• The Journal of Korea Assosiation for Disability and Oral Health
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• v.15 no.1
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• pp.50-54
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• 2019

Muscular dystrophy (MD) is a heterogeneous group of inherited neuromuscular disorders, characterized by progressive muscle weakness. Severity of the disease ranges from mild to severe, and the disease is mostly caused by mutations in a number of genes. These genetic mutations cause lack of proteins which are essential for muscle cell stability. Muscle fibers are gradually replaced by fat and fibrous tissue. The muscles of the head and neck are affected in several types of MD that manifest as altered craniofacial morphology and dental malocclusion. A 3-year-10-month old, 15.0 kg boy with MD presented to Seoul National University Dental Hospital, Seoul, South Korea because of extensive carious teeth. A number of dental caries in primary dentition were identified during clinical oral examination. Due to dental anxiety and underlying systemic disease, general anesthesia was considered. General anesthesia was induced and maintained with intravenous anesthetics, propofol and remifentanil. Caries treatments - resin restoration, pulpectomy, zirconia crown restoration, stainless steel crown restoration - were performed. Under general anesthesia, successful dental procedure was done. Total intravenous anesthesia (TIVA) was performed instead of inhalation anesthesia in order to avoid risk of complications such as malignant hyperthermia and life-threatening rhabdomyolysis. With decreasing muscle function, plaque control becomes more difficult and leads to gingivitis. Especially, the open-mouth posture worsens gingivitis and can leads to malocclusions and problems in swallowing. Regular and periodic dental care is essential for maintaining oral health for patients with MD.