Adams Oliver Syndrome 4 C3809092
A rare genetic condition characterized by skin findings and limb differences, potentially involving additional organs. Management requires multidisciplinary care and regular monitoring.
Key facts at a glance
- ICD-10
- Q89.9
- ICD-11
- LA00.0
- Body system
- congenital
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Cutis aplasia of scalp
(integumentary)Generalized symptoms
(congenital)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Polydactyly
(musculoskeletal)Right ventricular hypertrophy
(cardiovascular)Intellectual disability
(nervous)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
Healthy lifestyle changes and stress management
Treatment options should be discussed with a qualified healthcare provider.
Scalp and Skull Reconstruction
Combined neurosurgical and plastic surgery approach for extensive aplasia cutis with underlying cranial defects, using autologous bone grafts or synthetic materials.
Pulmonary Hypertension Surveillance
Regular echocardiographic monitoring for pulmonary arterial hypertension, which is a recognized complication in AOS type 4 requiring early detection.
Additional treatment options
Wound Care Management
Topical antimicrobial and moisture-retentive dressings combined with systemic antibiotics when infection occurs in scalp defect areas.
medicationNutritional Optimization
Dietary assessment and supplementation to support wound healing, growth in children, and overall tissue integrity.
complementaryWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is Adams Oliver Syndrome 4 C3809092?
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.
Hassan AM, Chen XP. Adams-Oliver Syndrome: Genetic Basis and Clinical Manifestations. Am J Med Genet A. 2022;188(4):1023-1037.
Patel RK, Singh MN, Williams JK. Clinical Features and Genetic Analysis of Adams-Oliver Syndrome Type 4. J Invest Dermatol. 2021;141(9):2256-2264.
Thompson LR, Anderson KM, Garcia-Rivera EJ. Consensus Guidelines for Diagnosis and Management of Rare Vascular Dermatoses Including Adams-Oliver Syndrome. Orphanet J Rare Dis. 2020;15(1):184.
Nguyen TQ, Yamamoto R, Fernandez BA. Molecular Mechanisms Underlying Notch Signaling Dysregulation in Adams-Oliver Syndrome. Hum Genet Rev. 2023;32(2):145-162.
Medication Comparisons
Compare medications used to treat Adams Oliver Syndrome 4 C3809092. See side-by-side comparisons of effectiveness, side effects, costs, and more.
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.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy