Volatile anesthesia for a pediatric patient with very long‐chain acyl‐coenzyme A dehydrogenase deficiency: A case report

Hess, Aaron S.; Rice, Gregory M.; Jochman, John; Muldowney, Bridget L.

doi:10.1111/pan.13314

Cited by 5 publications

(4 citation statements)

References 3 publications

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“…Volatile anesthetics could not be used for the majority of the case due to its inhibitory effect on the anterior horn cells of the spinal cord that preclude neuromonitoring. Despite previous conflicting recommendations due to concerns for rhabdomyolysis with volatile anesthetic use in VLCADD patients [ 12 ], recent literature reports and the review by Redshaw and Stewart have demonstrated that inhaled anesthetics are safe to use in VLCADD patients and were safely used at the end of our case as well [ 4 , 5 , 13 , 14 ]. The few existing reports on managing patients with VLCADD recommend strict intraoperative monitoring of glucose and serum CK levels along with continuous glucose infusions to prevent hypoglycemia [ 12 , 14 ].…”

Section: Discussionmentioning

confidence: 87%

Anesthetic Management and Neuromonitoring in a Patient with Very Long-Chain Acyl-Coenzyme A Dehydrogenase Deficiency Undergoing Scoliosis Surgery: A Case Report and Review of Literature

Tanaka,

Cai,

Platten

et al. 2024

Case Reports in Anesthesiology

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Patients with very long-chain acyl-CoA dehydrogenase deficiency (VLCADD) are prone to hypoglycemia and clinical decompensation when metabolic demands of the body are not met. We present a pediatric patient with VLCADD who underwent a posterior spinal fusion for scoliosis requiring intraoperative neurophysiology monitoring. Challenges included minimization of perioperative metabolic stressors and careful selection of anesthetic agents since propofol-based total intravenous anesthesia (TIVA) was contraindicated due to its high fatty acid content. This case is unique due to the sequential use of inhaled anesthetics after TIVA to allow for a rapid wakeup and immediate postoperative physical exam. Additionally, intraoperative neuromonitoring in the setting of VLCADD has not been reported in the literature. With communication among anesthesia, surgery, and neuromonitoring teams before and during the operation, the patient successfully underwent a major surgery without complications. This trial is registered with NCT03808077.

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Section: Discussionmentioning

confidence: 87%

Anesthetic Management and Neuromonitoring in a Patient with Very Long-Chain Acyl-Coenzyme A Dehydrogenase Deficiency Undergoing Scoliosis Surgery: A Case Report and Review of Literature

Tanaka,

Cai,

Platten

et al. 2024

Case Reports in Anesthesiology

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“…Considering that propofol is dissolved in 10% intralipid containing 100% long-chain fatty acid, it should be avoided in patients with carnitine deficiency or defects in the fatty acid β-oxidation pathway. This is because propofol increases long-chain fatty acid loading in patients with compromised fatty acid β-oxidation [25,29] . Thus, we surmised that only 6 (18.75%) of the 32 surgeries used propofol as an induction or maintenance agent of general anesthesia (Table 1).…”

Section: Discussionmentioning

confidence: 99%

“…This is because propofol increases long-chain fatty acid loading in patients with compromised fatty acid β-oxidation. [ 25 , 29 ] Thus, we surmised that only 6 (18.75%) of the 32 surgeries used propofol as an induction or maintenance agent of general anesthesia (Table 1 ). [ 7 , 12 , 13 , 20 , 28 , 30 ] Furthermore, the probability of the propofol infusion syndrome increased upon the intravenous administration of propofol (>4 mg/kg/h) for more than 48 hours.…”

Section: Discussionmentioning

confidence: 99%

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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“…Surgical procedures require perioperative management, including prolonged fasting, physical burden, and certain anesthetics that may include the use of lipid emulsions, which can lead to metabolic derangements [ 6 ]. Although there have been reports that focus on perioperative management of patients with VLCADD in adulthood [ 7 , 8 ], anesthesia of infant patients with VLCADD has rarely been reported [ 6 , 9 ]. Furthermore, the perioperative use of some medications is controversial.…”

Section: Introductionmentioning

confidence: 99%

The perioperative transition of serum biomarkers of a 1.5-year-old boy with very-long-chain acyl-CoA dehydrogenase deficiency

Awano

Yamada

et al. 2021

Molecular Genetics and Metabolism Reports

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Very long-chain acyl-coenzyme A dehydrogenase deficiency (VLCADD, OMIM 201475 ) is a congenital fatty acid oxidation disorder. Individuals with VLCADD should avoid catabolic states, including strenuous exercise and long-term fasting; however, such conditions are required when undergoing surgery. The perioperative management of VLCADD in infants has rarely been reported and details regarding the transition of serum biomarkers reflecting catabolic status have not been disclosed. Herein, we present the perioperative clinical and biological data of cryptorchidism in a 1.5-year-old boy with VLCADD. The patient was diagnosed through newborn screening and his clinical course was very stable. Genetic testing of ACADVL revealed compound heterozygous variants c.506 T > C (p.Met169Thr) and c.606-609delC (p.L216*). The enzyme activity of the patient with VLCAD was only 20% compared to that of healthy control. Left orchiopexy for the pediatric cryptorchidism was planned and performed at 1 and a half year of age. Induction anesthesia involved thiopental, fentanyl and rocuronium. The glucose infusion rate was maintained above 6.6 mg/kg/min starting the day before surgery until the operation was completed. Anesthesia was maintained with sevoflurane at approximately 2%. The serum concentration of tetradecenoylcarnitine were stable during the operation, ranging between 0.08 and 0.19 μM (cutoff <0.2 μM), and never deviated from the reference range. Concentration of other serum biomarkers including free fatty acid, 3-OH-butyrate, and creatine kinase, remained similarly unchanged. In this report, we describe the uneventful perioperative management of unilateral orchiopexy for left cryptorchidism in a 1.5-year-old boy with VLCADD using sufficient glucose infusion and volatile anesthesia.

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Volatile anesthesia for a pediatric patient with very long‐chain acyl‐coenzyme A dehydrogenase deficiency: A case report

Cited by 5 publications

References 3 publications

Anesthetic Management and Neuromonitoring in a Patient with Very Long-Chain Acyl-Coenzyme A Dehydrogenase Deficiency Undergoing Scoliosis Surgery: A Case Report and Review of Literature

Anesthetic Management and Neuromonitoring in a Patient with Very Long-Chain Acyl-Coenzyme A Dehydrogenase Deficiency Undergoing Scoliosis Surgery: A Case Report and Review of Literature

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

The perioperative transition of serum biomarkers of a 1.5-year-old boy with very-long-chain acyl-CoA dehydrogenase deficiency

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