Sana Shah Taizoon Dhoon Yeyoon Choi George Wang Govind Rajan

Keywords: RYR1 gene, Dantrolene, General Anesthesia, Pediatric Anesthesia, Anesthesia, Malignant Hyperthermia

Abstract

Malignant hyperthermia (MH) is a rare, life-threatening pharmacogenetic disorder triggered by exposure to volatile anesthetics and succinylcholine. It results from mutations in the RYR1 gene, leading to excessive intracellular calcium release, hypermetabolism, and potential multiorgan dysfunction. Prompt recognition and treatment are essential to reduce morbidity and mortality.

We report the case of an 11-year-old female undergoing pancreaticoduodenectomy who developed intraoperative MH following sevoflurane exposure. Early signs included rising end-tidal CO₂ (EtCO₂) despite increased ventilation, followed by tachycardia and metabolic acidosis, with a peak temperature of 37.1°C. MH was suspected approximately two hours after induction, leading to immediate discontinuation of sevoflurane, transition to total intravenous anesthesia, and administration dantrolene. The patient's vital signs stabilized, and the surgery was completed without further complications.

This case underscores the importance of early MH recognition, especially in pediatric patients, who may present with subtle or atypical symptoms. The MH Clinical Grading Score, designed for adults, may not reliably predict MH likelihood in children, necessitating a high index of suspicion. Given the variable presentation of MH in pediatric patients, anesthesiologists must be vigilant in monitoring intraoperative changes. Timely intervention and adherence to MH crisis protocols significantly improve outcomes. Further research is needed to optimize pediatric-specific MH diagnostic criteria and management strategies.

Abbreviations: MH: Malignant Hyperthermia, EtCO2: End Tidal Carbon Dioxide, RYR1: Ryanodine Receptor Gene

Keywords: Malignant Hyperthermia; Anesthesia; Pediatric Anesthesia; General Anesthesia; Dantrolene; RYR1 gene

Citation: Shah S, Dhoon T, Choi Y, Wang G, Rajan G. Pediatric malignant hyperthermia: recognizing early warning signs – a case report. Anaesth. pain intensive care 2025;29(4):446-452. DOI: 10.35975/apic.v29i4.2835

Received: May 09, 2024; Revised: October 26, 2024; Accepted: January 01, 2025