Bronchiectasis

Key Features

Essentials of Diagnosis

• Chronic cough with sputum production

• Rhonchi or wheezes (or both) on chest auscultation

• Diagnosis is confirmed by high-resolution CT scan

General Considerations

• Defined as the permanent dilation of bronchi resulting from airway obstruction by retained mucus secretions or inflammation in response to chronic or repeated infection

• Occurs either as a consequence of

  • Preceding illness (severe pneumonia or foreign body aspiration) or

  • Manifestation of an underlying systemic disorder (cystic fibrosis, primary ciliary dyskinesia, chronic aspiration, or immunodeficiency)

Clinical Findings

• Chronic cough, purulent sputum

• Fever

• Weight loss

• Recurrent respiratory infections and dyspnea on exertion

• Finger clubbing

• Rales, rhonchi, and decreased air entry often noted over the bronchiectatic areas

Diagnosis

Laboratory Findings

• Most common bacteria detected in cultures from the lower respiratory tract include

  • Streptococcus pneumoniae

  • Staphylococcus aureus

  • Nontypeable Haemophilus influenzae

  • Pseudomonas aeruginosa

• Nontuberculous mycobacterial species may also be detected

Imaging

• Chest radiographs

  • May be mildly abnormal with slightly increased bronchovascular markings or areas of atelectasis

  • May demonstrate cystic changes in one or more areas of the lung

• High-resolution CT

  • Best method to define extent of bronchiectasis

  • Often reveals far wider involvement of lung than expected from the chest radiograph

Diagnostic Studies

• Pulmonary function testing demonstrates airflow obstruction and air trapping

• Evaluation of lung function after use of a bronchodilator is helpful in assessing the benefit from bronchodilators

• Serial assessments of lung function help define the progression or resolution of the disease

Treatment

Medical

• Aggressive antibiotic therapy during pulmonary exacerbations and routine airway clearance is mainstay of treatmen

• Prolonged antibiotic use, anti-inflammatory therapy, hyperosmolar agents (hypertonic saline), and bronchodilators have not proven effective in non–cystic fibrosis bronchiectasis, although individual patients may benefit

• Prolonged azithromycin

  • Has been shown to reduce exacerbations in adults with non–cystic fibrosis bronchiectasis

  • Whether these results translate to children with idiopathic bronchiectasis is unknown

Surgical

• Removal of an area of lung affected with severe bronchiectasis is considered when the response to medical therapy is poor

• Other indications for surgery include

  • Severe localized disease

  • Repeated hemoptysis

  • Recurrent pneumonia in one area

• If bronchiectasis is widespread, surgical resection offers little advantage

Outcome

Prognosis

• Depends on the underlying cause and severity of bronchiectasis, the extent of lung involvement, and the response to medical management

• Good pulmonary hygiene and avoidance of infectious complications in the involved areas of lung may reverse cylindrical bronchiectasis

References