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KMID : 1144020200160020069
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Journal of Korean Association for Disability and Oral Health
2020 Volume.16 No. 2 p.69 ~ p.74
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Dental management of a patient with Rett syndrome 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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Rett syndrome (RTT) is a progressive neurological disorder affecting about 1 in 10000-15000 females. Affected individuals follow normal development until 6 to 18 months of life. After that, characteristic symptoms of the disease begin to appear such as growth retardation, loss of acquired purposeful hand skills, stereotypic hand movements, loss of language, and loss of motor coordination. Several oral manifestations of RTT are reported. Bruxism is the most common finding. Other frequently reported oral symptoms are gingivitis, non-physiological dental attrition, digit/hand sucking or biting, palatal shelving (high palate), anterior open bite, mouth breathing, drooling, and tongue thrusting. In this report, we describe the dental treatment of a child with RTT under general anesthesia, who presented with multiple caries lesions in primary and permanent teeth. Considering the extensive treatment need and uncooperative behavior of the patient, we decided to perform the dental treatment under general anesthesia. The patient was treated by extraction, pulp treatment, and restoration with stainless steel crowns. Intensive preventive measures, such as plaque control, dietary advice, and fluoride application, are vital to the management of the oral health in patients with RTT.
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KEYWORD
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Rett syndrome, Antiepileptic drugs, Dental treatment, General anesthesia
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