Multilevel airway involvement in children with Wegener's granulomatosis: clinical course and the utility of a multidisciplinary approach.

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. DESIGN Retrospective medical chart review. SETTING Tertiary children's hospital. PATIENTS Pediatric subjects with WG treated at a tertiary children's hospital over the past 15 years. MAIN OUTCOME MEASURES 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.