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Pulmonary Lymphangiectasia: diagnostic work-up |
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| Diagnostic test |
Main features |
Comment |
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| Chest x-ray |
Hyperinflation with interstitial markings |
Radiological findings in PL may improve over time. Longitudinal follow-up pointed to the possible progression of hazy infiltrates, that are usually seen during the neonatal period, to a more perihilar interstitial pattern with varying degrees of lung inflation |
| High Resolution Computed Tomography (HRCT) |
Diffuse thickening of the interstitium, both of the peribronchovascular interstitium and the septa surrounding the lobules |
HRCT is the technique of choice for diagnosing PL |
| Magnetic Resonance Imaging (MRI) |
Coronal MRI T1 may permit to show thickening of the interstitium, pleural fluid effusion, and atelectasia. Axial MRI T2 usually shows high-signal material within the pulmonary interstitium, which is very often associated with pleural effusion. |
HRCT is better than MRI not only in diagnosing PL, but, more in geeral, for the diagnosis of pediatric interstitial lung disease. |
| Lung biopsy |
Useful for demonstrating the presence of dilated lymphatic spaces in the sub-pleural connective tissue, along thickened interlobar septa, and around bronchovascular axes |
Great caution must be taken when preparing histological specimens and when interpreting lung biopsies or autopy samples |
| Lymphoscintigraphy |
Useful for evaluating lung lymph vessel involvement by showing radiotracer accumulation in the lung and by providing evidence of back-flow within the thoracic duct |
It provides valuable morpho-functional information regarding the lymphatic system |
| Bronchoscopic evaluation and lung function tests |
Not specific |
They may be useful for ruling out other pulmonary pathologies and for carrying out bronchial lavage in order to identify and isolate respiratory pathogens |
Bellini et al. Orphanet Journal of Rare Diseases 2006 1:43 doi:10.1186/1750-1172-1-43 |
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