Management of Non-Cystic Fibrosis Bronchiectasis in Children and Adolescents: Current Guidelines
Core Management Objectives
The primary goals are to optimize lung growth, preserve lung function, minimize exacerbations, optimize quality of life, prevent complications, and reverse structural lung injury when possible. 1
Early diagnosis and optimal treatment are critical because mild radiographic bronchiectasis is reversible if treated early, whereas untreated childhood bronchiectasis leads to worse disease and poorer prognosis in adulthood, with >60% of adults with bronchiectasis having symptoms from childhood. 1
Diagnostic Approach
Imaging Requirements
- High-resolution multidetector CT (MDCT) scans with HRCT are required for diagnosis, not chest radiography alone (up to 34% of chest radiographs are normal in CT-proven bronchiectasis). 2, 3
- Apply pediatric-specific broncho-arterial ratio (BAR) >0.8 to define abnormality, not the adult cut-off of >1.0-1.5. 2, 4
- Bronchiectasis is confirmed by bronchial dilatation showing BAR >0.8, lack of tapering, or visibility of airways within 1 cm of the costal pleura. 2, 3
Mandatory Etiological Investigation
All children with newly diagnosed bronchiectasis require systematic investigation for underlying causes. 2, 4, 3
The minimum investigation panel includes: 2, 4
- Sweat test (cystic fibrosis screening)
- Immunological assessment: total IgG, IgA, IgM, IgE, and specific antibodies to vaccine antigens
- Lower airway bacteriology (sputum or bronchoalveolar lavage when possible)
- Ciliary function evaluation when clinically indicated
- Assessment for aspiration risk and gastroesophageal reflux
- Full blood count
- Lung function tests (if age-appropriate)
Airway Clearance Techniques (Non-Pharmacological Cornerstone)
All children with bronchiectasis must be taught and receive regular airway clearance techniques, which represent the cornerstone of non-pharmacological management. 2, 4
Age-Appropriate Techniques
- Infants and young children: Gravity-assisted drainage and percussion 2, 4
- School-age children: Positive expiratory pressure (PEP) devices 2, 4
- Adolescents: Active cycle breathing or autogenic drainage 2, 4
Implementation Requirements
- Techniques must be taught by pediatric-trained chest physiotherapists. 2, 4
- Review airway clearance within 3 months of initial assessment and at least biannually thereafter. 2, 4
- During acute exacerbations, increase the frequency of airway clearance sessions beyond baseline regimen. 2, 4
Antibiotic Management
Acute Exacerbations
Treat all acute respiratory exacerbations with a 14-day course of systemic antibiotics (strong recommendation, moderate quality evidence). 2, 4
- Amoxicillin-clavulanate is the empiric antibiotic of choice, but selection should be guided by previous airway cultures and allergy history. 2, 4
- Use intravenous antibiotics when the child is hypoxic, severely ill, or fails to respond to oral therapy. 2
- Obtain lower airway specimens when possible to guide therapy. 4
Pathogen Eradication
Initiate eradication therapy promptly following initial or new detection of Pseudomonas aeruginosa (conditional recommendation, very low quality evidence). 2
- Eradication therapy is also recommended for new growth of MRSA, particularly with clinical deterioration. 3
Long-Term Antibiotic Prophylaxis
Use long-term macrolide antibiotics in children with recurrent exacerbations (strong recommendation, low quality evidence). 2, 4
- Continue for at least 6 months with regular reassessment. 4
- Obtain lower airway specimen to exclude non-tuberculous mycobacteria before commencing long-term macrolides. 4
- Monitor for antibiotic resistance development. 5
Medications to Avoid
Inhaled Corticosteroids
Do not routinely use inhaled corticosteroids in children with bronchiectasis alone (conditional recommendation against, very low quality evidence). 2
- Inhaled corticosteroids may be continued only when asthma coexists, as asthma represents a clear indication regardless of bronchiectasis presence. 2
Recombinant Human DNase
Do not use recombinant human DNase (rhDNase) routinely (strong recommendation against, very low quality evidence). 2
Vaccination Requirements
All bronchiectasis patients require annual influenza immunization and pneumococcal vaccination to reduce the risk of pneumonic exacerbations. 3
- Use 23-valent polysaccharide pneumococcal vaccine as standard. 3
- Consider 13-valent protein conjugate pneumococcal vaccine in patients without appropriate serological response to standard vaccine. 3
- Consider influenza vaccination in household contacts of immunodeficient patients to reduce secondary transmission. 3
Monitoring and Follow-Up
Assess patients at minimum annually, with more frequent monitoring (every 3-6 months) in severe disease. 2, 4, 6
Regular monitoring includes: 2, 4
- Sputum cultures to monitor for pathogen emergence, particularly P. aeruginosa
- Pulse oximetry to screen for respiratory failure
- Lung function testing when age-appropriate
- Assessment of exacerbation frequency and severity
Surgical Considerations
Surgery and lung transplantation are reserved for severe, refractory cases after failure of medical therapies. 5
Key Clinical Pitfalls
- Do not assume simple bronchiectasis without investigating underlying causes, as identifying treatable etiologies may slow or halt disease progression. 3
- Do not rely on chest radiography alone for diagnosis, as it misses up to one-third of cases. 3
- Do not use adult broncho-arterial ratio cut-offs (>1.0-1.5) in children, as this will miss cases; use pediatric cut-off of >0.8. 2, 4
- Do not delay eradication therapy for P. aeruginosa, as early treatment improves outcomes. 2, 3