Definition
Bronchiectasis is a pathological description of a disease process that
has many possible causes. The characteristic features are abnormally
dilated thick-walled bronchi that are inflamed and chronically infected
by bacteria. It was previously thought to be irreversible but reports
are emerging which challenge this dogma (Eastham 2004).
Causes
The causes of bronchiectasis are multifactorial and include: cystic fibrosis;
primary ciliary dyskinesia; gastro-oesophageal reflux; repeat chest
infections; inhaled foreign bodies and immunodeficiencies. In some
series, no cause has been found in up to 48% of children (Edwards 2003).
Clinical Presentations
Children usually present with sputum production, wheeze or shortness
of breath. They may be clubbed. Occasionally radiological changes are
detected early in at-risk groups in the absence of symptoms.
Investigations
Computerised tomography of the chest is the gold standard for the diagnosis
where dilatation of an airway greater than the accompanying vessel
fulfills the radiological criteria for bronchiectasis. Changes may
be evident on a chest X-ray but it may not be sensitive enough to detect
mild bronchiectasis. Other investigations are aimed at excluding known
causes e.g.: sweat test, nasal biopsy, nasal nitric oxide, reflux and
aspiration studies and investigations for immunodeficiencies.
Treatment
When no specific cause is found then the treatment approach is similar
to the management of pulmonary involvement in cystic fibrosis. Patients
should be taught appropriate physiotherapy techniques and exercise
encouraged. There should be a low threshold for oral antibiotic use
in infective exacerbations. Children with severe bronchiectasis may
require regular intravenous antibiotics. Some children will be treated
with prophylactic antibiotics. The use of macrolides, such as azithromycin
(Jaffe 2001) is increasing due to their potential anti-inflammatory
properties. Occasionally, surgical resection is an option if the disease
is localised.
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Useful references:
The need to redefine non-cystic fibrosis bronchiectasis
in childhood
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Eastham KM, Fall AJ, Mitchell L, Spencer DA
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Thorax 2004;59:324-327
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Retrospective review of children presenting with non
cystic fibrosis bronchiectasis: HRCT features and clinical
relationships
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Edwards EA, Metcalfe R, Milne DG, Thompson J, Byrnes
CA
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Pediatr Pulmonol 2003;36:87-93
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Anti-inflammatory effects of macrolides in lung disease
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Jaffe A, Bush A
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Pediatr Pulmonol. 2001;31:464-73
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Web links:
NELH
- Bronchiectasis
Lung
UK
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