2 Disorders of the Airways Increase in transradiancy of a lung field. Etiology, pathophysiology, pathogenesis One of several causes may be involved: Primary congenital pulmonary artery hypoplasia MacLeod/Swyer-James syndrome, i.e., hypoplasia of pulmonary parenchyma including the territory of the pulmonary artery secondary to viral infection in the first 8 years of life prior to complete maturation of the lung Perfusion defect from reflex vasoconstriction where a central bronchial obstruction (central bronchial carcinoma, bronchial adenoma, or endobronchial foreign body) creates a ventilation defect. Modality of choice CT. Radiographic findings Hypertransradiant hemithorax with hypoperfusion Signs of hyperinflation in the presence of a central bronchial obstruction. CT findings Depend on the cause Pulmonary artery hypoplasia: Hypoplastic pulmonary arterial system with otherwise normal parenchymal texture MacLeod/Swyer–James syndrome: Mixed picture with hyperinflated and normally inflated areas, bronchiectatic tissue remodeling with thickened bronchi in collapsed, scarred lung segments Central bronchial carcinoma with bronchial and/or pulmonary artery obstruction Endobronchial obstruction due to tumor or foreign body. Pathognomonic findings Depend on the cause. Typical presentation Asymptomatic incidental finding in MacLeod/Swyer–James syndrome and in pulmonary artery hypoplasia Symptomatic in tumor cases. Therapeutic options Depend on the cause and/or underlying disorder. Course and prognosis Depend on the cause and/or underlying disorder. What does the clinician want to know? Determine the cause and specifically exclude a tumor.
Hypertransradiant Hemithorax
Definition
Imaging Signs
Clinical Aspects
Differential Diagnosis
MacLeod/Swyer–James syndrome | – CT shows a mixed picture with hyperinflated and normally inflated areas – Bronchiectatic tissue remodeling with thickened bronchi in collapsed, scarred lung segments |
Pulmonary artery hypoplasia | – More common on the left than right; hypoplastic pulmonary artery system – Hemithorax smaller on the affected side – No air trapping |
Central bronchial carcinoma | – Tumor ventilation defect with perfusion defect from reflex vasoconstriction – Paradoxical hilus sign |
Endobronchial tumor (bronchial adenoma) | – Tumor ventilation defect with perfusion defect from reflex vasoconstriction – Hyperinflation with valve mechanism; CT shows endobronchial mass blocking the lumen |
Foreign body aspiration | – Ventilation defect from foreign-body aspiration with perfusion defect from reflex vasoconstriction – Hyperinflation with valve mechanism – Air trapping – History; CT shows endobronchial foreign body |
Tips and Pitfalls
Can be confused with off-center grid, post-mastectomy, and unilateral atrophy of the pectoralis muscles.
Selected References
Lucaya J et al. Spectrum of manifestations of Swyer-James-MacLeods syndrome. J Comput Assist Tomogr 1998; 22: 592–597
Bronchiectasis
Definition
Irreversible dilation of a bronchus or bronchi, especially large and small subsegmental bronchi Reid classification differentiates cylindrical, varicose, and cystic dilation.
Etiology, pathophysiology, pathogenesis
Congenital (Kartagener syndrome, mucociliary dysfunction, cystic fibrosis, etc.) Infectious (allergic bronchopulmonary aspergillosis, measles, whooping cough, tuberculosis, etc.) Bronchial obstruction or compression (tumor, foreign body, etc.) Pulmonary fibrosis (traction bronchiectasis) and/or thickening and inflammation of the bronchial wall Destruction of the bronchial wall Peribronchial fibrosis.
Imaging Signs
Modality of choice
CT.
Radiographic findings
Nonspecific findings Pronounced striped pattern Parallel stripes (“railroad track” sign) Ring shadows.
CT findings
“Signet ring” sign (bronchial diameter exceeds the diameter of the accompanying artery) Bronchi fail to taper peripherally Bronchi can be differentiated up to a subpleural depth of 1 cm Changes in bronchial contour (cylindrical bronchi appear as tracklike parallel lines, varicose bronchi as intermittent widening of the bronchial lumina or “strings of pearls,” and cystic bronchi as bunched “clusters of grapes,” because of shrinkage Nonspecific findings include bronchial thickening, fluid or mucus-filled bronchi, loss of volume, air trapping, and nodular bronchial dilation (“tree-in-bud” sign).
Pathognomonic findings
Predilection for the posterobasal segments of the lung “Signet ring” sign and “railroad track” sign (see above) Ring shadow.
Clinical Aspects
Typical presentation
Recurrent bronchopneumonia Voluminous expectorations Hemoptysis Dyspnea.
Therapeutic options
Surgery (segmental resection or lobectomy) Pulmonary toilet Bronchospasmolytic Specific antibiotic therapy Active immunization against influenza and pneumococcus.
Course and prognosis
Severity correlates with the diameter of the abnormal bronchi (cylindrical bronchiectasis has the best prognosis, cystic bronchiectasis the worst).
What does the clinician want to know?
Diagnosis of bronchiectasis Is resection of localized bronchiectatic areas indicated? Comorbidities detected (scarring, emphysema, abscess, empyema, broncholiths, pyemia).
Differential Diagnosis
Chronic bronchitis | – Involves the entire bronchial tree |
Bronchial asthma | – No productive cough |
Bullous emphysema | – No bronchial enlargement – No “signet ring” sign |
Scarring and traction | – Not a primary respiratory disease |
bronchiectasis | – Fibrotic changes in the structure of the lung |
Tips and Pitfalls
Motion artefacts on the CT due to breathing can be misinterpreted as a “railroad track” sign Reactive bronchial dilation (“reversible bronchiectasis”) occurs in the presence of atelectasis.
Selected References
Cartier Y et al. Bronchiectasis: Accuracy of high resolution CT in the differentiation of specific diseases. AJR Am J Roentgenol 1999; 173: 47–52
King P, Song X, Rockwood K. Bronchiectasis. Intern Med J 2006; 36: 729–737
Bronchiolitis
Definition
Epidemiology
Inflammation of the peripheral respiratory bronchioles (diameter < 2 mm).
Etiology, pathophysiology, pathogenesis
Acutely infectious (viruses, Mycoplasma, chlamydia, aspergillosis) Chronic inflammatory (asthma, chronic bronchitis) Panbronchiolitis Respiratory bronchiolitis (nicotine).
Imaging Signs
Modality of choice
CT.
Radiographic findings
Usually normal Reticulonodular shadowing Pneumonic infiltrate may be present Dystelectasis.
CT findings
Centrilobular and peribronchovascular nodules “Tree-in-bud” sign (usually peripheral) Ground-glass opacities Bronchial wall thickening Air trapping.
Pathognomonic findings
None Findings such as a mosaic pattern, “tree-in-bud” sign, or centrilobular nodules are nonspecific.
Clinical Aspects
Typical presentation
Dyspnea Nonproductive cough (productive cough occurs in diffuse panbronchiolitis) Fever.
Therapeutic options
Elimination of the noxious agent Steroids (inhalational or systemic) Bronchoalveolar lavage.
Course and prognosis
Prognosis is good in infections Course is more rapid in patients with bone marrow or stem cell transplantation In severe cases respiratory insufficiency andpulmonary fibrosis may develop Prognosis is poor in diffuse panbronchiolitis.
Differential Diagnosis
Extrinsic allergic alveolitis or bronchiolitis | – No thickening of the bronchial wall – Rarely nicotine use |
Air trapping | – Extrinsic allergic alveolitis (acute stage), no bronchial wall thickening, no nicotine use – Bronchial obstruction due to foreign body or mucusretention |
Bronchiolitis obliterans with organizing pneumonia | – Nodular, usually bilateral, nonsegmental densities with air bronchogram and pleural contact |
Tips and Pitfalls
False-negative diagnosis due to overlooking discrete changes.
Selected References
Hartmann TE et al. CT of bronchial and bronchiolar diseases. Radiographics 1994; 14: 991–1003
Howling SJ et al. Follicular bronchiolitis: thin-section CT and histologic findings. Radiology 1999; 212: 637–642
Müller NL, Miller RR. Diseases of the bronchioles: CT and histopathologic findings. Radiology 1995; 196: 3–12
Bronchiolitis Obliterans
Definition
Epidemiology
Depends on the etiology. Every second lung transplant recipient develops a bronchiolitis obliterans syndrome within 5 years of transplantation.
Etiology, pathophysiology, pathogenesis
Rarely idiopathic, usually secondary to infection (mycoplasmal, viral, in children especially respiratory syncytial virus), secondary to inhalation of noxious agents, as a hypersensitivity reaction (collagen diseases), secondary to transplantation (lung, heart, bone marrow), in the setting of chronic obstructive pulmonary disease Inflammation of the respiratory bronchioles Proliferation of submucosal and peribronchial tissue leading to concentric constriction of the respiratory bronchioles.
Imaging Signs
Modality of choice
CT on inspiration and expiration (to detect air trapping).
Radiographic findings
Signs of pulmonary hyperinflation of variable severity with diminished vascularity.
CT findings
Increased transparency of the pulmonary parenchyma with narrowed vessels and signs of air trapping On expiration the density of the affected areas fails to increase Affected areas show increased transparency as on inspiration Mosaic perfusion Peribronchiolar fibrosis (rare) In postinfectious bronchiolitis there is bronchial wall thickening and bronchiectasis “Tree-in-bud” sign is rare.
Pathognomonic findings
Increased transparency of the pulmonary parenchyma with narrowed vessels Severe air trapping Mosaic perfusion.
Clinical Aspects
Typical presentation
Cough Dyspnea Fever, resembles chronic obstructive pulmonary disease but with subacute course Primarily restrictive ventilation defect due to early complete occlusion.
Therapeutic options
Steroids Immunosuppression after lung transplant.
Course and prognosis
Prognosis is good with steroid therapy.
What does the clinician want to know?
Confirmation of tentative diagnosis in at-risk patients (post bone marrow, peripheral blood stem cell, or lung transplantation).
Differential Diagnosis
Mosaic pattern | – Constrictive bronchiolitis in graft-versus-host disease – Extrinsic allergic alveolitis (acute stage): no bronchial wall thickening, not associated with smoking – Pulmonary arterial hypertension and/or pulmonary embolism: mosaic pattern without air trapping, reduced caliber of vessels in the areas of increased transparency – Nodular ground-glass infiltrates in inflammatory lung disease |
Air trapping | – Constrictive bronchiolitis in graft-versus-host disease – Extrinsic allergic alveolitis (acute stage) – Bronchial obstruction due to foreign body, mucus plug, or tumor |
Panlobular emphysema | – Destruction of lung parenchyma – Predominantly in the basal lung segments – No mosaic perfusion |
Bronchiolitis obliterans with organizing pneumonia | – Nodular, usually bilateral, nonsegmental densities with air bronchogram and pleural contact |
Tips and Pitfalls
Generalized air trapping can escape detection, as can air trapping in an uncooperative patient (recognizable by the absence of impression of the membranous part of the trachea).
Selected References
Bankier AA et al. Bronchiolitis obliterans syndrome in heart-lung transplant recipients: diagnosis with expiratory CT. Radiology 2001; 218: 533–539
Choi YW et al. Bronchiolitis obliterans syndrome in lung transplant recipients: correlation of CT findings with bronchiolitis obliterans syndrome stage. J Thorac Imaging 2003; 18: 72–79
Hansell DM et al. Obliterative bronchiolitis: individual CT signs of small airway disease and functional correlation. Radiology 1997; 203: 721–726
Chronic Bronchitis (Chronic Obstructive Pulmonary Disease)
Definition
Pulmonary disorder characterized by increased resistance to airflow FEV1/FVC <70%.