Abstract
Monoamine oxidase (MAO) deficiency is an X-linked hereditary disease characterized by spontaneous deletion of MAO-A and/or MAO-B on the X chromosome. Here, we describe the first reported case of a patient with MAO-A and MAO-B deficiency managed under general anesthesia in dental treatment. The patient was aged 11 years old when he was scheduled for dental treatment. He was diagnosed with MAO-A and MAO-B deficiency on genetic testing at 2 years of age. He was not given premedication, and standard monitoring with noninvasive blood pressure monitoring, pulse oximetry, and ECG was instituted. We also preemptively prepared a cardioverter-defibrillator. General anesthesia was induced with propofol 46 mg (2 mg/kg), then rocuronium 10 mg (0.4 mg/kg) and remifentanil 0.30 μg/kg/min were administered via separate infusion pumps. Orotracheal intubation was performed without complications. Anesthesia was maintained uneventfully with a continuous infusion of remifentanil 0.15–0.2 μg/kg/min and propofol 5.0–7.0 mg/kg. Fresh gas flow included oxygen and air. End-tidal CO2 concentration was maintained at around 35 mmHg throughout the procedure. We administered sugammadex 92 mg (4 mg/kg) for reversal of neuromuscular blockade and the patient was extubated. We achieved successful anesthetic management without any appreciable clinical signs of fatal arrhythmias in this patient with MAO-A and MAO-B deficiency.
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Whibley A, Urquhart J, Dore J, Willatt L, Parkin G, Gaunt L, Black G, Donnai D, Raymond FL. Deletion of MAOA and MAOB in a male patient causes severe developmental delay, intermittent hypotonia and stereotypical hand movements. Eur J Hum Genet. 2010;18(10):1095–9.
Saito M, Yamagata T, Matsumoto A, Shiba Y, Nagashima M, Taniguchi S, Jimbo E, Momoi MY. MAOA/B deletion syndrome in male siblings with severe developmental delay and sudden loss of muscle tonus. Brain Dev. 2014;36(1):64–9.
Collins FA, Murphy DL, Reiss AL, Sims KB, Lewis JG, Freund L, Karoum F, Zhu D, Maumenee IH, Antonarakis SE. Clinical, biochemical, and neuropsychiatric evaluation of a patient with a contiguous gene syndrome due to a microdeletion Xp113 including the Norrie disease locus and monoamine oxidase (MAOA and MAOB) genes. Am J Med Genet. 1992;42(1):127–34.
Lenders JW, Eisenhofer G, Abeling NG, Berger W, Murphy DL, Konings CH, Wagemakers LM, Kopin IJ, Karoum F, van Gennip AH, Brunner HG. Specific genetic deficiencies of the A and B isoenzymes of monoamine oxidase are characterized by distinct neurochemical and clinical phenotypes. J Clin Invest. 1996;97(4):1010–9.
Hayashi Y, Sumikawa K, Tashiro C, Yamatodani A, Yoshiya I. Arrhythmogenic threshold of epinephrine during sevoflurane, enflurane, and isoflurane anesthesia in dogs. Anesthesiology. 1988;69(1):145–7.
Minami K, Yanagihara N, Segawa K, Tsutsui M, Shigematsu A, Izumi F. Inhibitory effects of propofol on catecholamine secretion and uptake in cultured bovine adrenal medullary cells. Naunyn Schmiedebergs Arch Pharmacol. 1996;353(5):572–8.
Watanabe K, Kashiwagi K, Kamiyama T, Yamamoto M, Fukunaga M, Inada E, Kamiyama Y. High-dose remifentanil suppresses stress response associated with pneumoperitoneum during laparoscopic colectomy. J Anesth. 2014;28(3):334–40.
Korczyn AD, Teplitsky I. Arrhythmogenic effect of epinephrine. J Cardiovasc Pharmacol. 1984;6(1):206–7.
Sonntagbauer M, Koch A, Strouhal U, Zacharowski K, Weber CF. Catecholamine crisis during induction of general anesthesia: a case report. Anaesthesist. 2018;67(3):209–15.
Holschneider DP, Scremin OU, Huynh L, Chen K, Seif I, Shih JC. Regional cerebral cortical activation in monoamine oxidase A-deficient mice: differential effects of chronic versus acute elevations in serotonin and norepinephrine. Neuroscience. 2000;101(4):869–77.
Scremin OU, Holschneider DP, Chen K, Li MG, Shih JC. Cerebral cortical blood flow maps are reorganized in MAOB-deficient mice. Brain Res. 1999;824(1):36–44.
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Hoshijima, H., Takeuchi, R., Kikuchi, K. et al. Anesthetic management in MAO-A and MAO-B deficiency: a case report. J Anesth 34, 773–776 (2020). https://doi.org/10.1007/s00540-020-02808-5
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DOI: https://doi.org/10.1007/s00540-020-02808-5