to pooling of blood inside the uterus (when there is a uterus)
• Presence of a dimple where normally the vaginal opening should be
• Pain during sex (because the vagina is too short or absent)

What are the findings of MRKH Syndrome?

Women with MRKH Syndrome have normal female chromosomes (46 XX) They have normal external genitelia, normal breast and pubic hair development. They have normal functioning ovaries. The uterus and vagina may be either underdeveloped or absent.

What are the treatment options for vaginal agnesis?

There are mainly two teatment options for vaginal agenesis:

• Self dilation: You may use small tubes called dilators to gradually create a vagina. They need to be used around 20-30 minutes each day. You may need to increase the size of the dilators as the vaginal opening enlarges.
• Surgery: There are several surgical options for the treatment of vaginal agenesis. The surgery to create a new vagina is named vaginoplasty. The newly created vagina is named as neovagina.

What are the surgical treatment options for the treatment of vaginal agenesis?

Mc Indoe Vaginoplasty: Vaginal canal is formed surgically by dissection of tissues between the bladder and the rectum. Skin from buttocks, outer thighs or abdomen may be used as a tissue graft to line the walls of the newly formed vagina.A mold or vaginal dilator is inserted into the vagina so that the new vagina will not close. It may need tos tay in the neovagina for a few months after surgery.

Laporoscopic Davydov’s Vaginoplasty In this procedure, peritoneal tissue is used to create neovagina. Laporoscopy is also known as closed surgery or keyhole surgery. This is a sophisticated new technique to create a new vagina in patients with vaginal agenesis. Small cuts made on the abdominal wall are used to insert a camera and other devices to dissect the peritoneum (a thin tissue layer covering the abdominal organs) and relocate this peritoneal tissue to cover the walls of the newly formed vaginal opening, so that this tissue will function as normal vaginal mucosa and the opening will not close again. The walls of the neovagina are lined with the peritoneal tissue flap which is still attached to the abdominal cavity and thus has normal vascularity, due to this vascularity it has better chance to persist than a skin graft. A mold is inserted into the neovagina and needs to stay there for a few months until healing.

Intestinal vaginoplasty: This is the most radical and invasive procedure to create a new vagina. An incision is made on the abdominal wall and a free segment of bowel is cut away to be inserted into the newly formed vaginal canal. This is also known as free tissue transfer. It needs a special microsurgical technique to attach the vessels of the bowel segment to the vessels of the newly formed vagina. Complications that may be seen after this surgery may be summarised as constipation, paralytic ileus, peritonitis and difficulty in urination. Since the bowel segment secretes a lot of mucus, the patients may have a lot of mucoid discharge from the neovagina and may need to use daily sanitary pads.

Inserting a medical traction device-Vecchietti Procedure: An olive shaped device is placed at the vaginal opening. Small cuts are made on the abdominal wall to insert a a camera (laparoscope) and other instruments to make traction on the olice shaped device up into the abdominal cavity. The traction device will gradually pull the olive inward to create a neovagina.

Is it possible to have a normal sex life after surgery for vaginal agenesis?

The patients have a totally normal and satisfactory sexual life after vaginoplasty. As a matter of fact, women do not need to have a vagina for a satisfactory sexual life. Because patients with vaginal agenesis have normal external genitelia including the clitoris. Clitoris is the most sensitive part of the female genital organs and it plays an important role in female sexual stimulation and orgasm. Since patients with vaginal agenesis have a normal clitoris, they can have orgasm even without penetrative intercourse.

However to be able to have penetrative intercourse with a sexual partner, vagina is necessary.

Can a future sexual partner understand that a woman had treatment for vaginal agenesis?

It is not possible for a future sexual partner to understand that a patient had surgery for vaginal agenesis. It is not possible to tell the difference between a normal vagina and a surgically formed neovagina. The function of the neovagina is also the same as a normal vagina.

Is it possible to give birth for patients with uterine agenesis?

It is not possible to give birth for patients with uterine agenesis. However there some treatment options available so that they can have children.

Can people with Mullerian agenesis have children? Can you get pregnant if you were born without a uterus?

There are two treatment options for patients born without a uterus to be able to have children:

Uterine transplantation: This is a very advanced surgery that can only be performed in specialized referral centers. Uterus is transplanted from a living or deceased donor to the patient and immunesupressive medications are given to the patient until birth to prevent the body to reject the newly transplanted uterus. Potential uterus donors are women aged 30-50 years of age in good health conditions who have completed their childbearing. After the transplantation, in vitro fertilisation (IVF) techniques are used and the embryo is transferred in to the transplanted uterus. During IVF the patient’s own eggs can be used. After the completion of child bearing, the transplanted uterus should be removed.

Host uterus: IVF techniques are used to form an embryo from the patient’s own eggs and the embryo is transferred to the uterus of a surrogate mother. A surrogate is a woman who carries and delivers a baby for a couple or an individual.

Do people with MRKH Syndrome have eggs?

Women with MRKH Syndrome have normal ovaries which function normally to produce eggs and hormones. These eggs can be used during IVF treatment. They can be removed from the ovaries by an ultrasound guided oocyte retrieval procedure, fertilised with sperm in the laboratory, the fertiliseed eggs form embryos which can either be transferred to a host uterus or a transplanted uterus.