Mullerian Agenesis (MRKH)

Definition - What does Mullerian Agenesis (MRKH) mean?

Mullerian agenesis is a congenital (from birth) defect in the development of the mullerian ducts, in which the uterus, cervix (opening of the uterus), and upper vagina fail to develop or do so only partially. There is no uterus and cervix at all, or if present these are very small and poorly formed. Usually, there is no apparent vagina but just a small vaginal opening or dimple. Women with MRKH have normal ovaries and will have normal development of their external genitalia i.e. breasts, clitoris and vulva.

Mullerian agenesis is also called also Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome.

FertilitySmarts explains Mullerian Agenesis (MRKH)

MRKH is a very rare condition and occurs in 1 in every 4000 female births. A woman is unaware she has MRKH until she is older. The main symptoms are the absence of the onset of periods. Pain may be experienced when attempting sexual intercourse.

Since the ovaries develop normally and can produce eggs, women with MRHK syndrome can have their own genetic children, but the absence of a uterus that can carry the pregnancy, will imply the need to use in vitro fertilization (IVF) and a gestational carrier.

A woman with MRKH can sometimes have satisfactory vagina created nonsurgically with successive vaginal dilation. However, if that does not work she may need surgery to enlarge the vagina.

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