Pompe's disease and anaesthesia

A case report of a child with Pompe's disease (glycogen storage disease Cori type II), who underwent two general anaesthetics, is presented. The progressive infiltration of heart and skeletal muscle with glycogen results in a severe form of cardiomyopathy and respiratory muscle weakness. Consequently, there are significant problems in the anaesthetic management of these patients. Although there are theoretical attractions for some anaesthetic drugs, the key factors in a successful outcome are attention to anaesthetic technique and close monitoring.