DP17: EEG-MONITORED TIVA FOR BILATERAL MYRINGOTOMY IN A VENEZUELAN CHILD WITH TRISOMY 21: BALANCING SAFETY AND EMERGING GENETIC CONCERNS
Joshua R Stanton, DO1; Ellen Basile, DO2
1Westside Anesthesia Residency Program; 2Nemours Children's Hospital
Introduction/Background: Children with trisomy twenty-one present unique anesthetic challenges including airway anomalies, hypotonia, developmental delay, and potential cardiac comorbidities. Preoperative assessment of pediatric patients also includes inquiry regarding prior anesthetic exposure and familial anesthetic complications. Recent updated joint communications from the American Society of Anesthesiologists (ASA) and the Society for Pediatric Anesthesia (SPA) describe rare but severe neurologic complications and deaths after routine general anesthesia in some adult and pediatric patients of Venezuelan ancestry. These reports have prompted increased vigilance, although insufficient data exist to mandate changes in anesthetic practice based solely on ancestry. Emerging information suggests mitochondrial genetic susceptibility specifically mitochondrial NADH dehydrogenase 4 (ND4) gene mutations such as mtND4 m.11232T>C may impair complex I oxidative phosphorylation and increase vulnerability to anesthetic neurotoxicity. This evolving safety signal creates uncertainty in anesthetic selection and depth management for affected populations.
Methods: A 5-year-old male with trisomy twenty-one and Venezuelan descent, presented for bilateral myringotomy with ear tube placement, auditory evoked potential (AEP) threshold estimation (multiple frequencies), for hearing assessment. The anesthetic plan included peripheral intravenous (IV) placement followed by total intravenous anesthesia (TIVA). Standard ASA monitoring was applied, with processed electroencephalographic (EEG) monitoring using Masimo SedLine®. Masimo SedLine® monitoring was used to titrate anesthetic depth and assessed for burst suppression. Nitrous oxide with oxygen was administered with intranasal dexmedetomidine to facilitate IV placement. A laryngeal mask airway (LMA) was placed atraumatically. Maintenance anesthesia was transitioned to TIVA with a propofol infusion. Multimodal analgesia included intravenous acetaminophen, ketorolac, ondansetron, and dexamethasone. Hemodynamic and respiratory parameters remained stable throughout the procedure.
Results: Intraoperative Masimo SedLine® monitoring demonstrated stable cortical activity without burst suppression or excessive anesthetic depth. No arrhythmias, metabolic instability, or unexpected anesthetic requirements were observed. Emergence was smooth, and the LMA was removed when the patient was awake with intact airway reflexes. In the post-anesthesia care unit, the patient returned to baseline neurocognitive function without delayed emergence or focal neurologic deficits.
Discussion/Conclusion: This case demonstrates safe anesthetic management of a pediatric patient with trisomy twenty-one and Venezuelan ancestry. While ASA/SPA communications acknowledge rare neurologic complications in some Venezuelan patients, causation remains unproven and no definitive practice changes are currently recommended. This case report highlights the ongoing discussion surrounding perioperative and intraoperative considerations in light of emerging recommendations regarding patients of Venezuelan descent. A propofol-based TIVA approach with EEG-guided titration avoided excessive anesthetic depth and resulted in full neurologic recovery. Anticipation of IV access challenges in trisomy twenty-one, individualized anesthetic planning, and careful depth monitoring remain paramount as research continues to clarify potential mitochondrial anesthetic interactions.
