Ovotesticular Disorder (True Hermaphroditism)

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In recent years, some groups have strongly advocated that individuals should be identified strictly according to their birth sex as male or female, and that gendered language should reflect this biological categorization—for instance, referring to males as boys or men and females as girls or women. While this viewpoint seeks clarity, it does not fully capture the complexities faced by individuals with Differences in Sex Development (DSD) such as "True Hermaphroditism," whose experiences challenge binary understandings of sex and gender.

Health care providers regularly encounter a diverse spectrum of individuals with various DSD conditions. These biological variations can present significant emotional and practical challenges, not just for the affected children but initially for their parents, who often bear the responsibility of navigating unfamiliar medical and social landscapes. As children mature, they also face important decisions about their bodies, identities, and care, often involving ongoing psychological and social support tailored to their unique needs.

Decisions about medical interventions—such as surgeries or hormone treatments—must be carefully managed by medical professionals through a thoughtful consultative process involving both the children and their parents. Such decisions carry profound physical and psychological implications, and medical teams are tasked with balancing the best clinical practices alongside respect for autonomy and consent as children grow.

This discussion centers on the psychological care component, which is equally critical. Psychological support should be individualized and sensitive to the child’s particular presentation of DSD, their developing sense of identity, and the interactions they have within their family and wider social environment. The psychological care team plays a vital role in assisting children and families to cope with the emotional complexities, social stigma, and potential identity questions that may arise.

Considering the broader social milieu is essential. Children with DSD and their families often operate within cultural, familial, and societal frameworks that may have varying levels of acceptance or understanding regarding sex and gender diversity. These social factors can influence the well-being of the child and family, as well as the experience of care. Health care providers must be attuned to these dynamics, fostering supportive communication and advocating for a respectful, inclusive environment.

Ethical decision-making is an integral part of managing DSD cases. Issues such as timing and necessity of medical interventions, informed consent, confidentiality, and balancing parental authority with the growing autonomy of the child require ongoing reflection and interdisciplinary collaboration. Ethical considerations also extend to language use and the importance of affirming each individual’s identity in a way that respects their lived experience.

A Summary of Ovotesticular Disorder (True Hermaphroditism)


Ovotesticular disorder of sex development (OT-DSD), historically referred to as true hermaphroditism, is a rare congenital condition characterized by the presence of both ovarian and testicular tissue in the same individual. This tissue may be found in separate gonads or combined within a single ovotestis. The condition affects fewer than 1 in 20,000 live births and accounts for approximately 3–10% of all DSD cases (Nistal et al., 2015; Zhao et al., 2023). Most individuals with OT-DSD have a 46,XX karyotype, although mosaic patterns such as 46,XX/46,XY and other chromosomal variations have been documented (Lee et al., 2006). The etiology is heterogeneous, involving factors such as translocation of the SRY gene, mutations in SOX9, RSPO1, and NR5A1, and chimerism resulting from the fusion of two zygotes (Barseghyan & Vilain, 2014).

Clinical presentation varies widely, ranging from ambiguous genitalia to phenotypically typical male or female characteristics. Gonads may be palpable or non-palpable, and internal reproductive structures such as a uterus or dysplastic cervix may be present. Fertility is rare but possible, with documented cases of ovulation and even pregnancy in individuals with functioning ovarian tissue (Kim et al., 2002; Matsui et al., 2011). Diagnosis typically involves karyotyping, hormonal assays, imaging studies, and histopathological examination of gonadal tissue (Zhao et al., 2023).

Coping with OT-DSD involves a multidisciplinary approach that integrates medical, psychological, and social support. Gender assignment is individualized and increasingly deferred until the child can participate in decision-making. Hormonal therapy and surgical interventions are tailored to the individual's phenotype and gender identity. Psychological counseling is essential to support identity development and address potential stigma or emotional distress (Lee et al., 2006; Sircili et al., 2014). Long-term studies suggest that individuals with OT-DSD can lead fulfilling lives when provided with appropriate care and support systems (Matsui et al., 2011). Advocacy groups and evolving clinical guidelines emphasize transparency, autonomy, and the minimization of non-consensual interventions, reflecting a shift toward more ethical and patient-centered care.



Note


This page is for education and not personal advice. Consult health care providers for the most recent information and personal concerns.



References

Barseghyan, H., & Vilain, E. (2014). The genetics of ovotesticular disorders of sex development. In Genetic Steroid Disorders (pp. 261–263). https://doi.org/10.1016/B978-0-12-416006-4.00020-X

Kim, K. R., Kwon, Y., Joung, J. Y., Kim, K. S., Ayala, A. G., & Ro, J. Y. (2002). True hermaphroditism and mixed gonadal dysgenesis in young children: A clinicopathologic study of 10 cases. Modern Pathology, 15(10), 1013–1019. https://doi.org/10.1097/01.MP.0000027623.23885.0D

Lee, P. A., Houk, C. P., Ahmed, S. F., & Hughes, I. A. (2006). Consensus statement on management of intersex disorders. Pediatrics, 118(2), e488–e500. https://doi.org/10.1542/peds.2006-0738

Matsui, F., Shimada, K., Matsumoto, F., Itesako, T., Nara, K., & Ida, S. (2011). Long-term outcome of ovotesticular disorder of sex development: A single center experience. International Journal of Urology, 18(3), 231–236. https://doi.org/10.1111/j.1442-2042.2010.02700.x

Nistal, M., Paniagua, R., González-Peramato, P., & Reyes-Múgica, M. (2015). Perspectives in pediatric pathology, chapter 7: Ovotesticular DSD (true hermaphroditism). Pediatric and Developmental Pathology, 18(5), 345–352. https://doi.org/10.2350/14-04-1466-PB.1

Sircili, M. H., Denes, F. T., Costa, E. M. F., et al. (2014). Long-term follow-up of a large cohort of patients with ovotesticular disorder of sex development. Journal of Urology, 191(5 Suppl), 1532–1536. https://doi.org/10.1016/j.juro.2013.10.037

Zhao, J., Zhu, J., Ding, S., & Li, H. (2023). Ovotesticular disorder of sex development presenting as a scrotal emergency. Pediatrics, 152(6), e2023061810. https://doi.org/10.1542/peds.2023-061810


Post Author

Geoffrey W. Sutton, Professor Emeritus of Psychology at Evangel University, holds a master’s degree in counseling and a PhD in psychology from the University of Missouri-Columbia. His postdoctoral work encompassed education and supervision in forensic and neuropsychology and psychopharmacology. As a licensed psychologist, he conducted clinical and neuropsychological evaluations and provided psychotherapy for patients in various settings, including schools, hospitals, and private offices. During his tenure as a professor, Dr. Sutton taught courses on psychotherapy, assessment, and research. He has authored over one hundred publications, including books, book chapters, and articles in peer-reviewed psychology journals. His website is https://suttong.com You can find Dr. Sutton's books on   AMAZON    and  GOOGLE. Many publications are free to download at ResearchGate   and Academia  

 







I write and give talks about Psychology and Culture--especially religion and spirituality and the places I have visited. I also write "how-to" books on research and statistics.

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