Generic term that refers to a group of genetic disorders in which the white matter of the central nervous system is affected either in isolation or predominantly. Main disorders: Krabbe leukodystrophy, metachromatic leukodystrophy, X-linked adrenoleukodystrophy, Pelizaeus-Merzbacher disease, Alexander disease, Canavan-Van Bogaert disease, Aicardi syndrome.

Anesthetic implications:

risk of pulmonary aspiration, abundant secretions, epilepsy, mental retardation.

References : 

-         Tobias JD. 
Anaesthetic considerations for the child with leukodystrophy. 
Can J Anaesth 1992; 39:394-7.  

-        Bascou NA, Marcos MC, Beltran Quintero ML, Roosen-Marcos MC, Cladis FP, Poe MD, Escolar ML.
General anesthesia safety in progressive leukodystrophies: a retrospective study of patients with Krabbe disease and metachromatic leukodystrophy.
Pediatr Anesth 2019; 29: 1053-9

Updated: November 2019