What Is Agenesis of the Vagina?
Vaginal agenesis is a rarely occurring congenital birth disorder occurring in females, in which the vagina does not develop or present with a very short vaginal canal, and the uterus may develop partially or absent. Additionally, this condition may be associated with skeletal or kidney problems. At times, vaginal agenesis is diagnosed at the time of birth, but most of the time, this condition is not diagnosed until puberty, when menstruation does not occur. Vaginal agenesis is also known as Mayer-Rokitansky-Kuster-Hauser syndrome, Mullerian aplasia, or Mullerian agenesis.
What Are the Causes of Vaginal Agenesis?
Vaginl agenesis is a congenital condition (present from birth), its exact cause of vaginal agenesis is not clear. Normally, the lower portion of the Mullerian ducts develops into the vagina and the uterus, and the upper portion of the ducts forms the fallopian tubes. If the ducts are underdevelopment, it can result in a partially closed vagina or absence of the vagina, as well as the absent or partial uterus, or both.
Vaginal agenesis can also occur as a symptom of a condition that involves several abnormalities of the female reproductive system. Those conditions include:
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Mayer-VON Rokitansky-Kuster-Hauser’s Syndrome (MRKH) - It is a condition that causes the uterus and the vagina to be underdeveloped or absent; it may include other abnormalities of female reproductive symptoms.
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Complete Androgen Sensitivity Syndrome (AIS) - In this condition, the patients have a female appearance, but there is an absence of a vagina, uterus, cervix, fallopian tubes, and ovaries.
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MURCS (Mullerian, Renal, Cervicothoracic, Somite) Association - It is a condition that includes MRKH syndrome abnormalities as well as several others, including short stature, spine abnormalities, and kidney defects.
What Are the Symptoms of Vaginal Agenesis?
In many patients, vaginal agenesis is not noticed until they reach their teens and menstruation does not occur but all other signs of puberty happen. Vaginal agenesis also includes the following features:
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Painful amenorrhea is if the uterus is present and the menstrual blood can not flow out.
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Monthly abdominal pain and cramps due to the menstrual blood build-up in the uterus.
Vaginal agenesis is seen associated with other symptoms, such as:
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Problems with the development of the kidneys and urinary tract.
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Developmental changes in the spine, wrists, and rib bones.
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Hearing trouble.
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It can also include other congenital conditions of the gastrointestinal tract, heart, and limb growth.
What Are the Diagnostic Procedures Used to Diagnose Vaginal Agenesis?
Vaginal agenesis is normally diagnosed during puberty when the menstrual periods do not occur, even after the signs of puberty like breast development and underarm and pubic hair development occur. At times, the vaginal agenesis can be identified at an earlier period when a doctor or parents notice a baby with the absence of vaginal opening or while evaluating other health problems.
The diagnostic tests for agenesis of the vagina may include:
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Ultrasound - With the help of ultrasound images the health care provider checks for the presence of ovaries and uterus and identifies any problems with them as well as the problems related to the kidneys.
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Blood Tests - It can assess the chromosomes and measure the hormone levels to rule out other conditions.
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Magnetic Resonance Imaging (MRI) - It provides a detailed image of the reproductive tract and kidneys.
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Other Testing - Tests to examine the heart, skeleton, and hearing abilities will be asked to be performed by the health care providers.
What Is the Treatment for Agenesis of the Vagina?
Vaginal agenesis treatment normally takes place during the late teens or early twenties. The health care provider will discuss the treatment options and depending on the individual condition and interest the treatment can be chosen. The treatment includes:
Vaginal Dilators -
Vaginal dilators are the most standard and efficient treatment for MRKH. The aim of this technique is to lengthen the vagina to a size that is comfortable for sexual intercourse. In this method, a firm smooth, round rod of plastic shaped similar to a tampon is used to pressure against the skin at the vaginal opening or inside the existing vagina. It is typically used for 10 to 20 minutes about one to three times a day. After a few days, larger dilators are recommended, and to see the visible results, it will take a few months. The main advantage of dilators is that it does not require surgery and the disadvantage is that it requires frequent use of the dilator until the vaginal canal becomes a normal length, problems with urinating, and vaginal bleeding and pain can be experienced in the beginning. Using artificial lubrication and a different type of dilator might be helpful.
Surgery (Vaginoplasty) -
Vaginoplasty is recommended for women with vaginal agenesis.
It can be done with different methods that include:
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Skin Graft - A graft is taken from the patient's own tissue to create a vagina. It is mostly taken from the skin from the buttocks, outer thigh, or lower abdomen. An incision is made to create the vaginal opening and the tissue is grafted over a mold to create the vagina and placed in the newly formed canal. The mold is left in place for about one week. After the surgery, bed rest is recommended and a catheter is attached to drain the urine.
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Bowel Vaginoplasty - In this method, a portion of the colon is moved to create an opening in the genital area to create a new vagina. Then one end of the colon is closed by the surgeon. The vaginal dilator need not be used every day after this surgery. A mold is placed for three days after the surgery and a catheter is attached for a week to drain the urine.
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Inserting a Medical Traction Device - A balloon device (balloon vaginoplasty) or an olive-shaped device (vecchietti procedure) is placed at the vaginal opening using a laparoscope as a guide, and a separate traction device is connected to the lower abdomen or through the navel. Tightening the traction device every day by the patient is needed which gradually pulls the device inward to create a vaginal opening. After the removal of the device, a mold of different sizes is used for about three months.
After the Surgery -
A vaginal dilator for three months after the surgery is recommended for most patients. It can be removed during showers, during bowel movements, and during sexual intercourse. After three months dilators are recommended to wear for another three months at night.
Conclusion -
Agenesis of the vagina is a rare condition, occuring before birth. It is often left unnoticed until the puberty period of a girl as menstruation is not possible due to a lack of vaginal opening. The treatment for vaginal agenesis is using a vaginal dilator or surgery to create a vaginal opening. Most doctors recommend vaginal dilators as an initial treatment option as it is an invasive method.
