Amelogenesis Imperfecta Hypoplastic Autosomal Dominant Local C0399368
Amelogenesis imperfecta is a genetic tooth enamel disorder. It causes thin, malformed enamel and tooth discoloration. Treatment involves restorative dental procedures to protect teeth.
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.
Enamel hypoplasia
(congenital)Generalized symptoms
(congenital)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Localized tooth discoloration
(congenital)Enamel fragility
(congenital)Patterned enamel defects following autosomal dominant inheritance
(congenital)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
Composite Resin Restorations
Direct application of tooth-colored composite resin to restore affected enamel surfaces and improve aesthetics. This approach is commonly used for mild to moderate hypoplastic amelogenesis imperfecta to protect tooth structure and enhance appearance.
Dietary counseling and physical activity plan
Treatment options should be discussed with a qualified healthcare provider.
Preventive Dental Surveillance
Regular dental examinations with radiographic monitoring to assess tooth development, detect caries early, and evaluate treatment outcomes. Includes professional cleaning and fluoride assessments.
Additional treatment options
All-Ceramic Crowns
Full coverage ceramic crowns placed over prepared teeth to provide durable protection and natural aesthetics. Recommended for severely affected teeth with significant enamel deficiency.
surgicalEnhanced Oral Hygiene Protocol
Implementation of specialized brushing techniques, interdental cleaning, and pH-neutral oral care products to minimize caries risk and protect compromised enamel surfaces.
lifestyleDental Veneers
Porcelain or composite veneers bonded to anterior teeth to achieve natural-looking results while preserving tooth structure. Appropriate for patients with primarily aesthetic concerns in visible areas.
procedureWhen 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 Amelogenesis Imperfecta Hypoplastic Autosomal Dominant Local C0399368?
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.
Crawford PJ, Aldred MJ, Bloch-Zupan A. Amelogenesis imperfecta: a review of the clinical manifestations and genetic heterogeneity. Orphanet J Rare Dis. 2020.
American Academy of Pediatric Dentistry. Clinical Guidelines for Management of Hereditary Enamel Defects. Pediatr Dent. 2022.
Simmer JP, Papagerakis P, Smith CE, et al. Molecular Mechanisms of Enamel Formation in Hereditary Conditions. J Dent Res. 2021.
Patir A, Seymen F, Yildirim M, et al. Enamel Formation and Genetic Disorders: Focus on Amelogenesis Imperfecta. Eur J Paediatr Dent. 2023.
Medication Comparisons
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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 25, 2026. · Read our editorial policy