Department of Respiratory Medicine, Juntendo University, School of Medicine, 2-1-1 Hongo, Bunkyo-Ku, Tokyo 113-8421, Japan.
Eur J Radiol. 2011 Mar;77(3):403-9. doi: 10.1016/j.ejrad.2009.09.004. Epub 2009 Sep 25.
To describe in detail the characteristic chest computed tomography (CT) findings of Birt-Hogg-Dubé (BHD) syndrome.
Thin-section chest CT scans of consecutive 12 patients with genetically diagnosed BHD syndrome were retrospectively evaluated by two observers, especially about the characteristics (distribution, number, size, shape and relation to pleura) of pulmonary cysts. Interobserver agreement in the identification of abnormalities on the CT images was achieved using the κ statistic, and the degree of interobserver correlation for the characterization of pulmonary cysts was assessed using the Spearman rank correlation coefficient.
Multiple pulmonary cysts were seen in all patients. The number of cysts in each patient was various (range, 29-407), and cysts of various sizes (from a few mm to 2 cm or more) were seen in all patient. 76.6% (mean) of cysts were irregular-shaped, and 40.5% (mean) of cysts were located along the pleura. The mean extent score of cysts was 13% of the whole lung, and the distribution of cysts was predominantly in the lower medial zone. Finally, cysts abutting or including the proximal portions of lower pulmonary arteries or veins were also seen in all patients.
Multiple, irregular-shaped cysts of various sizes with lower medial lung zone predominance are characteristic CT findings of BHD syndrome. Cysts abutting or including the proximal portions of lower pulmonary arteries or veins may also exist in this syndrome in a high probability.
详细描述 Birt-Hogg-Dubé（BHD）综合征的特征性胸部计算机断层扫描（CT）表现。
回顾性分析 12 例经基因诊断的 BHD 综合征患者的薄层胸部 CT 扫描，由两位观察者评估，特别关注肺囊肿的特征（分布、数量、大小、形状和与胸膜的关系）。使用κ统计量评估 CT 图像上异常的观察者间一致性，使用 Spearman 秩相关系数评估肺囊肿特征的观察者间相关性程度。
所有患者均可见多发肺囊肿。每个患者的囊肿数量不同（范围 29-407 个），大小不一（从数毫米到 2 厘米或更大）。76.6%（平均值）的囊肿为不规则形状，40.5%（平均值）的囊肿位于胸膜附近。囊肿的平均范围评分为全肺的 13%，分布主要在下中肺区。最后，所有患者均可见累及或包含下肺动静脉近端部分的囊肿。
多发、大小不一、形状不规则的囊肿，以下中肺区为主，是 BHD 综合征的特征性 CT 表现。该综合征中也可能存在累及或包含下肺动静脉近端部分的囊肿。
