Williams-Campbell Syndrome

From Dr. Max Gordon, we learned about a fascinating case of hemoptysis arising from Williams-Campbell Syndrome first diagnosed in an older patient. In this condition, a cartilaginous deficiency in the 4th to 6th order bronchi prevent adequate clearance of respiratory secretions, resulting in recurrent infections and thus bronchiectasis.

Bronchiectasis arising from this chronic progressive process is diffuse, cystic, and has a central predominance on imaging, characterized by air-fluid levels.

Below is some representative imaging similar to that of the case discussed:

Representative Radiographic Imaging- Radiopaedia.org

Representative Radiographic Imaging- Radiopaedia.org

Representative CT Imaging- Radiopaedia.org

Representative CT Imaging- Radiopaedia.org

We discussed some initial considerations when encountering a new case of hemoptysis:

Classification of Massive vs. Submassive: generally considered “massive” if >100cc/24 hrs

Location of bleed and potential etiologies:

  1. Lung parenchyma (Abscess, pneumonia, tuberculosis, Goodpasture, granulomatosis with polyangiitis)
  2. Subglottic airways (Bronchitis, bronchiectasis, tumor)
  3. Pulmonary vasculature (AVM, pulmonary embolism, mitral stenosis, left sided heart failure)
  4. Mimickers (GI tract, Nasopharynx, Serratia marcescens pneumonia (due to staining of pigmented bacteria))

Mechanism: Usually from bronchial arterial source (ie non-gas exchange vasculature).

  1. Either compromised pulmonary circulation in anastamotic vessels leading to rupture
  2. Chronic inflammatory or neoplastic related- angiogenic growth factors leading to proliferation of fragile new vessels which are prone to rupture

Thanks again to Dr. Gordon for this fascinating case and imaging.

Additional information above compiled from dynamed