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Congenital airway malacia is one of the few causes of irreversible airways obstruction in children, but the incidence in the general population is unknown. Severe airway malacia or malacia associated with specific syndromes is usually recognized and diagnosed early in infancy, but information about clinical features of children with primary malacia, often diagnosed only later in childhood, is scarce.We analyzed all flexible bronchoscopies performed between 1997 and 2004 in the Sophia Children's Hospital, summarized clinical features of children with primary airway malacia, estimated the incidence of primary airway malacia, and calculated the predictive value of a clinical diagnosis of airway malacia by pediatric pulmonologists.In a total of 512 bronchoscopies, airway malacia was diagnosed in 160 children (94 males) at a median age of 4.0 years (range, 0 to 17 years). Airway malacia was classified as primary in 136 children and secondary in 24 children. The incidence of primary airway malacia was estimated to be at least 1 in 2,100. When pediatric pulmonologists expected to find airway malacia (based on symptoms, history, and lung function) prior to bronchoscopy, this was correct in 74% of the cases. In 52% of the airway malacia diagnoses, the diagnosis was not suspected prior to bronchoscopy. Presenting clinical features of children with airway malacia were variable and atypical, showing considerable overlap with features of allergic asthma. Peak expiratory flow was more reduced than FEV(1).Primary airway malacia is not rare in the general population, with an estimated incidence of at least 1 in 2,100 children. Airway malacia is difficult to recognize based on clinical features that show overlap with those of more common pulmonary diseases. We recommend bronchoscopy in patients with impaired exercise tolerance, recurrent lower airways infection, and therapy-resistant, irreversible, and/or atypical asthma to rule out airway malacia.

The particles of a new hydrofluoroalkane-134a (HFA)-beclomethasone dipropionate (BDP) metered-dose inhaler (Qvar; 3M Pharmaceuticals; St. Paul, MN) are considerably smaller than those of chlorofluorocarbon (CFC)-BDP. This may improve lung deposition in infants who inhale nasally and have irregular breathing patterns and small airways.To compare the dose delivered to the lungs of HFA-BDP and CFC-BDP at different breathing patterns using an upper airway model of an infant.An anatomically correct upper airway model of a 9-month-old child with an open nasal airway was connected to an impactor and breathing simulator. HFA-BDP, 100 microg, and CFC-BDP, 100 micro g, were delivered to the model through a detergent-coated, small-volume spacer. The total dose leaving the model (lung dose), its particle size distribution, and median mass aerodynamic diameter (MMAD) were assessed during simulated tidal breathing with tidal volumes (VTs) of 50 mL, 100 mL, and 200 mL, and 30 breaths/min. Dose was expressed as percentage of nominal dose.Lung doses for HFA-BDP were 25.4%, 26.5%, and 30.7% compared with 6.8%, 4.8%, and 2.1% for CFC-BDP at VTs of 50 mL, 100 mL, and 200 mL, respectively. The dose of particles2.1 microm to the lung for HFA-BDP was 23 to 28% compared with 0.6 to 0.8% for CFC-BDP. The lung dose of CFC-BDP mainly consisted of particles between 2.1 microm and 4.7 microm. MMAD for HFA-BDP was 1.2 microm, and 2.6 to 3.3 microm for CFC-BDP depending on VT. The lung dose for CFC-BDP decreased significantly with increasing VT. HFA-BDP lung dose did not alter significantly with VT.In this infant model study, the use of HFA-BDP with a high dose of particles2.1 microm improves the dose delivered to the lungs substantially. Furthermore, the large proportion of extra-fine particles in HFA-BDP results in lung doses less dependent on breathing pattern compared with CFC-BDP.