TY - JOUR
T1 - CT bronchoscopy in the diagnosis of Williams-Campbell syndrome
AU - George, Jojy
AU - Jain, Rajeev
AU - Tariq, Syed M.
PY - 2006/1
Y1 - 2006/1
N2 - 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.
AB - 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.
KW - Bronchiectasis
KW - Bronchoscopy
KW - CT
KW - Williams-Campbell syndrome
UR - http://www.scopus.com/inward/record.url?scp=33645102967&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=33645102967&partnerID=8YFLogxK
U2 - 10.1111/j.1440-1843.2006.00793.x
DO - 10.1111/j.1440-1843.2006.00793.x
M3 - Article
C2 - 16423213
AN - SCOPUS:33645102967
SN - 1323-7799
VL - 11
SP - 117
EP - 119
JO - Respirology
JF - Respirology
IS - 1
ER -