Anesthetic management of a patient with mitochondrial encephalopathy, lactic acidosis and stroke-like symptoms (MELAS): a case study

  • Annabelle Lea Hicks Flanagan, DNP, CRNA Texas Christian University

Abstract

Introduction: Mitochondrial encephalopathy, lactic acidosis, stroke-like symptoms syndrome (MELAS) is a specific mitochondrial myopathy (MM) that results in defects in respiratory enzyme complexes I and IV. This ultimately causes aerobic metabolism defects especially in high energy requiring organs, leading to an obvious concern with anesthetic management. Some mitochondrial myopathies have speculative linkages to malignant hyperthermia.  This case report examines the anesthetic implications for a patient with MELAS.

Case Summary: A 39-year-old male with MELAS underwent a right hip fracture pinning.  The patient was managed with a successful general endotracheal anesthetic using a propofol infusion and < 1 MAC of sevoflurane gas. The patient was extubated without complications. 

Discussion: Anesthetic implications for the management of MELAS include a tailored preoperative assessment because of the different phenotypical presentations of mitochondrial diseases.  Avoidance of lactate in intravenous fluids, hypothermia, prolonged fasting, and postoperative nausea vomiting, all can decrease the metabolic burden in MELAS patients. Varied anesthetic techniques have been used in multiple case reports. Careful titration of muscle relaxants and opioids is paramount to prevent postoperative respiratory failure. According to the Malignant Hyperthermia Association of the United States, avoidance of volatile anesthetics is not necessary in MM patients.

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Published
2016-06-08
How to Cite
FLANAGAN, DNP, CRNA, Annabelle Lea Hicks. Anesthetic management of a patient with mitochondrial encephalopathy, lactic acidosis and stroke-like symptoms (MELAS): a case study. Anesthesia eJournal, [S.l.], v. 4, n. 1, june 2016. ISSN 2333-2611. Available at: <http://www.anesthesiaejournal.com/index.php/aej/article/view/33>. Date accessed: 13 dec. 2018.
Section
Articles

Keywords

MELAS, mitochondrial disorders, anesthetic management, mitochondrial myopathy