Kaneko K, Kudo S, Tashiro M, Kishikawa T, Nakanishi Y, Yamada H
Department of Radiology, Saga Medical School, Japan.
J Thorac Imaging. 1991 Apr;6(2):11-3.
Williams-Campbell syndrome is a rare type of cystic bronchiectasis that is due to defective cartilage of fourth-to sixth-order bronchi. The diagnosis has been made either at autopsy or by bronchographic findings. The article describes an adult case of this syndrome with CT findings that are characteristic of this entity. CT showed areas of emphysematous lung parenchyma distal to the dilated bronchi, suggesting bronchiolitis obliterans secondary to proximal cystic bronchiectasis. These findings are useful in differentiating Williams-Campbell syndrome from other causes of cystic bronchiectasis, thus obviating bronchography.
威廉姆斯-坎贝尔综合征是一种罕见的囊性支气管扩张症，由四至六级支气管软骨发育缺陷所致。该综合征的诊断通常在尸检时或通过支气管造影检查结果做出。本文描述了一例该综合征的成年病例，其CT表现具有该疾病的特征性。CT显示扩张支气管远端的肺实质出现肺气肿区域，提示近端囊性支气管扩张继发闭塞性细支气管炎。这些发现有助于将威廉姆斯-坎贝尔综合征与其他导致囊性支气管扩张的病因相鉴别，从而避免进行支气管造影检查。
