DENTAL TREATMENT IN A PATIENT WITH FUKUYAMA TYPE MUSCULAR DYSTROPHY UNDER TOTAL INTRAVENOUS ANESTHESIA USING PROPOFOL (후쿠야마 선천성 근이영양증 환자의 프로포폴을 이용한 전정맥마취 하 치과치료)

진달래; Shin Teo Jeon (신터전); Hong-Keun Hyun (현홍근); Kim, Young J (김영재); Kim, JungWook (김정욱); Sang-Hoon Lee (이상훈); 김종철; Jang, KiTaeg (장기택)

DENTAL TREATMENT IN A PATIENT WITH FUKUYAMA TYPE MUSCULAR DYSTROPHY UNDER TOTAL INTRAVENOUS ANESTHESIA USING PROPOFOL

Journal of the Korean academy of pediatric dentistry
Abbr : J Korean Acad Pediatr Dent
2013, 40(1), pp.66-71
Publisher : The Korean Academy Of Pediatric Dentistry
Research Area : Medicine and Pharmacy > Dentistry

진달래 ¹, Shin Teo Jeon ², Hong-Keun Hyun ², Kim, Young J ², Kim, JungWook ², Sang-Hoon Lee ², 김종철 ², Jang, KiTaeg ²

¹서울대학교 치의학대학원 소아치과학교실
²서울대학교

Accredited

ABSTRACT

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 3~3.5 μg/mL. 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.

KEYWORDS

Fukuyama type congenital muscular dystrophy, Propofol, Target controlled infusion, Multiple caries

Citation status

* References for papers published after 2023 are currently being built.

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Journal of the Korean academy of pediatric dentistry 2023 KCI Impact Factor : 0.48