Pavan Manohar Patil, Seema Pavan Patil. September 2020

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.