Remember that Malignant Hyperthermia may not have Hyperthermia
Nancy Sokal Hagerman MD
Malignant hyperthermia (MH) is a hypermetabolic disorder of skeletal muscle that is triggered in susceptible individuals by several inhalation anesthetic agents (sevoflurane, desflurane, isoflurane, halothane, enflurane, and methoxyflurane) and succinylcholine. These anesthetic triggers cause intracellular hypercalcemia in skeletal muscle by decreasing the uptake of calcium by the sarcoplasmic reticulum. The intracellular hypercalcemia activates metabolic pathways that result in adenosine triphosphate (ATP) depletion, acidosis, membrane destruction, and ultimately cell death. Susceptibility to MH is inherited as an autosomal dominant disorder and most susceptible individuals are completely asymptomatic until exposed to triggering agents.
Episodes of MH occur most often, but not exclusively, in children. The mean age for MH is 15 years, although cases have been reported in infants as well as the elderly. The incidence of MH ranges from approximately 1 in 10,000 to 1 in 50,000 individuals who are exposed to the triggering agents.
Signs and Symptoms
In most cases, the first signs and symptoms of the disorder are evident in the operating room. However, MH can also occur in the recovery room or even after the patient has been transferred to the patient floor. It is important to remember that none of the signs and symptoms occur in all cases. Initial signs usually include tachycardia (90% of cases) and tachypnea (80% of cases) due to the sympathetic nervous system response to the underlying hypermetabolism and hypercarbia (80% of cases). In the paralyzed patient under general anesthesia, the first sign of MH can be hypercarbia that is resistant to adjustments in the ventilator setting. Next, an increase in blood pressure, cardiac dysrhythmias, and muscle rigidity (80% of cases) are often seen. Patients may then become hyperthermic (70% of cases), with a rise of 1 to 2 degrees Celsius every 5 minutes. An arterial blood gas will usually show respiratory and metabolic acidosis. Other lab abnormalities include hyperkalemia, hypercalcemia, lactic acidemia and myoglobinuria. Creatinine kinase levels rise to 20,000 or more within 12 to
24 hours, putting the patient at risk for myoglobinuric renal failure. Isolated myoglobinuria in the early postoperative period should make the anesthesiologist and surgical team suspicious for MH. Masseter muscle rigidity shortly after the administration of succinylcholine has also been associated with MH as well.
24 hours, putting the patient at risk for myoglobinuric renal failure. Isolated myoglobinuria in the early postoperative period should make the anesthesiologist and surgical team suspicious for MH. Masseter muscle rigidity shortly after the administration of succinylcholine has also been associated with MH as well.