J Korean Dent Soc Anesthesiol.  2007 Jun;7(1):18-21. 10.17245/jkdsa.2007.7.1.18.

Dental Treatment of a Patient with Pelizaeus-Merzbacher Disease under Outpatient General Anesthesia: A Case Report

Affiliations
  • 1Department of Anesthesiology and Pain Medicine, Seoul National University Hospital, Seoul, Korea.
  • 2Department of Dental Anesthesiology, Seoul National University, School of Dentistry, Seoul, Korea. stone90@snu.ac.kr
  • 3Department of Pediatric Dentistry, Seoul National University, School of Dentistry, Seoul, Korea.

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.

Keyword

Dental procedure; General anesthesia; Leukodystrophy; Pelizaeus-Merzbacher disease
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