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KMID : 1144020180140010017
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Journal of Korean Association for Disability and Oral Health
2018 Volume.14 No. 1 p.17 ~ p.21
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DENTAL TREATMENT FOR A PATIENT WITH WILLIAMS SYNDROME UNDER GENERAL ANESTHESIA : CASE REPORT
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Seo Mee-Kyung
Song Ji-Soo
Shin Teo-Jeon
Hyun Hong-Keun
Kim Jung-Wook
Jang Ki-Taeg
Lee Sang-Hoon
Kim Young-Jae
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Abstract
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Williams syndrome (WS) is a rare congenital disorder which is caused by microdeletion of approximately 1.6 MBP from the long arm of chromosome 7 at 7q11.23. It is characterized by cardiovascular anomalies, elfin face and mental retardation. The most typical oral signs in patient with WS are hypodontia, reduced mesio-distal dimensions both in the primary and permanent teeth, macroglossia, excessive interdental spacing, enamel hypoplasia and enamel hypomineralization. The majority of children with WS have mild to moderate mental retardation, generalized anxiety disorder, hyperactivity disorder and sensitivity to sounds. The purpose of this presentation is to describe dental treatment for a child with WS. A 9-year-old boy diagnosed with WS had caries on his first permanent molars. Because of the poor cooperation, these teeth were filled temporarily with glass ionomer, and treatment under general anesthesia was planned. Under general anesthesia, caries treatment of first permanent molar and extraction of primary molar was successfully performed and there was no postoperative complications related to general anesthesia. Open bite, hypodontia, excessive dental space, enamel hypoplasia, enamel hypomineralization were observed which were characteristic in WS.
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KEYWORD
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Williams syndrome, Dental treatment, General anesthesia, Oral manifestation
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