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

Abstract

any problems. General anaesthesia was maintained with 2% sevoflurane and 50% O 2 in the air, remifentanil infusion (0.01–0.5 μg/kg per hour) and rocuronium infusion (0.3 mg/kg). Proper ventilation settings were arranged. Gastric contents were suctioned. Since these patients have a low body temperature, we used a circulating-water mattress and a forced-air warming device to prevent hypothermia and followed up with a nasopharyngeal temperature probe. Haemodynamic changes were monitored with Pulse index Contour Continuous Cardiac Output (PiCCO) measured parameters, including stroke volume, stroke volume variation, pulse pressure variation and cardiac output. Fluid therapy was administered, guided by an algorithm depending on the PiCCO parameters. In total, 110 ml of the red 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.