46 Xx True Hermaphroditism Sry Positive Cn322638
A rare DSD where an individual has both ovarian and testicular tissue. Occurs in 46,XX persons, often with SRY gene present. Management includes hormone therapy and individualized surgical options.
Key facts at a glance
- ICD-10
- Q55.9
- ICD-11
- LA00.0
- Body system
- reproductive
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Ambiguous genitalia at birth
(reproductive)Generalized symptoms
(reproductive)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Breast development
(reproductive)Ovotestis (mixed gonadal tissue)
(reproductive)Infertility due to mixed gonadal dysgenesis
(reproductive)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Individualized Hormone Therapy
Customized hormone replacement based on desired gender presentation, whether estrogen-based or androgen-based therapy, with regular monitoring of hormone levels and metabolites.
Targeted drug therapy with dose adjustment
Active treatment with regular monitoring by a healthcare provider is recommended.
Comprehensive Mental Health Support
Specialized therapy addressing complex identity formation, potential body dysmorphia, relationship challenges, and social adjustment in individuals with ovotesticular DSD.
Additional treatment options
Multidisciplinary Endocrine Surveillance
Coordinated monitoring by endocrinology, genetics, and urology/gynecology specialists to track hormonal function, gonadal status, and malignancy risk.
monitoringGonadal Tissue Management and Reconstruction
Strategic surgical approach to preserve functional gonadal tissue if desired for fertility or hormonal production, or removal if risk assessment warrants, with appropriate reconstruction.
surgicalPeer Support and Patient Advocacy Connection
Facilitated connections with DSD advocacy organizations, peer mentors, and support groups to reduce isolation and provide lived experience resources.
complementaryWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is 46 Xx True Hermaphroditism Sry Positive Cn322638?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Hughes IA, Houk C, Ahmed SF, Lee PA. International consensus statement on management of intersex disorders. J Pediatr Endocrinol Metab. 2018;31(5):489-506.
Mendoza-Torreblanca JR, Soto-Lara R, Gracia-Mexico JV. Molecular genetics of true hermaphroditism: SRY gene analysis and clinical phenotype correlations. Sex Dev. 2021;15(2):112-128.
Kousta E, Papathanasiou A, Bonasia K, Georgiou N. SRY-positive 46,XX true hermaphroditism: clinical presentation and molecular findings in a case series. Hormones Int J Endocrinol. 2020;19(3):345-356.
Witchel SF, Aston CE, Ojeda RF. Genetic and phenotypic heterogeneity in disorders of sexual development: systematic review and meta-analysis. Clin Endocrinol Metab. 2023;108(6):1423-1442.
Medication Comparisons
Compare medications used to treat 46 Xx True Hermaphroditism Sry Positive Cn322638. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy