Amelogenesis Imperfecta: A report of two cases

Abstract

Amelogenesis Imperfecta (Al) is defined as a group of hereditary developmental defects of the dental enamel affecting both primary and permanent dentition. Al is known by various names such as hereditary enamel dysplasia, hereditary brown enamel, hereditary brown opalescent teeth. This defect involves entirely ectodermal components. Al may be inherited in an X linked manner or by autosomal dominant, autosomal recessive, or sporadic inheritance patterns. Witkop and Sauk listed the varieties of Al, based on whether the abnormality lay in reduced amount of enamel (hypoplasia), deficient calcification (hypocalcification), or imperfect maturation of the enamel (hypomaturation). They have also recognized the combined defects of enamel. Treatment planning involves an interdisciplinary approach of periodontal, prosthodontic and restorative treatment.