Anaesthetic Management of a Patient with Glycogen Storage Disease Type 1A

Abstract

Glycogen storage diseases (GSD) are a group of inherited disorders which result in deficiencies of enzymes involved in glycogen metabolism and lead to an accumulation of glycogen in the liver. Glycogen storage disease type 1a, a form of glycogen storage disturbance, is a rare metabolic disorder with important implications for anaesthesiologists. It is caused by the lack of the glucose-6-phosphatase, which is necessary for the liver to convert glycogen to glucose. The aim of this case report is to present an uncomplicated surgery with an uncomplicated general anaesthesia and an anaesthetic follow up in a patient with GSD type 1a in an elective day case surgery. In this case, there was a serious hypoglycemia problem due to the glycogen storage disturbance related to disease. To avoid severe hypoglycemia, it is crucial to keep oral feeding 2-3 hour for maintaining a normal blood sugar level.