Diagnosing and preventing chronic suppurative lung disease (CSLD) and bronchiectasis

Abstract

Current diagnostic labelling of childhood bronchiectasis by radiology has substantial limitations. These include the requirement for two high resolution computerised tomography [HRCT] scans (with associated adversity of radiation) if criteria is adhered to, adoption of radiological criteria for children from adult data, relatively high occurrence of false negative, and to a smaller extent false positive, in conventional HRCT scans when compared to multi-detector CT scans, determination of irreversible airway dilatation, and absence of normative data on broncho-arterial ratio in children. A paradigm presenting a spectrum related to airway bacteria, with associated degradation and inflammation products causing airway damage if untreated, entails protracted bacterial bronchitis (at the mild end) to irreversible airway dilatation with cystic formation as determined by HRCT (at the severe end of the spectrum). Increasing evidence suggests that progression of airway damage can be limited by intensive treatment, even in those predestined to have bronchiectasis (eg immune deficiency). Treatment is aimed at achieving a cure in those at the milder end of the spectrum to limiting further deterioration in those with severe 'irreversible' radiological bronchiectasis.

Figure 1

HRCT of the lower lobes of a child aged 3 years and 7 months during routine investigation for a chronic wet cough that was found to be related to common variable immunodeficiency. The HRCT shows borderline bronchiectasis. Most respiratory paediatricians considered bronchiectasis was present in this HRCT scan but two paediatric radiologists reported absence of abnormal airway dilatation (and thus absence of bronchiectasis). Following treatment (immunoglobulin replacement, intravenous antibiotics and airway clearance), the cough cleared after 2 weeks. Child is currently well (now aged 5.5 years), cough free and has no abnormality spirometry.