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High-Resolution CT of Patients with Primary Ciliary Dyskinesia

Marcus P. Kennedy1,2, Peadar G. Noone1, Margaret W. Leigh3, Maimoona A. Zariwala1, Susan L. Minnix1, Michael R. Knowles1 and Paul L. Molina4

1 Division of Pulmonary and Critical Care Medicine, University of North Carolina, Chapel Hill, NC.
2 Present address; Division of Pulmonary Medicine, M. D. Anderson Cancer Center, 1400 Holcombe Blvd., Unit 403, Houston, TX 77030-4009.
3 Division of Pediatric Pulmonology, University of North Carolina, Chapel Hill, NC.
4 Department of Radiology, University of North Carolina at Chapel Hill, Chapel Hill, NC.


Figure 1
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  		Fig. 1 —Graph shows lobar distribution of bronchiectasis by anatomic distribution on high-resolution CT scans for all (n =45) (black), adult (n =29) (white), and pediatric (n =16) (gray) patients with primary ciliary dyskinesia. RUL = right upper lobe, RML = right middle lobe, RLL = right lower lobe, LUL = left upper lobe, LLL = left lower lobe.

 

Figure 2
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  		Fig. 2A —57-year-old woman with primary ciliary dyskinesia. High-resolution CT scans show bronchiectasis distributed in typical pattern (severity score, 7) with disease identified in middle (B) and lower (C) lobes but not in upper lobes (A). Peribronchial consolidation and collapse (arrow) are evident in left lower lobe.

 

Figure 3
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  		Fig. 2B —57-year-old woman with primary ciliary dyskinesia. High-resolution CT scans show bronchiectasis distributed in typical pattern (severity score, 7) with disease identified in middle (B) and lower (C) lobes but not in upper lobes (A). Peribronchial consolidation and collapse (arrow) are evident in left lower lobe.

 

Figure 4
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  		Fig. 2C —57-year-old woman with primary ciliary dyskinesia. High-resolution CT scans show bronchiectasis distributed in typical pattern (severity score, 7) with disease identified in middle (B) and lower (C) lobes but not in upper lobes (A). Peribronchial consolidation and collapse (arrow) are evident in left lower lobe.

 

Figure 5
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  		Fig. 3 —34-year-old woman with primary ciliary dyskinesia. High-resolution CT scan shows severe saccular bronchiectasis with air-fluid levels (severity score, 13). Patient subsequently underwent successful bilateral lung transplantation.

 

Figure 6
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  		Fig. 4 —41-year-old man with primary ciliary dyskinesia Contrast-enhanced CT scan shows polysplenia (S).

 

Figure 7
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  		Fig. 5 —61-year-old woman with Kartagener's syndrome. Contrast-enhanced CT scan shows extensive bibasilar calcification. Situs inversus totalis and pectus excavatum are evident. H = heart, L = liver, a = aorta.

 

Figure 8
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  		Fig. 6A —Correlation of bronchiectasis severity score with age and lung function in patients with primary ciliary dyskinesia. Graph shows bronchiectasis severity score correlates (r = 0.54) with older age at CT (n =45).

 

Figure 9
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  		Fig. 6B —Correlation of bronchiectasis severity score with age and lung function in patients with primary ciliary dyskinesia. Graph shows bronchiectasis severity score correlates (r = 0.6) with worsening forced expiratory volume in 1 second (FEV1) (n =40).

 

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