CT bronchoscopy in the diagnosis of Williams-Campbell syndrome

Jojy George, Rajeev Jain, Syed M. Tariq

Research output: Contribution to journalArticle

17 Citations (Scopus)

Abstract

Williams-Campbell syndrome, a rare disorder, is characterized by a congenital deficiency of cartilage, typically involving the fourth to the sixth order bronchi, and resulting in expiratory airway collapse and bronchiectasis. The authors report a patient with Williams-Campbell syndrome with type II respiratory failure due to extensive cystic bronchiectasis and secondary emphysema. CT of the thorax showed the affected bronchi had characteristic ballooning on inspiration and collapse on expiration. Three-dimensional images of the tracheobronchial tree were constructed from a volume of data acquired by thin-slice CT scanning. Apart from confirming expiratory collapse of the affected bronchi, these images revealed an absence of the cartilage ring impressions in the bronchial wall, extending bilaterally from the mainstem down to subsegmental bronchi, suggesting cartilage deficiency.

Original languageEnglish
Pages (from-to)117-119
Number of pages3
JournalRespirology
Volume11
Issue number1
DOIs
Publication statusPublished - Jan 2006

Keywords

  • Bronchiectasis
  • Bronchoscopy
  • CT
  • Williams-Campbell syndrome

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

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