Bronchiectasis is defined as irreversible abnormal dilatation of the bronchial tree. It has a variety of underlying causes, with a common etiology of chronic inflammation. High resolution CT is the most accurate modality for diagnosis.
As there are many causes of bronchiectasis, which may occur at essentially any age, no single demographic is particularly involved. Two groups make up the majority of cases: post-infectious and cystic fibrosis 3.
Bronchiectasis typically presents with recurrent chest infections, production of copious amounts of sputum, and hemoptysis.
Bronchiectasis is the common response of bronchi to many different insults. Causes include 1-7,9:
- post-infective (most common)
- cystic fibrosis (CF) (most common cause in children in resource-rich countries)
- congenital cystic bronchiectasis
- ciliary dysfunction syndromes, e.g. Kartagener syndrome
- bronchial cartilage deficiency, e.g. Williams-Campbell syndrome
- abnormal lung, e.g. pulmonary sequestration
- abnormal physiology, e.g. alpha-1-antitrypsin deficiency
- Mounier-Kuhn syndrome
- bronchial obstruction
- loss of surrounding lung volume
Mnemonic to remember common causes: CAPT KANGAROO HAS MOUNIER KUHN.
- HIV/AIDS: may be due to recurrent opportunistic infections such as PJP or associated with lymphocytic interstitial pneumonitis 15,16
According to macroscopic morphology, three types have been described, which also represent a spectrum of severity 8:
- cylindrical bronchiectasis
- relatively uncommon
- beaded appearances where dilated bronchi have interspersed sites of relative narrowing
- severe form with cyst-like bronchi that extend to the pleural surface
- air-fluid levels are commonly present
According to one study, the relative prevalence of bronchiectatic changes were 14:
- cylindrical: ~ 47%
- varicose: ~ 9.9%
- cystic: ~ 45.1%
- multiple types: ~ 24.3%
Additionally, bronchiectasis can result from the traction of fibrotic lung surrounding an airway. This is known as traction bronchiectasis.
There is dilation of bronchi and bronchioles due to elastic tissue and smooth muscle destruction.
Chest x-rays are usually abnormal but are inadequate in the diagnosis or quantification of bronchiectasis. Tram-track opacities are seen in cylindrical bronchiectasis, and air-fluid levels may be seen in cystic bronchiectasis. Overall there appears to be an increase in bronchovascular markings, and bronchi seen end-on may appear as ring shadows 8. Pulmonary vasculature appears ill-defined, thought to represent peribronchovascular fibrosis 8,9.
CT and HRCT especially excel at demonstrating the airways and is able to a greater or lesser degree to distinguish some of the various underlying causes.
A number of features are helpful in diagnosing bronchiectasis 8,9:
- bronchus visualized within 1 cm of pleural surface
- especially true of lung adjacent to costal pleura
- most helpful sign for early cylindrical change
- lack of tapering
- increased bronchoarterial ratio 9
- diameter of a bronchus should measure approximately 0.65-1.0 times that of the adjacent pulmonary artery branch
- between 1 and 1.5 may be seen in normal individuals, especially those living at high altitude
- greater than 1.5 indicates bronchiectasis
A number of ancillary findings are also recognized:
- bronchial wall thickening: normally wall of bronchus should be less than half the width of the accompanying pulmonary artery branch
- mucoid impaction
- air-trapping and mosaic perfusion
Signs described on CT include:
Treatment and prognosis
In general treatment of bronchiectasis is medical and involves promoting sputum clearance, using positional physiotherapy and early and aggressive treatment of pulmonary infections. In some cases, chronic prophylactic administration of antibiotics may be required.
In cases where bronchiectasis is severe and significant morbidity is present, surgical resection of the affected lobe may be of benefit provided adequate respiratory reserve exists.
In cases where both lungs are extensively involved (e.g. cystic fibrosis) lung transplantation can be considered 10.
The differential is in two parts:
- causes of particular distribution of bronchiectasis
- mimics of bronchiectasis
Causes of particular distribution of bronchiectasis
- central bronchiectasis
- upper lobe bronchiectasis
- middle lobe bronchiectasis
- lower lobe bronchiectasis
Mimics of bronchiectasis
Conditions that may mimic cystic bronchiectasis include 8: