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Sex Health Blog

Vaginal Agenesis

Oct 15, 2013

Jocelyn’s daughter was growing up fast. Now sixteen, Abby was active in her school theater program, played softball, and worked at the local diner on the weekends. Her grades were good and she was thinking about college. And she had her first serious boyfriend.

Jocelyn was proud of her daughter and so pleased that Abby was happy and healthy. There was one thing, though, that puzzled her. Abby hadn’t started her menstrual periods yet. It was strange, since everything else about Abby’s puberty had seemed normal.

Jocelyn knew that some girls started their periods late, but it troubled Abby. She felt awkward at school, especially during gym class when other girls shared their experiences with menstruation.

“We’ll call the doctor,” Jocelyn told her. “Just to make sure everything is all right.”

Jocelyn and Abby weren’t prepared for the diagnosis: vaginal agenesis. Abby had been born without a properly-formed vagina. She would need treatment to create one, if she ever hoped to have sexual relationships and, if possible, children.

Vaginal agenesis is rare. According to the Urology Care Foundation, it affects about 1 in 5,000 females. But it can be difficult for families to accept. Today, we’ll cover some of the basics of vaginal agenesis and how it is treated.

What is vaginal agenesis?

Vaginal agenesis is a birth defect in which girls are born either without a vagina or with a remnant of one. There might be a “dimple” where the vagina is supposed to be. Some girls with vaginal agenesis are born without a fully-formed uterus as well.

Often, vaginal agenesis isn’t caught right away. In fact, for many girls like Abby, the condition isn’t found until their teen years when they haven’t started menstruating.

Why? Girls with vaginal agenesis do have functioning ovaries, which secrete hormones. As a result, girls with vaginal agenesis go through puberty just like girls without it.

One of the most common types of vaginal agenesis is called Mayer-von Rokitansky-Küster-Hauser syndrome (MRKH). Some girls with MRKH also have kidney problems, hearing loss, and spinal curvature.

What causes vaginal agenesis?

Unfortunately, no one knows what causes vaginal agenesis. It is a congenital condition - a birth defect - that just happens. It is not hereditary. And there is nothing a pregnant woman can do to prevent it.

What can be done about vaginal agenesis?

There are several treatment options for vaginal agenesis. Treatment decisions often depend on the severity of the condition and the maturity and readiness of the girl.

Many girls with vaginal agenesis start treatment in their teenage years, when they are old enough to handle both the physical and emotional aspects.

  • Self-dilation (the Frank method)is usually the first treatment considered. This procedure involves pressing a tampon-shaped plastic tube called a dilator against the area where the vagina should be. This process stretches the area. Larger dilators are introduced gradually. Using this method for 15-20 minutes a day, a new vagina is created over the course of several months to a year.
  • Continuous dilation (the Vecchietti procedure) creates a vagina in about ten days through a combination of surgery and dilation. With this method, an olive-shaped bead is placed where the vagina should be. This bead is attached to strings that are surgically implanted in the abdomen. The strings then exit the body and are attached to a traction device. Each day, the patient “cranks” the device, pulling the bead upward to create the vagina. Once finished, the equipment is surgically removed.
  • Surgery for vaginal agenesis can take many forms. For example, a new vagina may be created with a skin graft from the buttocks. The tissue is placed over a mold and inserted where the vagina should be. Another surgical procedure uses part of the bowel to create a vagina. Recovery after surgery takes time. Bed rest and catheterization might be necessary. Some girls need to wear a dilator almost continuously for a few months. Girls who have a bowel vagina will likely need to wear pads for the rest of their lives, as chronic vaginal discharge is a common problem.

No matter what treatment path a girl takes, emotional and psychological support is critical. The diagnosis takes time to sink in and the treatment might be difficult. Patient respect and privacy are important, but so is being willing to listen and talk. Some girls find counseling helpful as they enter this new part of their lives and process what it may mean for the future.

What about the future?

It may take some time, but women who have been treated for vaginal agenesis can go on to have satisfying sexual relationships. Sometimes, male partners aren’t even aware that women have a surgically constructed vagina.

However, fertility might be difficult. If a woman treated for vaginal agenesis does have a fully-formed uterus, she might be able to have fertility treatment and carry a baby.

If she doesn’t have a uterus – or if the one she has is too small – carrying a baby won’t be possible. Fertility experts may be able to harvest eggs produced by her ovaries and fertilize them with a partner’s sperm. But the baby would need to be carried by a surrogate. Some couples in this situation consider adoption as well.

Vaginal agenesis can be a trying situation for girls and their families. With proper care and solid support, however, girls can move forward.

Resources

Advanced Reproductive Medicine – University of Colorado

“Vaginal Agenesis”

http://arm.coloradowomenshealth.com/resources/medical/vaginal-agenesis

American Congress of Obstetricians and Gynecologists

“Müllerian Agenesis: Diagnosis, Management, and Treatment”

(Committee Opinion. May 2013.)

http://www.acog.org/~/media/Committee%20Opinions/Committee%20on%20Adolescent%20Health%20Care/co562.pdf?dmc=1&ts=20130923T1459185227

Center for Young Women’s Health

“MRKH: A Guide for Parents and Guardians”

(Updated: October 20, 2011)

http://www.youngwomenshealth.org/PDFs/Full%20PDF's%20of%20Broken%20Out%20Guides/MRKH%20Parent_Full.pdf

Fertility and Sterility

Kimberley, Natalie, B.Med.Sci, et al.

“Well-being and sexual function outcomes in women with vaginal agenesis”

(Full-text. January 2011)

Article in press accessed via MRKH Canada Blog: http://mrkhcanada.files.wordpress.com/2010/08/well-being-and-sexual-function-outcomes-in-women-with-vaginal-agenesis.pdf

Johns Hopkins Medicine

“Treating all facets of a rare malformation: vaginal agenesis”

(March 1, 2011)

http://www.hopkinsmedicine.org/news/publications/physician_update/physician_update_winter_2011/treating_all_facets_of_a_rare_malformation_vaginal_agenesis

North Shore LIJ Health System

“Vaginal Agenesis”

http://www.northshorelij.com/wh/wh-our-services/wh-ped-adol-gynecology-sexual-dev-vaginal-agenesis

Urology Care Foundation

“Vaginal Abnormalities: Vaginal Agenesis”

(Last updated: April 2013)

http://www.urologyhealth.org/urology/index.cfm?article=50