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Amelogenesis imperfecta is a tooth development disorder. It causes the tooth enamel to be thin and abnormally formed. Enamel is the outer layer of the visible crown portion of the teeth.
Causes
Amelogenesis imperfecta is passed down through families as a dominant trait. That means you only need to get the abnormal gene from one parent in order to get the disease.
Symptoms
The enamel of the tooth is soft and thin. The teeth appear yellow and are easily damaged. Both baby teeth and permanent teeth can be affected.
Exams and Tests
A dentist can identify and diagnose this condition.
Treatment
The treatment depends on how severe the problem is. Full crowns may be necessary to improve the appearance of the teeth and protect them from further damage. Eating a diet that is low in sugar and practicing very good oral hygiene can reduce the chance of developing cavities.
Outlook (Prognosis)
Treatment is often successful in protecting the teeth.
Possible Complications
The enamel is easily damaged, which affects the appearance of the teeth, especially if left untreated.
When to Contact a Medical Professional
Contact your dentist if you have symptoms of this condition.
Alternative Names
AI; Congenital enamel hypoplasia
References
Dhar VK. Development and developmental anomalies of the teeth. In: Kliegman RM, St. Geme JW, Blum NJ, et al, eds. Nelson Textbook of Pediatrics. 22nd ed. Philadelphia, PA: Elsevier; 2025:chap 353.
National institute of health website. Amelogenesis imperfecta. rarediseases.info.nih.gov/diseases/5791/amelogenesis-imperfecta. Updated August 2024. Accessed September 4, 2024.
Regezi JA, Sciubba JJ, Jordan RCK. Abnormalities of teeth. In: Regezi JA, Sciubba JJ, Jordan RCK, eds. Oral Pathology: Clinical Pathologic Correlations. 7th ed. St Louis, MO: Elsevier; 2017:chap 16.
Review Date 3/31/2024
Updated by: Michael Kapner, DDS, General Dentistry, Norwalk Medical Center, Norwalk CT. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.