title: Multilevel airway involvement in children with Wegener's granulomatosis: clinical course and the utility of a multidisciplinary approach.
abstract: OBJECTIVE
  To examine the prevalence of airway involvement in children with Wegener's granulomatosis (WG) at our institution and to evaluate the utility of a treatment paradigm involving a multidisciplinary team.
  
  
  METHODS
  Retrospective medical chart review.
  
  
  METHODS
  Tertiary children's hospital.
  
  
  METHODS
  Pediatric subjects with WG treated at a tertiary children's hospital over the past 15 years.
  
  
  METHODS
  The medical records of all subjects with airway lesions were reviewed for clinical characteristics of airway involvement and for the medical and surgical treatment regimens.
  
  
  RESULTS
  Seven of 28 pediatric patients with WG were identified to have airway lesions (25%), including vocal fold granuloma, subglottic stenosis, and multilevel stenoses. Three of these patients had isolated and limited lesions. The 4 remaining patients are the focus of this study. One patient underwent a cricotracheal resection. All patients underwent repeated surgical treatment from combined services (otolaryngology and pulmonology) with flexible and rigid endoscopy, dilatation, and corticosteroid injection to manage subglottic, tracheal, and bronchial stenoses. Operative findings were communicated to the rheumatology service for manipulation of medical therapy. The 3 most recent patients received the anti-CD20 chemotherapeutic agent rituximab. This treatment approach led to temporary remission in some of the patients.
  
  
  CONCLUSIONS
  Airway involvement in pediatric patients with WG is known to occur, but multilevel airway involvement is rare and clinically challenging. We advocate a combined surgical approach involving otolaryngology, pulmonology, and rheumatology in managing aggressive cases of multilevel airway involvement in WG.
authors.affiliation: Department of Otolaryngology, University of Colorado Denver, School of Medicine, Denver, Colorado, USA.
authors.forename: Marcia E
authors.initials: ME
authors.lastname: Eustaquio
authors.forename: Kenny H
authors.initials: KH
authors.lastname: Chan
authors.forename: Robin R
authors.initials: RR
authors.lastname: Deterding
authors.forename: Roger J
authors.initials: RJ
authors.lastname: Hollister
mesh_terms: D000293:Adolescent; D002648:Child; D003131:Combined Modality Therapy; D005260:Female; D006099:Granuloma / Q000209:etiology / Q000628:therapy; D014890:Granulomatosis with Polyangiitis / Q000150:complications*; D006801:Humans; D007818:Laryngeal Diseases / Q000209:etiology* / Q000628:therapy*; D007829:Laryngostenosis / Q000209:etiology / Q000628:therapy; D008297:Male; D010348:Patient Care Team; D015995:Prevalence; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult