Amelogenesis imperfecta with coronal resorption: report of three cases

Amelogenesis Imperfecta (AI) is a group of relatively rare inherited defects that affect the quantity and/or quality of enamel formation. The disorder is generalized, predominantly affecting all teeth in both primary and permanent dentitions. The prevalence of AI is known to vary by population, ranging from 1:700 to 1:14000.1 Its mode of inheritance can be autosomal dominant, autosomal recessive or X-linked. Sporadic cases, representing spontaneous mutation, may also present.2

AI arises as a result of genetic alteration associated with the process of formation and maturation of enamel.3 Alterations occurring at different levels of enamel formation and maturation result in clinically different presentations. To date, 14 different types of AI have been identified on the basis of genetic pattern, clinical and radiological features, and histological changes.4 In the classifications most commonly used by clinicians, the main types of AI are initially described phenotypically as hypoplastic or hypomineralized; hypomineralized AI can be further sub-classified into hypocalcified and hypomaturation types, with hypocalcified being a more severe type. A combination of hypoplasia and hypomineralization can also be observed in some patients with AI.