Pediatric Cystic Fibrosis

Etiology:
— Thick exocrine secretions leads to thick mucous leads to decreased mucocilliary clearance leads to bacterial colonization leads to bronchiectasis
— Thick exocrine secretions leads to thick mucous leads to mucous plugging leads to ball valving leads to air trapping
Imaging CXR:
— Follow chronic changes
— Surveil for new bacterial pneumonia and pulmonary hemorrhage
Imaging CT:
— Inspiratory CT: Look for bronchiectasis – to diagnose bronchiectasis on CT need diameter of bronchus greater than diameter of adjacent pulmonary artery
— Expiratory CT: Look for air trapping
Imaging: Sinusitis
DDX:
Complications:
— Pulmonary hemorrhage from bronchial artery hypertrophy caused by infection as focal infection can erode into artery
— Pulmonary infection (colonized by Haemophilus influenzae, Staphylococcus aureus, Pseudomonous aeruginosa (most common))
— Bronchiectasis
— Sinusitis
— Uncomplicated meconium ileus and complicated meconium ileus and distal intestinal obstruction syndrome
— Malabsorption
— Liver cirrhosis
— Pancreatic insufficiency
— Infertility
Treatment:
Clinical:

Radiology Cases of Cystic Fibrosis

CXR of pneumothorax — Upright CXR shows a distinct right pleural edge with no lung markings lateral to it and the right lung collapsing inferiorly and medially.

CXR and CT of cystic fibrosis — CXR AP and lateral (above) show bilaterally hyperexpanded lungs with a coarse interstitial pattern, bronchial wall thickening and bronchiectasis. Axial CT without contrast of the chest show thickened interlobular septa and bronchiectasis in the bilateral upper (below left) and lower (below right) lobes.

CT of bronchiectasis — Axial CT without contrast of the chest shows extensive bronchiectasis throughout the lungs.

Radiology Cases of Pulmonary Hemorrhage in Cystic Fibrosis

CXR and angiogram of pulmonary hemorrhage in cystic fibrosis — CXR (left) shows chronic changes of cystic fibrosis. Bronchial artery angiogram (right) shows hypertrophy of the right-sided bronchial arteries.

CXR of pulmonary hemorrhage after cystic fibrosis — CXR AP and lateral show bilaterally hyperexpanded lungs with a coarse interstitial pattern, bronchial wall thickening and bilateral lower lobe bronchiectasis. The opacity in the medial aspect of the right lower lobe was new when compared to prior serial CXRs.

Radiology Cases of Lung Abscess in Cystic Fibrosis

CXR of lung abscess — CXR shows a round cavitary lesion with an air-fluid level in the left upper lobe.

Radiology Cases of Chronic Fungal Pulmonary Infection Causing Osteomyelitis in Cystic Fibrosis

CXR of osteomyelitis of the rib — CXR PA and magnified view of the ribs (below) shows dense opacity in the right upper lobe and marked periosteal reaction of the right 4th rib posteriorly.

Gross Pathology Cases of Cystic Fibrosis

CXR of chest tube malposition with chest tube in the lung parenchyma — CXR AP (left) shows chronic interstitial fibrosis and scarring in the lungs, a left-sided chest tube, and a moderately-sized basilar left pleural air collection manifesting as a deep sulcus sign. Gross pathological specimen (right) shows the left chest tube entering the upper lobe of the left lung.

Gross pathological image of bronchiectasis in cystic fibrosis — Gross pathological image of a sectioned lung shows marked dilation of the airways in the middle and lower lung with many of the dilated airways containing mucous within them.