WILLIAMS SYNDROME - A CASE REPORT
and weakness in visuospatial construction . They also exhibit personality traits such as empathy , overfriendliness , attention problems and anxiety . 8 This is an important factor to consider in the behavior management of children during dental treatment . Many studies have reported increased frequency of dental abnormalities including malocclusion , hypodontia , malformed teeth , taurodontism , pulp stones , increased space between teeth , enamel hypoplasia , and high prevalence of dental caries . Affected patients usually have normal periodontium and are not predisposed to increased periodontal destruction , despite the presence of elastin gene haploinsufficiency . 6 , 11 A high caries index is reported due to the enamel hypoplasia and hypomineralization which can be attributed to the hypercalcemia seen in these individuals . 12 The reported patient presented enamel hypoplasia , dental caries and a Class III malocclusion commonly seen in this condition . Periodontal evaluation revealed a healthy periodontium . Individuals with WS can exhibit hyperacusis , which is hypersensitivity to certain sound frequencies . 13 This is of great significance in the dental environment where instruments like the dental hand piece , ultrasonic scalers and high volume section generate high pitched sounds . The Tell Show Do behavior management approach 14 is very helpful where these instruments and their sounds are first demonstrated to the patient before being used . The patient was diagnosed with anxiety disorder for which she was undergoing psychological therapy . The Tell Show Do behavior management technique and reassurance helped allay her anxiety and she was very cooperative during the dental treatment . The risk of sudden cardiac death is 25 – 100 times greater in patients with WS compared to the general population 15 , which confers a significant risk for patients undergoing general anesthesia . This is an important consideration for clinicians planning procedures requiring general anesthesia . 16 Early dental examinations and parent counseling are important in the management of individuals with WS . Preventive and dietary programs must be customized for these patients and reinforced in children with severe enamel hypoplasia , high caries rates and vulnerable cardiac condition to help minimize the risk of infection in the oral cavity . 16
4 . Conclusion
There is no cure for Williams syndrome . The care for these patients is mainly symptomatic and supportive . Individuals with WS need regular monitoring for potential medical and dental problems by health care professionals who are familiar with the disorder . The dental treatment protocol may require alterations or necessary precautions to be taken due to the underlying medical conditions . The pediatric dentist thus plays an important role in the overall care of these patients .
Acknowledgments
The authors declare no conflict of interest related to this study . There are no conflicts of interest and no financial interests to be disclosed .
REFERENCES
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