Children's Hospital Colorado
Aerodigestive

Pediatric Bronchiectasis

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What is pediatric bronchiectasis?

Bronchiectasis is a condition in which the small airways of the lungs become large and dilated. Bronchiectasis occurs when repeated injury to the airways makes the airway tissue irritated and inflamed. In some cases, this irritation causes the airway wall to become thick and the airway becomes larger. The abnormally large airways then collect secretions (mucus, phlegm, pus) that contribute to a chronic wet cough. Bacteria can get stuck in these airway secretions and cause recurrent infections and further contribute to the irritation and inflammation.

We often describe bronchiectasis as a cycle of airway injury: airway irritation, mucus collection and infection. When the cycle worsens, people experience increased coughing, known as an exacerbation.

What causes bronchiectasis?

Bronchiectasis may be caused by an inherited or genetic condition, such as cystic fibrosis, or repeated airway injury. People with cystic fibrosis have thick mucus that is difficult to clear from their airways. The mucus contributes to inflammation and infection that cause repeated airway injury resulting in bronchiectasis.

The amount of inflammation and irritation created by airway injury is related to the development of bronchiectasis. Some children with repeated airway injury develop bronchiectasis and some children who seem to have the same amount of injury may not develop bronchiectasis.

  • Airway injury may be caused by:
    • Repeated pneumonia or other lung infections
    • Trouble fighting infections (immunodeficiency)
    • Severe asthma
    • A very severe lung infection (called bronchiolitis obliterans)
    • Repeated aspiration (food, liquid or saliva being inhaled into the lungs)
    • Aspiration of a foreign object that becomes stuck and causes local airway injury
    • Tracheoesophageal fistula (abnormal connection between the esophagus and trachea)
  • Abnormal inflammation may be caused by:
    • Autoimmune or rheumatologic diseases
  • Abnormal mucus collection in the airways may be caused by:
    • Abnormal airway anatomy like tracheomalacia, airway stenosis and esophageal atresia that makes it hard for children to cough out mucus
    • Trouble coughing to clear mucus, which can occur in those with muscle weakness
    • Primary ciliary dyskinesia, a rare inherited disease where the cilia (hair cells) that line the airways do not move correctly to clear mucus

Some children who develop bronchiectasis may have more than one cause. It is important to determine the cause of bronchiectasis as it can affect treatment. Because it’s hard to predict who will develop bronchiectasis over time, our goal is to prevent bronchiectasis by preventing or treating airway injury and inflammation whenever possible.

Who gets bronchiectasis?

Bronchiectasis develops at different ages depending on the injury, but it may be worse in children who are older and have had repeated airway injury for a longer period. Bronchiectasis appears worse in certain ethnic groups including Aboriginal Australians and Alaska Natives. There is no clear difference in bronchiectasis by gender.

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