Anesthesia management in a patient with Canavan disease

Abstract

Canavan disease is an autosomal recessive leukodystrophy, with accumulation of N-acetylaspartic acid. Anaesthetist should be careful abouth the high prevalence of seizure disorders, gastroesophageal reflux, poor pharyngeal muscle control, abundant oral secretions releated with the risk of aspiration. In addition, severe macrocephaly may be seen in these patients. During the preoperative examinations, determination of the releated conditions with this disease will allow safe anaesthesia for these children. In this case report, the anaesthetic manegement of a pediatric patient with Canavan disease is described.