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A malignant hyperthermia-like reaction in a six-month-old female with a 5q chromosomal deletion

Mark Greenberg, MD^*, Alvin Faierman, MD, Brock Fisher, MD and Bryan Harris, MD^*

^* University of California, San Diego, USA
Children’s Hospital and Heath Center, San Diego, USA, E-mail: mgreenberg{at}ucsd.edu

To the Editor:

We encountered a six-month-old female with 5q-syndrome, who developed progressive tachycardia, hyper-carbia and hyperthermia following a general anesthetic given for repair of a cleft palate. Two weeks prior, the infant had received a sevoflurane anesthetic during a magnetic resonance imaging scan of the brain. Immediately following this procedure, she experienced a self-limited episode of fever and tachycardia that were attributed to overzealous warming of the infant by the nursing staff. Body stiffness was not described. In accordance with our Human Research Protection Program, formal review and approval of this document were not required.

General anesthesia for the cleft palate repair was induced using sevoflurane and nitrous oxide, followed by neuromuscular blockade with of rocuronium 0.8 mg·kg^–1 iv. Following laryngoscopy and intubation, anesthesia was maintained with isoflurane, nitrous oxide and oxygen. During the case all physiologic parameters were within normal limits. Upon completion of the repair the trachea was extubated. Vital signs on admission to the recovery room were unremarkable: heart rate 105 beats·min^–1, respiratory rate 20 breaths·min^–1, blood pressure 96/40 mmHg, temperature 37°C.

Approximately one hour later, examination revealed a heart rate of 220 beats·min^–1, respiratory rate of 60 breaths·min^–1, blood pressure of 126/62 mmHg, and an axillary temperature of 39.9°C. Inspection of the child showed her to be in severe respiratory distress with hypertonia. A venous blood gas sample was obtained: pH 7.12 /CO₂ 270 /PO₂ 271 mmHg /base excess –6.0. The child was intubated and cooling measures rapidly instituted. The hyper-metabolic state rapidly resolved following the administration of dantrolene sodium (3 mg·kg^–1). The infant was admitted to the pediatric intensive care unit and subsequently received three more doses of dantrolene (1 mg·kg^–1). Serial laboratory examinations at 12, 24 and 36 hr revealed a peak serum creatine phosphokinase (CPK) of 260 IU·L^–1 with consistently normal serum electrolytes. She exhibited a full recovery, and was discharged from the hospital five days later, in good condition.

This is the first report of a malignant hyperthermic reaction occurring in a patient with a chromosomal deletion of 5q. Interestingly, the infant’s serum CPK level increased only modestly following the anesthetic. We attribute this to the young age of the patient, prompt treatment, and the rapid resolution of symptoms following institution of therapy. It is possible that older infants and adults exhibit more marked elevations in serum CPK due to their relative increased muscle mass. Also noteworthy, was the self-limited fever and tachycardia following her initial anesthetic. Unexplained fever and tachycardia after general anesthesia, in patients as young as six months of age should be considered possible precursors to malignant hyperthermia.

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