title: Anesthetic agents in patients with very long-chain acyl-coenzyme A dehydrogenase deficiency: a literature review.
abstract: Very long-chain acyl-coenzyme A dehydrongenase deficiency (VLCADD) is a rare disorder of fatty acid metabolism that renders sufferers susceptible to hypoglycemia, liver failure, cardiomyopathy, and rhabdomyolysis. The literature about the management of these patients is hugely conflicting, suggesting that both propofol and volatile anesthesia should be avoided. We have reviewed the literature and have concluded that the source papers do not support the statements that volatile anesthetic agents are unsafe. The reports on rhabdomyolysis secondary to anesthesia appear to be due to inadequate supply of carbohydrate not volatile agents. Catabolism must be avoided with minimal fasting, glucose infusions based on age and weight, and attenuation of emotional and physical stress. General anesthesia appears to be protective of stress-induced catabolism and may offer benefits in children and anxious patients over regional anesthesia. Propofol has not been demonstrated to be harmful in VLCADD but is presented in an emulsion containing very long-chain fatty acids which can cause organ lipidosis and itself can inhibit mitochondrial fatty acid metabolism. It is therefore not recommended. Suxamethonium-induced myalgia may mimic symptoms of rhabdomyolysis and cause raised CK therefore should be avoided. Opioids, NSAIDS, regional anesthesia, and local anesthetic techniques have all been used without complication.
authors: Department of Anaesthesia, Princess Royal Hospital, Telford, UK.
authors: Charlotte
authors: C
authors: Redshaw
authors: Catherine
authors: C
authors: Stewart
mesh_terms: D044942:Acyl-CoA Dehydrogenase, Long-Chain / Q000172:deficiency*; D000698:Analgesia / Q000379:methods*; D018685:Anesthetics, Inhalation; D018686:Anesthetics, Intravenous; D002648:Child; D002675:Child, Preschool; D000080984:Congenital Bone Marrow Failure Syndromes; D006801:Humans; D007223:Infant; D008052:Lipid Metabolism, Inborn Errors / Q000601:surgery*; D028361:Mitochondrial Diseases / Q000601:surgery*; D009135:Muscular Diseases / Q000601:surgery*; D019148:Neuromuscular Blockade / Q000379:methods*
chemical_list: D018685:Anesthetics, Inhalation; D018686:Anesthetics, Intravenous; D044942:Acyl-CoA Dehydrogenase, Long-Chain
keywords: VLCADD; metabolic disease; propofol; rhabdomyolysis; volatile anesthesia