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The need to redefine non-cystic fibrosis bronchiectasis in childhood
K M Eastham1, A J Fall, L Mitchell
1Sir James Spence Institute of Child Health, Royal Victoria Infirmary, Newcastle upon Tyne NE1 4LP, UK.
Insights
Pediatric non-cystic fibrosis bronchiectasis is more common than previously thought, often diagnosed late. Some cases show resolution on high-resolution computed tomography (HRCT) scans, suggesting it may not always be permanent.
Area of Science:
- Pediatric Pulmonology
- Radiology
- Respiratory Medicine
Background:
- Non-cystic fibrosis (CF) bronchiectasis in children was considered rare and progressive in Western societies.
- This study investigates the prevalence and characteristics of non-CF bronchiectasis in a pediatric population.
Purpose of the Study:
- To evaluate the incidence and clinical features of radiologically defined non-CF bronchiectasis in children.
- To assess diagnostic delays and the utility of imaging modalities.
Main Methods:
- A retrospective review of 93 children diagnosed with non-CF bronchiectasis using high-resolution computed tomography (HRCT) scans.
- Analysis of clinical data, referral diagnoses, associated conditions, and imaging findings.
Main Results:
- Non-CF bronchiectasis represented 9.6% of new pediatric respiratory referrals.
- Previous pneumonia was the most common association (30%); diagnostic delay was significant.
- Chest radiography showed poor agreement with HRCT; HRCT demonstrated resolution in some cases.
Conclusions:
- Radiologically defined non-CF bronchiectasis is not uncommon in children.
- Diagnostic delays and the limited value of chest radiography highlight challenges in pediatric respiratory care.
- The term 'bronchiectasis' may require re-evaluation for pediatric cases due to potential for resolution.
Background:
Non-cystic fibrosis (CF) bronchiectasis has previously been reported to be rare and progressive in children living in western societies.
Method:
A clinical and radiological review was undertaken of 93 children with non-CF bronchiectasis defined by high resolution computed tomographic (HRCT) scanning presenting to a tertiary paediatric respiratory centre since 1996.
Results:
Cases constituted 9.6% of all new referrals. Male to female ratio was 2:1. Median age at symptom onset was 1.1 years (range 0-16) and of HRCT diagnosis was 7.2 years (1.6-18.8). The most common referral diagnosis of asthma was refuted in 39 of 45 cases. Associations were previous pneumonic illness (30%), immunocompromise (21%), obliterative bronchiolitis (9%), congenital lung abnormality (5%), chronic aspiration (3%), eosinophilic oesophagitis (2%), familial syndrome (2%), primary ciliary dyskinesia (1%), and right middle lobe syndrome (1%). 8% had two associated diagnoses and 18% were idiopathic. There was agreement between the chest radiograph and HRCT scan for diagnosis and lobe affected in only five cases (5%). A repeat HRCT scan in 18 cases at a minimum interval of 18 months showed total resolution of the changes in six, improvement in one, progression in five, and was unchanged in six.
Conclusions:
Radiologically defined non-CF bronchiectasis in children is not uncommon. Diagnostic delay is a problem. The most common association is a previous pneumonia. Chest radiography is of little diagnostic value, but resolution is possible on HRCT scanning. Bronchiectasis is currently defined as a condition which is both permanent and progressive. This term is not necessarily appropriate for all paediatric patients for whom we suggest an alternative nomenclature.
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