What is the recommended treatment for a child with bronchiectasis?

Management of Bronchiectasis in Children and Adolescents

Children and adolescents with bronchiectasis require a comprehensive treatment approach centered on regular airway clearance techniques, prompt antibiotic treatment of exacerbations for 14 days, investigation of underlying causes, and avoidance of routine inhaled corticosteroids unless asthma coexists. 1

Core Treatment Components

Airway Clearance Techniques (ACT)

• All children with bronchiectasis must be taught and receive regular airway clearance techniques, which represent the cornerstone of non-pharmacological management 1
• ACT should be individualized based on developmental stage and age, taught by paediatric-trained chest physiotherapists, and reviewed at least biannually 1
• During acute exacerbations, increase the frequency of ACT sessions beyond the baseline regimen 1
• Age-appropriate techniques include: gravity-assisted drainage and percussion for infants/young children, positive expiratory pressure devices for school-age children, and active cycle breathing or autogenic drainage for adolescents 1

Antibiotic Management

Acute Exacerbations:

• Treat all acute respiratory exacerbations with a 14-day course of systemic antibiotics (strong recommendation, moderate quality evidence) 1
• Amoxicillin-clavulanate is the empiric antibiotic of choice, but selection should be guided by previous airway cultures and allergy history 1
• Use intravenous antibiotics when the child is hypoxic, severely ill, or fails to respond to oral therapy 1
• Obtain sputum or airway samples for culture before initiating antibiotics whenever feasible 2

Pseudomonas aeruginosa Eradication:

• Initiate eradication therapy promptly following initial or new detection of P. aeruginosa (conditional recommendation, very low quality evidence) 1
• This recommendation is based on indirect evidence from adult studies but represents critical practice given the association of Pseudomonas with increased mortality 2

Long-term Macrolide Therapy:

• Use long-term macrolide antibiotics in children with recurrent exacerbations (strong recommendation, low quality evidence) 1
• Reserve for patients with >1 hospitalized exacerbation or ≥3 non-hospitalized exacerbations in the previous 12 months 1
• Continue for at least 6 months with regular reassessment of clinical benefit 1
• Exclude non-tuberculous mycobacteria (NTM) before initiating long-term macrolides when possible 1
• Monitor for antibiotic resistance development in both the patient and community 1

Medications to Avoid or Use Selectively

Inhaled Corticosteroids (ICS):

• Do not use ICS routinely in children with bronchiectasis alone (conditional recommendation against, very low quality evidence) 1
• Exception: Continue ICS when asthma coexists, as asthma represents a clear indication regardless of bronchiectasis presence 2
• Consider ICS only in patients with documented eosinophilic airway inflammation 1

Mucoactive Agents:

• Do not use recombinant human DNase (rhDNase) routinely (strong recommendation against, very low quality evidence) - it increases exacerbation rates and worsens lung function 1
• Do not use bromhexine routinely (conditional recommendation against) 1
• Do not use hypertonic saline or inhaled mannitol routinely (conditional recommendation against) 1
• Exception for hypertonic saline/mannitol: Consider in selected patients with high daily symptoms, frequent exacerbations, difficulty expectorating, or poor quality of life 1, 3
• When using hypertonic saline (6-7%) or mannitol, always pre-treat with short-acting β2-agonists and administer the first dose under medical supervision 1, 3

Bronchodilators:

• No recommendation for routine short-acting β2-agonist (SABA) use due to lack of evidence 1
• SABA may be beneficial before airway clearance techniques or nebulized therapies to optimize delivery and prevent bronchospasm 1, 2

Investigation of Underlying Causes

• All children with newly diagnosed bronchiectasis require systematic investigation for underlying causes 1
• Essential investigations include: immunological assessment (immunoglobulin levels, vaccine responses), cystic fibrosis testing (sweat chloride, genetic testing), ciliary function evaluation when indicated, and assessment for aspiration risk 1
• Treating identified underlying conditions (e.g., primary immunodeficiency) is warranted regardless of bronchiectasis presence 1

Diagnostic Considerations

• Use high-resolution multidetector CT (MDCT) scans with HRCT for diagnosis (conditional recommendation, very low quality evidence) 1
• Apply paediatric-specific broncho-arterial ratio (BAR) >0.8 to define abnormality, not the adult cut-off of >1.0-1.5 1

Monitoring and Follow-up

• Assess patients at minimum annually, with more frequent monitoring in severe disease 2
• Obtain regular sputum cultures to monitor for pathogen emergence, particularly P. aeruginosa 2
• Review airway clearance technique within 3 months of initial assessment and annually thereafter 2
• Perform pulse oximetry to screen for respiratory failure 2

Critical Pitfalls to Avoid

• Never discontinue inhaled corticosteroids simply because bronchiectasis is present if the patient has coexisting asthma - asthma remains a clear indication 2
• Never use antibiotic courses shorter than 14 days for exacerbations, particularly with Pseudomonas infection 2
• Never extrapolate cystic fibrosis treatment protocols directly to non-CF bronchiectasis - treatment responses differ significantly 2
• Never administer nebulized hypertonic saline or mannitol without bronchodilator pre-treatment in patients with reactive airways 2, 3
• Never use rhDNase in non-CF bronchiectasis - it causes harm by increasing exacerbations 1

Special Considerations

• Vaccinations (pneumococcal and influenza) are crucial for preventing infections and complications 4
• Surgery and lung transplantation are reserved for severe, refractory cases after failure of medical therapies 4
• Early diagnosis combined with optimal management offers the prospect of reversing bronchiectasis in some patients, particularly when changes are mild 5, 6