Amelogenesis imperfecta

Amelogenesis imperfecta (AI) represents a group of developmental conditions, genomic in origin, which affect the structure and clinical appearance of enamel of all or nearly all the teeth in a more or less equal manner, and which may be associated with morphologic or biochemical changes elsewhere in the body. The prevalence varies from 1:700 to 1:14,000, according to the populations studied. The enamel may be hypoplastic, hypomineralised or both and teeth affected may be discoloured, sensitive or prone to disintegration. AI exists in isolation or associated with other abnormalities in syndromes. It may show autosomal dominant, autosomal recessive, sex-linked and sporadic inheritance patterns. In families with an X-linked form it has been shown that the disorder may result from mutations in the amelogenin gene, AMELX. The enamelin gene, ENAM, is implicated in the pathogenesis of the dominant forms of AI. Autosomal recessive AI has been reported in families with known consanguinity. Diagnosis is based on the family history, pedigree plotting and meticulous clinical observation. Genetic diagnosis is presently only a research tool. The condition presents problems of socialisation, function and discomfort but may be managed by early vigorous intervention, both preventively and restoratively, with treatment continued throughout childhood and into adult life. In infancy, the primary dentition may be protected by the use of preformed metal crowns on posterior teeth. The longer-term care involves either crowns or, more frequently these days, adhesive, plastic restorations

Amelogenesis imperfecta—towards a new classification

This editorial reviews the history of the classification of amelogenesis imperfecta (Al). The limitations of the existing classification systems are discussed. An alternative classification is proposed based upon the molecular defect, biochemical result, mode of inheritance and phenotype in the family involved. While not all of the criteria for the proposed classification can yet be addressed, this scheme is proposed for future classification of At cases and families

Amelogenesis imperfecta with coronal resorption: report of three cases

Intracoronal resorption of the permanent dentition in cases of amelogenesis imperfecta (AI) is a rare finding which poses an added complication to the already complex management of this condition. This paper presents three cases of AI associated with delayed eruption of permanent teeth in which asymptomatic intracoronal resorption occurred. CPD/Clinical Relevance: This paper highlights the fact that teeth affected with amelogenesis imperfecta may undergo asymptomatic intracoronal resorption which is only identifiable radiographically

Oral Rehabilitation of a Young Adult with Amelogenesis Imperfecta: A Clinical Report

This clinical report describes a multidisciplinary approach for the oral rehabilitation of a young adult patient diagnosed with hypoplastic amelogenesis imperfecta with a skeletal Class III malocclusion. The specific objectives of this treatment were to eliminate tooth sensitivity while enhancing esthetics and restoring masticatory function. The reverse horizontal overlap of posterior teeth was maintained. Treatment included removal of few teeth, lengthening of the maxillary and mandibular clinical crowns, and placement of anterior and posterior metal-ceramic fixed partial dentures. The third month recall examination revealed no pathology associated with the rehabilitation, and the patient’s esthetic and functional expectations were satisfied