General anesthesia for an adolescent with Pelizaeus-Merzbacher disease: A case report.
10.17085/apm.2019.14.1.44
- Author:
Hyuckgoo KIM
1
;
Chaeseok LIM
Author Information
1. Department of Anesthesiology and Pain Medicine, Yeungnam University College of Medicine, Daegu, Korea. rlagurrn@hanmail.net
- Publication Type:Case Report
- Keywords:
General anesthesia;
Genetic disease;
Leukodystropy;
Pelizaeus-Merzbacher disease
- MeSH:
Adolescent*;
Anesthesia;
Anesthesia, General*;
Anesthetics;
Central Nervous System;
Chromosome Disorders;
Exotropia;
Female;
Humans;
Incidence;
Intubation;
Monitoring, Intraoperative;
Muscle Spasticity;
Myelin Sheath;
Oxygen;
Pelizaeus-Merzbacher Disease*;
Perioperative Period;
Propofol;
Pyridostigmine Bromide;
Seizures
- From:Anesthesia and Pain Medicine
2019;14(1):44-47
- CountryRepublic of Korea
- Language:English
-
Abstract:
Pelizaeus-Merzbacher disease (PMD) is a progressive and degenerative chromosomal disorder of the central nervous system caused by defective myelin production. Few case reports have been issued on the anesthetic management of PMD, because of its extremely low incidence. We anesthetized a 13-year-old female patient diagnosed with PMD for ophthalmic surgery because of intermittent exotropia. General anesthesia was induced and maintained with propofol and sevoflurane in air and oxygen. Rocuronium was administered to facilitate orotracheal intubation, and residual neuromuscular blockage was reversed with pyridostigmine. Between emergence to 24 hours postoperatively, her muscle power completely recovered and no unpredictable events occurred. Summarizing, anesthesiologists should be concerned about the high possibility of aspiration, spasticity, and seizure during the perioperative period in patients with even mild PMD. Appropriate preoperative evaluation, intraoperative monitoring, and choice of proper anesthetic drugs enable safe anesthesia in patients with PMD.
