Necrotizing epiglottitis and hemophagocytic lymphohistiocytosis

SummaryWith the introduction of vaccination for haemophilus influenza, the epidemiology of epiglottitis in children has changed. Classic childhood epiglottitis is now rare, and unusual forms of the disease may be seen. Nectrotizing epiglottitis is an extremely rare form of epiglottitis. Only four cases of nectrotizing epiglottitis have been previously reported, and all cases were in immunocompromised adult patients. Hemophagocytic lymphohistiocytosis is a rare condition characterized by high fevers, hepatosplenomegaly, and cytopenias caused by an abnormal proliferation and activation of macrophages. We report the first case of a previously healthy 5-year-old male presenting with acute onset of airway distress and pancytopenia. Subsequent airway evaluation led to the diagnosis of nectrotizing epiglottitis, and he was simultaneously diagnosed with infection-induced hemophagocytic lymphohistiocytosis. The patient recovered with intubation and antibiotics. Follow-up direct laryngoscopy revealed an epiglottis remnant with approximately 50% loss of epiglottic tissue. The presentation and pathophysiology of this unusual manifestation of epiglottitis and hemophagocytic lymphohistiocytosis will be reviewed. In the post-haemophilus influenze vaccination era, the pediatric otolaryngologist must be familiar with unusual forms of epiglottitis and its associated manifestations.