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KMID : 0358920130400010066
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Journal of the Korean Academy of Pedodontics
2013 Volume.40 No. 1 p.66 ~ p.71
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DENTAL TREATMENT IN A PATIENT WITH FUKUYAMA TYPE MUSCULAR DYSTROPHY UNDER TOTAL INTRAVENOUS ANESTHESIA USING PROPOFOL
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Jin Dal-Lae
Shin Teo-Jeon
Hyun Hong-Keun
Kim Young-Jae
Kim Jung-Wook
Lee Sang-Hoon
Kim Chong-Chul
Jang Ki-Taeg
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Abstract
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Muscular dystrophy is a genetically heterogeneous group of disorders characterized by progressive muscle weakness of variable distribution and severity. Fukuyama type congenital muscular dystrophy (FCMD) is an unusual form of muscular dystrophy with autosomal recessive inheritance and is clinically characterized by an early age of onset, severe central nervous system involvement, facial muscle weakness, and multiple joint contractures. Muscular dystrophy is susceptible to perioperative respiratory, cardiac and other complications. Patients with FCMD have upper airway muscle weakness, therefore general anesthesia is preferred to sedation regarding maintaining the airway when treating these patients. The development of malignant hyperthermia in general anesthesia for patients with muscular dystrophy is a concern. Total intravenous anesthesia should be used instead of inhaled anesthetics because of the risk of malignant hyperthermia. A 3-year-9-month old, 13kg girl with Fukuyama type congenital muscular dystrophy was scheduled for dental treatment under general anesthesia. She had multiple caries and 14 primary teeth needed caries treatment. Prior to general anesthesia, oral premedication with 9 mg midazolam was given. General anesthesia was induced and maintained with target controlled infusion of propofol . The patient with progressive muscular dystrophy was successfully treated under total intravenous anesthesia with a target controlled infusion of propofol. There were no complications related to anesthesia and dental treatment during or after the operation. This case suggests that target controlled infusion of propofol is a safe and appropriate anesthetic technique in FCMD patients for dental treatment.
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KEYWORD
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Fukuyama type congenital muscular dystrophy, Propofol, Target controlled infusion, Multiple caries
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