What is MRKH?
MRKH is a congenital disorder that affects the female reproductive tract. Congenital means that it’s acquired during development and present at birth. About 1 in every 5,000 female babies has this condition. MRKH is a syndrome (group of symptoms). We don’t know the cause of this syndrome, but we do know that when a baby grows in their mother’s uterus (womb), organs and systems develop. One of the systems is called the reproductive system, which in female babies includes the uterus, cervix, vagina, fallopian tubes, and ovaries. The reproductive system is formed during the first few months of “fetal” life (while a baby is still in her mother’s womb). With MRKH, the reproductive system starts to grow but doesn’t completely develop.
People with MRKH typically have normal ovaries and fallopian tubes. Most often the uterus is absent or tiny. The vaginal canal is typically shorter and narrower than usual or it may be absent. Sometimes, there may be one kidney instead of two. About 3% of people diagnosed with MRKH will have a minor hearing loss and some may have spinal problems such as scoliosis (curvature of the spine). People with MRKH have normal external genitalia, which means everything on the outside of the vagina is not affected. This part of your body is called the vulva and includes what you can see – clitoris, urethra, labia, vaginal opening, hymen and anus. (Source: Boston Children’s Hospital)