Eosinophilic Granulomatosis With Polyangiitis: An Unusual Case of Pediatric Subglottic Stenosis. 2021

Tara E Mokhtari, and Lauren E Miller, and Asitha D L Jayawardena, and Peter M Sadow, and Stephen C Hardy, and Deborah Rothman, and Christopher J Hartnick
Department of Otolaryngology, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, U.S.A.

Systemic disease is an uncommon cause of subglottic stenosis (SGS). We report a case of severe SGS due to underlying eosinophilic granulomatosis with polyangiitis (EGPA) in a child presenting with isolated stridor. EGPA is a rare systemic vasculitis with very limited cases reported in the pediatric population. While surgical intervention was required given the degree of stenosis in this case, medical management of the underlying systemic disease process is critical when there is clinical suspicion of SGS in the context of systemic vasculitis. Laryngoscope, 131:656-659, 2021.

UI MeSH Term Description Entries
D007829 Laryngostenosis Developmental or acquired stricture or narrowing of the LARYNX. Symptoms of respiratory difficulty depend on the degree of laryngeal narrowing. Laryngeal Stenosis,Acquired Laryngeal Stenosis,Acquired Subglottic Stenosis,Congenital Subglottic Stenosis,Acquired Laryngeal Stenoses,Acquired Subglottic Stenoses,Congenital Subglottic Stenoses,Laryngeal Stenoses,Laryngeal Stenoses, Acquired,Laryngeal Stenosis, Acquired,Laryngostenoses,Stenoses, Acquired Laryngeal,Stenoses, Acquired Subglottic,Stenoses, Congenital Subglottic,Stenoses, Laryngeal,Stenosis, Acquired Laryngeal,Stenosis, Acquired Subglottic,Stenosis, Congenital Subglottic,Stenosis, Laryngeal,Subglottic Stenoses, Acquired,Subglottic Stenoses, Congenital,Subglottic Stenosis, Acquired,Subglottic Stenosis, Congenital
D008297 Male Males
D012135 Respiratory Sounds Noises, normal and abnormal, heard on auscultation over any part of the RESPIRATORY TRACT. Breathing Sounds,Crackles,Lung Sounds,Pleural Rub,Rales,Rhonchi,Stridor,Wheezing,Breathing Sound,Crackle,Lung Sound,Pleural Rubs,Rale,Respiratory Sound,Rhonchus,Rub, Pleural,Sound, Breathing,Sound, Lung,Sound, Respiratory,Sounds, Breathing,Sounds, Lung,Stridors,Wheezings
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D004803 Eosinophilic Granuloma The most benign and common form of Langerhans-cell histiocytosis which involves localized nodular lesions predominantly of the bones but also of the gastric mucosa, small intestine, lungs, or skin, with infiltration by EOSINOPHILS. Granuloma, Eosinophilic,Eosinophilic Granulomas,Granulomas, Eosinophilic
D005931 Glottis The vocal apparatus of the larynx, situated in the middle section of the larynx. Glottis consists of the VOCAL FOLDS and an opening (rima glottidis) between the folds. Rima Glottidis,Glottidi, Rima,Glottidis, Rima,Rima Glottidi
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D014135 Tracheal Stenosis A pathological narrowing of the TRACHEA. Stenoses, Tracheal,Stenosis, Tracheal,Tracheal Stenoses
D014890 Granulomatosis with Polyangiitis A multisystemic disease of a complex genetic background. It is characterized by inflammation of the blood vessels (VASCULITIS) leading to damage in any number of organs. The common features include granulomatous inflammation of the RESPIRATORY TRACT and KIDNEYS. Most patients have measurable autoantibodies (ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES) against MYELOBLASTIN. Granulomatosis, Wegener's,Wegener Granulomatosis,Wegener's Granulomatosis,Granulomatosis with Polyangiitides,Granulomatosis, Wegener,Polyangiitides, Granulomatosis with,Polyangiitis, Granulomatosis with,with Polyangiitides, Granulomatosis,with Polyangiitis, Granulomatosis

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