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

• Published in: Special care in dentistry : official publication of the American Association of Hospital Dentists, the Academy of Dentistry for the Handicapped, and the American Society for Geriatric Dentistry the Academy of Dentistry for the Handicapped, and the American Society for Geriatric Dentistry, 2024, Vol.44 (2), p.438-449
• Main Authors: Vavetsi, Konstantina, Panagopoulou, Olga, Koromantzos, Panagiotis, Fryssira, Ηelen, Bobetsis, Spyridon A, Emmanouil, Dimitris, Bobetsis, Yiorgos A
• Format: Report
• Language: English
• ISSN: 1754-4505
• DOI: 10.1111/scd.12881

AIMSWilliams 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 RESULTSA 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.CONCLUSIONDue 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.