Pulmonary function and long-term follow-up of children with tracheobronchomalacia.

BACKGROUND

Primary tracheobronchomalacia (TBM) is a disease of the large airways. Long-term follow-up studies of TBM patients have not been reported. This study was undertaken to further elicit the natural history of this condition and the presence of concomitant reactive airways disease through clinical profiling and pulmonary function testing.

METHODS

Twenty-one children diagnosed with TBM by bronchoscopy between 1998 and 2001 in Queensland were recruited in 2008. Parents completed a questionnaire detailing their child's respiratory symptoms over the previous 12 months. Children then undertook pulmonary function and flow-volume loop classification. Mannitol bronchial provocation testing or post-bronchodilator spirometry was performed to assess for the confounding presence of reactive airways disease.

RESULTS

Data from 19 children (12 males) were able to be analyzed. The median age was 9.4 (range 7.6-14.3) years. 15 parents indicated their child's symptoms were unresolved. The mean FEV(1) was 81% predicted with 7 <80% predicted. This was significantly lower than the percent predicted population mean (P = 0.0005). Mean FEV(1) /FVC, FEF(25-75) , and PEF were also significantly reduced (P = < 0.0001). Four participants had a classical TBM flow-volume loop on analysis. One of 15 (6.7%) participants recorded a positive test for reactive airways disease.

CONCLUSIONS

Clinical symptom profiles and pulmonary function indicate persistent functional mechanical abnormalities of the large and small airways in TBM patients, and the absence of reactive airways disease.