Williams-Campbell Syndrome

(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]])