[An analysis of the prevalence of isolated dental anomalies and of those associated with hereditary syndromes: a model for evaluating the genetic control of the dentition characteristics].

Tooth ontogenesis, morphogenesis, and eruption are under a multifactorial (genetic, epigenetic, environmental) control. The aim of this study has been a genetic appraisal of tooth developmental characteristics by means of the prevalence and patterns of association of tooth anomalies in a large sample of hereditary syndromes. MATERIALS AND METHODS. A sample of 169 hereditary syndromes exhibiting tooth abnormalities was collected from the literature: original articles were controlled in order to achieve uniformity of judgement about the definition of the dental defects. Tooth anomalies were classified according to anatomoclinical criteria: number (hypodontia/hyperdontia), shape, position, structure (enamel/dentin/cementum), size (micro-/macrodontism), eruption (precocious/delayed including unerupted teeth). Prevalence data were calculated for: 1) modes of syndrome inheritance; 2) syndromes exhibiting a single dental anomaly vs. syndromes exhibiting associated tooth abnormalities; 3) different types of tooth abnormalities; 4) isolated and associated tooth abnormalities.