Anesthetic implications of Leigh's syndrome.
Leigh's syndrome (LS) is a mitochondrial disorder characterized by progressive neurodegenerative changes with loss of developmental milestones, abnormalities of central control of respiration, and metabolic derangements. The primary genetic defect involves the respiratory chain complex and pyruvate dehydrogenase complex resulting in abnormal mitochondrial function and defective oxidative phosphorylation. While most patients have respiratory and neurological impairment, involvement of the cardiovascular and musculoskeletal systems may also occur. The authors present the use of spinal anesthesia for muscle biopsy in a 19 month old with LS and review the potential anesthetic implications of this syndrome.
Shear
Torin
T
University of Missouri School of Medicine, Columbia, MO 65212, USA.
Tobias
Joseph D
JD
D000775:Anesthesia, Spinal / Q000379:methods*; D000779:Anesthetics, Local / Q000627:therapeutic use; D001706:Biopsy / Q000379:methods; D001993:Bronchodilator Agents / Q000627:therapeutic use; D004837:Epinephrine / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007223:Infant; D007888:Leigh Disease / Q000601:surgery*; D008012:Lidocaine / Q000627:therapeutic use; D000077442:Lidocaine, Prilocaine Drug Combination; D016343:Monitoring, Intraoperative / Q000379:methods; D009132:Muscles / Q000473:pathology; D011318:Prilocaine / Q000627:therapeutic use; D013748:Tetracaine / Q000627:therapeutic use
D000779:Anesthetics, Local; D001993:Bronchodilator Agents; D000077442:Lidocaine, Prilocaine Drug Combination; D011318:Prilocaine; D013748:Tetracaine; D008012:Lidocaine; D004837:Epinephrine