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KMID : 1144020200160020089
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Journal of Korean Association for Disability and Oral Health
2020 Volume.16 No. 2 p.89 ~ p.93
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Dental treatment of a patient with GM1 gangliosidosis under general anesthesia: A case report
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Kim In-Ki
Song Ji-Soo
Shin Teo-Jeon
Hyun Hong-Keun
Kim Young-Jae
Kim Jung-Wook
Jang Ki-Taeg
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Abstract
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GM1 gangliosidosis is a lysosomal storage disease caused by various mutations in the GLB1 gene leading to deficiency in ßgalactosidase activity. Reported oral manifestations of GM1 gangliosidosis are gingival hyperplasia, enlarged tongue, disturbance in tooth eruption, dystonic movements of jaw and tongue, and mastication impairment. This report presents the dental treatment of a 4-year-old girl with GM1 gangliosidosis under general anesthesia. Caries lesions were detected on the primary second molars, and calculus was deposited around anterior teeth. Red granular spots around the marginal gingiva were observed, and gingival bleeding was easily triggered by simple stimulation. Such bleeding tendency might have been related to the use of valproic acid. The patient was treated by stainless steel crown restorations on the primary molars and scaling. Intensive oral hygiene care is a crucial duty of the oral health providers to reduce the risk of aspiration pneumonia in tube-feeding patients. To understand the medical status of patients and make appropriate modifications to the treatment plan, dental professionals should properly communicate with pediatricians.
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KEYWORD
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GM1 gangliosidosis, Valproic acid, Aspiration pneumonia, Tube-feeding patients
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