Dental Anomalies in Opitz G or BBB Syndrome and Cleft Lip and Palate associated with Hypertelorism - Abstract
G/BBB is a heterogeneous disorder, with variant X-linked inheritance associated with the chromosomal region Xp22. The aim of this cross-sectional study was to determine the prevalence of dental anomalies in individuals with Opitz G/BBB syndrome (G/BBB) and cleft lip and palate associated with hypertelorism (CLP/H) compared to two control groups. 22 individuals with G/BBB and 14 with CLP/H, above eight years of age, and two control groups paired for gender and age - non-syndromic cleft lip and palate (CLEFT), and without morphofunctional alterations (WMFA) composed the sample. Hypoplastic, hyperplastic and heterotopic anomalies were analyzed on panoramic radiographs, obtained before orthodontic treatment. The results were analyzed by descriptive statistics. Comparisons between groups was performed by the Dunn’s method, and the number of dental anomalies was evaluated by the Kruskal-Wallis test (p<0.05). All individuals with G/BBB syndrome and CLP/H presented at least one dental anomaly, with predominance of hypoplastic alterations, mainly anomalies of number as hypodontia and supernumerary teeth, followed by heterotopic alterations with predominance of rotation. The frequency of hypodontia and supernumerary teeth was significantly higher compared with WMFA group and without significant difference from the CLEFT group. Eight supernumerary teeth were identified in the mandibular midline and three mesiodens in the maxillary region. For diagnostic evaluation of individuals suspected to have these syndromes, it is suggested to investigate the occurrence of supernumerary teeth in the midline in both deciduous and permanent dentitions.