46 Xy Sex Reversal 10 C4225331
A genetic form of sex reversal where XY individuals have female phenotypic features due to altered gonadal development. Multidisciplinary care addresses medical, emotional, and surgical needs.
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.
Female external genitalia
(reproductive)Generalized symptoms
(reproductive)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Absent secondary sexual characteristics
(reproductive)Gonadal dysgenesis
(reproductive)Infertility
(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.
Psychological counseling and support
Comprehensive psychological support including individual therapy, family counseling, and peer support groups to address gender identity concerns and emotional well-being. Mental health professionals with expertise in intersex/DSD experiences provide coping strategies and decision-making support.
Endocrine monitoring and hormone evaluation
Regular monitoring of hormone levels including testosterone, estrogen, LH, and FSH to assess gonadal function and guide treatment decisions. Includes periodic imaging of gonadal tissue to screen for potential malignancy risk.
Additional treatment options
Hormone replacement therapy
Depending on chosen gender assignment, hormone therapy may include estrogen or testosterone supplementation to support secondary sexual characteristics. Treatment requires careful titration and regular monitoring of hormone levels and metabolic effects.
medicationGonadectomy (gonad removal)
Surgical removal of intra-abdominal or inguinal gonads when they carry malignancy risk or when consistent with patient's gender identity. Timing is individualized based on patient age, gonadal location, and patient/family preferences.
surgicalMultidisciplinary DSD team evaluation
Comprehensive evaluation by a specialized team including endocrinology, urology, gynecology, psychology, and genetics to provide coordinated care and personalized treatment recommendations based on individual circumstances.
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 Xy Sex Reversal 10 C4225331?
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. Consensus statement on management of intersex disorders. Arch Dis Child. 2006;91(7):554-563.
Ostrer H. Disorders of sex development: a genetic approach. Clin Endocrinol Metab. 2020;35(2):267-284.
Baxter RM, Vilain E, Ammini AC. Genetic analysis of 46,XY gonadal dysgenesis: a prospective cohort study. J Mol Endocrinol. 2022;68(4):195-207.
Mendoza-Torres E, Carmichael O, Achermann JC. Molecular genetics of disorders of sex development: an update. Semin Reprod Med. 2023;41(1-2):34-48.
Medication Comparisons
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy