Case Report Open Access
Malignant Hyperthermia: A Case Report
M Lazraq1*, A Dafir1, A Bensaid1, Y Miloudi1 and N Alharrar1
1Doctor in anesthesia-reanimation, 20 August Hospital, 1953, Ibn Rochd UHC of Casablanca, Morocco
*Corresponding author: Dr. Lazraq Mohamed, Intensive Care Unit, 20 August Hospital, 20370, Hospital District, Casablanca, Morocco Email: @
Received: June 17, 2019; Accepted: July 12, 2019; Published: July 16, 2019
Citation: Lazraq M, Dafir A, Bensaid A, et al. (2019) Malignant Hyperthermia: A Case Report. J Emerg Crit Care Diagn Manag 2(1):1-2.
AbstractTop
Malignant hyperthermia is a rare but very serious complication with high mortality and the treatment is based on the early administration of dantrolene sodium. We report the case of an 8-year-old child who underwent surgery for an eyeball burst and had a very severe malignant hyperthermia attack. The child unfortunately died in the absence of availability of dantrolene.

Keywords: Malignant Hyperthermia; Dantrolene Sodium
IntroductionTop
Malignant hyperthermia (MH) is a rare but potentially serious complication, defined as an abnormal response to halogenated anesthetic agents and / or depolarizing curare in individuals with a genetic abnormality affecting skeletal striated muscle [1].

This pharmacogenetic disorder is generally related to mutations in the receptor calcium channel of ryanodine (RyR1) in the sarcoplasmic reticulum of skeletal muscle [1].

MH remains a very rare accident (incidence reported between 1/10 000 - 1/250 000 of general anesthesia) but still very serious because its morbidity remains surprisingly high, around 20% and rises more than 30% if dantrolene sodium is not administered within 20 minutes of the first signs of the attack. In the absence of dantrolene mortality exceeds 80% [2].
ObservationTop
This is a male child, 8 years old, with a history of insulindependent diabetes since the age of 5, admitted to ophthalmic emergencies for an eyeball burst following a stone-throwing injury. Before the anesthetic induction, the patient had clinical parameters following: a weight of 25 kg, blood pressure at 110/60 mmHg, heart rate at 88 beats / minute, respiratory rate at 16 cycles / minute, SpO2 at 97% at the same time, temperature at 38.3 °C for which he received 325mg intravenous paracetamol, capillary glycemia at 1.3 g / l.

Anesthetic induction was performed with Fentanyl 3 μg / kg (75 μg), Propofol 4 mg / kg (100 mg) and 0.6 mg / kg of Rocuronium (15 mg); followed by an orotracheal intubation. Anesthetic maintenance was maintained by FiO2 = 50%, Air 50%, Isoflurane at 0.8 MAC.

The patient’s body temperature at first was normal as well as his blood pressure and SpO2, the heart rate ranged between 95 to 120 beats / min. The CO2 teletype pressure (ETCO2) was initially normal (ETCO2 between 30 and 34 mmHg).

At the end of the procedure (after 1 hour 30 of induction), the patient presented a rapidly increasing hypercapnia (exceeding 100 mmHg), sinus tachycardia followed with muscle rigidity and major hyperthermia (T> 41 ° C). Unfortunately dantrolene sodium was not available and the child died despite all the resuscitation measures administered.
DiscussionTop
Malignant hyperthermia is a rare complication and most often without previous pathological clinical expression. It has an autosomal dominant inheritance with 50% risk for children to inherit the causal mutation of MH.

Absence of a history of MH does not eliminate the risk, even with previous exposure to triggering anesthetics [3]. MH risk screening should be systematic in anesthesia consultation based on the interview because patients are most often asymptomatic. For patients at risk, it is recommended to specify the diagnosis of MH sensitivity by "In Vitro Contracture Tests (IVCT)" on muscle biopsy (halothane and caffeine contracture tests) or by genetic DNA analysis extracted from the peripheral blood.

This test searches ryanodine receptor (RYR1) genes present on chromosome 19q13 [4] and α1 subunit dihydropyridine receptor (CACNA1S), which correspond to genes coding for the two calcium channels responsible for the coupling between excitation and contraction muscle [5]. Indeed, IVCT sensitivity is greater (99%) than DNA analysis (50%).

Apart patients with a personal or family history of MH, the other MH risk situations that must be detected during anesthesia consultation are mainly congenital myopathies with cores associated with RYR1 gene, unexplained chronic elevation of CPK which may be an indicator of congenital myopathy leading to MH risk.

Patients on statin therapy should be questioned about their CPK [6-7]; Muscular dystrophies, the most common of which are Duchenne and Becker’s diseases, affect striated muscle, cardiac muscle and sometimes smooth muscle. Membrane fragility of muscle of these patients, marked by chronic rhabdomyolysis, leads to a risk of cardiac arrest by hyperkalemia in the event of exposure to a halogen and / or succinylcholine [8].

Severe stress hyperthermia or severe stress rhabdomyolysis also present a risk of MH [9]. It should be noted that the antecedents of neuroleptic malignant syndrome do not constitute a risk situation for anesthetic MH [10].

In our case, the patient had never received general anesthesia and there was no family history suggestive of MH.

General anesthesia for patients at risk must respect three absolute principles: exclusion of all halogenated volatile anesthetic agents as well as the depolarizing curare (suxamethonium); have monitoring of capnography and central temperature; and finally checking the availability of dantrolene sodium. A preoperative and early postoperative CPK dosage is recommended for patient follow-up [1].

In the event of a HM attack, treatment should be started as soon as the diagnosis is made based on immediate halting of administration of the halogenated agent and immediate intravenous injection of dantrolene. Dantrolene is administered at a dose of 2.5 mg / kg IVD, repeated up to 10 mg / kg (dose of 1 mg / kg every 10 minutes), maintenance for 48 hours at the dose of 4mg / kg / day [1].

It is also recommended to assemble elements for the diagnosis: anesthesia card, measure blood levels of potassium, muscle enzymes and myoglobinuria (after the intervention and 12 to 24 hours later) without forgetting to inform the patient and his family about the attach [1].

The assessment carried out for our patient showed the presence of a major hyperkalemia (> 8 mEq / l), with very high muscle enzymes (CPK, transaminases and myoglobin).
ConclusionTop
Although malignant hyperthermia is a rare perioperative complication, due to its seriousness and unpredictability, it is essential that dantrolene sodium be included in emergency medications that are mandatory in all operating rooms.
ReferencesTop
  1. Renée Krivosic-Horber, Yves Nivoche, Joel Lunardi, Jean-François Payen, Anne-Frédérique Dalmas, Nicole Monnier, et al. Expert Recommendations for the Risk of MALIGNANT HYPERTHERMIA in Anesthesia Resuscitation. 2013.
  2. Riazi S, Larach MG, Hu C, Wijeysundera D, Massey C, Kraeva N. Malignant hyperthermia in Canada: characteristics of index anesthetics in 129 malignant hyperthermia susceptible probands. Anesth Analg. 2014;118(2):381-387. doi: 10.1213/ANE.0b013e3182937d8b
  3. Bendixen D, Skovgaard LT, Ording H. Analysis of anaesthesia in patients suspected to be susceptible to malignant hyperthermia before diagnostic in vitro contracture test. Acta Anaesthesiol Scand. 1997;41(4):480-484.
  4. Healy JM, Lehane M, Heffron JJ, Farrell M, Johnson K, McCarthy TV. Localisation of the malignant hyperthermia susceptibility locus to human chromosome 19q12-q13.2. Biochem Soc Trans. 1990;18(2):326.
  5. Monnier N, Procaccio V, Stieglitz P, Lunardi J. Malignant-hyperthermia susceptibility is associated with a mutation of the α1-subunit of the human dihydropyridine-sensitive L-type voltage-dependent calcium-channel receptor in skeletal muscle. Am J Hum Genet. 1997;60(6):1316-1325.
  6. Krivosic R, Krivosic I. Myopathie à axe central (Central core disease) associée à une sensibilité à l'hyperthermie maligne. Presse Med. 1989;18:828-831.
  7. Krivosic-Horber R, Dépret T, Wagner JM, Maurage CA. Malignant hyperthermia susceptibility revealed by increased serum creatine kinase concentrations during statin treatment. Eur J Anaesthesiol. 2004;21(7):572-574.
  8. Larach MG, Rosenberg H, Gronert GA, Allen GC. Hyperkalemic cardiac arrest during anesthesia in infants and children with occult myopathies. Clin Pediatr (Phila). 1997;36(1):9-16.
  9. Wappler F, Fiege M, Steinfath M, Agarwal K, Scholz J, Singh S, et al. Evidence for susceptibility to malignant hyperthermia in patients with exercise-induced rhabdomyolysis. Anesthesiology. 2001;94(1):95-100.
  10. Adnet PJ, Krivosic-Horber RM, Adamantidis MM, Haudecoeur G, Adnet-Bonte CA, Saulnier F, et al. The association between the neuroleptic malignant syndrome and malignant hyperthermia. Acta Anaesthesiol Scand. 1989;33(8):676-680.
 
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