Anesthetic management for a patient with medium-chain acyl-CoA dehydrogenase deficiency in an outpatient setting using ketamine and fentanyl

Prabhu, Shamit S.; Khan, Saad; Doudnikoff, Alexander L.; Mueller, Nicholas; Reebye, Uday N.

doi:10.1016/j.jclinane.2019.01.031

Cited by 2 publications

(1 citation statement)

References 5 publications

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“…Various anesthetic agents were used, including midazolam, propofol, thiopental, and sevoflurane. [6][7][8][9][10] The present report is the first case to describe the use of remimazolam for a laparoscopic percutaneous extraperitoneal closure for an inguinal hernia in a pediatric patient with MCAD deficiency.…”

Section: Discussionmentioning

confidence: 81%

A Remimazolam and Remifentanil Anesthetic for a Pediatric Patient With a Medium-Chain Acyl-CoA Dehydrogenase Deficiency: A Case Report

et al. 2022

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Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency is one of the most common fatty acid oxidation disorders. The choice of anesthetics and blood glucose management are crucial to prevent metabolic decompensation. A 5-year-old Japanese boy with MCAD deficiency was scheduled to undergo surgery for an inguinal hernia. Glucose was continuously infused perioperatively, and his glucose concentrations were within the normal range. Anesthesia was induced and maintained with remimazolam, remifentanil, and intermittent rocuronium. No metabolic decompensation was observed. This case indicates the importance of a continuous intravenous glucose infusion, and that remimazolam can be the first-line anesthetic for a patient with MCAD deficiency. (A&A Practice. 2022;16:e01646.) GLOSSARY BIS = bispectral index; GA = general anesthesia; MCAD = medium-chain acyl-CoA dehydrogenase; PACU = postanesthesia care unit; PONV = postoperative nausea and vomiting

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Section: Discussionmentioning

confidence: 81%

A Remimazolam and Remifentanil Anesthetic for a Pediatric Patient With a Medium-Chain Acyl-CoA Dehydrogenase Deficiency: A Case Report

et al. 2022

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Anesthetic management of patients with carnitine deficiency or a defect of the fatty acid β-oxidation pathway

Kim

et al. 2022

Medicine

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Carnitine is essential for the transport of long-chain fatty acids from the cytoplasm to the mitochondrial matrix. The carnitine shuttle transports long-chain fatty acylcarnitine to the mitochondrial matrix. Subsequently, long-chain fatty acyl CoA, which is split from long-chain fatty acylcarnitine by carnitine palmitoyltransferase II, undergoes fatty acid β-oxidation. Acetyl CoA is produced from long-chain fatty acyl CoA via fatty acid β-oxidation and aids in the synthesis of adenosine triphosphate via the tricarboxylic acid cycle and electron transport chain. In addition, in the fasting state, it leads to ketone body production in the liver and glucose production via gluconeogenesis. However, patients with compromised fatty acid β-oxidation, owing to carnitine deficiency as well as defects in carnitine transport and the fatty acid β-oxidation pathway, develop hypoglycemia, cardiomyopathy, arrhythmia, and hypotonia. These conditions are attributed to the accumulation of released fatty acids and acylcarnitine. This review aimed to shed light on the anesthetic management of patients with compromised fatty acid β-oxidation undergoing various surgeries by assessing relevant case reports associated with fatty acid β-oxidation disorder in PubMed. Pre-anesthetic and intraoperative evaluation should include monitoring of glucose and carnitine levels and specific cardiac tests, such as echocardiography. Considering that propofol is dissolved in 10% long-chain fatty acids, propofol infusion should be avoided because of increased long-chain fatty acid loading in patients with compromised fatty acid β-oxidation. Thus, anesthesia using opioids (remifentanil and fentanyl), midazolam, dexmedetomidine, etomidate, and non-depolarizing neuromuscular blocking agents would be appropriate in such patients.

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Anesthetic management for a patient with medium-chain acyl-CoA dehydrogenase deficiency in an outpatient setting using ketamine and fentanyl

Cited by 2 publications

References 5 publications

A Remimazolam and Remifentanil Anesthetic for a Pediatric Patient With a Medium-Chain Acyl-CoA Dehydrogenase Deficiency: A Case Report

A Remimazolam and Remifentanil Anesthetic for a Pediatric Patient With a Medium-Chain Acyl-CoA Dehydrogenase Deficiency: A Case Report

Anesthetic management of patients with carnitine deficiency or a defect of the fatty acid β-oxidation pathway

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