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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\u2010induced catabolism and may offer benefits in children and anxious patients over regional anesthesia. Propofol has not been demonstrated to be harmful in <jats:styled-content style=\"fixed-case\">VLCADD<\/jats:styled-content> but is presented in an emulsion containing very long\u2010chain fatty acids which can cause organ lipidosis and itself can inhibit mitochondrial fatty acid metabolism. It is therefore not recommended. Suxamethonium\u2010induced myalgia may mimic symptoms of rhabdomyolysis and cause raised <jats:styled-content style=\"fixed-case\">CK<\/jats:styled-content> therefore should be avoided. Opioids, <jats:styled-content style=\"fixed-case\">NSAIDS<\/jats:styled-content>, regional anesthesia, and local anesthetic techniques have all been used without complication.<\/jats:p>","DOI":"10.1111\/pan.12474","type":"journal-article","created":{"date-parts":[[2014,7,29]],"date-time":"2014-07-29T11:30:42Z","timestamp":1406633442000},"page":"1115-1119","source":"Crossref","is-referenced-by-count":18,"title":["Anesthetic agents in patients with very long\u2010chain acyl\u2010coenzyme A dehydrogenase deficiency: a literature review"],"prefix":"10.1111","volume":"24","author":[{"given":"Charlotte","family":"Redshaw","sequence":"first","affiliation":[{"name":"Department of Anaesthesia Princess Royal Hospital Telford UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Catherine","family":"Stewart","sequence":"additional","affiliation":[{"name":"Department of Anaesthesia Princess Royal Hospital Telford UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2014,7,29]]},"reference":[{"key":"e_1_2_5_2_1","unstructured":"LeslieND TinkleBT StraussAWet\u00a0al.Very long chain acyl\u2010coenzyme A dehydrogenase deficiency. 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