Case report | Volume 7, Article 12, 17 Nov 2021 | 10.11604/pamj-cm.2021.7.12.31045

Malignant hyperthermia revealed by cardiac arrest after paediatric sevoflurane induction: a case report

Anouar Jarraya, Manel Kammoun, Bilel Louati, Saloua Ammar, Hsairi Manel, Loulik Mouna, Kamel Kolsi

Corresponding author: Anouar Jarraya, Anesthesiology Department, Hedi Chaker University Hospital, Sfax, Tunisia

Received: 03 Aug 2021 - Accepted: 30 Sep 2021 - Published: 17 Nov 2021

Domain: Intensive care medicine

Keywords: Malignant hyperthermia, sevoflurane, cardiac arrest, pediatric anesthesia, case report

©Anouar Jarraya et al PAMJ - Clinical Medicine (ISSN: 2707-2797). This is an Open Access article distributed under the terms of the Creative Commons Attribution International 4.0 License (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Cite this article: Anouar Jarraya et al . Malignant hyperthermia revealed by cardiac arrest after paediatric sevoflurane induction: a case report. PAMJ - Clinical Medicine. 2021;7:12. [doi: 10.11604/pamj-cm.2021.7.12.31045]

Available online at: https://www.clinical-medicine.panafrican-med-journal.com/content/article/7/12/full

Home | Volume 7 | Article number 12

Case report

Malignant hyperthermia revealed by cardiac arrest after paediatric sevoflurane induction: a case report

Malignant hyperthermia revealed by cardiac arrest after paediatric sevoflurane induction: a case report

Anouar Jarraya1,&, Manel Kammoun1, Bilel Louati1, Saloua Ammar², Hsairi Manel3, Loulik Mouna3, Kamel Kolsi1

 

1Anesthesiology Department, Hedi Chaker University Hospital, Sfax, Tunisia, ²Pediatric Surgery Department, Hedi Chaker University Hospital, Sfax, Tunisia, 3Pediatric Department, Hedi Chaker University Hospital, Sfax, Tunisia

 

 

&Corresponding author
Anouar Jarraya, Anesthesiology Department, Hedi Chaker University Hospital, Sfax, Tunisia

 

 

Abstract

Malignant hyperthermia (MH) is a rare and severe pharmacogenetic disorder triggered by volatile anesthetics and succinylcholine. Clinical symptoms of MH are highly variable. Classic clinical symptoms of MH include rapidly increasing hyperthermia, hypercarbia, sinus tachycardia, masseter spasm with acidosis and hyperkalemia. We report a case of a one year boy scheduled for inguinal hernia who presented a MH revealed by a cardiac arrest immediately after sevoflurane administration which is uncommon. After successful cardiac arrest resuscitation, the patient had tachycardia with inadequate tachypnea and hypercarbia. Few hours later, he presented hyperthermia up to 40°C, severe rhabdomyolysis and acidosis with hyperkalemia and renal failure. MH was suspected. To confirm the diagnosis, muscular biopsy was done and showed muscular dystrophy and genetic mutations in ryanodine receptor type 1 (RYR1) was tested positive. Our patient had symptomatic treatment but Dantrolene is not available in our country. The patient presented persistent hyperthermia, with severe metabolic disorders and died 72 hours later.

 

 

Introduction    Down

Malignant hyperthermia (MH) is a rare and severe pharmacogenetic disorder of skeletal muscle [1] that manifests clinically as a hypercatabolic crisis caused by volatile anesthetic or succinylcholine exposure [1]. The prevalence is about 1/100 000 administered anesthetics in general population [2] but its mortality remains high (10% to 70%) [2].Classic clinical symptoms of MH include hypercarbia, tachycardia, masseter contraction and hyperthermia [1]. Severe cardiac arrhythmia leading to cardiac arrest may be seen later [3], but the occurrence of cardiac arrest with asystole immediately after sevoflurane administration is original. We describe the case of one-year boy who presented immediate cardiac asystole with delayed classic symptoms of MH after sevoflurane exposure.

 

 

Patient and observation Up    Down

Patient information: we present a case report of a 14 months boy scheduled for inguinal hernia cure. The patient had no co-morbid condition with any past history of surgery or any previous anesthetic exposure. However, the patient had good psychomotor development and acquired walk at the age of one year. Our patient had correct preoperative hemostasis blood analysis. On the day of surgery, preoperative temperature was 37°C and starvation instructions were respected.

 

Clinical findings: in the operation room, a basic monitoring was attached to the patient before inhalatory anesthesia induction. Blood pressure was 105/50mmHg, heart rate 112 beats/min and oxygen saturation were 100%. Anesthesia was induced with progressive doses of sevoflurane (3% then 6%) with 100% of oxygen. A 24 gauge venous cannula was put in place. The patient presented immediately a severe bradycardia 30b/min and untaken oxygen saturation and capnography. We stopped any anesthesia drug administration, we ventilated the patient with adequate facial mask with pure oxygen, a bolus of 250μg of adrenaline was given and the bradycardia was corrected instantly. Then, 60mg of propofol (5mh/kg) and 250μg of Alfentanil (20μg/kg) were given and we intubated the patient to secure the airway with size 3.5 ID cuffed polyvinyl endotrachial tube and connected to ventilator for protective ventilation. The patient was stable with correct hemodynamic status (HR = 122b/min, BNIP = 100/49mmHg) and oxygen saturation (99%). The central temperature was 37.5°C. No rising trend in end tidal CO² was noted (34 mmHg). Two minutes later, we noted a sudden cardiac arrest with asystole and skin mottling.

We begin external cardiac massage and manual ventilation with 100% oxygen and adrenaline administration (250 μg/bolus/2min then continuous adrenaline infusion 1000μg/h). The cardiac resuscitation lasted 25 minutes, then, a cardiac activity was noted with tachycardia up to 160b/min, with a high blood pressure 132/80 mmHg that allowed the progressive withdrawal of adrenalin infusion. A rising trend in end tidal CO² was noticed (40 mmHg). A small rise in temperature with maximum 38°C was recorded by nasopharyngeal probe.

Two hours after, we tried to awake the patient but he was always unconscious and developed a tachycardia with 180 b/min and tachypnea 50c/min, so a propofol infusion (10mg/kg/h) was restarted, and the patient was referred to the Pediatric Intensive Care Unit (PICU). In the PICU, 5 hours after sevoflurane exposure, the patient had increasing hyperthermia up to 40°C, tachycardia up to 200/min, polypnea (70/min) and 99% oxygen saturation. Active cooling was started. Blood sample showed severe acidosis (pH 7.22 and lactate: 3.8mmol/l) with hypercarbia (63mmHg) hyperkalemia (5.8 mEq/L) and rahabdomyolysis and renal failure (creatine phosphokinase (CPK) up to 2936 and creatinine:104μmol/L).

Diagnostic assessments: to confirm the diagnosis of MH, muscular biopsy was done and showed muscular dystrophy. Genetic mutations in ryanodine receptor type 1 (RYR1) were tested positive. MH was confirmed.

Therapeutic interventions: the patient was referred to the intensive care unit for to monitor the temperature, blood pressure, heart rate, breathing and response to treatment. We opted to symptomatic treatment by body cooling and mechanical ventilation with high concentration of oxygen. The patient gets extra fluids through a central IV line. The collapsus was treated by saline co-load and noradrenaline infusion. However, Dantrolene, drug of choice for malignant hyperthermia that acts by stopping the release of calcium into the muscle, is unavailable in our country.

Follow-up and outcomes: the patient developed multivisceral failure and died 72 hours later. All family members of the patient were made aware of suspected diagnosis of malignant hyperthermia and risks of recurrence in other family members on future exposure to anesthesia.

 

 

Discussion Up    Down

Malignant hyperthermia (MH) is a potentially fatal pharmacogenetic disease [4]. The initial signs of MH generally occur soon after triggering agents´ exposure or at any time during anaesthesia maintenance and rarely few hours later [4]. The first signs of MH are hypercarbia, sinus tachycardia and masseter spasm [4]. However, peaked T waves and arrhythmias may occur later due to acute hyperkalemia. In addition, the youngest children have small muscular mass that may explain that the metabolic disorders may occur later [4]. In our case, these severe disorders appeared within 2 minutes and caused cardiac asystole immediately which seems to be original and which make the diagnosis of malignant hyperthermia difficult. Using clinical grading scale to assess the likelihood of a MH may be useful in these cases [5]. This scale will gather different clinical and biological elements in favor of MH and take consideration to related diseases like muscular dystrophy [5,6].

As the majority of reported cases are caused by mutations in two genes: RYR1 gene (ryanodine receptor type 1) and CACNA1S gene (voltage-dependent L type calcium channel alpha 1S subunit), genetic study can help in the diagnosis and prevent similar accidents in the family [7]. While invitro contracture test remains the gold standard for malignant hyperthermia susceptibility (MHS) diagnosis [8]. Symptomatic resuscitation of MH according to the recent guidelines is mandatory [9], and Dantrolene remains the drug of choice for this disease. It must be introduced as soon as the diagnosis is suspected. Since its introduction, MH mortality has fallen from 80% to less than 5% [10]. Its mechanism of action is to bind to ryanodine receptors and block calcium release causing muscle relaxation [10], unfortunately, this drug was not available in our country and the evolution was fatal for our patient in spite of resuscitation.

 

 

Conclusion Up    Down

The presented case highlights that MH is a rare but severe pharmacogenetic disorder that manifestations may vary. Classic signs of MH were delayed few hours after sevoflurane exposure and the immediate signs were cardiac asystole which is original. The treatment of MH is based on symptomatic resuscitation but also Dantrolene administration that should be present in all anesthesiology departments.

 

 

Competing interests Up    Down

The authors declare no competing interests.

 

 

Authors' contributions Up    Down

Anouar Jarraya and Bilel Louati contributed to the redaction of the manuscript. Manel Kammoun contributed to the correction of the manuscript. Saloua Ammar contributed to data collection. Hsairi Manel and Loulik Mouna contributed to the medical resuscitation and was in charge the patient.

 

 

References Up    Down

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Case report

Malignant hyperthermia revealed by cardiac arrest after paediatric sevoflurane induction: a case report

Case report

Malignant hyperthermia revealed by cardiac arrest after paediatric sevoflurane induction: a case report

Case report

Malignant hyperthermia revealed by cardiac arrest after paediatric sevoflurane induction: a case report

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Key words

Malignant hyperthermia

Sevoflurane

Cardiac arrest

Pediatric anesthesia

Case report

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