Vaginal Agenesis

The Pediatric and Adolescent Gynecology specialists at the Steven and Alexander Cohen Children’s Medical Center of North Shore University Hospital and Long Island Jewish Medical Center are experienced in treating vaginal agenesis. We offer comprehensive non-surgical, minimally invasive, and surgical approaches to treating vaginal agenesis and other congenital abnormalities of the reproductive system.

 

What Is Vaginal Agenesis?

 

Vaginal agenesis, or absent vagina, is a relatively rare disorder of the female reproductive system. We do not know the cause of vaginal agenesis. What we do know is that it is a birth defect that is most commonly associated with absence of the uterus in a syndrome called MRKH, or Mayer Rokitansky Kuster Hauser Syndrome. Absence of the vagina can also be associated with defects of other organs systems including kidney, bone, and hearing. Rarely, vaginal agenesis is associated with abnormal development of the anus and rectum. Girls with MRKH have normal breast development, normal ovaries and normal female hormones. The uterus is usually very small or absent and the vagina is typically absent but can be short and narrow.

 

How Is Vaginal Agenesis Diagnosed?

 

Girls with MRKH usually are diagnosed between 15 and 18 years old. Most girls find out that the vagina is missing after the doctor checks to see why the period has not started. The diagnosis can be made by the doctor’s examination. Most doctors will order a pelvic ultrasound or MRI to check if there is a uterus and to look at the ovaries and kidneys.

 

How Is Vaginal Agenesis Treated?

 

Treatment for vaginal agenesis typically occurs in adolescence. The exact timing depends on the motivation and maturity of the individual. There are several options for treating vaginal agenesis. Vaginal dilation upon approaching sexual maturity is the most appropriate first-line treatment for vaginal agenesis. This can be done by the patient herself over the course of six to twelve months, or it can be performed using a laparoscopic traction device over a 10-day period. Vaginoplasty or surgical grafts can be used to create a vagina using skin, bowel, or peritoneum.

Intermittent self dilation

Many girls with vaginal agenesis choose to make a vagina using dilators. This is an effective and non invasive method where the patient will use a dilator to stretch the skin where the vagina belongs. The patient applies pressure for 15-20 minutes twice a day. Several months to over a year may be required to obtain a functional vagina.

Continuous dilation (Vecchietti procedure)

A dilator is placed at the vaginal opening or vaginal dimple and attached to two sutures which are placed through the abdomen laparoscopically and attached to a device. Continuous traction is placed on the dilator allowing the skin where the vagina belongs to stretch, resulting in a functional vagina in seven to ten days. The technique is minimally invasive and achieves the same results as self dilation in a much faster time frame. Intercourse or manual dilation is required to maintain the vagina.

Skin graft vaginoplasty (McIndoe procedure)

A skin graft is taken from the buttocks and placed on a mold. Other materials that act like skin can also be used. The mold is inserted in a space that is surgically created where the vagina should be located. 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 at all times for a while. Manual dilation is required to maintain the vagina. The new vagina is functional after approximately two months.

Bowel graft vaginoplasty

A portion of the bowel is transplanted to a space where the vagina is normally located. This is a major operation requiring abdominal surgery. Many girls will have excessive discharge after this procedure. This procedure does not typically require maintenance or manual dilation.

Laparoscopic assisted peritoneal neovagina (Davydov)

The space where the vagina should be located is surgically dissected and the inner wall of the abdomen is laparoscopically mobilized to fill that space. The new vagina must be maintained with intermittent manual dilation. The new vagina is functional after six weeks.

 

How Often Do I Need to See My Doctor?

 

Regardless of whether you choose to use dilators or have surgery, it is very important to see your doctor frequently. Your doctor will guide you in proper usage of the dilators and monitor your progress. A physician who specializes in treating patients with vaginal agenesis plays an important role in the successful outcome of any method used to creating a vagina.

 

Will I Be Able to Have Children?

 

If you have a uterus but no vagina, a new vagina can be made and the uterus can be used to carry a pregnancy. On the other hand, if you were born without a uterus, you will not be able to carry a pregnancy. Because your ovaries are normal, your eggs can be fertilized outside of your body and the pregnancy can be carried by someone else. This person is called a surrogate. In this case, you are the biological parent. Adoption is another option.

North Shore-LIJ gynecologists are experienced in the diagnosis and surgical repair of vaginal agenesis. For more information on vaginal agenesis or to schedule a consultation, please contact the Division of Pediatric and Adolescent Gynecology at Cohen Children’s Medical Center of New York at 516-390-9258.  For an emergency call 911 or go to the Cohen Children's Medical Center Emergency Room.

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