Bronchoscopic evaluation of airway obstruction in campomelic dysplasia

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 di...

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Bibliographic Details
Published inPediatric pulmonology Vol. 3; no. 5; p. 364
Main Authors Grad, R, Sammut, P H, Britton, J R, Goodrich, P, Hoyme, H E, Dambro, N N
Format Journal Article
LanguageEnglish
Published United States 01.09.1987
Subjects
Airway Obstruction - pathology
Bronchi - pathology
Bronchoscopy
Fiber Optic Technology
Humans
Infant, Newborn
Laryngoscopy
Osteochondrodysplasias - pathology
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Summary: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.
ISSN:8755-6863
DOI:10.1002/ppul.1950030514