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Canadian Journal of Anesthesia, Vol 39, 394-397, Copyright © 1992 by Canadian Anesthesiologists' Society

ARTICLES

Anaesthetic considerations for the child with leukodystrophy

JD Tobias
Department of Anesthesiology, Vanderbilt University, Nashville, Tennessee 37232.

The author presents a four-year-old boy with Pelizaeus-Merzbacher disease who required anaesthesia during placement of PE (pressure equalization) tubes and a permanent silastic intravascular device (Broviac catheter). Pelizaeus-Merzbacher is one of a group of progressive, degenerative disorders of the cerebral white matter known as the leukodystrophies. They include metachromatic leukodystrophy, adrenoleukodystrophy, Krabbe's disease, Canavan's disease, Alexander's disease and Pelizaeus-Merzbacher disease. Due to the progressive nature of the disorders and their devastating effects on the central nervous system, these children frequently require anaesthesia during imaging procedures such as MRI or during various surgical procedures. Of concern to the anaesthetist is the high prevalence of seizure disorders, gastroesophageal reflux with the risk of aspiration, and airway complications related to poor pharyngeal muscle control and copious oral secretions. In addition, adrenal involvement and hypofunction may be seen in patients with adrenoleukodystrophy. Identification of these associated conditions during the preoperative examination will allow safe anaesthesia for these children.


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A. Ioscovich, A. Briskin, A. Abrahamov, S. Halpern, A. Zimran, and D. Elstein
Uncomplicated outcome after anesthesia for pediatric patients with Gaucher disease: [Evolution sans complications apres l'anesthesie de patients pediatriques atteints de la maladie de Gaucher]
Can J Anesth, October 1, 2005; 52(8): 845 - 847.
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Copyright © 1992 by the Canadian Anesthesiologists' Society.