Anaesthetic management for orthotopic liver transplantation in a patient with glycogen storage disease type IIIa

Abstract

Glycogen storage disease (GSD) type III is a metabolic disorder caused by a deficiency in amylo-1,6-glucosidase enzyme, which is responsible for the breakdown of the glycogen molecule, resulting in glycogen accumulating in the organs, hypoglycaemia, muscle weakness, liver dysfunction, delayed anaesthetic recovery, excessive surgical bleeding, cardiomyopathy and end-organ dysfunction. This case report presents a child with GSD type IIIa who underwent orthotopic liver transplantation (OLT) with her mother as a donor. A multidisciplinary approach should be provided to optimise the preoperative period and minimise complications in these patients.