What is Mayer-Rokitansky-Küster-Hauser syndrome?

This rare reproductive disorder affects 1 in 5000 women.

Mayer-Rokitansky-Küster-Hauser syndrome. Not a name that exactly trips of the tongue. And perhaps that’s why this reproductive disorder remains relatively unknown. Also known as the less tongue-twisting MRKH or Mullerian agenesis, this syndrome causes the vagina and uterus to be underdeveloped or absent, making it is difficult for women who live with it to become pregnant or carry a child. The diagnosis of MRKH is usually made between 16 and 18 when a girl fails to start having periods despite other normal signs of puberty. In this blog, we take a closer look at the MRKH, its diagnosis and treatments.


hands holding uterus female reproductive system


What is MRKH syndrome?

MRKH or Mayer-Rokitansky-Küster-Hauser syndrome is a congenital abnormality which affects around 1 in 5000 women. Congenital abnormality means that the disorder is present from birth and develops in the womb as the foetus grows. MRKH causes the uterus, vagina, and cervix to be underdeveloped or absent entirely. This makes it difficult for women who have the disorder to have sex without treatment or to conceive and carry a child. The external reproductive structures, ovaries and fallopian tube are usually fully developed and functioning normally. Some women with MRKH also suffer from related problems with their kidneys, spine and hearing. The cause is unknown, and MRKH is usually not hereditary.

How is MRKH diagnosed?

MRKH is often diagnosed during late puberty when a girl’s period fails to develop or after reports of painful sex. GPs who suspect a diagnosis of Mayer-Rokitansky-Küster-Hauser syndrome are likely to refer patients to a specialist gynaecologist where it will be confirmed via ultrasound and blood tests to rule out genetic abnormalities. Other investigations and tests may be required if it is suspected that a patient has any of the associated renal, spinal or hearing problems associated with MRKH.

What are the treatments for MRKH?

Mayer-Rokitansky-Küster-Hauser syndrome can lead to painful or difficult sex because of the absence of a fully developed vagina. Most women with MRKH, even those with just a small amount of vaginal tissue are able to achieve a comfortable and enjoyable sex life through dilator therapy. This is where a specialist vaginal dilator is used to slowly expand the vaginal opening. This treatment may not be suitable for a small number of women and surgical intervention may be necessary. Other treatments may be suggested, specific to the unique symptoms of the patient. Many women go through a period of shock and distress on being diagnosed with MKRH and they may also be referred for psychological therapy to help them come to terms with their diagnosis.

Can women with MRKH ever have babies?

As women with Mayer-Rokitansky-Küster-Hauser syndrome are born without a fully developed vagina or womb it is very unlikely that they will be able to become pregnant or successfully carry a baby to term. However, as the ovaries usually remain functioning and intact it is possible to harvest a woman’s eggs for fertilisation using her partner’s sperm and appropriate fertility treatment. This will require the use of a surrogate but does mean that women with MRKH can have their own biological children. In the future, this could change. A revolutionary group of researchers in Sweden have successfully carried out seven uterus transplants, followed by the birth of eight healthy children. Although this treatment is in its early stages, it may offer hope for women born with MRKH in the future.

I have been diagnosed with MRKH. Can I get in touch with other women in the same position?

There are a number of online communities for women with Mayer-Rokitansky-Küster-Hauser syndrome. These can be a useful resource for women going through diagnosis or living with the reality of MRKH day to day. These communities can help women access further information on the disorder and reach out to other women with similar stories. MRKH Connect is a registered UK charity that helps women around the world with MRKH connect and form friendships in a safe online community.

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