Preoperative Evaluation of Pediatric Patients with Muscle Disorders

Abstract

The preoperative assessment of pediatric patients with myopathy begins with a description of which muscle groups are impacted and the degree of disability present. Particular focus on respiratory status is necessary, as weak muscles of respiration can cause ventilatory insufficiency. This is exacerbated by swallowing difficulty, poor clearance of secretions, inadequate cough, and spinal and rib cage deformity. Restrictive lung disease may be severe. Cardiac dysfunction often accompanies skeletal muscle disease. Cardiomyopathy and conduction defects are prevalent in patients with muscle disorders and require preoperative evaluation. There is wide phenotypic variability among patients with muscle disease. By grouping myopathies according to pathophysiology, we can understand both the presentation and the most significant comorbidities associated with specific types of muscle disease. Finally, we can consider the specific anesthesia considerations that are pertinent to each, highlighting the risks of malignant hyperthermia and anesthesia-induced rhabdomyolysis. This review contains 3 tables and 38 references. Keywords: anesthesia considerations, congenital myopathy, malignant hyperthermia, mitochondrial disorders, muscular dystrophy, myotonia, succinylcholine, anesthesia-induced rhabdomyolysis