A Rare Cause of Upper Airway Obstruction Isolated Demyelinating Brainstem Lesion

Abstract

Acute upper airway obstruction derived from any cause can be a life-threatening emergency in pediatric patients. The major causes are infection, foreign body aspiration, and trauma. However, acute neurological disorders occasionally may manifest as severe airway obstruction. In our case, we report a 10-year-old patient presenting with prominent upper airway obstruction requiring intubation and respiratory support. He also had fever, encephalopathy, and involvement of multiple cranial nerves. Cranial magnetic resonance imaging showed a hyperintense lesion covering all of the medulla oblongata on T2-weighted images and a lesion that was hypointense on T1-weighted images with no enhancement. Diffusion maps were normal. High-dose steroids and then intravenous immunoglobulin were given because these findings were mostly consistent with an inflammatory demyelinating process in the brainstem. A dramatic improvement was observed, and he was extubated on the 10th day. He completely recovered within 3 weeks without any neurological deficit. We emphasize that emergency physicians should be familiar with the airway manifestations of acute neurological disorders. Although rare, acute demyelinating brainstem disorders should be considered a part of the differential diagnosis of upper airway obstruction in children. Prompt recognition of this entity in light of additional neurological findings, neuroimaging, and other laboratory test results is critical. Early immunomodulatory treatment of these patients can be lifesaving.