Amelogenesis imperfecta (AI) represents a group of inherited conditions that affect the structure and appearance of the enamel of all or nearly all the teeth, with a prevalence in the United States of approximately one in 14,000.1,2 The variable range of AI enamel defects involves abnormalities that are classified as hypoplastic (defect in quantity of enamel), hypomaturation (defect in final growth and development of enamel crystallites), and hypocalcified (defect in initial crystallite formation followed by impaired growth), with diagnosis traditionally determined by clinical presentation.2,3
Four main types of AI classifications exist, based on the type of enamel defect. Fourteen unique subtypes exist and are based on the clinical appearance and mode of inheritance.3,4 Although AI primarily affects enamel formation, AI has been associated with a more constricted maxillary arch as well as with a skeletal open bite with anterior space, resulting in occlusal instability.5-8
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