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Dental management of patient with Duchenne Muscular Dystrophy under general anesthesia in a child: A case report

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¼º¿µÁ¦ ( Sung Young-Jae ) - Seoul National University School of Dentistry Department of Pediatric Dentistry
¼ÛÁö¼ö ( Song Ji-Soo ) - 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
ÀÌ»óÈÆ ( Lee Sang-Hoon ) - Seoul National University School of Dentistry Department of Pediatric Dentistry
½ÅÅÍÀü ( Shin Teo-Jeon ) - Seoul National University School of Dentistry Department of Pediatric Dentistry

Abstract


Duchenne Muscular Dystrophy (DMD) is a disease caused by a mutation in a gene on X chromosome, which prevents the production of dystrophin, a normal protein in muscle tissue. This gender-linked, recessive disorder affects males at a rate of one in 3,500 births. Patients affected by DMD have progressive loss of muscle functions and weakness in their early childhood.
A 5-year-old boy with DMD visited Seoul National University Dental Hospital Department of pediatric dentistry for caries treatment. Clinical and radiographic examinations revealed multiple carious lesions on deciduous teeth. Based on patient¡¯s underling disease, age, and level of distress tolerance, dental treatment under general anesthesia with total intravenous anesthesia was scheduled. All posterior molars were treated with pulpectomy and stainless-steel crown. Maxillary incisors were treated with zirconia crown.
Macroglossia, open-bite, maxillary and mandibular arch expansion, facial muscle malfunction, delayed tooth emergence are commonly observed in patients with DMD. Loss of muscle function in arms and hands often results in poor oral hygiene level. Therefore, dental and prophylactic treatment should be provided earlier. Recent case reports suggest that some patients with DMD are at risk of developing malignant hyperthermia related to general anesthesia with inhaled anesthetics. When planning general anesthesia for dental treatment, total intravenous anesthesia should be considered as an anesthetic technique for these patients.

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Duchenne Muscular Dystrophy (DMD); Dental treatment; Total intravenous anesthesia

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