Perioperative anesthetic management of very long-chain acyl-coenzyme a dehydrogenase deficiency

Abstract

Very long-chain acyl-coenzyme A dehydrogenase deficiency is a rare disorder of β-oxidation fatty acid metabolism that results in susceptibility to hypoglycemia, liver failure, cardiomyopathy and rhabdomyolysis during catabolic situations. We report the case of a 10-year-old male undergoing a totally implanted central venous catheter placement during hospitalisation for rhabdomyolysis, who was successfully managed with general anesthesia with nitrous oxide, sevoflurane and remifentanil. No hypoglycemia occurred and creatine kinase levels did not increase in the perioperative period. We describe the challenges encountered and the strategies used to avoid further decompensation of the disease due to surgical stress.

Keywords: Anestesia; Anesthesia; Deficiencia de acil-coenzima A deshidrogenasa de cadena muy larga; Enfermedad metabólica; Fatty acids; Lipid metabolism; Metabolic disease; Metabolismo lipídico; Rabdomiolisis; Rhabdomyolysis; Very long-chain acyl-coenzyme A dehydrogenase deficiency; Ácidos grasos.