PMC4501538_01
Chest X-ray on admission, and light microscopic and electron microscopic examination of transbronchial lung biopsy specimens of the patient, a 15-yr-old female nonsmoker. (A) Chest X-ray shows cystic changes with an air-fluid level localized in the right lower lung field. (B) Light microscopic examination of the transbronchial lung biopsy specimen. Noncaseating granuloma in the alveolar space (left panel) and neutrophil infiltrations (arrowheads, right panel) are observed. Hematoxylin and eosin staining. Scale bars: 50 mum. (C) Electron microscopic examination of transbronchial lung biopsy specimens. The cross-section of cilia shows the absence of inner dynein arms.
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A chest X-ray revealed cystic changes with an air-fluid level localized in the right lower lung field (Fig. 1A). The pathologic examination of transbronchial lung biopsy (TBLB) specimens obtained from the lateral segmental bronchus demonstrated noncaseating granuloma in the alveolar spaces and neutrophil infiltration (Fig. 1B). An electron microscopic examination of the biopsy specimens obtained from the bronchial mucosa showed deficiency of the inner dynein arm in the cilia (Fig. 1C).
High-resolution CT of the thorax and CT angiography. (A) Chest CT on admission. The chest CT shows complex cystic lesions with a high air-fluid level in the right lower lobe and diffuse nodular shadows in the whole lobe of the lung. (B) Chest CT after 6-month administration of clarithromycin. Reductions in both diffuse nodular shadows and the fluid in the cystic lesions are observed. (C) CT angiography after 6-month administration of clarithromycin. The CT angiography demonstrates an aberrant systemic artery arising from the celiac trunk and supplying the cystic mass lesions with systemic blood flow.
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The chest CT scan on admission showed complex cystic lesions with an air-fluid level in the right lower lobe, and diffuse centrilobular nodules in the whole lobe of the lung (Fig. 2A). In addition, the 6-month chest CT scan revealed that the diffuse nodular shadows and the fluid in cystic lesions were diminished (Fig. 2B), whereas CT angiography demonstrated an aberrant systemic artery arising from the celiac trunk and supplying the cystic mass lesions with systemic blood flow (Fig. 2C).
The pathology of the resected lung specimens. (A) Gross appearance of the resected right lower lobe. Cut sections show multiple cystic lesions without pleural covering and an aberrant artery penetrating the right lower lobe. Bar scale: 10 mm. (B) Microscopic appearance of the resected right lower lobe. The multiple cystic lesions are located in the normal lobe and lacked their own pleural covering, and an aberrant artery was found in the cystic lesion (left panel, bar scale = 5 mm). The resected lung exhibited extensive infiltrations of inflammatory cells in the wall of the cystic lesion (right panel; bar scale = 200 mum).
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