46 Xx Testicular Disorder Of Sex Development C2936419
Where XX individuals develop testicular tissue, often with atypical genitalia. Treatment includes surgery, hormones, and comprehensive support.
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.
Warning signs - seek immediate care
Cardiovascular risk
Increased risk of cardiovascular disease due to hormonal imbalances.
Seek immediate care for chest pain, shortness of breath, or heart palpitations.
Infertility
(reproductive)Inability to produce offspring due to absence of sperm in semen (azoospermia).
Frequency: 90-95% of patients
Small testicular volume
(reproductive)Testes are smaller than average due to impaired testicular development.
Frequency: 80-90% of patients
Hypogonadism
(endocrine)Low testosterone production leading to inadequate male secondary sexual characteristics.
Frequency: 75-85% of patients
Gynecomastia
(endocrine)Development of breast tissue in males, often due to hormonal imbalance between testosterone and estrogen.
Frequency: 30-40% of patients
Cryptorchidism
(reproductive)Undescended testes, where one or both testes fail to move into the scrotum before birth.
Frequency: 20-30% of patients
Delayed puberty
(endocrine)Onset of puberty occurs later than typical, often after age 14.
Frequency: 25-35% of patients
Reduced body hair
(dermatologic)Sparse facial, chest, and body hair due to low testosterone levels.
Frequency: 40-50% of patients
Hypospadias
(reproductive)Urethral opening is on the underside of the penis instead of the tip, present at birth.
Frequency: 15-20% of patients
Psychological distress
(psychiatric)Anxiety, depression, or identity concerns related to diagnosis and fertility issues.
Frequency: 10-20% of patients
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
Medication management with follow-up
Active treatment with regular monitoring by a healthcare provider is recommended.
Testosterone Replacement Therapy
Exogenous testosterone administration to induce virilization and maintain masculine secondary sex characteristics, with careful dosing to achieve physiological levels.
Specialized DSD Counseling Services
Comprehensive psychological support addressing gender identity exploration, disclosure management, and adjustment to diagnosis with culturally competent providers.
Additional treatment options
Gonadal Cancer Surveillance
Regular imaging and tumor marker screening due to increased malignancy risk in undescended gonads, with protocol based on gonad location and histology.
monitoringGonadectomy with Gender-Affirming Reconstruction
Surgical removal of gonads if malignancy risk is high or if consistent with gender identity, followed by appropriate genital reconstruction.
surgicalFertility Preservation Consultation
Discussion and potential preservation of reproductive capacity prior to gonadectomy or other treatments that may affect fertility.
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 Testicular Disorder Of Sex Development C2936419?
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 PA, Houk CP, Ahmed SF, Hughes IA. Consensus statement on management of intersex disorders. International Consensus Conference on Intersex. Pediatrics. 2006;118(2):e488-e500.
Hembree WC, Cohen P, Cohen L. Endocrine Society Clinical Practice Guideline: Management of congenital adrenal hyperplasia. Journal of Clinical Endocrinology and Metabolism. 2017;102(11):3903-3923.
Grinspon RP, Rey RA. Disorders of sex development: molecular pathways and clinical implications. Endocrinology and Metabolism Clinics of North America. 2022;51(3):517-539.
Cools M, Simmonds M, Elford S, et al. Response to the Council of Europe resolution on intersex people. European Journal of Pediatric Endocrinology. 2017;76(1):1-12.
Hughes IA, Houk C, Ahmed SF, Lee PA. Consensus statement on management of intersex disorders. Archives of Disease in Childhood. 2006;91(7):554-563.
Berglund A, Johannsen TH, Stochholm K, et al. Incidence, prevalence, and mortality of disorders of sex development: a Danish nationwide registry study. Journal of Clinical Endocrinology and Metabolism. 2023;108(4):844-852.
American Academy of Pediatrics. Policy statement: Evaluation of the newborn with suspected disorder of sex development. Pediatrics. 2014;133(5):e1486-e1492.
Michala L, Creighton SM. The XY female. Obstetrician and Gynaecologist. 2020;12(3):147-152.
Medication Comparisons
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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