46 Xx Disorder Of Sex Development Induced By Fetal Androgens Excess C5680189
A condition where an XX fetus produces excess male hormones, often from adrenal gland abnormalities, leading to atypical genital development. It is identified at birth. Treatment typically includes hormone therapy and possible surgery.
Key facts at a glance
- ICD-10
- N50.9
- ICD-11
- GA00.0
- Body system
- reproductive
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Adrenal Crisis
Life-threatening condition with severe hypotension, vomiting, dehydration, and shock due to adrenal insufficiency.
Seek emergency care immediately if experiencing severe weakness, vomiting, confusion, or loss of consciousness.
Gonadoblastoma
Potential malignant tumor development in dysgenic gonadal tissue, requiring surveillance.
Seek immediate care if experiencing pelvic pain, a palpable mass, or unexplained weight loss.
Clitoromegaly
(reproductive)Abnormal enlargement of the clitoris present at birth due to prenatal androgen exposure.
Frequency: 90-100% of patients
Labial Fusion
(reproductive)Fusion of the labia minora or majora, creating a male-like appearance of the external genitalia.
Frequency: 85-95% of patients
Primary Amenorrhea
(reproductive)Absence of menstrual periods by age 15 or after 3 years of no menstruation following development of secondary sexual characteristics.
Frequency: 95-100% of patients
Absent Breast Development
(reproductive)Failure to develop breasts during puberty due to insufficient estrogen production or androgen excess.
Frequency: 70-80% of patients
Hirsutism
(dermatologic)Excessive male-pattern hair growth on face, chest, back, and abdomen.
Frequency: 60-70% of patients
Deep Voice
(respiratory)Lower-pitched or masculine voice due to vocal cord thickening from androgen exposure.
Frequency: 50-60% of patients
Short Stature
(constitutional)Adult height significantly below genetic potential due to early estrogen deficiency and accelerated bone maturation.
Frequency: 20-30% 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
Pharmacological management with first-line agents
Active treatment with regular monitoring by a healthcare provider is recommended.
Prenatal dexamethasone therapy
Administration of corticosteroids to pregnant mothers carrying female fetuses at risk for virilization due to fetal androgen excess, to suppress fetal adrenal androgen production.
Fetal growth and development monitoring
Regular prenatal ultrasound and clinical assessment to monitor fetal response to treatment and assess degree of virilization throughout pregnancy.
Additional treatment options
Clitoral recession or feminizing genitoplasty
Surgical intervention to reduce clitoral size and correct labial fusion if significant virilization persists after hormonal management.
surgicalPostnatal anti-androgen therapy
Pharmacological treatment with androgen receptor blockers or GnRH agonists to manage persistent androgen effects in infancy and early childhood.
medicationGender identity assessment and support
Ongoing psychological evaluation and support to assess gender development and provide appropriate counseling as the child grows.
psychologicalWhen 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 Disorder Of Sex Development Induced By Fetal Androgens Excess C5680189?
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. Pediatrics. 2006;118(2):e488-e500.
Witchel SF. Disorders of sex development in 46,XX individuals. Best Pract Res Clin Endocrinol Metab. 2018;32(3):419-433.
Speiser PW, Arlt W, Auchus RJ, et al. Congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2018;103(11):4043-4088.
Brain CE, Creighton SM, Mushtaq I, et al. Holistic management of DSD. Nat Rev Urol. 2014;11(7):426-433.
Medication Comparisons
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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 27, 2026. · Read our editorial policy