title: 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.
authors: Department of Anesthesiology, Academic Medical Centre, University of Amsterdam, Meibergdreef 9, 1105 AZ, Amsterdam, The Netherlands.
authors: M M
authors: MM
authors: Welsink-Karssies
authors: Department of Anesthesiology, Academic Medical Centre, University of Amsterdam, Meibergdreef 9, 1105 AZ, Amsterdam, The Netherlands. j.a.polderman@amc.nl.
authors: J A W
authors: JAW
authors: Polderman
authors: Department of Surgery, Academic Medical Centre, Amsterdam, The Netherlands.
authors: E J
authors: EJ
authors: Nieveen van Dijkum
authors: Department of Anesthesiology, Academic Medical Centre, University of Amsterdam, Meibergdreef 9, 1105 AZ, Amsterdam, The Netherlands.
authors: B
authors: B
authors: Preckel
authors: Department of Anesthesiology, Academic Medical Centre, University of Amsterdam, Meibergdreef 9, 1105 AZ, Amsterdam, The Netherlands.
authors: W S
authors: WS
authors: Schlack
authors: Department of Metabolic Diseases, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, The Netherlands.
authors: G
authors: G
authors: Visser
authors: Division of Endocrinology and Metabolism, Department of Internal Medicine, Academic Medical Centre, Amsterdam, The Netherlands.
authors: C E
authors: CE
authors: Hollak
authors: Department of Anesthesiology, Academic Medical Centre, University of Amsterdam, Meibergdreef 9, 1105 AZ, Amsterdam, The Netherlands.
authors: J
authors: J
authors: Hermanides
keywords: Adults; Metabolic derangement; Perioperative management; Propofol; VLCADD (very long-chain acyl-CoA dehydrogenase deficiency); Volatile agents