Thank you for your question. MRKH (Mayer Rokitansky Kuster Hauser Syndrome) is a congenital (occurs during fetal development) problem that affects the female reproductive tract. One in 5,000 females is born with MRKH. MRKH begins during early “fetal” life, when the baby is in the mother’s uterus (womb) and the reproductive tract is beginning to form. The female reproductive tract is typically made up a uterus, cervix, vagina, two fallopian tubes, and two ovaries. Girls who are born with MRKH typically have normal ovaries and fallopian tubes. However, most have no uterus or a just a remnant of a tiny uterus; the vagina is short, narrow, or absent. Some girls born with MRKH may also have only one kidney instead of two. A small number of girls born with MRKH also have trouble with their ability to hear as well as spinal problems such as scoliosis (a curvature of the spine). Because a girl with MRKH has normal ovaries that make estrogen, she will have a growth spurt at the time of puberty, develop normal breasts, and may also feel moodiness once a month. Her ovaries also produce eggs.
However, despite how a girl with MRKH looks like on the inside, it’s impossible to tell a girl has MRKH from the outside. This means that girls with MRKH look just like girls without MRKH. They develop just like any other girls, by beginning with breasts and pubic hair. If you think you may have MRKH because you haven’t started your menstrual periods, it’s important to schedule an appointment with your health care provider (HCP) to discuss your concerns.