Oral and dental development in X chromosome aneuploidy.

Though frequently noted, orofacial anomalies in poly X syndromes have been seldom reviewed. In a study of patients with an X chromosome aneuploidy (XXXXX, XXXX, XXXXY, XXXY) the following facial and dental defects were found to be characteristic features of these syndromes: mainly bifid uvula and macroglossia, enamel defects, dental taurodontism and abnormal roots. These observations are discussed in the light of the possible role of X-linked genes.