Real Evidence and Misconceptions about Malignant Hyperthermia in Children: A Narrative Review

Abstract

Malignant hyperthermia is a rare but life-threatening pharmacogenetic disorder triggered by exposure to specific anesthetic agents. Although this occurrence could affect virtually any patient during the perioperative time, the pediatric population is particularly vulnerable, and it has a five-fold higher incidence in children compared to adults. In the last few decades, synergistic efforts among leading anesthesiology, pediatrics, and neurology associations have produced new evidence concerning the diagnostic pathway, avoiding unnecessary testing and limiting false diagnoses. However, a personalized approach and an effective prevention policy focused on clearly recognizing the high-risk population, defining perioperative trigger-free hospitalization, and rapid activation of supportive therapy should be improved. Based on epidemiological data, many national scientific societies have produced consistent guidelines, but many misconceptions are common among physicians and healthcare workers. This review shall consider all these aspects and summarize the most recent updates.

Keywords: dantrolene; general anesthesia; genetic screening; malignant hyperthermia; patient safety; pediatric anesthesia; personalized medicine.

Figure 1

Role of dysfunction in the mechanism of excitation–contraction coupling in skeletal muscles primarily due to mutations in type 1 RyR1, Cav1.1, or STAC3 accessory protein in determining susceptibility to MH. Calcium release and uptake (arrows) is in equilibrium.

Figure 2

Diagnostic pathway for investigation of MH susceptibility.