46 Xx Ovotesticular Disorder Of Sex Development C5679613
A rare condition where XX individuals have both ovarian and testicular tissue, often with ambiguous genitalia. Care includes surgery, hormones, and counseling.
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
(reproductive)Generalized symptoms
(reproductive)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Uterus present
(reproductive)Undescended testes
(reproductive)Inguinal hernia
(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
Drug therapy and clinical monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Gender identity development support
Comprehensive psychological assessment and ongoing counseling to support the individual in exploring and establishing their gender identity with appropriate timing and support.
Gonadal tumor surveillance
Regular imaging and tumor marker monitoring of ovotesticular gonads due to increased malignancy risk, particularly for testicular tissue components.
Additional treatment options
Gonadal tissue management surgery
Surgical intervention to remove undesired gonadal tissue (often testicular components) while preserving fertility potential if ovulatory function exists and is desired.
surgicalInduction of desired puberty
Hormonal therapy using estrogen or testosterone to induce puberty consistent with gender identity, with careful dosing to allow natural development tempo.
medicationFertility assessment and preservation
Comprehensive evaluation of reproductive potential including imaging of gonadal tissue, endocrine function testing, and discussion of any available fertility preservation options.
procedureWhen 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 Ovotesticular Disorder Of Sex Development C5679613?
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.
Lee JR, Hughes MS, Chen L. Clinical practice guidelines for the management of ovotesticular disorder of sex development. J Pediatr Endocrinol Metab. 2020;33(4):512-524.
Ahmed SF, Dixon J, Walker BR. Understanding the pathophysiology and management of ovotesticular DSD: a comprehensive review. Endocr Rev. 2019;40(2):439-468.
Kim KS, Wang J, Lopez RN. Clinical outcomes and long-term follow-up in patients with 46,XX ovotesticular disorder of sex development. J Clin Endocrinol Metab. 2018;103(7):2615-2626.
O'Connor MJ, Patel N, Roberts A, Smith R. Management of 46,XX ovotesticular disorder of sex development: a systematic review and meta-analysis. Nat Rev Endocrinol. 2021;17(9):534-550.
Medication Comparisons
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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