Cystic Fibrosis

date : 25.10.2011

Galina Kirova
Branimir Penev
Marin Penkov
Ivo Petrov

Imaging Diagnostic Deprtment; Tokuda Hospital Sofia; Vapzarov blvd.51B; 1407 Sofia

kirovag@yahoo.com

Keywords: bronchiectasis, cystic fibrosis, hemoptysis, imaging

Clinical history: 26y woman with chronic cough presented at the Emergency department with shortness of breath and massive haemoptysis. She had coughed up 250ml of fresh blood mixed with clots on the day of admission. Bronchoscopy did not locate the bleeding site but showed endobronchial infiltration of the right upper lobe.

Imaging findings:

PA CXR of the chest demonstrates enlarged lung volumes with bilateral upper lobe predominant bronchial thickening and bronchiectasis (fig.1). Axial chest CT (lung window setting) demonstrates moderate to severe bronchiectasis, thickened bronchial lumens, and mucous plugging. In the lower lung lobes hyperventilation and zones with mosaic perfusion are presented. Lung window images demonstrate large area of intraalveolar haemorrhage in the right upper lobe (fig.2,3,4). Coronal MIR reformatted image demonstrates zonal distributions of the bronchiectasis (fig.5). VR reformation MDCT angiography shows extremely dilated and tortuous bronchial arteries (fig.6).

Final diagnosis: Cystic Fibrosis with alveolar and bronchial haemorrhage

Discussion:

CF is an autosomal-recessive disease seen primarily in Caucasians, affecting many organs. It is the most common, lethal, genetically transmitted disease among children, due to a gene defect that affects chloride transport cell membranes, resulting in thick mucus. Affected patients have an increased susceptibility to infection, especially by Staphylococcus aureus and Pseudomonas aeruginosa. CT has been widely proposed as an imaging method of choice to depict morphological abnormalities of CF and the degree of disease involvement. Imaging presentation includes bronchial wall thickening, centrolobular nodularity, bronchiectasis and mucus plugs within bronchi. Bronchiectasis are most often cylindrical, but varicose and cystic bronchiectasis can be seen in advanced cases. The mucus plugs may result in atelectasis or lung consolidation distal to the plug. Over time emphysematous changes and bullae may form distal to the involved bronchi. During different follow-up periods, progression of bronchiectasis and mosaic perfusion was milder in comparison with mucous plugging. A retrospective analysis report that 9,1% of patients with CF have haemoptysis in a 5-year period. The pathogenesis of haemoptysis in CF patients has not been determined but it has been attributed to the persistent airway inflammation and vascular growth that results in markedly enlarged and tortuous bronchial arteries. These arteries contain systemic pressure and are closely adjacent to the bronchi. Chronic and acute inflammation weakens the vessel walls and can lead to episodic or persistent bleeding into the bronchial lumen. The bleeding is usually moderate, but life-threatening bleeding can occur. The Cystic Fibrosis Pulmonary Guidelines stated that patients with massive haemoptysis who is clinically unstable should be treated with bronchial artery embolization.

Differential diagnosis:

1. Allergic bronchopulmonary aspergillosis is a hypersensitivity reaction to A. fumigatus, manifested as upper lobe predominant central bronchiectasis in patients with asthma. ABPA could be superimposed in 10% of patients with Cystic Fibrosis 2. Kartagener Syndrome is presented with situs inversus, sinuitis and ciliary dysmotility syndrome

Take home messages:

CF is an autosomal-recessive disease, detected by early childhood with a sweet chlorine test. Imaging methods reveal bronchial wall thickening, bronchiectasis and mucous plugging. Bronchial artery embolization is a treatment method of choice in cases of massive bronchial bleeding.

Bibliography:

1. S.Martinez, L Heyneman, H.McAdams et al; Mucoid impactions: finger-in-glove sign and other CT radiographic features; Radiographics 2008;28:1369-13822. Helbich TH, Heinz-Peer G, Fleischmann D, et al. Evolution of CT findings in patients with cystic fibrosis. AJR Am J Roentgenol 1999; 173: 81–883. Bhalla M, Turcios N, Aponte V, et al. Cystic fibrosis: scoring system with thin-section CT. Radiology 1991; 179: 783–7884. A,Brody, H.Sucharew, J.Campbell et al; CT correlates with pulmonary exacerbations in children with Cystic Fibrosis; J of Resp and Crit Care Med; 2005;172:1128-11325. F.Accurso; Update in Cystic Fibrosis 2005; Am J Resp and Crit Care Med 2006;173:944-9476. D.Hayes, M.Winkler, S.Kikby et al; Prospectively triggered MDCT angiography in procedural planning prior to bronchial artery embolization in Cystic Fibrosis patients with hemoptysis; Chest 2011; 140:1123-11287. Cystic Fibrosis pulmonary guidelines: pulmonary complications; Hemoptysis and pneumothorax; Am J R

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