Something is missing in the bronchus-Williams-Campbell syndrome

A 60-year-old female who was a former smoker was admitted with a five-day history of progressive dyspnea, cough, sputum production, and fever. She had a history of recurrent pneumonia since her childhood. Physical examination revealed expiratory wheezes and an SpO₂ of 91% on room air. A chest CT scan was performed (Figure 1). On the basis of the radiological findings and the exclusion of other causes, the patient was diagnosed with Williams-Campbell syndrome (WCS). After 10 days of antibiotic therapy, she was discharged with improved symptoms.

Figure 1
Axial CT scans of the chest taken during inhalation (in A) and exhalation (in B). Note complete or partial airway collapse during exhalation. In C, coronal CT scan of the chest with minimum intensity projection to improve visualization of the airways. Note bronchiectasis in both lungs, involving fourth- to sixth-order bronchi. The peripheral and central airways, including the trachea, main bronchi, and lobar bronchi, remain unaffected by bronchiectasis.

WCS is a rare congenital disorder characterized by the absence of cartilage in subsegmental bronchi, leading to bronchiectasis.^1,2 The pathophysiology of WCS involves airway collapse caused by deficiency of cartilage, resulting in chronic respiratory symptoms such as dyspnea and recurrent pulmonary infections.¹ Diagnosis is typically based on clinical manifestations and characteristic radiological findings on chest HRCT scans, as well as on exclusion of other causes of bronchiectasis.³ Management of WCS remains challenging because of its rarity, being based on the use of antimicrobials. Treatments such as noninvasive positive pressure ventilation have shown promise in managing respiratory failure, and lung transplantation may be considered in severe cases.^1,2

REFERENCES

• ¹
  Noriega Aldave AP, William Saliski D. The clinical manifestations, diagnosis and management of williams-campbell syndrome. N Am J Med Sci. 2014;6(9):429-432. https://doi.org/10.4103/1947-2714.141620
  » https://doi.org/10.4103/1947-2714.141620
• ²
  Rohilla M, Previgliano C, Geimadi A, Sangster G. Williams-Campbell syndrome: an unusual presentation in an adult patient. BJR Case Rep. 2020;7(1):20200052. https://doi.org/10.1259/bjrcr.20200052
  » https://doi.org/10.1259/bjrcr.20200052
• ³
  George J, Jain R, Tariq SM. CT bronchoscopy in the diagnosis of Williams-Campbell syndrome. Respirology. 2006;11(1):117-119. https://doi.org/10.1111/j.1440-1843.2006.00793.x
  » https://doi.org/10.1111/j.1440-1843.2006.00793.x

Publication Dates

• Publication in this collection
  21 Aug 2023
• Date of issue
  2023