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.
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