Wegener's granulomatosis, acute laryngotracheal airway obstruction and death in a 17-year-old female

Case report and review of the literature

Research output: Contribution to journalArticle

23 Citations (Scopus)

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 languageEnglish
Pages (from-to)163-172
Number of pages10
JournalInternational Journal of Pediatric Otorhinolaryngology
Volume37
Issue number2
DOIs
StatePublished - Oct 1996

Fingerprint

Granulomatosis with Polyangiitis
Airway Obstruction
Community Hospital
Respiratory Sounds
Trachea
Granuloma
Autopsy
Fibrosis
Nasal Septal Perforation
A 17
Hoarseness
Antineutrophil Cytoplasmic Antibodies
Serology
Larynx
General Anesthesia
Mucous Membrane
Observation
Kidney
Biopsy
Growth

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

@article{0a5f1fb5d1194a1bad5e68e04e588b98,
title = "Wegener's granulomatosis, acute laryngotracheal airway obstruction and death in a 17-year-old female: Case report and review of the literature",
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.",
keywords = "Larynx, Subglottic stenosis, Sudden airway obstruction, Trachea, Wegener's granulomatosis",
author = "Bruce Matt",
year = "1996",
month = "10",
doi = "10.1016/0165-5876(96)82359-7",
language = "English",
volume = "37",
pages = "163--172",
journal = "International Journal of Pediatric Otorhinolaryngology",
issn = "0165-5876",
publisher = "Elsevier Ireland Ltd",
number = "2",

}

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

UR - http://www.scopus.com/inward/record.url?scp=0030271436&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0030271436&partnerID=8YFLogxK

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 -