RESUMO
La deficiencia de acil-coenzima A deshidrogenasa de cadena muy larga (VLCADD) es un trastorno infrecuente del metabolismo de β-oxidación de los ácidos grasos que origina susceptibilidad a hipoglucemia, fallo hepático, cardiomiopatía y rabdomiólisis durante las situaciones catabólicas. Reportamos el caso de un varón de 10 años de edad programado para la colocación de catéter venoso central totalmente implantado durante su hospitalización por rabdomiólisis, que fue exitosamente gestionada con anestesia general con óxido nitroso, sevoflurano y remifentanilo. No se produjo hipoglucemia y los niveles de creatina quinasa no se incrementaron durante el periodo perioperatorio. Describimos las dificultades a que nos enfrentamos, y las estrategias utilizadas para evitar mayor descompensación de la enfermedad debida al estrés quirúrgico.(AU)
Very long-chain acyl-coenzyme A dehydrogenase deficiency (VLCADD) 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 hospitalization 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.(AU)
Assuntos
Humanos , Masculino , Criança , Período Perioperatório , Acil-CoA Desidrogenase de Cadeia Longa , Anestesia , Rabdomiólise , Cardiomiopatias , Hipoglicemia , Anestesiologia , Doenças Metabólicas , Metabolismo dos LipídeosRESUMO
Very long-chain acyl-coenzyme A dehydrogenase deficiency is a rare disorder of ß-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.
