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PubMed This is a summary of 16 peer-reviewed journal articles Updated
Pediatrics

Navigating Gender Assignment and Psychological Support

At a Glance

Navigating 46,XX ovotesticular DSD involves complex decisions about gender assignment at birth based on anatomy and fertility potential. Ongoing psychological support is essential for patients and families to process the diagnosis, navigate gender identity, and build a healthy body image.

Navigating gender identity and the psychological impact of a DSD diagnosis requires deep sensitivity and strong support. Whether you are a parent deciding on a sex of rearing for an infant, or an adolescent/adult processing what this diagnosis means for your own identity, this is a highly individualized journey [1][2].

Gender Assignment for Infants

For parents of newborns, deciding whether to raise your child as a boy or a girl is a complex process made in partnership with the multidisciplinary team. The medical team looks at several functional factors to help guide the recommendation:

  • Anatomical Findings: The team assesses the internal reproductive organs and the external appearance of the genitals [3][2].
  • Fertility Potential: Because 46,XX individuals cannot produce sperm but often have functional ovarian tissue and a uterus, a female sex of rearing is frequently recommended to preserve the possibility of future pregnancy [4][5].

While the team provides a recommendation, the decision is a shared one that respects your family’s values and your child’s future autonomy [6][7].

The Evolution of Gender Identity

Biological sex (chromosomes and anatomy) and gender identity (one’s internal sense of being male, female, or another gender) are related but distinct.

  • Identity Development: In most cases, children grow up to be comfortable in the gender they were assigned at birth [6]. However, in some individuals with 46,XX OT-DSD, a different gender identity may emerge, particularly during adolescence when the body undergoes hormonal changes [8][9].
  • Fluidity and Support: If a child expresses gender dysphoria (distress caused by a mismatch between gender identity and assigned sex), or if an older patient decides to transition, it is not a “failure” of the initial decision. It is a natural, recognized path of development that requires supportive, non-judgmental care from the multidisciplinary team [10][8].

The Role of Psychological Support

A DSD diagnosis can be overwhelming, often causing significant distress [11][12]. Psychological support is not “extra” care—it is an essential part of the treatment plan [13][14].

  • For Parents: Counseling helps you process the diagnosis, manage the pressure of medical decisions, and prepare for future conversations with your child [11][14].
  • For Adolescents and Adults: Working with a therapist who understands DSDs can help you develop a healthy body image, process the reality of your unique biology, and navigate complex questions about dating and identity [13][15].

Talking About the Condition

Openness is the foundation of trust and empowerment. Experts strongly recommend age-appropriate disclosure for children, meaning sharing information in small, understandable pieces as they grow [15].

  • Early Childhood: Use simple terms to explain that everyone’s body is a little bit different, and their body has some unique features [15].
  • Adolescence and Beyond: As patients get older, discussions become more detailed regarding hormones, fertility, and genetic switches, helping them become the lead advocate for their own health [16][15].

By fostering an environment of honesty and support, families and patients can ensure that an individual grows up feeling empowered by their identity, rather than defined by a medical label [6][15].

Common questions in this guide

How is a sex of rearing chosen for an infant with 46,XX OT-DSD?
The medical team evaluates internal reproductive organs, external genitals, and fertility potential. Because many individuals with 46,XX OT-DSD can preserve fertility, a female sex of rearing is often recommended, but the final decision is shared with the parents.
Will my child's gender identity match their assigned sex?
In most cases, children with 46,XX OT-DSD grow up comfortable with the gender they were assigned at birth. However, some may develop a different gender identity as they grow, which is a recognized and natural path of development that requires supportive care.
Why is psychological support important for DSD diagnoses?
A DSD diagnosis can cause significant emotional distress for families. Counseling helps parents navigate medical decisions and helps patients develop a healthy body image while processing their unique biology.
How should I talk to my child about their DSD diagnosis?
Experts recommend age-appropriate disclosure, starting with simple conversations in early childhood about how everyone's body is different. As the child grows, discussions can include more details about genetics and hormones to empower them in their health care.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.What specific biological factors (hormones, anatomy, genetics) are used to suggest a sex of rearing for infants?
  2. 2.How often will gender identity be formally assessed or discussed as the patient grows?
  3. 3.What are the most recent research findings regarding gender identity stability for 46,XX OT-DSD?
  4. 4.Can you help us find a therapist who has worked specifically with other families and patients navigating DSD diagnoses?

Questions For You

Tap a prompt to share your answer — we'll use it plus this page's context to start a tailored conversation.

References

References (16)
  1. 1

    Disorders of sex development.

    Witchel SF

    Best practice & research. Clinical obstetrics & gynaecology 2018; (48()):90-102 doi:10.1016/j.bpobgyn.2017.11.005.

    PMID: 29503125
  2. 2

    Ovotesticular Disorder of Sex Development in Korean Children: A Single-Center Analysis over a 30-Year Period.

    Kim HI, Lee I, Kim SH, et al.

    Journal of pediatric and adolescent gynecology 2021; (34(5)):626-630 doi:10.1016/j.jpag.2021.02.105.

    PMID: 33667640
  3. 3

    Baseline Characteristics of Infants With Atypical Genital Development: Phenotypes, Diagnoses, and Sex of Rearing.

    Finlayson C, Rosoklija I, Aston CE, et al.

    Journal of the Endocrine Society 2019; (3(1)):264-272 doi:10.1210/js.2018-00316.

    PMID: 30623164
  4. 4

    Laparoscopic gonad-sparing procedure in Ovotesticular disorder of sex development - Case video technique.

    Rombaldi MC, da Silva Azenha MV, de Paula LCP, et al.

    Journal of pediatric urology 2021; (17(4)):583-584 doi:10.1016/j.jpurol.2021.06.034.

    PMID: 34284957
  5. 5

    An Unusual and Late Presentation of 46, XY Ovotesticular Difference of Sex Development.

    Elsa Mathew U, Arun CS, George V, et al.

    JCEM case reports 2025; (3(8)):luaf115 doi:10.1210/jcemcr/luaf115.

    PMID: 40520042
  6. 6

    Ovotesticular Difference of Sex Development: Genetic Background, Histological Features, and Clinical Management.

    Syryn H, Van De Vijver K, Cools M

    Hormone research in paediatrics 2023; (96(2)):180-189 doi:10.1159/000519323.

    PMID: 34469891
  7. 7

    46,XY ovotesticular disorders of sex development: A therapeutic challenge.

    Scarpa MG, Grazia MD, Tornese G

    Pediatric reports 2017; (9(4)):7085 doi:10.4081/pr.2017.7085.

    PMID: 29285340
  8. 8

    Gender Dysphoria in a Patient With Ovotesticular Disorder of Sex Development.

    Moreno T, Ribeiro S, Rodrigues P

    JCEM case reports 2024; (2(1)):luad159 doi:10.1210/jcemcr/luad159.

    PMID: 38143928
  9. 9

    The Spectrum of Ovotesticular Disorders of Sex Development in South Africa: A Single-Centre Experience.

    Ganie Y, Aldous C, Balakrishna Y, Wiersma R

    Hormone research in paediatrics 2017; (87(5)):307-314 doi:10.1159/000466693.

    PMID: 28376506
  10. 10

    Phenotypic spectrum and long-term outcomes of patients with 46,XX disorders of sex development.

    Yoon H, Kim D, Kim JH, et al.

    Annals of pediatric endocrinology & metabolism 2025; (30(2)):77-85 doi:10.6065/apem.2448122.061.

    PMID: 40335043
  11. 11

    Prenatal detection and evaluation of differences of sex development.

    Finney EL, Finlayson C, Rosoklija I, et al.

    Journal of pediatric urology 2020; (16(1)):89-96 doi:10.1016/j.jpurol.2019.11.005.

    PMID: 31864813
  12. 12

    Predictors of Psychosocial Distress in Parents of Young Children with Disorders of Sex Development.

    Perez MN, Delozier AM, Aston CE, et al.

    The Journal of urology 2019; (202(5)):1046-1051 doi:10.1097/JU.0000000000000424.

    PMID: 31268850
  13. 13

    Psychological support for individuals with differences of sex development (DSD).

    Bennecke E, Strandqvist A, De Vries A, et al.

    Journal of psychosomatic research 2024; (179()):111636 doi:10.1016/j.jpsychores.2024.111636.

    PMID: 38507969
  14. 14

    Differences of Sex Development: Current Issues and Controversies.

    Johnson EK, Whitehead J, Cheng EY

    The Urologic clinics of North America 2023; (50(3)):433-446 doi:10.1016/j.ucl.2023.04.010.

    PMID: 37385705
  15. 15

    Exploring caregiver experiences of communicating with children about differences of sex development (DSD).

    Kimball A, Rutter MM, Kavanaugh G, Fishler K

    Patient education and counseling 2025; (130()):108470 doi:10.1016/j.pec.2024.108470.

    PMID: 39405587
  16. 16

    'It was Supposed to be a Secret': a study of disclosure and stigma as experienced by adults with differences of sex development.

    Mediå LM, Fauske L, Sigurdardottir S, et al.

    Health psychology and behavioral medicine 2022; (10(1)):579-595 doi:10.1080/21642850.2022.2102018.

    PMID: 35898596

This page provides information on navigating gender assignment and psychological support for educational purposes. Always consult a multidisciplinary medical team for personalized DSD care and psychological counseling.

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