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

Abstract

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

Remimazolam as an Adjunct to General Anesthesia in Children: Adverse Events and Outcomes in a Large Cohort of 418 Cases

“…Without pediatric approval, the off-label use of remimazolam for general anesthesia has been limited to published accounts of case reports and small series [ 4 , 5 , 6 , 7 , 16 ]. At our institution, remimazolam has been approved for routine pediatric anesthesia use since 2020.…”

“…To date, there are no published pediatric studies that report a broad experience with remimazolam when administered as part of a general anesthetic. With only approval for adult administration, the off-label experience in the pediatric population has been limited to case reports only [ 4 , 5 , 6 , 7 ]. At our hospital, remimazolam is approved for off-label use as an adjunct to general anesthesia.…”

Clinical Application and Research Progress of Remimazolam for Pediatric Patients

“…Studies of adults have shown that remimazolam and its antagonist flumazenil have no significant effects on fatty acid metabolism and are good options for anesthesia management in children with muscular dystrophy and medium-chain acyl-CoA dehydrogenase (MCAD) deficiency. [30][31][32] In conclusion, remimazolam is suitable for patients who need MEP monitoring and avoid inhaled anaesthetics.…”

“…Flumazenil can quickly reverse the hypnotic effect of remimazolam, shorten the postoperative extubation time in children, allow spontaneous breathing after failed intubation and avoid delayed awakening in children with congenital metabolic diseases, which improves the safety and controllability of remimazolam anesthesia. 32 , 37 , 41 The FDA-approved doses of remimazolam for benzodiazepine reversal when used for pediatric patients (children 1 year and older and adolescents) receiving conscious sedation or general anesthesia are the initial dose of 0.01 mg/kg given over 15 seconds (up to a maximum dose of 0.2 mg). If no desired return of consciousness is noted after 45 seconds, repeat 0.01 mg/kg (up to 0.2 mg) at 1-minute intervals as needed.…”

“…To date, most clinical studies of children have administered remimazolam according to the recommended dose for adults and adjusted the dosing rate according to the BIS value. 24 , 32 , 39 The BIS, however, may be insufficient due to the lack of age-related covariates for assessing the extent of sedation under remimazolam. 40 A study 23 showed that the modified observer’s assessment of alertness/sedation (MOAA/S) scale may be the best index for optimal indice assessing the extent of sedation under remimazolam in adults, but the accuracy remains to be proven in children.…”