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.

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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Copyright © 2024 Anna Tanaka et al.

Déclaration de conflit d'intérêts

Author LET is a grant recipient through Merck Investigator Studies Program (MISP) to fund clinical trial at MSKCC (NCT03808077). LET serves a consultancy and advisory role for Merck & Co. Pharmaceutical Company. LET serves a consultancy and advisory role for GE Healthcare. LET receives stipend for his role as examiner with The American Board of Anesthesiology. LET is an expert testimony witness and receives compensation for this role. The remaining authors declare that they have no conflicts of interest regarding the publication of this article.