Amelogenesis imperfecta

Abstract

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. Disease name Amelogenesis imperfecta (AI) is a term for a clinically and genetically heterogeneous group of conditions that affect the dental enamel, occasionally in conjunction with other dental, oral and extraoral tissues. Definition and diagnosis criteria AI represents a group of conditions, genomic in origin, which affect the structure and clinical appearance of the 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 [1]. AI is a developmental condition of the dental enamel (characterised by hypoplasia and/or hypomineralisation) that shows autosomal dominant, autosomal recessive, sexlinked and sporadic inheritance patterns, as well as sporadic cases.

Interdisciplinary Approach to Management of Amelogenesis Imperfecta: A Case Series

“…Preventive aspects include dietary advice on limited sugar intake and nonerosive diet, fluoride supplements and oral hygiene maintenance. 16 The periodontal health and eruptive process are important for the success of the restorative treatment. And if any restoration margins become visible, then additional treatment will be required at regular follow-up intervals.…”

“…Veneers are a good option in mixed dentition compared to full crowns but porcelain veneers, full crowns, overdentures, and complete dentures are preferred in permanent dentition. 4,16,23,25,27 The option of extraction and construction of complete or removable partial dentures in AI, affects the psychological status of the adolescent patients and should be dealt with utmost care. 4,22,24,27 conclusIon Management of patients with AI requires careful planning and requires a stepwise approach with most conservative treatment options first.…”

“…According to other reports, the anterior open bite results from tongue thrusting and thumb sucking habits. 16 Children with AI have a variety of oral difficulties including increased incidence of caries, delayed tooth eruption, or even teeth retention, the sensitivity of teeth, loss of occlusal vertical dimension, unsatisfactory aesthetics. [17][18][19] Due to unsatisfactory esthetics the subjects may also have a psychological related problem and lack ratio, decreased bond strength of resin to enamel and bonding to enamel due to rapid loss of enamel.…”