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KMID : 1144020220180010018
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Journal of Korean Association for Disability and Oral Health
2022 Volume.18 No. 1 p.18 ~ p.24
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Dental Management of a Patient with Peters-plus syndrome under General Anesthesia: A Case Report
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Oh Seung-Hyun
Kim Hyun-Tae
Shin Teo-Jeon
Hyun Hong-Keun
Kim Young-Jae
Kim Jung-Wook
Jang Ki-Taeg
Song Ji-Soo
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Abstract
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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 β-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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KEYWORD
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Peters-plus syndrome, Antiepileptic drugs, Dental treatment, General anesthesia
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