Mayer-Rokitansky-Kuster-Hauser syndrome - Having no uterus.


Q: I am 20 years old. Im a female. I went to the doctors when I was 17 because I didn't have a women's cycle, a period. The doctors took tests and an ultra sound. They found out that my uterus is gone, that I don't have one OR it is extremely small due to a birth defect. The doctor said I won't be able to have kids in the future, but I still have my ovaries and I could do serogation or adoption. What I do not understand is HOW my ovaries are just sitting there not connected to my uterus since I don't have one. Can my uterus eventual get bigger?? Can you help me better understand this situation?


A:   It is difficult for someone to go through what you are experiencing and I do understand your concern. It is likely that you have Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome. This is is a disorder that occurs in females and mainly affects the reproductive system. This condition causes the vagina and uterus to be underdeveloped or absent. Affected women usually do not have menstrual periods due to the absent uterus. Often, the first noticeable sign of MRKH syndrome is that menstruation does not begin by age 16 (primary amenorrhea). Women with MRKH syndrome have a female chromosome pattern (46,XX) and normally functioning ovaries. They also have normal female external genitalia and normal breast and pubic hair development. MRKH syndrome affects approximately 1 in 4,500 newborn girls. The cause of MRKH syndrome is unknown, although it probably results from a combination of genetic and environmental factors.
It is unlikely that your uterus will get bigger. Although women with this condition are usually unable to carry a pregnancy, they may be able to have children through assisted reproduction. Women with Mayer-Rokitansky-Kuster-Hauser syndrome can become pregnant by having oocytes harvested, fertilized, and implanted in a surrogate. I hope I could be of some help, take care.

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