Bronchiectasis

Between 4% and 27% of children born with OA/TOF across various research had radiological evidence of bronchiectasis, and whilst data is lacking in older patients, one can extrapolate that this is likely to increase with age. (17)

As in other patients, signs of bronchiectasis include:

• Cough
• Daily sputum production, lasting months to years
• Haemoptysis (blood-streaked sputum) during acute exacerbations
• Dyspnoea, chest pain, wheezing, fever, fatigue and weight loss (26)

Flares of bronchiectasis may result in:

• Increased sputum
• Increased thickness of sputum
• A foul-smelling sputum occasionally
• Low-grade fever (rare)
• Increased fatigue, malaise
• Increased dyspnoea, shortness of breath, wheezing or pleuritic pain

There should be a high index of suspicion for bronchiectasis in adults with repaired OA/TOF with recurrent episodes of chest infections, and/or cough with daily purulent or mucopurulent sputum and consideration of CT scan or respiratory referral. The British Thoracic Society (BTS) recommends investigating patients with chronic purulent or mucopurulent cough and risk factors such as GORD and chronic obstructive pulmonary disease (COPD). (27)

Investigations

The BTS guideline recommends the following investigations:

1. Full blood count, urea and electrolytes, serum total IgE, specific IgE to Aspergillus fumigatus.
2. Serum immunoglobulins
3. Sputum cultures for routine and mycobacterial culture
4. CT scan (27)

• Normal CXR does not rule out bronchiectasis but may show increased pulmonary markings, honeycombing, atelectasis, tram tracking.
• Chest CT without contrast is the best way to diagnose bronchiectasis and may show parallel tram track lines, signet ring appearance, dilated bronchus lumen, cystic spaces and honeycomb appearance. There may also be bronchial wall thickening, mucous impaction and air trapping on expiratory CT. (26,27)
• Chest CT is recommended in any adult born with OA/TOF with irreversible changes on CXR. (12)
• Pulmonary function tests may show obstructive changes and a yearly decline in FEV1.

Treatment

• Supportive – smoking cessation and avoidance of second-hand smoke, immunisations for pneumonia, influenza, measles, rubeola, pertussis (whooping cough).
• Chest physiotherapy. Active cycle of breathing techniques or oscillating positive expiratory pressure should be taught to all patients with bronchiectasis, and sitting airway clearance techniques rather than postural drainage due to adults born with OA/TOF’s GORD. (27)
• Mucolytics – see TM section.
• Broad spectrum antibiotics for acute exacerbations/flares for seven to ten days, eg amoxicillin, clarithromycin.
• Regular antibiotics to control the infectious process are needed in some patients, eg macrolides. The BTS advise this for those with three or more exacerbations per year when physiotherapy or mucolytics have not improved this. (26,27)