George Jojy, Jain Rajeev, Tariq Syed M
Department of Medicine, Sultan Qaboos University Hospital, Muscat, Sultanate of Oman.
Respirology. 2006 Jan;11(1):117-9. doi: 10.1111/j.1440-1843.2006.00793.x.
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.
威廉姆斯 - 坎贝尔综合征是一种罕见疾病，其特征为先天性软骨缺乏，通常累及第四至第六级支气管，导致呼气时气道塌陷和支气管扩张。作者报告了一名患有威廉姆斯 - 坎贝尔综合征的患者，因广泛的囊性支气管扩张和继发性肺气肿而出现II型呼吸衰竭。胸部CT显示，受累支气管在吸气时呈特征性扩张，呼气时塌陷。通过薄层CT扫描获取的容积数据构建了气管支气管树的三维图像。这些图像除了证实受累支气管呼气时塌陷外，还显示支气管壁从主支气管向下至亚段支气管双侧均无软骨环压迹，提示软骨缺乏。
