Wegener's granulomatosis, acute laryngotracheal airway obstruction and death in a 17-year-old female: Case report and review of the literature

Objective: To alert practitioners to the risk of sudden airway obstruction and death in patients with Wegener's granulomatosis. Design: Case report and literature review. Settings: University and Community hospitals. Patient: A 17-year-old white female. Intervention: (1) evaluation and treatment for mental status changes over 2 months. (2) Evaluation and surgical biopsy of nasal septal perforation under general anesthesia at a university children's hospital. (3) Evaluation and observation at a community hospital 2 days later. (4) autopsy. Results: Serology performed 3 days ante-mortem revealed (at 2 days post-mortem) cytoplasmic anti-neutrophil cytoplasmic antibody positive at 1:128. Autopsy was significant for microscopic fibrosis and granulomas in the kidneys and essentially total obstruction of the subglottis and upper trachea by a 3.5 x 1 x 1 cm mass of fibrosis and granulomas overlying circumferentially necrotic mucosa. This mass was centered on the crico-tracheal junction. Conclusions: Wegener's granulomatosis can lead to proliferative tissue growth with acute airway obstruction in the larynx and trachea, and death. Any patient with WG under age 20, as well as patients with WG and laryngotracheal symptoms (e.g. stridor, hoarseness, wheezing) would benefit from evaluation of the airway.