- Imaging findings in Cystic Fibrosis:
- Chest X-ray: It may be normal in early stages Fig. 5 or may show nonspecific linear images in the lower lobes.
As the disease progresses,
thickening of these linear images and even tubular lesions with the appearance of "rail station",
characteristic of cylindrical bronchiectasis Fig. 6 are identified.
In advanced stages saccular bronchiectasis as annular images in both lungs,
sometimes full of mucous or fluid levels inside Fig. 7 Fig. 8 Fig. 9 are found.
Others common findings are hyperinflation areas and oligohemia in the periphery of the lung due to reducing pulmonary arterial perfusion Fig. 10.
In late stages of the disease are frequent retractions by fibrous scarring,
loss of volume and an important parenchymal destruction Fig. 11.
- High resolution lung CT: numerous radiological studies have reported the diagnostic superiority of CT over chest radiograph for the pulmonary involvement in CF.
The most frequently morphologic CT findings are bronchiectasis,
peribronchial wall thickening,
mosaic perfusion,
mucous plugging,
air trapping and parenchymal opacities.
The most typical finding of this disease are bronchiectasis.
The classification of Lynne
Reid that divided them into the following groups:
a) Cylindrical or tubular bronchiectasis: the bronchi have regular contours,
uniform diameter and terminating at the periphery.
Inside you can see mucus plugs Fig. 12 Fig. 13.
b) Saccular bronchiectasis: It is characterized by the presence of dilated airway with irregular contours like a rosary.
Fluid levels may occur Fig. 14 (Fig 14).
c) Varicose bronchiectasis: It is characterized by progressive dilatation to the periphery of the airways,
which end in large cysts,
bags,
or as clusters grapes (this finding is always indicative of the most severe form of bronchiectasis).
Another typical finding in these patients is the air trapping which is shown in HRCT as patchy areas of higher and lower attenuation,
and that is called "mosaic pattern" Fig. 15.
These density differences between areas are also favored by the vasoconstriction that accompanying the air trapping areas,
where there are not gas exchange and arterial vasoconstriction.
In very advanced disease is often found adhesive atelectasis due to bronchiectasis Fig. 16,
parenchyma retractions,
bullae and diffuse emphysema Fig. 17.
- Radiological findings in the acute episode:
- Consolidations or pulmonary infiltrates,
as areas of increased attenuation with or without air bronchogram .
Sometimes it is difficult to assess this finding due to the structural damage to the lung parenchyma.
Comparison with previous radiographs of the patient can help.
- Pneumothorax, due to subpleural bullae rupture,
which may occur in up to 19% of CF patients,
especially in adults Fig. 20 Fig. 21.
- Atelectasis,
lobar or segmental,
present between 5-10% of respiratory infections Fig. 22 Fig. 23.
Hemoptysis.
There are two types,
minor hemoptysis which is common and often accompanies respiratory infection episodes and greater hemoptysis (Fig 24),
in which there is bleeding of 240-300 ml/24 h and the patient's life is in danger.
In this last type,
the mortality is very high (85%).
Approximately 1% of patients with CF will have an episode of massive hemoptysis each year.
In these patients,
the realization of an CT angiography of can help locate the affected vessel and planning the treatment (Fig 25).