Journal of The Korean Dental Society of Anesthesiology (대한치과마취과학회지)

The Korean Dental Society of Anesthsiology (대한치과마취과학회)
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Dental Treatment of a Patient with Pelizaeus-Merzbacher Disease under Outpatient General Anesthesia -A Case Report-

Pelizaeus-Merzbacher 병 환자의 외래전신마취 하 치과치료 -증례보고-

  • Kim, Tae-Kyung (Department of Anesthesiology and Pain Medicine, Seoul National University Hospital) ;
  • Shin, Cha-Uk (Pediatric Dentistry, Seoul National University, School of Dentristry) ;
  • Kim, Hyun-Jeong (Department of Dental Anesthesiology, Seoul National University) ;
  • Yum, Kwang-Won (Department of Dental Anesthesiology, Seoul National University) ;
  • Seo, Kwang-Suk (Department of Dental Anesthesiology, Seoul National University)
  • 김태경 (서울대학교병원 마취통증의학과) ;
  • 신차욱 (서울대학교 소아치과학교실) ;
  • 김현정 (서울대학교 치의학대학원 치과마취과학교실) ;
  • 염광원 (서울대학교 치의학대학원 치과마취과학교실) ;
  • 서광석 (서울대학교 치의학대학원 치과마취과학교실)
  • Published : 2007.06.23
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Abstract

Pelizaeus-Merzbacher disease (PMD) can be defined as an X-linked recessive leukodystrophy that is caused by a mutation in the proteolipid protein gene on chromosome Xq22. PMD is one of a group of progressive, degenerative disorders of the cerebral white matter known as the leukodystrophies. Due to the progressive nature of the disorders and their devastating effects on the central nervous system, these children frequently require anesthesia during imaging procedures such as MRI or during various surgical procedures. Anesthetic concerns in theses cases include high prevalence of seizure disorders, gastroesophageal reflux with the risk of aspiration, airway complications related to poor pharyngeal muscle control and copious oral secretions, and mental retardation. We report a successful anesthetic management in a patient with PMD for dental procedures.

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