A decade of non-cystic fibrosis bronchiectasis 1996-2006

This study aimed to determine the aetiology, clinical presentation, co-morbidity, severity and the lobar distribution of non cystic fibrosis bronchiectasis (NCFB). We performed a retrospective review of clinical, radiological, immunological and microbiological data from 92 non-CF patients with a High resolution thoracic CT (HRCT) diagnosis of bronchiectasis in the three Dublin Children's referral Hospitals for the period 1996-2006. Of 92 patients (50 female), the median age at diagnosis was 6.4 years. The aetiology of bronchiectasis was as follows; idiopathic 29 (32%), post-pneumonia 16 (17%), immune deficiency 15 (16%), recurrent aspiration 15 (16%), primary ciliary dyskinesia 8 (9%), chronic aspiration with immune deficiency 5 (5%), post foreign body inhalation 2 (2%), tracheomalacia 1 (1%) and Obliterative bronchiolitis 1 (1%). Bronchial asthma and gastroesophageal reflux disease (GORD) were concurrently present in 18 (20%) and 10 (11%) respectively. Left lower lobe was commonly involved followed next by the right middle lobe. The common isolates were Haemophilus influenza (50), Streptococcus pneumoniae (34) and Staphylococcus aureus (14), Moraxella catarrhalis (9) and Pseudomonas auerginosa (8). Surgical interventions were performed in 23 (25%) of patients, lobectomy 11 (12%), pneumectomy 2 (2%), laryngeal cleft repair 4 (5%), rigid bronchoscopy for foreign body removal 2 (2%), Nissan's fundoplication 2 (2%), tracheoesophageal fistula repair 2 (2%). We conclude NCFB is under-recognised in Irish children and diagnosis is often delayed and Bronchial Asthma and GORD are common co morbidity. A high index of suspicion and early HRCT can expedite the diagnosis.