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

Abstract

Surgery and anesthesia pose a threat to patients with very long-chain acyl-CoA dehydrogenase deficiency (VLCADD), because prolonged fasting, stress, and pain are known risk factors for the induction of metabolic derangement. The optimal perioperative management in these patients is unknown and the use of volatile agents and agents dissolved in fatty acids has been related to postoperative metabolic complications. However, the occurrence of metabolic derangement is multifactorial and depends, amongst others, on the severity of the mutation and residual enzyme activity. Current guidelines suggest avoiding both volatile anesthetics as well as propofol, which seriously limits the options for providing safe anesthesia. Therefore, we reviewed the available literature on the perioperative management of patients with VLCADD. We concluded that the use of some medications, such as volatile anesthetics, in patients with VLCADD might be wrongfully avoided and could in fact prevent metabolic derangement by the adequate suppression of pain and stress during surgery. We will illustrate this with a case report of an adult VLCADD patient undergoing minor surgery. Besides the use of remifentanil, anesthesia was uneventfully maintained with the use of sevoflurane, a volatile agent, and continuous glucose infusion. The patient was monitored with a continuous glucose meter and creatinine kinase measurements.

The perioperative transition of serum biomarkers of a 1.5-year-old boy with very-long-chain acyl-CoA dehydrogenase deficiency

“…Similarly, it has been reported that volatile anesthetics are associated with significant increases in FFA concentrations during the first phases of anesthesia, which in the past has resulted in the development of rhabdomyolysis [ 6 ]. Our results are consistent with the results of recent publications that reported relatively safe volatile agents [ 7 , 8 ].…”

“…Propofol includes lipid emulsions; and therefore, it can impair mitochondrial entry of long-chain fatty acids and inhibit the respiratory chain at several points [ 6 , 8 ]. However, there are case reports demonstrating the uneventful use of propofol in individuals with VLCADD and other fatty acid oxidation disorders [ 7 , 19 ]. This makes the use of propofol in patients with VLCADD controversial.…”

“…Surgical procedures require perioperative management, including prolonged fasting, physical burden, and certain anesthetics that may include the use of lipid emulsions, which can lead to metabolic derangements [ 6 ]. Although there have been reports that focus on perioperative management of patients with VLCADD in adulthood [ 7 , 8 ], anesthesia of infant patients with VLCADD has rarely been reported [ 6 , 9 ]. Furthermore, the perioperative use of some medications is controversial.…”

Anesthesia management in a patient with very long-chain acyl-Coenzyme A dehydrogenase deficiency

“…As for anesthetics, benzodiazepines, barbiturates, nondepolarizing muscle relaxants, and opioids have been previously used without problems [6,[11][12][13]. Propofol was not selected in the present case because it contains long-chain fatty acids in the solvent [6,13]. There have been conflicting results of using inhaled anesthetics for patients with VLCAD deficiency.…”

“…Kleemann et al [15] measured plasma concentration of free fatty acids as a marker of catabolism secondary to stress during enflurane anesthesia in patients without VLCAD deficiency and reported that free fatty acids transiently increased around tracheal intubation, but decreased 10 min following the start of enflurane and intraoperatively, suggesting that enflurane anesthesia could suppress stress-induced catabolism [15]. Almost all papers demonstrated that inhaled anesthetics were safely used for patients with VLCAD deficiency [6,13,16]. Iwata et al [16] reported an intraoperative lactate increase in a patient with VLCAD deficiency.…”