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Dental Management of a Patient with Peters-plus syndrome under General Anesthesia: A Case Report

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¿À½ÂÇö ( Oh Seung-Hyun ) - Seoul National University School of Dentistry Department of Pediatric Dentistry
±èÇöÅ ( Kim Hyun-Tae ) - Seoul National University School of Dentistry Department of Pediatric Dentistry
½ÅÅÍÀü ( Shin Teo-Jeon ) - Seoul National University School of Dentistry Department of Pediatric Dentistry
ÇöÈ«±Ù ( Hyun Hong-Keun ) - Seoul National University School of Dentistry Department of Pediatric Dentistry
±è¿µÀç ( Kim Young-Jae ) - Seoul National University School of Dentistry Department of Pediatric Dentistry
±èÁ¤¿í ( Kim Jung-Wook ) - Seoul National University School of Dentistry Department of Pediatric Dentistry
Àå±âÅà( Jang Ki-Taeg ) - Seoul National University School of Dentistry Department of Pediatric Dentistry
¼ÛÁö¼ö ( Song Ji-Soo ) - Seoul National University School of Dentistry Department of Pediatric Dentistry

Abstract


Peters-plus syndrome is a very rare disease with fewer than 80 cases reported worldwide. It is an autosomal recessive disorder caused by mutations in ¥â-1,3-glucosyltransferase gene (B3GALTL) located on chromosome 13q12.3. The clinical features are ocular anterior segment dysgenesis, also known as Peters anomaly, short limb with brachydactyly, intellectual disability, Cupid¡¯s bow-shaped upper lip, and cleft lip and palate. This report shows dental treatment of a 7-year-boy with Peters-plus syndrome under general anesthesia. Because of frequent seizure attack caused by Peters-plus syndrome, he was prescribed anti-epileptic drugs. In clinical and radiographic examinations, the patient was found to have multiple caries in primary molars. Concerning the patient¡¯s uncooperative behavior related to intellectual disability, an extensive dental restorative treatment was performed under general anesthesia. Intensive preventive treatments such as plaque control, diet consultation, and topical fluoride application would be necessary to maintain the patient¡¯s oral health.

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Peters-plus syndrome; Antiepileptic drugs; Dental treatment; General anesthesia

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