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.

Very Long-Chain Acyl-Coenzyme A Dehydrogenase Deficiency and Perioperative Management in Adult Patients

“…Preoperative Care Stress prior to surgery is a known trigger for metabolic derangement (Vellekoop et al 2011;Redshaw and Stewart 2014). Preventing stress with the use of premedication could be effective.…”

“…The use of propofol in patients with VLCADD is controversial (Leslie et al 1993;Steiner et al 2002;Vellekoop et al 2011;Redshaw and Stewart 2014). Propofol is dissolved in a solution containing 0.1 g fat/ml.…”

“…During a one-hour procedure, the minimum dosage fat within propofol may equate a sixth of the daily fat allowance of an adult. Since VLCADD patients may have a fat restricted diet to prevent organ lipidosis and the accumulation of fat oxidation intermediates, it is recommended to avoid the use of propofol, to improve long-term morbidity and mortality (Redshaw and Stewart 2014).…”

“…There is evidence of more general mitochondrial dysfunction in these disorders, including generation of reactive oxygen species and calcium imbalance (Wajner and Amaral 2015). During catabolic circumstances such as prolonged fasting, stress, illness, and surgery, metabolic derangement can occur, resulting in hypoglycemia, myopathies (including cardiomyopathy), metabolic acidosis, and rhabdomyolysis (Leslie et al 1993;Redshaw and Stewart 2014). Current treatment consists of avoiding catabolism with regular feedings and in some patients a restriction of long chain fatty acids, supplementation of medium chain triglycerides, and frequent carbohydrate intake to prevent activation of fatty acid metabolism (Arnold et al 2009;Das et al 2010).…”

“…In patients with a very long-chain acyl-CoA dehydrogenase deficiency (VLCADD; EC # OMIM201475) the enzyme responsible for one of the first steps in the metabolism of fatty acids is deficient (Leslie et al 1993;Redshaw and Stewart 2014). VLCADD is an autosomal recessive d i s o r d e r w i t h a n e s t i m a t e d p r e v a l e n c e o f 1:31,500-1:85,000 (Lindner et al 2010;Arnold et al 2009).…”