ABSTRACT
La ataxia de Friedreich (AF) es una enfermedad hereditaria autosómica recesiva poco frecuente caracterizada por un proceso neurodegenerativo del cerebelo, la médula espinal y nervios periféricos, asociado con ataxia, disartria, alteraciones del tracto piramidal, pérdida de la sensibilidad, escoliosis, miocardiopatía y diabetes. Existen muchas posibilidades de complicaciones perioperatorias graves durante el manejo anestésico de estos pacientes. Presentamos el caso de una adolescente de 14 años con AF, sometida a cirugía de reparación de escoliosis dorsolumbar bajo anestesia total intravenosa (AU)
Friedreich ataxia (FA) is an inherited autosomal recessive disease characterized by a neurological degenerative process of the cerebellum, spinal cord, and peripheral nerves. FA is associated with ataxia, dysarthria, motor and sensory impairment, scoliosis, cardiomyopathy, and diabetes. There is a significant risk of perioperative major complications during the anesthetic management of these patients. We present the case of a fourteen-year-old patient with FA, who had a posterior spinal fusion and instrumentation underwent to total intravenous anesthesia (AU)
Subject(s)
Humans , Female , Adolescent , Scoliosis , Anesthesia, General/instrumentation , Anesthesia, General/methods , Anesthesia, Intravenous/instrumentation , Anesthesia, Intravenous/methods , Radiography, Thoracic/methods , Myocardial Ischemia/complications , Myocardial Ischemia , Diabetes Complications/drug therapy , Lidocaine/therapeutic use , Gentamicins/therapeutic use , Propofol/therapeutic useABSTRACT
Friedreich ataxia (FA) is an inherited autosomal recessive disease characterized by a neurological degenerative process of the cerebellum, spinal cord, and peripheral nerves. FA is associated with ataxia, dysarthria, motor and sensory impairment, scoliosis, cardiomyopathy, and diabetes. There is a significant risk of perioperative major complications during the anesthetic management of these patients. We present the case of a fourteen-year-old patient with FA, who had a posterior spinal fusion and instrumentation underwent to total intravenous anesthesia.
