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What is MRKH?
- MRKH occurs in people with XX sex chromosomes when the internal reproductive tract develops without a full uterus, cervix and upper vagina.
- Usually people with MRKH are assigned female at birth and have one or two ovaries, will ovulate but not menstruate.
- People with MRKH have typical vulva and clitoris and all the usual secondary sex characteristics at puberty such as breasts, and body shape.
- MRKH occurs in around 1 in 4000-5000 female births annually.
- Most people find out they have MRKH or a related trait in their mid to late teenage years, but earlier detection can be due to type 2 MRKH.
- In some cases, other parts of the body have differences including issues related to skeletal, ears, heart and kidneys.
Type 1
- Characterised by the absence of the upper 2/3 of the vagina, cervix and uterus.
- Other sex characteristics (such as the external genitalia, ovaries and secondary sex characteristics meet typical female norms)
- Some people with Type 1 MRKH may have a small amount of uterine tissue, however, the absence of a complete uterus and the absence of a cervix (blind end) means that pregnancy is not possible
- Ovaries may be ectopic which means that they may not be found in the typical location, and therefore difficult to locate on an ultrasound. This sometimes results in assuming only one ovary is present.
- Ovaries are functional, producing hormones and releasing eggs on a regular basis and then producing progesterone after ovulation, allowing for puberty, hormonal health and fertility options through assisted reproductive therapies and surrogacy.
- People with MRKH will not need HRT until symptoms of perimenopause or menopause become apparent and interrupt well-being
Type 2
Type 2 MRKH, is similar to type 1, but also includes differences in other parts of the body.
Most commonly these differences are in the skeletal, urinary tract, kidneys and less often hearing and heart.
Where Does MRKH Come From?
- MRKH and related variations relate to the partial formation of the Mullerian duct during the first 6-8 weeks of a pregnancy.
- The Mullerian duct is the structure that develops into the female reproductive tract forming the uterus, cervix, and upper portion of the vagina.
- The reasons behind this are not understood, but clinical research presently seeks to identify any genetic origins.
Recognising MRKH
Type 1
Type 2
Urinary and kidney variations
- Urinary tract variations (40%).
- Absence of one kidney (23-28%).
- Ectopic kidneys (one or both kidneys are in atypical place) (17%).
- Underdeveloped kidneys (4%).
- Horseshoe kidney (when the kidneys are fused together).
- Hydronephrosis (when urine is unable to be drained properly and travels back up the urinary tract and into the kidney).
Other possible anatomical variations:
- Hearing impairment from differences in the middle or inner ear (10-20%).
- Skeletal differences in the spine or ribs (less commonly seen in the face and limbs).
- Heart anomalies including aorto-pulmonary window, atrial septal defects, or pulmonary valvular stenosis (though this is rare).
Health Considerations
Most people with MRKH live full, happy, and healthy lives. However, there are some health considerations to be aware of:
- Kidney and urinary conditions
- Hearing impairment
- Mobility considerations due to skeletal differences
- Heart defects
- Fertility considerations
- Psychosocial impacts
Treatment Considerations
You can speak with your doctor about your options should you wish to address or manage the above aspects of your variation. It is also perfectly acceptable not to opt for any intervention or medical management.
The Australian Human Rights Commission recommends minimal medical intervention for people with intersex variations until a person is of an age to consent to treatment unless there is a clear medical reason to intervene. You should be supported to make the decisions that are best for you.
Talk with a doctor you trust to establish a healthcare plan that is individualised to your needs. Your healthcare plan might include regular check-ups, any required regular testing or health screening, and medications that you may need. Although people with MRKH don’t have a cervix, screening tests are still recommended. Refer to this fact sheet to learn more or visit https://www.mrkhaustralia.org/mrkh. It is important that your doctor or health provider listens and responds to your preferences, explains the benefits and risks of any treatment they propose, and provides information about any alternative available options, including the decision to not undertake treatments.
It is vital to seek psychological and/or peer support as soon as you are ready. Peer support networks can provide emotional support, information and referrals to professionals and help you feel less alone, develop the tools to talk about your body, prepare for medical appointments and normalise your feelings or experiences related to having MRKH. A knowledgeable counselor can help you make sense of your feelings and adjust to the changes taking place along your journey.
Some treatment pathways are predicated on the idea that it is preferable to make bodies fit stereotypical characteristics as much as possible.
Typical assumptions for MRKH are centred around the core idea that everyone with MRKH identifies or understands themselves as a girl/woman in line with their anatomy and sex of rearing. While this may be true for many, or even most people, and these assumptions exist, it is important to remember that people understand themselves in many different ways, and all understandings are equally valid.
This assumption also influences the categorisation of MRKH as an intersex variation.
- Categorisation of MRKH as an intersex variation is often disputed due to incorrect assumptions that the word intersex means something about an individual’s identity.
- MRKH fits most working definitions of intersex because it is an innate variation of sex characteristics.
- Like other intersex characteristics, MRKH is associated with medicalisation and experiences of stigma and shame due to innate sex characteristics.
- These factors mean that people with MRKH can benefit from intersex support services and groups.
Dilation
For people with MRKH a non-surgical treatment called dilation may be suggested. Dilation is a process of gradually stretching and opening the vaginal canal with a series of cylinder or tube-shaped devices called dilators.
It is important that dilation is only undertaken by choice when you are old enough to make an informed decision that this is something that you want to do and that is important to you. Dilation is mainly intended to help people who wish to receive penetrative intercourse where this may be uncomfortable, painful, or otherwise limited. For people who have had a vaginoplasty this is an important element of post-operative care and treatment, though it may be chosen even without surgery. It can be quite an involved process and can be as clinical or informal as is comfortable for you. Many MRKH woman reported dilating “naturally” with their partners without the use of dilators.
Surgical Interventions
Most people with MRKH will not require surgical interventions. Where there are genuine health risks to an individual this should be addressed in an appropriate time frame as determined by the treating medical team/specialists.
While surgery is not routinely necessary for most people with MRKH it is something that may be presented as an option or be sought out to facilitate penetrative sex. Surgical options should be carefully considered and not be seen as the first choice. The option of vaginal reconstruction surgeries should first be weighed up with the alternative option of vaginal dilation only. The latter is a less invasive option, and dilation can be done at a slower pace with support from medical practitioners or even with your partner with great results. It also opens up the conversation around sexual pleasure and alternative ways to enjoy sex apart from penetrative sex.
Any surgical decision making should be directed by the individual, with their full and informed consent, and where they are unable to provide informed consent, deferred until a point where they are able to do so. It is also important to consider when any proposed treatment should occur and what follow-up care is required. Many decisions can be delayed until an individual is old enough to participate fully in decision-making and make their own decisions about their body and healthcare. There are plenty of ways to explore intimacy and enjoy wonderful sex without a long vagina.
Unnecessary surgery causes additional stress and may need to be followed by more treatments and surgeries later in life. These surgeries are intrusive and are often harmful to physical sensation and sexual enjoyment later in life and take away decision-making and options from the individual, who may grow up to understand their identity and values in ways that are incongruent with the proposed treatment/s.
Fertility Treatment
This can include:
- Egg retrieval with IVF and surrogacy.
- Reciprocal IVF if you have a partner with a uterus.
- Uterus transplant and IVF with donor egg.
Other options may also be available to you depending on your circumstances and any fertility concerns should ideally be raised with a doctor early if you are considering having your own biological children. Where possible, your doctors can guide you through the steps and decisions you may need to make.
You may also wish to explore alternate family-building options. Parenting takes many shapes and forms such as adoption, step-parenting, foster care, parenting pets, being a godparent or playing an active role with nieces, nephews or cousins. Life is rich with opportunities to nurture, care for and provide guidance to others. Our families can be the ones we are born with but also the ones we choose for ourselves. This can include choosing to have a family without children.
When making any decisions about starting a family, take time to consider your view on your role you want to play in your family and acknowledge the impact of social pressures to conform to traditional ideals about parenthood.
Health Monitoring and Other Treatments
Examinations and Photography
You may need to undergo regular examinations to understand how your body is functioning. This includes genital examinations. Ask how these can be reduced in number, or if they need to go ahead at all.
If you are a parent or carer, you may be asked if photographs can be taken of your child’s genitals, this is not usually necessary, so it is important to ask why it is being suggested. This is sometimes done to minimise the need for future genital examinations, however, should be fully considered due to the distress they can cause the individual later in life. These photographs will not be distributed without your explicit consent as the parent/individual. If you’re uncomfortable with a decision your parents made, you can ask for the photographs to be destroyed.
You can choose not to have genital examinations, or photography altogether, and may wish to ask whether the doctor is able to get the same information another way. More guidance on paediatric genital examinations and photography is available at View Link
Ongoing Care
You may see many different kinds of doctors and health workers. Some people that may be involved in your care may include:
- A psychologist or psychiatrist, who can help you consider your own treatment options, your wishes and support you and your family.
- A social worker, peer navigator and peer support workers who can help you navigate health systems, connect you with community, and address any challenges.
- Your general practitioner or family doctor.
- An endocrinologist (to help with any hormonal management).
- A gynaecologist (for anything to do with your anatomy, menstruation or sex).
- A urologist (for anything to do with your anatomy, bladder or urination).
Surgeons involved in your care. - Fertility specialists (for any fertility support or assisted reproduction).
- Sexual health specialists.
- Pelvic health physiotherapists.
- Sexologists and sexological bodyworkers.
You can ask your regular doctor about creating a care plan, and for referrals to community/peer support organisations. Other people have been through these circumstances before, and they can help you navigate medical and support systems.
If you are maintaining healthy hormone levels, and your body is functioning and developing in ways you are happy with, there is likely no need for any medical interventions. As always you should ask for written copies of the latest reports on outcomes so that you can access all the information about your body and health if and when you need access to it. It’s important for everyone to have adequate information about their body, in order to best manage your physical and mental health. Finding health professionals who are knowledgeable about MRKH and understand the person’s emotional needs is vital for the general practitioner who might know that you are perfectly healthy in general, but might lack the awareness of the psychological impact.
When seeking medical care you may be confronted by medical professionals who lack awareness of MRKH and/or show a lack of interest in doing additional research to address their knowledge gap. This can make the patient feel discouraged or compelled to educate the professional who is supposed to be looking after them. Experiences like this can be traumatic, creating distrust when asking for help and barriers to accessing medical care. Finding referrals to knowledgable services from your peer network can help you avoid being repeatedly disappointed.
Working with a doctor is important to understand which of these health concerns are relevant to your body. Learning how to monitor and manage these conditions is an important aspect of living with MRKH.
Community and Other Supports
Supports Available for Individuals and Families in Australia
- MRKH Australia – https://www.mrkhaustralia.org/
- InterLink psychosocial support service – https://ilink.net.au
- InterAction for Health and Human Rights – https://interaction.org.au/
- IPSA Intersex Peer Support Australia (formerly AISSGA) – https://isupport.org.au/
- +61 7 3017 1724
- info@ilink.net.au
- PO Box 1372 Eagle Farm QLD 4009
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