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.