Overview
Definition:
Bronchiectasis is a chronic lung disease characterized by irreversible dilatation of bronchi and bronchioles, resulting from destruction of bronchial wall cartilage, elastic tissue, and smooth muscle
In children, it represents a persistent, pathological widening of airways, often leading to chronic cough, sputum production, and recurrent infections.
Epidemiology:
The incidence and prevalence of bronchiectasis in children vary globally, with higher rates in low-income countries due to increased prevalence of severe childhood infections like pneumonia and tuberculosis
Specific etiologies like cystic fibrosis, primary ciliary dyskinesia (PCD), and primary immunodeficiencies (PIDs) contribute significantly to its burden
Post-infectious causes, particularly following adenoviral or pertussis infections, are also common.
Clinical Significance:
Pediatric bronchiectasis is a serious condition impacting lung function and quality of life
Early diagnosis and effective management are crucial to prevent progressive lung damage, reduce exacerbations, and improve long-term outcomes
Understanding its diverse etiologies is paramount for targeted treatment and genetic counseling, vital for DNB and NEET SS preparation in pediatric respiratory medicine.
Etiologies
Infectious Causes:
Common infectious causes include severe or recurrent pneumonia (bacterial or viral, e.g., adenovirus, pertussis), tuberculosis, and measles
Post-infectious bronchiectasis is a significant entity, where a severe initial insult leads to airway damage and subsequent colonization.
Cystic Fibrosis Cf:
Cystic fibrosis is the most common inherited cause of bronchiectasis in children
Genetic mutations in the CFTR gene lead to thick, viscous mucus production, airway obstruction, chronic infection, and inflammation, ultimately causing bronchiectasis.
Primary Ciliary Dyskinesia Pcd:
PCD is a group of autosomal recessive disorders affecting motile cilia function
Impaired mucociliary clearance leads to chronic airway infections, sinusitis, otitis media, and situs inversus in some cases, frequently resulting in bronchiectasis.
Primary Immunodeficiencies Pids:
Conditions like Common Variable Immunodeficiency (CVID), X-linked agammaglobulinemia (XLA), and Selective IgA deficiency impair the immune system, making children susceptible to recurrent sinopulmonary infections and subsequent bronchiectasis.
Other Causes:
Less common causes include congenital anomalies (e.g., tracheobronchomegaly), foreign body aspiration, allergic bronchopulmonary aspergillosis (ABPA), connective tissue disorders, and gastroesophageal reflux disease (GERD) with aspiration.
Clinical Presentation
Symptoms:
Chronic daily cough, often productive of purulent sputum, especially in the morning
Recurrent lower respiratory tract infections (pneumonia, bronchitis)
Wheezing
Dyspnea
Hemoptysis (less common in children)
Failure to thrive or poor weight gain
Sinusitis and otitis media are common comorbidities.
Signs:
Crackles on lung auscultation, particularly at the lung bases
Clubbing of fingers (in severe or long-standing cases)
Signs of chronic respiratory distress
Possible extrapulmonary manifestations depending on etiology (e.g., nasal polyps in CF or PCD).
Diagnostic Criteria:
Diagnosis is primarily based on clinical symptoms and confirmation by high-resolution computed tomography (HRCT) of the chest, which demonstrates thickened bronchial walls, peribronchial thickening, and irreversible bronchial dilatation (CT index >1.5 or presence of airways larger than adjacent pulmonary arteries).
Diagnostic Approach
History Taking:
Detailed birth history (prematurity, meconium aspiration)
History of severe childhood infections (pertussis, measles, pneumonia)
Family history of lung disease, CF, PID, or PCD
Presence of extra-pulmonary symptoms (sinusitis, otitis, diarrhea)
Immunization status
Environmental exposures.
Physical Examination:
Complete respiratory system examination focusing on auscultation for crackles and wheezes
Assess for clubbing and signs of respiratory distress
Examine ears and sinuses for signs of chronic infection
Assess growth parameters.
Investigations:
HRCT chest is the gold standard for diagnosis and assessment of severity
Sputum culture and sensitivity (if productive) for microbiology
Pulmonary function tests (PFTs) may be useful in older children to assess airflow limitation and reversibility
Sweat chloride test for CF
Immunoglobulin levels (IgG, IgA, IgM) and lymphocyte subsets for PIDs
Ciliary beat frequency analysis or genetic testing for PCD
Allergy testing for ABPA
pH-impedance studies for GERD.
Differential Diagnosis:
Asthma, chronic bronchitis, cystic fibrosis (if initial workup is negative), recurrent pneumonia, tuberculosis, foreign body aspiration, allergic bronchopulmonary aspergillosis, immunodeficiency disorders.
Management
Airway Clearance Techniques:
The cornerstone of management, aiming to mobilize and clear secretions
Techniques include: Chest physiotherapy (CPT) - percussion, postural drainage, vibration
Positive expiratory pressure (PEP) therapy - e.g., Flutter valve, Acapella device
Autogenic drainage
Active Cycle of Breathing Techniques (ACBT)
High-frequency chest wall oscillation (HFCWO).
Pharmacological Treatment:
Antibiotics: For acute exacerbations (oral or intravenous, guided by culture and sensitivity)
Prophylactic antibiotics may be used in select cases
Bronchodilators: May be used if there is a component of reversible bronchoconstriction
Mucolytics: Such as nebulized hypertonic saline or recombinant human DNase (especially in CF)
Anti-inflammatory agents: Corticosteroids may be used in specific situations (e.g., ABPA).
Management Of Exacerbations:
Prompt treatment with antibiotics, enhanced airway clearance, and often bronchodilators
Hospitalization may be required for severe exacerbations, IV antibiotics, and respiratory support
Nutritional support is crucial.
Surgical Management:
Rarely indicated in pediatric bronchiectasis
Surgical resection of a localized, non-resolving area of bronchiectasis may be considered if there is a clear focal cause (e.g., retained foreign body) or for recurrent life-threatening hemoptysis from a specific segment.
Supportive Care:
Nutritional support is vital for growth and immune function
Pneumococcal and influenza vaccinations are recommended
Education of parents/caregivers on airway clearance techniques and recognizing signs of exacerbation is essential
Psychosocial support.
Key Points
Exam Focus:
Recognizing the diverse etiologies of bronchiectasis in children, differentiating them based on clinical presentation and initial investigations, and understanding the principles of airway clearance
HRCT is diagnostic
CF, PCD, and PIDs are critical differential diagnoses to rule out.
Clinical Pearls:
In a child with chronic cough and recurrent chest infections, always consider bronchiectasis
Thorough history taking focusing on infections, family history, and associated symptoms is crucial
Partner with respiratory therapists for effective airway clearance strategies
Regular follow-up is key to monitor progression and adjust management.
Common Mistakes:
Misdiagnosing bronchiectasis as simple asthma or recurrent pneumonia
Inadequate or inconsistent application of airway clearance techniques
Failing to investigate for underlying etiologies like CF or PIDs
Delaying antibiotic treatment for exacerbations.