Anesthesia Considerations 

Glycogen Storage Disorders

 

 

Background 

 

• Inherited disorders caused by abnormalities of enzymes that regulate glycogen synthesis & breakdown

• 3 common features with all these disorders: 

  • Acidosis secondary to fat & protein metabolism

  • Risk of hypoglycemia secondary to the failure to metabolize glycogen to glucose

  • Cardiac & hepatic dysfunction secondary to destruction of normal tissue by accumulated glycogen

 

 

Considerations

 

• Careful monitoring of glucose & give D5W during fasting periods 

• Careful evaluation of cardiac & hepatic function 

• Careful review of previous anesthetics & obtain endocrinology consultation before surgery 

• Monitor glucose & pH intra-op

• Avoid lactate containing solutions (they cannot convert lactate to glycogen)