Characteristics of pulmonary cysts in Birt-Hogg-Dubé syndrome: thin-section CT findings of the chest in 12 patients.
OBJECTIVE
To describe in detail the characteristic chest computed tomography (CT) findings of Birt-Hogg-Dubé (BHD) syndrome.


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 κ 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.


CONCLUSIONS
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.
Department of Respiratory Medicine, Juntendo University, School of Medicine, 2-1-1 Hongo, Bunkyo-Ku, Tokyo 113-8421, Japan. tobino@juntendo.ac.jp
Kazunori
K
Tobino
Yoko
Y
Gunji
Masatoshi
M
Kurihara
Makiko
M
Kunogi
Kengo
K
Koike
Noriyuki
N
Tomiyama
Takeshi
T
Johkoh
Yuzo
Y
Kodama
Shin-ichiro
S
Iwakami
Mika
M
Kikkawa
Kazuhisa
K
Takahashi
Kuniaki
K
Seyama
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D058249:Birt-Hogg-Dube Syndrome / Q000000981:diagnostic imaging*; D003560:Cysts / Q000000981:diagnostic imaging*; D005260:Female; D006801:Humans; D008171:Lung Diseases / Q000000981:diagnostic imaging*; D008297:Male; D008875:Middle Aged; D013902:Radiography, Thoracic / Q000379:methods*; D014057:Tomography, X-Ray Computed / Q000379:methods*