Oral manifestations of nine individuals with Williams syndrome. A case series.

AIMS: Williams syndrome (WS) is a congenital developmental disorder characterized, mainly, by distinctive facial features, cardiovascular anomalies, growth delay and a typical neurobehavioral profile. The oral manifestations have not been sufficiently described and, therefore, the aim of the current study was to present the clinical, radiographic and microbiological findings of individuals with WS. METHODS AND RESULTS: A series of nine WS individuals (seven females) with mean age 21 years-old were evaluated. A complete intraoral clinical examination, a radiographic analysis using panoramic and cephalometric x-ray and a supra- and sub-gingival microbiological profiling were performed. We observed abnormal tooth morphology, excessive interdental spacing, congenitally missing permanent teeth and malocclusion. High levels of DMFT were observed and gingivitis was present in all subjects. Bacteria related to periodontal disease were detected in dental plaque. Three patients were classified with a gingival phenotype type I according to the Maynard and Wilson classification. The sella turcica bridging was a novel finding for this group of patients. CONCLUSION: Due to the elevated prevalence of gingivitis, caries and malocclusion, a multidisciplinary approach including dental follow-ups should be the standard of care in WS patients.

Oral manifestations of eleven individuals with Noonan syndrome. A case series.

AIMS: Noonan syndrome (NS) is a clinically and genetically heterogeneous condition characterized by distinctive facial features, short stature, and congenital heart defects. The oral manifestations have not been sufficiently described. In an attempt to enrich our understanding regarding the oral manifestations, the aim of the current study was to present the clinical, radiographic, and microbiological findings of eleven subjects with NS syndrome. METHODS AND RESULTS: A complete intraoral clinical evaluation, a radiographic analysis using panoramic and cephalometric x-rays, and a supra- and sub-gingival microbiological profiling of 20 periodontal and cariogenic microbiota using the checkerboard DNA-DNA hybridization technique were performed in a series of 11 NS individuals. Compared to previous reports very few dental findings were present. Gingivitis and carious lesions were present in all subjects and could be related to poor oral hygiene. High-arched palate and malocclusions such as crossbite, open-bite, and deep-bite were very common findings but without a specific pattern. Two findings that have not been reported before included the different shapes of the condyles and the irregular shape of the sella turcica. CONCLUSION: Due to the elevated prevalence of gingivitis, caries, and malocclusion, a multidisciplinary approach including dental follow-ups should be the standard care in NS patients.

Caries prevalence and caries experience (ICDAS II criteria) of 5-, 12- and 15-year-old Greek children in relation to socio-demographic risk indicators. Trends at the national level in a period of a decade.

PURPOSE: To study the caries status of 5, 12 and 15-year-old Greek children, assess how disease parameters are related to socio-demographic indicators and identify relevant trends at the national level. METHODS: A stratified cluster sample of 3702 children in total was randomly selected and examined clinically for caries (ICDAS II criteria). Caries experience was outlined by adapting ICDAS0-6 criteria to the d/D component of the WHO dmf/DMF index configuration. Percentages (%) of caries experience-free children, of children with initial caries (ICDAS1-2), and the mean d1-2t/D1-2T, d3-6mft/D3-6MFT and d3-6mfs/D3-6MFS indices were calculated. The probability of presenting with d1-2t/D1-2T ≥ 1 was assessed by binary logistic regression analysis, whereas negative binomial regression models examined the effect of socio-demographic parameters on d3-6mfs/D3-6MFS indices (level of significance: p ≤ 0.05). RESULTS: 60.1%, 48.1%, and 34.7% of the 5, 12, and 15-year-old children, respectively, had no caries experience at the defect level (d3-6mft/D3-6MFT = 0). Initial lesions (ICDAS1-2) were detected in 17.7%, 19.3% and 17.4% of the 5, 12 and 15-year-olds, accordingly. Mean d1-2t/D1-2T was 0.93, 1.70, and 2.51, whereas mean d3-6mft/D3-6MFT was 1.48, 1.61, and 2.46 for the 5, 12, and 15-year-olds, respectively. Children with higher educated parents and 15-year-old urban residents exhibited significantly less caries experience at the defect level. Initial caries lesions presented a significantly higher probability of being detected in urban-residing 5- and 15-year-olds, while no consistent trend could be identified for parental education level. Caries prevalence and experience levels declined for all age groups in ten years. CONCLUSION: Although the dental health of Greek children has improved disparities remain, calling for organised primary and secondary preventive interventions.