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.