(aka Congenital Cartilage Deficiency)
Epidemiology
- First reported in 1960
- Congenital
Physiology
- Abnormal or deficienct cartilage in 2nd-3rd generation airways
Diagnosis
- FOB: normal
- CXR/Chest CT Patterns:
- Bronchogram: dilated but collapsible 2nd-3rd generation bronchi (with abnormal cartilage) by bronchography
Clinical
- Present with recurrent pulmonary infections in infancy
- Bronchiectasis (see [[Bronchiectasis]])