[ANCA-negative subglottic laryngeal stenosis in childhood]

A 15-year-old female, having developed recurrent infections of the upper airway, hoarseness, dyspnea, and nasal congestion, was referred to our department. There was no history of trauma or intubation. The subglottic space was circularly narrowed. The test for c-ANCA was negative. Chest X-ray and renal function were normal. A tracheotomy was performed; the histology showed infiltrating plasma cells, but no signs of vasculitis or granulomatous inflammation. One year later the patient developed acute renal failure. Biopsy of the kidney confirmed Wegener's disease. The laryngeal stenosis completely resolved after therapy with cyclophosphamide. Juvenile Wegener's granulomatosis is extremely rare; the larynx and trachea seem to be involved more frequently in children than in adults. The positive testing of c-ANCA can support the diagnosis; however, even when c-ANCA do not test positive, the disease can never be excluded. Surgical interventions within the larynx or trachea might only be considered after ineffective therapy with immunosuppressive drugs.