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Characteristics of pulmonary cysts in Birt-Hogg-Dubé syndrome: Thin-section CT findings of the chest in 12 patients |
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| Authors: | Kazunori Tobino Yoko GunjiMasatoshi Kurihara Makiko KunogiKengo Koike Noriyuki TomiyamaTakeshi Johkoh Yuzo KodamaShin-ichiro Iwakami Mika KikkawaKazuhisa Takahashi Kuniaki Seyama |
| Affiliation: | a Department of Respiratory Medicine, Juntendo University, School of Medicine, 2-1-1 Hongo, Bunkyo-Ku, Tokyo 113-8421, Japan b Pneumothorax Center, Nissan Tamagawa Hospital, 4-8-1 Seta, Setagaya-Ku, Tokyo 158-0095, Japan c Department of Radiology, Osaka University Graduate School of Medicine, 2-2 Yamadaoka, Suita, Osaka 565-0871, Japan d Department of Radiology, Kinki Central Hospital of Mutual Aid Association of Public School Teachers, Kurumazuka 3-1, Itami, Hyogo 664-0872, Japan e Department of Respiratory Medicine, Juntendo University, Shizuoka Hospital, 1129 Nagaoka, Izunokuni, Shizuoka 410-2295, Japan f Biochemical Research Center, Division of Proteomics and Biomolecular Sciences, Juntendo University, Graduate School of Medicine, Bunkyo-Ku, Tokyo, Japan g The Study Group of Pneumothorax and Cystic Lung Diseases, 4-8-1 Seta, Setagaya-Ku, Tokyo 158-0095, Japan |
| Abstract: | PurposeTo describe in detail the characteristic chest computed tomography (CT) findings of Birt-Hogg-Dubé (BHD) syndrome.Materials and methodsThin-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.ResultsMultiple 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.ConclusionMultiple, 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. |
| Keywords: | Birt-Hogg-Dubé syndrome Pneumothorax Computed tomography Pulmonary cyst |
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