Perioperative measures in very long chain acyl-CoA dehydrogenase deficiency

Vellekoop, P.; Diekman, Eugène F.; Tuijl, I. van; Vries, M.M.C. de; Hasselt, Peter M. van; Visser, Gepke

doi:10.1016/j.ymgme.2011.01.010

Cited by 15 publications

(25 citation statements)

References 9 publications

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“…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.…”

Section: Principles Of Perioperative Managementmentioning

confidence: 99%

“…According to several authors, volatile agents should be avoided, because they are associated with a significant rise of free fatty acid concentrations and this could lead to metabolic derangement (Leslie et al 1993;Steiner et al 2002;Vellekoop et al 2011). However, the assumption is based on the study of Kleeman et al, who investigated plasma concentrations of free fatty acids as the marker of catabolism secondary to stress at multiple points during surgery in a small cohort.…”

Section: Volatile Agentsmentioning

confidence: 99%

“…Another mentioned risk of volatile agents is malignant hyperthermia, which could cause rhabdomyolysis (Fierobe et al 1998;McKenney and Holman 2002;Vellekoop et al 2011;Rosenberg et al 2015). Malignant hyperthermia is a hereditary disorder that is characterized by a rise of body temperature (1 C/5 min) and can be fatal if not timely detected and treated (Vanholder et al 2000;Rosenberg et al 2015).…”

Section: Volatile Agentsmentioning

confidence: 99%

“…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.…”

Section: Propofolmentioning

confidence: 99%

“…Additionally, cases demonstrating the uneventful use of propofol have been described as well (Vellekoop et al 2011;Martin et al 2014;Redshaw and Stewart 2014). Most likely, induction with propofol will not lead to immediate problems when sufficient glucose is provided (explained below) and its use in VLCADD is therefore only relatively contraindicated.…”

Section: Propofolmentioning

confidence: 99%

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

et al. 2016

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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.

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Section: Principles Of Perioperative Managementmentioning

confidence: 99%

Section: Volatile Agentsmentioning

confidence: 99%