Williams-Beuren syndrome: A complete guide for oral healthcare

Abstract

Introduction: Williams-Beuren syndrome is a rare, congenital, multisystem disorder, resulting from genetic alterations on chromosome 7q11.23. Characteristic features of WBS are the developmental, physical and mental abnormalities associated with it. Typical facial features and a spectrum of tooth disorders are associated with this condition. Cardiac and renal involvement may be so severe that sudden death during oral healthcare procedures is a possibility. Photophobia and hyperacusis further make dental treatment a challenge in these patients. Corpus: Routine dental treatment in the dental office pose a significant risk, depending upon the mental and physical compromise of the patient, thereby making hospital admission a safer approach. A multispeciality approach is necessary to provide effective and safe oral healthcare to such patients. This article provides undergraduates, residents, general dental practitioners, and specialists involved in oral healthcare with a comprehensive overview of the condition with emphasis on its genetic basis, pathology, clinical features, diagnosis, and management of general and oral health. Conclusion: Adequate knowledge regarding the various aspects of Williams-Beuren syndrome allows the oral health care student or specialist to plan and manage oral procedures safely and effectively. Specialist referral and multidisciplinary care may be considered when appropriate.