Abstract
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.
| Original language | English |
|---|---|
| Pages (from-to) | 163-172 |
| Number of pages | 10 |
| Journal | International Journal of Pediatric Otorhinolaryngology |
| Volume | 37 |
| Issue number | 2 |
| DOIs | |
| State | Published - Oct 1996 |
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Keywords
- Larynx
- Subglottic stenosis
- Sudden airway obstruction
- Trachea
- Wegener's granulomatosis
ASJC Scopus subject areas
- Otorhinolaryngology
- Pediatrics, Perinatology, and Child Health
- Pulmonary and Respiratory Medicine
- Surgery
Cite this
Wegener's granulomatosis, acute laryngotracheal airway obstruction and death in a 17-year-old female : Case report and review of the literature. / Matt, Bruce.
In: International Journal of Pediatric Otorhinolaryngology, Vol. 37, No. 2, 10.1996, p. 163-172.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Wegener's granulomatosis, acute laryngotracheal airway obstruction and death in a 17-year-old female
T2 - Case report and review of the literature
AU - Matt, Bruce
PY - 1996/10
Y1 - 1996/10
N2 - 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.
AB - 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.
KW - Larynx
KW - Subglottic stenosis
KW - Sudden airway obstruction
KW - Trachea
KW - Wegener's granulomatosis
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U2 - 10.1016/0165-5876(96)82359-7
DO - 10.1016/0165-5876(96)82359-7
M3 - Article
VL - 37
SP - 163
EP - 172
JO - International Journal of Pediatric Otorhinolaryngology
JF - International Journal of Pediatric Otorhinolaryngology
SN - 0165-5876
IS - 2
ER -