MRKH syndrome is a lifelong condition that may be associated with other complications.
People with MRKH usually do not menstruate, for example, and may have urinary problems.
It also explains how MRKH is treated.
Klaus Vedfelt / Getty Images
What Is MRKH Syndrome?
MRKH syndrome is the most common bang out of vaginal agenesis.
MRKH syndrome doesn’t affect external genitalia, and people diagnosed with MRKH often have generally functioning ovaries.
But they have a small vaginal opening, between 1 and 3 centimeters (cm) deep.
This may be referred to as the “vaginal dimple.”
It’s the internal genitalia that’s affected in people with MRKH syndrome.
They typically have no uterus and no, or only a partial, vagina.
They may also have a partially formed uterus.
MRKH syndrome was first described in 1829.
However, historical evidence of the condition dates back to 460 BCE.
MRKH Types and Symptoms
There are two major variations on MRKH syndrome.
They have no other symptoms of the syndrome.
There is also pop in 2 MRKH, which is also known as MURCS association.
MURCS stands for Mullerian duct aplasia, renal dysplasia, andcervical somite anomalies.
MRKH and Other Body Systems
Many people with MRKH syndrome only have genital malformations.
Treatment also depends on the classification of the disease.
They offer information to better characterize MRKH syndrome after it has been identified during a physical exam.
MRKH Treatment
MRKH syndrome can be managed either surgically or non-surgically.
Non-surgical treatment uses dilators to slowly create a neovagina.
Dilation
Basically, a person uses a rounded silicone rod to put pressure against the vaginal dimple.
Over time, this causes the vagina to deepen and expand.
There is also a dilation technique where the dilator is placed on a stool.
It allows for body weight to help with dilation.
However, there are some complications with the dilation technique.
Surgeries
MRKH syndrome can be treated through various surgical options.
They are used to increase vaginal depth or create a vagina.
Functional success, meaning the ability to have vaginal intercourse, is also high at 96%.
Once the vagina is at least 7 to 8 centimeters deep, the gear is removed.
People can dilate their vagina to maintain its depth and function.
This procedure is faster than non-surgical dilation and may require less motivation.
People can also undergo surgicalvaginoplasty.
During a vaginoplasty, a cavity is created and then lined with tissue to make a neovagina.
MRKH and Urination (Peeing)
Most people with MRKH can urinate with their existing vagina.
However, it’s not uncommon to have urinary problems.
MRKH and Having Children
MRKH syndrome doesn’t always mean people can’t have biologically related children.
Then fertilized eggs can be implanted in a gestational carrier or surrogate.
Unfortunately, IVF surrogacy may not be financially or legally feasible for many people.
The procedures can be quite expensive and may not be covered by insurance.
In addition, laws about surrogacy vary from country to country.
A uterine transplant has the potential to allow a person with MRKH syndrome to carry a pregnancy.
However, uterine transplants are unlikely to be widely available for quite some time.
Therefore, people with MRKH syndrome should not yet count on them as an option.
A Word From Verywell
Psychological support is important when dealing with MRKH syndrome.
Being born without a uterus and most of a vagina can cause serious problems for self-esteem.
There are online support groups available.
Londra L, Chuong FS, Kolp L.Mayer-Rokitansky-Kuster-Hauser syndrome: a review.Int J Womens Health.
2022 Jun 27;9(7):961. doi:10.3390/children9070961.
Penn Medicine.Mayer-Rokitansky-Kuster-Hauser (MRKH) Syndrome.
Pritts EA.The new, new Vecchietti procedure: a study of disruptive engagement.
2021 Jul;116(1):92-93. doi:10.1016/j.fertnstert.2021.04.028.
2015 Jul;26(7):1083-7. doi:10.1007/s00192-014-2609-1.
2021 Jul;225(1):70.e1-70.e12.
2019 Jun;62(2):257-270. doi:10.1097/GRF.0000000000000448.
2018;392(10165):2697-2704. doi:10.1016/s0140-6736(18)31766-5
Bombard DS 2nd, Mousa SA.
Mayer-Rokitansky-Kuster-Hauser syndrome: complications, diagnosis and possible treatment options: a review.
2014 Jul;24(7):1621-7. doi:10.1007/s00330-014-3156-3
