Cystic bronchiectasis - cystic fibrosis

Case contributed by Naim Qaqish
Diagnosis certain


Known bronchiectasis and mitral regurgitation presents with shortness of breath to the ED

Patient Data

Age: 40 years
Gender: Male

Multiple air cysts are seen scattered in the right hemithorax and in the left lower zone medially suggesting cystic bronchiectasis and fibrosis.

Loss of volume of the right hemithorax with an ipsilateral shifting of the mediastinum and blunting of the right costophrenic angle.

Severe fibrotic changes of the entire right lung associated with volume loss and marked ipsilateral mediastinal shift, with cystic bronchiectasis throughout (signet ring sign).

Massive compensatory hyperinflation of the left upper lobe is observed, with cystic and tractional cylindrical bronchiectasis seen mainly involving the anteromedial basal segment of the left lower lobe and, to a lesser degree, the lingula.

Few atelectatic bands of the left lower lobe.

A small air bubble is seen at the right posterolateral aspect of the trachea at the thoracic inlet level suggestive of a paratracheal air cyst or tracheal diverticulum.

No pleural effusion.

No significant hilar or mediastinal lymphadenopathy.

Case Discussion

This patient with known bronchiectasis presented with shortness of breath, upon evaluation of his x-ray it was evidently involving the entire right lung and a high-resolution chest CT was further requested for better assessment of lung disease demonstrating variable subtypes of bronchiectasis with distorted lung parenchymal architecture.

The sweat chloride test was advised by the radiology resident on call, which confirmed the diagnosis of cystic fibrosis.

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