Bronchoscopic evaluation of airway obstruction in campomelic dysplasia

Roni Grad, Paul H. Sammut, John R. Britton, Paul Goodrich, H. Eugene Hoyme, Nancy N. Dambro

Research output: Contribution to journalArticle

8 Scopus citations

Abstract

Campomelic dysplasia is a generalized disorder of cartilaginous growth and development, leading to early death from pulmonary insufficiency. We describe the airway dynamics as observed bronchoscopically in two affected infants. Both infants demonstrated anatomic compromise of the upper airway and diffuse laryngotracheobronchomalacia. Additionally, both had a characteristically small, bell‐shaped thoracic cage. The abnormal airway dynamics produced serious inspiratory and expiratory obstruction in these infants and, in combination with the restrictive chest wall defect, led rapidly to the development of respiratory failure. While palliative procedures such as tracheostomy may temporarily improve airway dynamics, future respiratory tract insults may prove fatal. Pediatr Pulmonol 1987; 3:364–367.

Original languageEnglish (US)
Pages (from-to)364-367
Number of pages4
JournalPediatric pulmonology
Volume3
Issue number5
DOIs
StatePublished - Jan 1 1987

Keywords

  • Laryngotracheobronchomalacia
  • airway anatomy and dynamics
  • respiratory failure

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Pulmonary and Respiratory Medicine

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    Grad, R., Sammut, P. H., Britton, J. R., Goodrich, P., Hoyme, H. E., & Dambro, N. N. (1987). Bronchoscopic evaluation of airway obstruction in campomelic dysplasia. Pediatric pulmonology, 3(5), 364-367. https://doi.org/10.1002/ppul.1950030514