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 neu...

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Main Authors: Anna Tanaka, Tim Cai, Michael Platten, Luis E. Tollinche, Samuel J. DeJoy
Format: Article
Language:English
Published: Wiley 2024-01-01
Series:Case Reports in Anesthesiology
Online Access:http://dx.doi.org/10.1155/2024/1050279
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author Anna Tanaka
Tim Cai
Michael Platten
Luis E. Tollinche
Samuel J. DeJoy
author_facet Anna Tanaka
Tim Cai
Michael Platten
Luis E. Tollinche
Samuel J. DeJoy
author_sort Anna Tanaka
collection DOAJ
description 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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spelling doaj-art-99c702df00294525baf6d6197f4fe6862025-02-03T01:29:50ZengWileyCase Reports in Anesthesiology2090-63902024-01-01202410.1155/2024/1050279Anesthetic Management and Neuromonitoring in a Patient with Very Long-Chain Acyl-Coenzyme A Dehydrogenase Deficiency Undergoing Scoliosis Surgery: A Case Report and Review of LiteratureAnna Tanaka0Tim Cai1Michael Platten2Luis E. Tollinche3Samuel J. DeJoy4Case Western Reserve University School of MedicineDepartment of AnesthesiologyDepartment of AnesthesiologyDepartment of AnesthesiologyDepartment of AnesthesiologyPatients 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.http://dx.doi.org/10.1155/2024/1050279
spellingShingle Anna Tanaka
Tim Cai
Michael Platten
Luis E. Tollinche
Samuel J. DeJoy
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
Case Reports in Anesthesiology
title 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
title_full 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
title_fullStr 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
title_full_unstemmed 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
title_short 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
title_sort 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
url http://dx.doi.org/10.1155/2024/1050279
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