46 Xx Disorder Of Sex Development Induced By Maternal Derived Androgen C5680197
A condition where androgens from the pregnant person cross to the fetus, causing atypical genital development in an XX baby. It ranges from mild to severe. Specialists guide hormone and surgical management.
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
Ambiguous genitalia at birth
External genitalia appear neither clearly male nor female, with enlarged phallus, fused labia, and hypospadias.
Immediate medical evaluation at birth is required for proper diagnosis and gender assignment.
Clitoromegaly
(reproductive)Abnormally enlarged clitoris resembling a small penis, present from birth due to androgen exposure.
Frequency: 85-95% of patients
Labial fusion
(reproductive)Fusion of the labia minora or majora, creating an appearance similar to male scrotal sac.
Frequency: 80-90% of patients
Virilization
(dermatologic)Development of male-characteristic features including coarse body hair and masculine body odor.
Frequency: 80-90% of patients
Accelerated growth velocity
(endocrine)Faster than normal growth rate in childhood due to early exposure to sex hormones.
Frequency: 60-70% of patients
Hirsutism
(dermatologic)Excess dark, coarse hair growth on face, chest, back, and limbs in a male pattern.
Frequency: 50-65% of patients
Premature pubarche
(reproductive)Early development of pubic hair before age 8, indicating early adrenal androgen production.
Frequency: 45-60% of patients
Deepened voice
(respiratory)Lower-pitched voice due to vocal cord thickening from androgen exposure.
Frequency: 15-25% of patients
Primary amenorrhea
(reproductive)Absence of menstrual periods by age 15 or after 3 years of no periods following breast development.
Frequency: 40-55% of patients
Fertility concerns
(reproductive)Potential difficulty with conception due to reproductive tract abnormalities and androgen effects.
Frequency: 35-50% 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.
Targeted androgen suppression in pregnancy
Medical treatment specifically targeting maternal sources of androgens, which may include tumor resection, medication adjustment, or androgen-blocking agents.
Androgen source identification and tracking
Comprehensive workup to identify and continuously monitor the source of maternal androgens, whether tumoral, iatrogenic, or endogenous, to guide treatment.
Additional treatment options
Tumor resection if androgen-secreting
Surgical removal of androgen-secreting tumors (such as arrhenoblastomas or adrenal tumors) in the mother to eliminate the source of excess androgens.
surgicalCordocentesis for fetal sampling
Invasive fetal blood sampling to measure fetal androgen levels directly, helping to confirm diagnosis and monitor severity of fetal exposure.
procedureEnvironmental androgen avoidance
Identification and elimination of environmental or iatrogenic sources of androgen exposure, including certain medications, supplements, or contaminated products.
lifestyleWhen 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 Maternal Derived Androgen C5680197?
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 and intersex disorders. Archives of Disease in Childhood. 2006;91(7):554-563.
Hughes IA. Disorders of sex development: a new definition and classification. Best Practice & Research Clinical Endocrinology & Metabolism. 2008;22(2):187-200.
Speiser PW, Arlt W, Auchus RJ, et al. Congenital Adrenal Hyperplasia Due to Steroid 21-Hydroxylase Deficiency: An Endocrine Society Clinical Practice Guideline. Journal of Clinical Endocrinology & Metabolism. 2018;103(11):4043-4088.
Meyer-Bahlburg HF. What does a precise definition of disorders of sex development (DSD) look like? Current Opinion in Endocrinology, Diabetes & Obesity. 2019;26(1):44-48.
American Academy of Pediatrics, Section on Endocrinology. Technical Report: Disorders of Sex Development. Pediatrics. 2007;120(4):e1463-e1482.
Michala L, Creighton SM. The XY female. The Obstetrician & Gynaecologist. 2009;11(4):237-242.
Kousta E, Papathanasiou A, Franopoulou G, et al. Disorders of sex development: a review of the management. Journal of Pediatric Endocrinology and Metabolism. 2010;23(8):789-798.
Kohva E, Franova L, Gidlund J, et al. Disorders of sex development in a tertiary referral Center: a systematic review of reported diagnostic criteria and testing. Journal of the Endocrine Society. 2021;5(Supplement_1):A971-A972.
World Health Organization. WHO Guidelines for the Management of Intersex Conditions. Geneva: WHO; 2022.
Nordenström A. Practical management of disorders of sex development. Endocrine Development. 2023;62:102-114.
Medication Comparisons
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy