Hereditary amelogenesis imperfecta. I. Oral health in children.

An epidemiological study of hereditary enamel defects comprising more than 400 000 children, 3-19 years of age, was performed in 1982-1983 in the middle of Sweden. 105 children were diagnosed as having Hereditary Amelogenesis Imperfecta (HAI). Based on clinical and genetic data a classification of HAI could be carried out based on the hypoplastic and the hypomineralized type. 99 out of the 105 children were available for a thorough clinical and radiographical examination concerning oral health and these children were analysed in terms of carious lesions, restorations, dental plaque, calculus, gingival health and the occurrence of pulpal calcifications and taurodontism. A low number of carious lesions were particularly seen in children characterized by severe hypoplastic and hypomineralized enamel defects. A high number of restored proximal and buccal/lingual tooth surfaces could be recorded predominantly in children age 13-16 years associated to the hypomineralized type of HAI, where the number of decayed and filled proximal surfaces was 3.7 in the hypoplastic type and 17.1 in the hypomineralized type of HAI, respectively. In the age-group 6-19 years, 36 out of 91 children had totally 265 crowns and/or veneers. Crowns, anterior as well as posterior, were more common in children associated to the hypomineralized type compared to the hypoplastic type. Cosmetic treatment with composite materials was preferrably the choice of treatment in children with the hypoplastic type of HAI. Plaque and gingivitis were predominantly recorded in children with severe hypomineralized enamel defects. Excessive amounts of supracalculus deposition were also recorded in these children. Pulpal calcification and taurodontism could be scarcely diagnosed in the material and the findings could not be related to any specific type of HAI. The oral health data indicated a need for early therapy planning in children with HAI.