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.

Take-Home Message

Despite the conflicting literature addressing the perioperative management in patients with VLCADD (very long-chain acyl-CoA dehydrogenase deficiency), volatile agents can be used safely if other important precautions are provided, such as an adequate glucose infusion and the minimization of the fasting period and surgical stress, taking into account the severity of the mutation.

• Author contributions: All authors contributed to the writing and editing of the manuscript:

  • Contribution of the individual authors:

  • MM Welsink-Karssies: Data collection, reviewing the available literature and writing a first draft of the manuscript, and processing the adjustments of the other authors

  • JAW Polderman: data collection, critically reviewing the manuscript

  • EJ Nieveen van Dijkum: surgeon performing the procedure, critically reviewing the manuscript

  • BP Preckel: critically reviewing the manuscript

  • WS Schlack: critically reviewing the manuscript, data collection

  • G Visser: reviewing the available literature, critically reviewing the manuscript

  • CE Hollak: reviewing the available literature, critically reviewing the manuscript, data collection, and treating metabolic specialist of the patient

  • J Hermanides: anesthesiologist during the procedure, reviewing the available literature, critically reviewing the manuscript, and data collection

  • J. Hermanides serves as guarantor for the article, accepts full responsibility for the work and/or the conduct of the study, had access to the data, and controlled the decision to publish.

  • The conflict of interest form of the authors is included in the online submission.

  • The authors hereby confirm independence from the sponsors and state that the content of the article has not been influenced by the sponsors.

  • The informed consent of the patient is available.

  • The authors hereby state that an ethics approval was not required.

  • Keywords: VLCADD (very-long-chain-acyl-CoA dehydrogenase deficiency), perioperative management, metabolic derangement, volatile agents, propofol, adults.