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Table of Contents
CASE REPORT
Year : 2019  |  Volume : 3  |  Issue : 3  |  Page : 178-180

Perioperative management in a malignant hyperthermia susceptible patient


1 Department of Anesthesiology and Intensive Care, Medical Faculty, Universitas Indonesia-Cipto Mangunkusumo Hospital, Jakarta, Indonesia
2 Department of Orthopedic and Traumatology, Medical Faculty, Universitas Indonesia-Cipto Mangunkusumo Hospital, Jakarta, Indonesia

Date of Submission05-Nov-2019
Date of Acceptance05-Dec-2019
Date of Web Publication23-Jan-2020

Correspondence Address:
Dr. Fildza Sasri Peddyandhari
Department of Anesthesiology and Intensive Care, Medical Faculty, Universitas Indonesia/Cipto Mangunkusumo Hospital, Jl. Salemba Raya 5, Jakarta 10430
Indonesia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/BJOA.BJOA_2_19

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  Abstract 


Malignant hyperthermia (MH) is a rare genetic disorder. Susceptible one could suffer fatal condition if the patient is exposed to triggers. MH can be seen in many general anesthesia procedures using volatile anesthetics. In susceptible patients who will be undergoing surgery, the most important is to prevent intraoperative MH despite overcoming the crisis. This case will discuss an elective spine surgery performed in a patient with a family history of MH.

Keywords: Anesthesia, complication, family, malignant hyperthermia, risk


How to cite this article:
Soenarto RF, Auerkari AN, Peddyandhari FS, Lunaesti C, Rahyussalim AJ. Perioperative management in a malignant hyperthermia susceptible patient. Bali J Anaesthesiol 2019;3:178-80

How to cite this URL:
Soenarto RF, Auerkari AN, Peddyandhari FS, Lunaesti C, Rahyussalim AJ. Perioperative management in a malignant hyperthermia susceptible patient. Bali J Anaesthesiol [serial online] 2019 [cited 2020 May 25];3:178-80. Available from: http://www.bjoaonline.com/text.asp?2019/3/3/178/276623




  Introduction Top


Malignant hyperthermia (MH) is a dominant, autosomal inherited disorder. There are approximately 1:50,000 up to 1:100,000 incidences.[1] This disorder impacts all races worldwide, and the greater incidence is among young adults. This disorder involves skeletal muscle. Ryanodine receptor was activated by agents that will cause excessive Ca 2+ efflux to the skeletal muscle cytosol and cause cell hypercontraction.[1] Many widely known literature show that this disorder can be caused by volatile anesthetics, depolarizing muscle relaxant, caffeine, and m-chlorocresol.

This life-threatening condition will cause a metabolic crisis in skeletal muscle and can be occurred during intraoperative period until after the emergence. The early signs are muscle rigidity, tachycardia, hypercarbia, and elevated body temperature (1°C of core body temperature could be rise for every 5 min).[1],[2] Despite low incidence, MH crisis can cause serious morbidity if not well managed. Proper diagnosis and early rapid management could decrease the mortality rate to 1.4%.[3],[4] In susceptible patients, the most important thing to do is to prevent the crisis from happening not only intra-anesthesia but also preanesthesia period.


  Case Report Top


A 16-year-old girl diagnosed with adolescent idiopathic scoliosis (Lenke Classification 5B) was scheduled for scoliosis corrective procedure. Physical status was concluded as the American Society of Anesthesiologists 2 with MH susceptible. Back pain was the only complaint of this patient. She has not experienced any surgery, but four of her family members, including one of her siblings, had MH crisis intraoperative; therefore, their surgeries were canceled. They are inherited from the same paternal side [Figure 1]. For this reason, we believed this patient as “MH susceptible.”
Figure 1: Family tree assessment

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A day before the surgery, our anesthesia team ensured that all the management elements are available, including operating theater (OT) preparation. The vaporizer was disconnected from the anesthesia machine, 100% oxygen was flushed, and CO2 absorber was replaced with the new absorber. These were to dispose all volatile anesthetics. Other preparations include end-tidal CO2 monitoring, the availability of both sterile and nonsterile ice packs for surface cooling, and gastric lavage. Above all, we have to be sure that dantrolene is available.

According to the MH Association of the United States recommendation,[5] flushing of 100% oxygen 10 l/min is mandatory. Despite performing measures to ensure the remaining volatile agents were flushed, the anesthesia team has to be sure that backup oxygen source, resuscitation bag, and another free-volatile-anesthetic oxygen source are on hand.

All involved OT personnel (anesthetist, nurse, surgeon, and OT management) already have a simulation drill about managing the MH crisis. The simulation includes emergency system activation, dantrolene preparation, and surface cooling among other drills regarding the acute management of MH. Other OT staffs have to be trained for preparing the ice packs for surface cooling and also cold-sterile water for gastric lavage.

On the day of surgery, total intravenous anesthesia (TIVA) was performed. After the administration of 100 mcg of fentanyl, we induced the patient with 60 mg of propofol, followed by 60 mg of rocuronium for facilitating endotracheal intubation. A nasogastric tube was inserted after intubation along with an oropharyngeal airway. The anesthesia was maintained by target-controlled infusion of propofol at 1.5–3.5 mcg/mL and a continuous dose of fentanyl at 50–150 mcg/h. Intraoperative anesthesia depth was monitored using the bispectral index (BIS) with a target 40–60. The surgery lasted went for 4 h, and the anesthesia lasted for around 6 h and 25 min. Hemodynamic was stable, and there were no signs of the MH crisis intraoperatively.

The patient then admitted to the intensive care unit (ICU) right after the surgery, and she was monitored for 24 h. Hemodynamic monitoring and body temperature were maintained in a normal range. Both surgery and postsurgery treatments were uneventful.


  Discussion Top


Despite the genetic disorders' rarity, MH can be fatal. The mortality of MH can be as high as 70%.[6] As this disorder is inherited in the autosomal dominant pattern, each child of an MH parent has a 50% chance of inheriting.[1]

MH diagnosis is initially based on the clinical parameters during the crisis. To confirm, a muscle biopsy is needed.[6],[7],[8] Caffeine-halothane contracture test (CHCT) is the current gold standard for the MH diagnosis. There is yet any chromosomal test to replace CHCT. In our case, the patient has no history of previous anesthesia. All family members have not yet undergone any diagnostic tests to confirm MH. However, since there is a history of MH crisis in the family, we considered this patient as an MH suspect. Overdiagnosis is much safer than underdiagnosis in patients with a family history of MH crisis.

Establishing a strong suspicion of MH has direct consequences. The patient should be treated as an MH patient, and preoperative preparation should be made with this in mind. Given its rarity, the anesthesia and surgical team should be briefed about MH and its crisis management. To prepare, we conduct a short course for the assigned anesthesia team for the case. Protocols to prevent and address the MH crisis are already available at the time of the surgery but should be re-socialized. The more health-care practitioners trained to handle the MH crisis, the better; however, as time is limited, we can only provide the training for the anesthesia team. If time permits, the course should be expected to nurse and or management team to anticipate crises. For example, a special code for MH can be used to alert and activate the anesthesia and surgical team during an MH crisis.

Most MH crises are associated with the use of volatile agents during general anesthesia.[1] The incidence varies from 1:4,500 to 1:60,000 during general anesthesia.[8] The onset of clinical symptoms in this disorder can occur in an acute and fulminant fashion, or even late. MH crisis can take place at any time during surgery and was even reported as late as 24 h postsurgery.[8] Early signs of an MH crisis are tachycardia, tachypnea, hypercarbia, and masseter rigidity. Core temperature increases in 15 min after exposure to a precipitating agent. Rapid saturation of CO2 absorber can be one of the signs of MH crisis.[8] Therefore, it is important that in this case, we ensure the use of a well-functioning capnograph and thermometer. We also inserted a nasogastric tube despite it is not for routine use in spine surgery. The tube is inserted as preparation for cold saline gastric lavage if needed. After intubation, the oropharyngeal airway device is inserted to prevent a kink in the endotracheal tube if masseter rigidity was to occur.

An early detection of signs and symptoms is a crucial part of MH crisis management. The short course that we prepared addresses the early detection as one of the most important parts of MH management. If the MH crises were to occur, it is imperative that the precipitating factor is stopped and complications are managed.[2] Dantrolene can reduce mortality from 70% to<5%.[8] In the short course, dantrolene preparation and administration are covered. A mock drug, using instant drink powder and 100 ml glass bottle, was used to simulate dantrolene preparation in the course.

TIVA combined with a nondepolarizing muscle relaxant is the commonly chosen anesthesia technique for patients with a suspicion of MH. Volatile anesthetic agents and depolarizing muscle relaxants are two well-known precipitators for the MH crisis. It is imperative to ensure that the anesthesia circuit is completely free from volatile agents. Therefore, in preparation, the vaporizer of the anesthesia machine of the assigned surgical theatre is removed 1 day prior to surgery. Removing the vaporizer can prevent anesthesia personnel from accidentally opening the vaporizer dial.[9]

During surgery, the depth of anesthesia is maintained with a BIS monitor with a target range of 40–60. Analgesia is also important to monitor, as pain can activate the sympathetic system and misdiagnose as an MH crisis. In this case, we assess the adequacy of analgesia from clinical signs. Ideally, if available, the analgesia monitor can be used. Body temperature should be closely monitored throughout the surgery. Even though an increase in temperature can be slow, it is important to be vigilant for the increase in temperature. As patients tend to be hypothermic during surgery, an increase in temperature should raise suspicion. During intraoperative monitoring, there is no sign of the MH crisis in this patient. We continue to closely monitor the patient in the ICU for 24 h to anticipate for late MH crisis. The patient was doing well throughout his postoperative period.


  Conclusion Top


All patients with suspicion of MH can safely undergo anesthesia and surgery. Preoperative preparation is an important part of perioperative management in MH. All anesthesia personnel should be trained to anticipate and address the MH crisis. Knowledge in pathophysiology and management of MH should be well known by all members of the anesthesia team. Written protocols should be well socialized, and perioperative MH crisis simulation should be conducted regularly to ensure team aptitude. There is no such thing as being overly prepared when ensuring patient safety.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Rosenberg H, Pollock N, Schiemann A, Bulger T, Stowell K. Malignant hyperthermia: A review. Orphanet J Rare Dis 2015;10:93.  Back to cited text no. 1
    
2.
Kim DC. Malignant hyperthermia. Korean J Anesthesiol 2012;63:391-401.  Back to cited text no. 2
    
3.
Osman BM, Saba IC, Watson WA. A case report of suspected malignant hyperthermia: How will the diagnosis affect a patient's insurability? Case Rep Anesthesiol 2018;2018:6532821.  Back to cited text no. 3
    
4.
Lee YS, Kim WY, Lee SH, Baek SM, Ok SJ, Kim JH, et al. A case of malignant hyperthermia during anesthesia induction with sevoflurane -A case report-. Korean J Anesthesiol 2010;59 Suppl:S6-8.  Back to cited text no. 4
    
5.
Preparation of Anesthesia Workstations to Anesthetize MH Susceptible Patients. MHAUS. Available from: https://www.mhaus.org/healthcare-professionals/mhaus-recommendations/preparation-of-anesthesia-workstations-to-anesthetize-mh-susceptible-patients/. [Last accessed on 2019 Mar 13].  Back to cited text no. 5
    
6.
Sinha AK, Kumari P, Vaghela MM, Sinha C, Kumar B. Postoperative malignant hyperthermia-A medical emergency: A case report and review of literature. J Clin Diagn Res 2017;11:PD01-2.  Back to cited text no. 6
    
7.
Iqbal A, Badoo S, Naqeeb R. A case report of suspected malignant hyperthermia where patient survived the episode. Saudi J Anaesth 2017;11:232-5.  Back to cited text no. 7
    
8.
Ferhi F, Dardour L, Tej A, Kebaili R, M'aaref S, Jazia KB. Malignant hyperthermia in a 4-year-old girl during anesthesia induction with sevoflurane and succinylcholine for congenital ptosis surgery. Saudi J Ophthalmol 2019;33:183-7.  Back to cited text no. 8
    
9.
Kim TW, Nemergut ME. Preparation of modern anesthesia workstations for malignant hyperthermia-susceptible patients: A review of past and present practice. Anesthesiology 2011;114:205-12.  Back to cited text no. 9
    


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