What is the recommended treatment approach for a pediatric patient with bronchiectasis, potentially caused by underlying conditions such as cystic fibrosis or primary ciliary dyskinesia?

Treatment of Pediatric Bronchiectasis

For pediatric patients with bronchiectasis, implement a comprehensive treatment strategy centered on airway clearance techniques, prompt antibiotic therapy for exacerbations, investigation and management of underlying causes (particularly cystic fibrosis and primary ciliary dyskinesia), and avoidance of routine mucoactive agents like rhDNase. 1

Immediate Diagnostic Priorities

Confirm Underlying Etiology

• All children with bronchiectasis must undergo sweat testing to exclude cystic fibrosis, which occurs in 1 per 2,000-3,000 live births and is one of the most common identifiable causes in developed countries 2, 3
• Test for primary ciliary dyskinesia if there is chronic rhinosinusitis, neonatal respiratory distress, situs abnormalities, or consanguinity, as these patients have higher risk of chronic Pseudomonas aeruginosa colonization 2, 4
• Minimum investigation panel should include: chest CT scan, sweat test, lung function tests, full blood count, immunological tests (immunoglobulins and IgG subclasses), and lower airway bacteriology 2
• Immunological evaluation provides a diagnosis in approximately 42% of children when systematically investigated, with antibody deficiency syndromes accounting for 8.6-9.7% of pediatric cases 2

Core Treatment Components

Airway Clearance Techniques (ACT)

Airway clearance therapy is a cornerstone intervention that must be implemented in all pediatric bronchiectasis patients, despite very low quality evidence, due to consistent benefits and risk of harm if omitted. 1

• ACT should be individualized, age-appropriate, and taught by paediatric-trained chest physiotherapists 1
• Review ACT at least biannually to ensure proper technique and adherence 1
• Age-specific techniques include:
  • Infants: positioning, modified gravity-assisted drainage, assisted autogenic drainage 1
  • Toddlers: chest percussion with/without expiratory vibration, positive expiratory pressure (PEP) via bottle/mouthpiece/mask 1
  • Children: forced expirations, huffing, active cycle of breathing, oscillating PEP devices, bouncing on fitball 1
  • Adolescents: autogenic drainage, high-frequency chest wall oscillation, exercise, musical wind instruments 1
• Increase ACT frequency during exacerbations when airway secretions are increased 1

Antibiotic Management

For Acute Exacerbations

Treat all exacerbations with antibiotics for 14 days, with agent selection based on likely pathogens and previous culture results. 1, 4

• Obtain sputum culture before starting antibiotics to guide treatment 4
• For patients with primary ciliary dyskinesia or risk factors for Pseudomonas aeruginosa (previous colonization, ≥3 exacerbations/year, previous antibiotic use, recent hospitalization):
  • First-line oral: Ciprofloxacin 500-750 mg twice daily for 14 days 4
  • Alternative oral: Levofloxacin 500-750 mg once daily 4
  • If hospitalization required: Ceftazidime or meropenem IV 4
• For patients without Pseudomonas risk factors: Amoxicillin-clavulanate can be used, though quinolones are preferred in bronchiectasis 4
• Do not use courses less than 14 days with Pseudomonas infection, as this increases relapse risk 4

Long-term Antibiotic Prophylaxis

Consider long-term maintenance antibiotics only after optimizing airway clearance in patients with ≥3 exacerbations per year. 4

• For chronic Pseudomonas aeruginosa infection: Inhaled colistin (nebulized) is first-line 4, 5
• For patients without Pseudomonas infection: Azithromycin 250 mg three times weekly 4
• Review patients on long-term antibiotics every 6 months to evaluate efficacy, toxicity, and continued need 4
• Monitor for antimicrobial resistance, though in vitro resistance may not correlate with clinical response in vivo, particularly with P. aeruginosa 4

Mucoactive Agents

Do NOT use recombinant human DNase (rhDNase) routinely in pediatric bronchiectasis—this is a strong recommendation despite its use in cystic fibrosis. 1

Do NOT use bromhexine routinely in pediatric bronchiectasis. 1

The evidence for other mucoactive agents in non-CF bronchiectasis is insufficient to support routine use 1

Asthma-Type Therapies

The 2021 ERS guidelines address inhaled corticosteroids and bronchodilators, though specific recommendations require assessment of individual bronchodilator responsiveness and asthma-like features 1

Disease Monitoring and Follow-up

• Monitor patients every 3-6 months, with closer monitoring for severe disease 6
• Send sputum culture immediately before and after completing antibiotics to determine treatment outcome 4
• Consider periodic surveillance of colonization in patients with bronchiectasis 4
• Assess lung function regularly when age-appropriate 2

Surgical Considerations

Reserve surgery for selected patients with localized idiopathic bronchiectasis causing intolerable symptoms despite maximal medical therapy. 1, 3

Surgery and lung transplantation are reserved for severe, refractory cases after failure of medical therapies 7

Common Pitfalls to Avoid

• Do not assume bronchiectasis is irreversible in children—mild bronchiectasis may be reversible with early, aggressive treatment 8, 6
• Do not extrapolate CF treatment protocols directly to non-CF bronchiectasis (e.g., rhDNase is contraindicated) 1
• Do not neglect underlying cause investigation—52-86% of pediatric cases have identifiable causes 2
• Do not use short antibiotic courses for Pseudomonas infections 4
• Do not forget vaccinations—pneumococcal and influenza vaccines are crucial for preventing infections and complications 7

Special Populations

Tuberculosis or HIV Endemic Regions

Testing for these infections should be included in the minimum diagnostic panel 2

Neuromuscular Disorders

Use inspiratory and expiratory strategies such as breath stacking, manually assisted cough, and mechanical insufflation/exsufflation techniques 1