Bronchoscopic evaluation of airway obstruction in campomelic dysplasia

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

doi:10.1002/ppul.1950030514

Bronchoscopic evaluation of airway obstruction in campomelic dysplasia

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

Pediatrics

Research output: Contribution to journal › Article › peer-review

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 language	English (US)
Pages (from-to)	364-367
Number of pages	4
Journal	Pediatric pulmonology
Volume	3
Issue number	5
DOIs	https://doi.org/10.1002/ppul.1950030514
State	Published - 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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10.1002/ppul.1950030514

Cite this

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title = "Bronchoscopic evaluation of airway obstruction in campomelic dysplasia",

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.",

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AU - Grad, Roni

AU - Sammut, Paul H.

AU - Britton, John R.

AU - Goodrich, Paul

AU - Hoyme, H. Eugene

AU - Dambro, Nancy N.

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N2 - 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.

AB - 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.

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Bronchoscopic evaluation of airway obstruction in campomelic dysplasia

Abstract

Keywords

ASJC Scopus subject areas

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