[ACUTE COURSE OF INFANTILE LARYNGEAL DYSPNEAS (CLINICO-STATISTICAL STUDY ON 521 CASES)].
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Pseudomyotonia is a syndrome characterized by progressive muscle stiffness, weakness, myokymia, hyperhydrosis, and wasting of muscle. It especially affects the distal aspect of the extremities. The electromyogram is characteristic, and shows continuous muscle activity at rest. We discuss a case that is believed to be the first recorded instance of pseudomyotonia complicated by airway obstruction secondary to laryngeal spasm. Treatment with phenytoin sodium resulted in rapid improvement of the peripheral symptoms and simultaneous resolution of the laryngeal spasm.
Thirteen infant and juvenile monkeys and baboons were studied in procedures designed to assess the functional characteristics of the upper airway, with special attention to the consequences of laryngeal nerve stimulation. In young animals, stimulation of the recurrent laryngeal nerve produced closure of the airway in sufficient degree and duration to cause death. Older juvenile monkeys were resistant to fatal laryngospasm induced by nerve stimulation. A variety of patterns of general autonomic response accompanied the airway changes. These results are consistent with (1) a laryngospasm hypothesis to account for certain terminal events in sudden infant death syndrome, and (2) prior observations of age-related general autonomic stability.
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Tonsillectomy and adenoidectomy can predispose to stridor and laryngospasm in the immediate postextubation period. A prospectively randomized study of 133 patients undergoing tonsillectomy and adenoidectomy was undertaken to determine if the topical application of 4 mg/kg of 4% lidocaine at the time of intubation would decrease the incidence of postoperative stridor and laryngospasm. Taken together, eight (12%) of 67 control patients suffered stridor or laryngospasm vs two (3%) of 66 patients receiving lidocaine. It is concluded that lidocaine administered topically at the time of intubation for adenotonsillectomy helps prevent postoperative stridor and laryngospasm. Surgery of the upper aerodigestive tract often involves not only the technical aspects of the surgical procedure but also concomitant management of the airway. This can present a challenge to even the most adept otolaryngologist and anesthesiologist. Accidental extubation is always possible, and reintubation can be difficult in the presence of blood and saliva. The difficulty is often compounded with a patient who is not completely paralyzed.
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A 20-month-old girl with developmental delay, dysmorphic features, horizontal supranuclear gaze palsy, retrocollis, and episodes of laryngospasm was diagnosed with variant neuronopathic Gaucher disease. The diagnosis was made enzymatically. Mutation analysis showed compound heterozygosity for D409H and a previously unreported mutation C16S. The presence of dysmorphic features, laryngospasm, absent cardiac findings, and the severe clinical phenotype distinguishes our case from other cases of variant neuronopathic Gaucher disease. We therefore propose to extend the spectrum of variant Gaucher disease.
Few serious adverse reactions associated with the use of gadopentetate dimeglumine in magnetic resonance imaging have been reported. The present case involves an 8-year-old girl who developed laryngospasm after administration of gadopentetate dimeglumine.
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