Characteristics of pulmonary cysts in Birt-Hogg-Dube syndrome: Thin-section CT findings of the chest in 12 patients
Characteristics of pulmonary cysts in Birt-Hogg-Dube syndrome: Thin-section CT findings of the chest in 12 patients
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DOI：
10.1016/j.ejrad.2009.09.004
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发表时间：
2011-03-01
影响因子：
3.3
通讯作者：
Seyama, Kuniaki
中科院分区：
文献类型：
--
作者：
Tobino, Kazunori;Gunji, Yoko;Seyama, Kuniaki
目的：详细描述Birt-Hogg-Dube(BHD)综合征的胸部CT表现。材料与方法：由两位观察者对连续12例BHD综合征患者的胸部薄层CT扫描进行回顾性分析，特别是对肺囊肿的分布、数量、大小、形态及其与胸膜的关系进行分析。结果：所有患者均可见多发性肺囊肿，观察者间对CT图像异常的判断采用kappa统计量，对肺囊肿定性的观察者间关联度采用Spearman等级相关系数进行评估。每例囊肿数不一(29~407个)，大小不一(从几毫米到2厘米或更大)。76.6%(平均)为不规则形，40.5%(平均)位于胸膜。囊变范围评分平均为全肺的13%，主要分布在下内侧带。所有患者均可见与下肺动、静脉近端相邻或包括在内的囊性病变。结论：BHD综合征的CT表现为多发、大小不一、形状不规则、以下肺内侧带为主的囊性病变。毗邻或包括下肺动、静脉近端的囊肿也可能存在于本综合征中。(C)2009爱思唯尔爱尔兰有限公司。保留所有权利。
Purpose: To describe in detail the characteristic chest computed tomography (CT) findings of Birt-Hogg-Dube (BHD) syndrome.Materials and methods: 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 kappa statistic, and the degree of interobserver correlation for the characterization of pulmonary cysts was assessed using the Spearman rank correlation coefficient.Results: 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.Conclusion: 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. (C) 2009 Elsevier Ireland Ltd. All rights reserved.