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Anesthetic Management of a Child with Family History of Malignant Hyperthermia: Case Report

Received: 27 August 2013     Published: 10 November 2013
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Abstract

Malignant hyperthermia (MH) is a rare and autosomal dominant myopathy triggered by volatile anesthetics and depolarizing neuromuscular blocking agents such as succinylcholine and is characterized by an acute hypermetabolic clinical state. This report presents the importance of preoperative preparation and perioperative management of patients with a family history of MH, in which there is no possibility of a diagnostic confirmation. Attention must be directed to the preparation of the anesthetic machine because modern workstations need longer cleansing times than their predecessors.Case Report: A two-year-old male child, weighing 16 kg, with a family history of MH, confirmed by muscular biopsy, underwent an elective umbilical and unilateral inguinal hernioplasty, and postectomy under intravenous general anesthesia associated with a caudal block. The preanesthetic care involves the preparation of the surgical environment and assessment of possible perioperative events. The patient's exhaled CO2 fraction and body temperature were continuously monitored throughout the surgery and immediate postoperative period. The patient recovered without further events and was discharged from the hospital after two days.In a patient with family history of MH the administration of intravenous general anesthesia with the adequate preparation of the surgical environment allowed safe anesthetic management for the proposed surgical procedure.

Published in Journal of Anesthesiology (Volume 1, Issue 3)
DOI 10.11648/j.ja.20130103.11
Page(s) 21-23
Creative Commons

This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.

Copyright

Copyright © The Author(s), 2013. Published by Science Publishing Group

Keywords

Malignant Hyperthermia, Anesthetic Management, Anesthetic Machine

References
[1] Hopkins PM – Malignant hyperthermia: advances in clinical management and diagnosis. Br J Anaesth, 2000; 85:118.
[2] Larach MG, Gronert GA, Allen GC, Brandom BW, Lehman EB – Clinical Presentation, Treatment, and Complications of Malignant Hyperthermia in North America from 1987 to 2006. Anesth Analg, 2010; 110:498-507.
[3] Denborough MA, Lovell RRH – Anaesthetic deaths in a family. Lancet, 1960; 2:45.
[4] Allen GC, Brubaker CL – Human malignant hyperthermia associated with desflurane anesthesia. Anesth Analg, 1998; 86:1328-1331.
[5] Ranklev-Twetman E – Malignant hyperthermia: the clinical syndrome. Acta Anaesthesiol Belg, 1990; 41(2):79-82.
[6] Britt B, Orkin FK, Cooperman LH – Complications in Anesthesiology. Philadelphia: JB Lippingcott, 1993; 291-313.
[7] European Malignant Hyperpyrexia Group: A protocol for the investigation of malignant hyperpyrexia. Br J Anaesth, 1984; 56:1267
[8] Kim TW, Nemergut ME – Preparation of Modern Anesthesia Workstations for Malignant Hyperthermia-susceptible Patients. Anesthesiology, 2011; 114:205-212.
[9] Schonell LH, Sims C, Bulsara M – Preparing a new generation anaesthetic machine for patients susceptible to malignant hyperthermia. Anaesth Intensive Care, 2003; 31:58-62.
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  • APA Style

    Tiago Freire da Fonte, Lucas Gianni Menegaz, Marcos Lopes de Miranda, Rodrigo Montebello de Araújo, Carlos Darcy Alves Bersot. (2013). Anesthetic Management of a Child with Family History of Malignant Hyperthermia: Case Report. International Journal of Anesthesia and Clinical Medicine, 1(3), 21-23. https://doi.org/10.11648/j.ja.20130103.11

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    ACS Style

    Tiago Freire da Fonte; Lucas Gianni Menegaz; Marcos Lopes de Miranda; Rodrigo Montebello de Araújo; Carlos Darcy Alves Bersot. Anesthetic Management of a Child with Family History of Malignant Hyperthermia: Case Report. Int. J. Anesth. Clin. Med. 2013, 1(3), 21-23. doi: 10.11648/j.ja.20130103.11

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    AMA Style

    Tiago Freire da Fonte, Lucas Gianni Menegaz, Marcos Lopes de Miranda, Rodrigo Montebello de Araújo, Carlos Darcy Alves Bersot. Anesthetic Management of a Child with Family History of Malignant Hyperthermia: Case Report. Int J Anesth Clin Med. 2013;1(3):21-23. doi: 10.11648/j.ja.20130103.11

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  • @article{10.11648/j.ja.20130103.11,
      author = {Tiago Freire da Fonte and Lucas Gianni Menegaz and Marcos Lopes de Miranda and Rodrigo Montebello de Araújo and Carlos Darcy Alves Bersot},
      title = {Anesthetic Management of a Child with Family History of Malignant Hyperthermia: Case Report},
      journal = {International Journal of Anesthesia and Clinical Medicine},
      volume = {1},
      number = {3},
      pages = {21-23},
      doi = {10.11648/j.ja.20130103.11},
      url = {https://doi.org/10.11648/j.ja.20130103.11},
      eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ja.20130103.11},
      abstract = {Malignant hyperthermia (MH) is a rare and autosomal dominant myopathy triggered by volatile anesthetics and depolarizing neuromuscular blocking agents such as succinylcholine and is characterized by an acute hypermetabolic clinical state. This report presents the importance of preoperative preparation and perioperative management of patients with a family history of MH, in which there is no possibility of a diagnostic confirmation. Attention must be directed to the preparation of the anesthetic machine because modern workstations need longer cleansing times than their predecessors.Case Report: A two-year-old male child, weighing 16 kg, with a family history of MH, confirmed by muscular biopsy, underwent an elective umbilical and unilateral inguinal hernioplasty, and postectomy under intravenous general anesthesia associated with a caudal block. The preanesthetic care involves the preparation of the surgical environment and assessment of possible perioperative events. The patient's exhaled CO2 fraction and body temperature were continuously monitored throughout the surgery and immediate postoperative period. The patient recovered without further events and was discharged from the hospital after two days.In a patient with family history of MH the administration of intravenous general anesthesia with the adequate preparation of the surgical environment allowed safe anesthetic management for the proposed surgical procedure.},
     year = {2013}
    }
    

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    AU  - Tiago Freire da Fonte
    AU  - Lucas Gianni Menegaz
    AU  - Marcos Lopes de Miranda
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    JF  - International Journal of Anesthesia and Clinical Medicine
    JO  - International Journal of Anesthesia and Clinical Medicine
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    PB  - Science Publishing Group
    SN  - 2997-2698
    UR  - https://doi.org/10.11648/j.ja.20130103.11
    AB  - Malignant hyperthermia (MH) is a rare and autosomal dominant myopathy triggered by volatile anesthetics and depolarizing neuromuscular blocking agents such as succinylcholine and is characterized by an acute hypermetabolic clinical state. This report presents the importance of preoperative preparation and perioperative management of patients with a family history of MH, in which there is no possibility of a diagnostic confirmation. Attention must be directed to the preparation of the anesthetic machine because modern workstations need longer cleansing times than their predecessors.Case Report: A two-year-old male child, weighing 16 kg, with a family history of MH, confirmed by muscular biopsy, underwent an elective umbilical and unilateral inguinal hernioplasty, and postectomy under intravenous general anesthesia associated with a caudal block. The preanesthetic care involves the preparation of the surgical environment and assessment of possible perioperative events. The patient's exhaled CO2 fraction and body temperature were continuously monitored throughout the surgery and immediate postoperative period. The patient recovered without further events and was discharged from the hospital after two days.In a patient with family history of MH the administration of intravenous general anesthesia with the adequate preparation of the surgical environment allowed safe anesthetic management for the proposed surgical procedure.
    VL  - 1
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    ER  - 

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Author Information
  • Department of Anesthesia, Hospital Federal da Lagoa, Rio de Janeiro, Brazil

  • Department of Anesthesia, Hospital Federal da Lagoa, Rio de Janeiro, Brazil

  • Department of Anesthesia, Hospital Federal da Lagoa, Rio de Janeiro, Brazil

  • Department of Anesthesia, Hospital Federal da Lagoa, Rio de Janeiro, Brazil

  • Department of Anesthesia, Hospital Federal da Lagoa, Rio de Janeiro, Brazil

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