Late bloomer or MRKH?

Girls who have not started their periods by the appropriate age should be checked for Mayer Rokitanksy Kuster Hauser Syndrome (MRKH). MRKH, named after four men who studied this birth defect, describing a female born without a uterus or only part of one, no cervix, and no vagina or a very short one and normal ovaries. Doctors can't usually tell anything by a pelvic exam since the vagina is closed off.

Ultrasound, magnetic resonance imaging (MRI), and laparoscopy are all ways to determine what reproductive organs the females have. These are usually done after blood tests show the woman is genetically a female.

As for treatment, there are three treatment options to open the vagina: dilation, surgery, and nothing. For girls who have some vaginal opening, dilation with plastic dilators in graduated sizes are an option. Those who have no opening at all can have surgery. Of course, teenage girls may wish to put off one of these treatments until older or do nothing at all.

A hysterectomy may have to be performed on some girls who have uterine remnants which may cause pain that mimic menstrual cramps.

Then there are other medical problems that come from this condition. Kidney and skeletal problems are the most common followed by hearing loss. The symptoms vary as much as the women themselves. Some have no problems other than the gynecological ones.

Parents should be supportive of their teenagers and not pressure them with vaginal treatments and the "having children" issue.

Besides being aware of physical problems and how to treat them, MRKH women may suffer mental/ emotional issues as well. Family reactions can bother a teenage girl. Many mothers blame themselves for something they did during their pregnancies that may have caused this. Yet MRKH has no known cause. Chromosomal studies have not found a genetic cause for MRKH.

Three choices exist for having children as well. Adoption is an option of course. Also gestational surrogacy has been successful. This is when the man's sperm and the MRKH woman's eggs are combined to form an embryo which is implanted into another woman's host uterus. The child is the MRKH woman's biological child. However, the success rate for this procedure is low. Living childfree is the third option.

Some doctors don't realize this is a syndrome, much less that it's MRKH. They just think of the reproductive aspects. Hopefully, doctors and other medical staff will become more educated about MRKH as a syndrome which may have physical and mental repercussions beyond the "female problems."

The internet is a wonderful source of information with not only support groups but websites devoted to the MRKH cause. With the Internet, supportive parents and partners, and understanding medical staff, teen girls with MRKH should grow up to be normal, healthy, and happy.

Niki Taylor is a writer, book reviewer, and website content manager. She has degrees in English and Library Science. Having MRKH gives her a special insight into this syndrome.

Some of the medical information was found in the MRKH FAQ compiled by Kathy Willis, Programs Director of the MRKG Foundation.

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