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Ovotesticular Variations of Sex Characteristics

(Diagnostic terms change over time, and this trait is also known as: Ovotestis, Ovotesticular Disorder of Sex Development, True Gonadal Intersex, True Hermaphroditism, an Intersex Variation)

What are Ovotesticular Variations of Sex Characteristics?

  • Ovotesticular variations involve the presence of mixed gonads, other than a pair of testes or pair of ovaries.
  • This might include the presence of an internal testis and an ovary, or the presence of mixed gonads called “ovotestes”.
  • Ovotestes have a mixture of ovarian and testicular tissue.
  • This is estimated to occur in around 1 per 20,000 births.
  • Ovotesticular variations often present with other variations in sex characteristics.
  • Most people with ovotesticular variations have 46,XX chromosomes.
  • In rare cases they may be 46,XX/46,XY (chimerism) or 46,XX/47,XXY (mosaic).

Where do Ovotesticular Variations Come From?

  • Ovotesticular variations are poorly understood due to their relative rarity, even among other innate variations of sex characteristics.
  • There appears to be a number of different genetic causes and mechanisms that produce these variations including:
    the translocation of the SRY gene onto the X chromosome, or
    duplication, deletion or mutation of other genes.
  • These mechanisms are likely to be the result of external factors rather than heritable traits.
  • Ovotesticular variations appear to be more prevalent in some communities, specifically in some populations with a heritage in Kenya and South Africa.
  • New research continues to explore genetic avenues and mechanisms behind ovotesticular variations.

Recognising Ovotesticular Variations

As ovotesticular variations largely relate to internal gonads and chromosomes it may be difficult to recognise these variations. Ovotesticular variations may be identified when diagnosed with another innate variation of sex characteristics.
  • A common indicator of ovotesticular variations are genital differences.
  • Most people with ovotesticular variations are assigned female at birth, though genital anatomy can range anywhere from typically female to typically male.
  • In most cases the genital anatomy of people with ovotesticular variations are termed “atypical”.
  • “Atypical genitalia” refers to genital anatomy that cannot be easily classified as male or female.
  • This kind of anatomy may also be understood as a large clitoris or small penis (perhaps with hypospadias).
  • ovotesticular variations are diagnosed using a combination of tests including chromosome and genetic analysis, ultrasound or MRI, hormone testing and gonadal biopsy.

Health Considerations

Most people with ovotesticular variations 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 issues.
  • Fertility considerations.
As with all innate variations of sex characteristics, these differences can be understood and managed allowing people to live fulfilling lives. There are a range of treatments available to address and manage these conditions should they arise. We need to be able to understand our bodies to be able to look after ourselves, and there is nothing inherently shameful about how your body exists.

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. 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.

Treatment and Gender

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 ovotesticular variations are centred around the core idea that everyone identifies or understands themselves as a girl/woman or boy/man 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.

Many people with ovotesticular variations do not identify as female despite their sex of rearing it is therefore important to take time to consider your options, feelings and identity before proceeding with any non-urgent medical options. Access to psychosocial support, peers and community can help you to understand these options.

Hormone Replacement Therapy

For Children and Adolescent

  • Hormone production for people with ovotesticular variations is varied, however the presence of ovarian or testicular tissue is generally associated with hormone production during puberty.
  • If no interventions are undertaken, increased hormone production during puberty will drive both breast development and/or genital growth depending on what gonadal tissue is present, and in what quantity.
  • Other secondary sex characteristics also influenced by this such as:
    shorter stature (due to premature closure of the skeletal growth plates)
    deepened vocal pitch
    development of facial and body hair
As many of these changes are permanent, they can be undesirable when they do not align with a person’s gender identity. This can be managed in a number of ways such as through the use of puberty suppressing medications (sometimes called puberty blockers).
These medications are designed for temporary use and allow individuals more time to make decisions about the kinds of pubertal changes they want for themselves. Suppressing hormones in the body before masculinisation or feminisation occurs can take the pressure off needing to make big healthcare decisions right away. Eventually a decision will need to be made whether to opt for no treatment and experience all potential changes that will result from puberty, or to opt for medical intervention to direct a masculinising or feminising puberty.

For Adults

If your body does not produce enough sex hormones, or if you have had a gonadectomy (a surgery that removes the testes) you may need to start, or continue, hormone replacement therapy. This is especially important if you experience some of the impacts of low sex hormones.

All bodies require a balance of estrogen and testosterone, amongst other sex hormones. Impacts of low sex hormones can include:

  • Loss of bone density, which can lead to osteoporosis. Osteoporosis causes the bones to become brittle and more vulnerable to fractures and broken bones.
  • Impaired cognition.
  • Low mood.
  • Low sex drive.
  • Fertility challenges.

Some people may like to take supplementary hormones to help with some of these issues.
The benefits of hormonal therapy can include:

  • Maintaining bone health.
  • Initiating, progressing or altering puberty.
  • Alleviating low mood or personal distress.
  • Increasing sex drive (if this is beneficial for you).
People can respond in different ways to different hormone treatments. Therefore, you and your healthcare provider should understand and manage the side effects and consequences of undergoing hormone replacement.
Typically, most women are only prescribed estrogen and progesterone, while men are prescribed testosterone, though some people will prefer to use different hormones in accordance with their identity, tolerances and preferences, and may benefit from changing their hormone regime.
Importantly not all people with ovotesticular variations want or need hormone replacement therapy. Counselling and peer support can help determine the best decision for you.

Surgical Interventions

Most people with ovotesticular variations 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 of specialists.
While surgery is not routinely necessary for most people with ovotesticular variations, it is something that may be presented or become relevant for an individual. Traits associated with ovotesticular variations are usually picked up from birth, so people with this variation may experience a higher incidence of early medical intervention. The decision to pursue non-urgent surgical interventions should be thoroughly considered, and alternatives explored. This 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. 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.
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. They may be illegal in some jurisdictions in Australia.

Genital Surgeries

Gonadectomy may be recommended for people with ovotesticular variations based on the assumption that atypical gonads pose a substantial risk of cancer. It is important to note that in most cases ovotestes and ovotesticular variations only pose a low to negligible risk for cancers. The presence of a Y chromosome however does increase this risk. Another assumption that underpins such recommendations is that puberty can be influenced by the “wrong” hormones. In general, it is preferable to not remove gonads that have potential for future function, even if this may be incongruent with sex of rearing.
A gonadectomy is not only a sterilising procedure, but also creates the need for lifelong hormone replacement therapy, and comes with the associated risks of osteopenia and osteoporosis. These conditions can lead to increased fractures and broken bones.
It is therefore important to be guided by accurate, current and unbiased medical and scientific information in these conversations, and that input from the individual be centred in these conversations.
Risk factors for gonadal cancers are individual and should be discussed and understood on a case-by case basis, and potential cancer risks can be managed through routine monitoring and other non-surgical methods.
Decisions around hormone production and puberty should always involve and be made by the individual, and can be deferred for longer if needed through the use of puberty suppressing medication.

Fertility Treatment

Fertility can be challenging or distressing for many people with ovotesticular variations. Your fertility is highly dependent on your variation, anatomy and medical history.
Unfortunately, very little is known about the fertility of people with ovotesticular variations.
  • For people with predominantly testicular tissue, fertility is most impacted as there are no known cases of sperm formation.
  • People with adequate ovarian tissue may have eggs and menstruate.
  • Ovarian tissue may however be susceptible to “premature ovarian” failure, and subsequent loss of reproductive function.
If having children is important to you, you may wish to consider options that utilise Assisted Reproductive Technologies. A fertility specialist will be able to discuss the options available to you depending on your body and circumstances.
Ideally fertility concerns should ideally be raised with a doctor early if you are considering having children. Where possible, your doctors can guide you through the steps and decisions you may need to make. You may need to consider and make decisions about egg preservation ( which is usually done prior to 21 years of age). This consideration is also shared by people with 45, X0 Turner Syndrome and some people with Gonadal Dysgenesis.
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
As an adult, your permission must be sought to be examined by doctors, or medical students for education purposes. You should not feel compelled to agree.

Ongoing Care

It may be helpful to prepare yourself before medical appointments by making a list of questions, topics or information you want to cover, taking notes, or to consider bringing along a support person with you to your appointment. These actions can help you to feel supported and in control of these interactions, and the information you have to make decisions with.

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 that 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.
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 ovotesticular variations.

Community and Other Supports

There are other people with ovotesticular variations, including in Australia’s intersex community.
Although ovotesticular variations may be different to other innate variations, the many struggles faced from the medical community and society more generally, are universally experienced by the intersex community. There are great benefits in connecting with people with ovotesticular variations, and also with people who have other innate variations. Seeing, being and interacting with people with similarly diverse bodies can help you make informed decisions free from external pressure through families, doctors or societal norms.

Supports Available for Individuals and Families in Australia

  • 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/

CONTACT

  • +61 7 3017 1724
  • info@ilink.net.au
  • PO Box 1372 Eagle Farm QLD 4009
InterLink is a program of InterAction for Health and Human Rights. We respect your rights when using  our services.
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