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Bronchoscopic evaluation of airway obstruction in campomelic dysplasia
Author(s) -
Grad Roni,
Sammut Paul H.,
Britton John R.,
Goodrich Paul,
Hoyme H. Eugene,
Dambro Nancy N.
Publication year - 1987
Publication title -
pediatric pulmonology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.866
H-Index - 106
eISSN - 1099-0496
pISSN - 8755-6863
DOI - 10.1002/ppul.1950030514
Subject(s) - medicine , airway obstruction , airway , dysplasia , bronchopulmonary dysplasia , respiratory tract , respiratory failure , palliative care , respiratory system , surgery , pediatrics , pregnancy , nursing , biology , genetics , gestational age
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 .