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Tracheomalacia and bronchomalacia in children: incidence and patient characteristics
Ruben Boogaard1, Sjoerd H Huijsmans, Marielle W H Pijnenburg
1Erasmus MC-Sophia Children's Hospital, Erasmus University Medical Centre, Department of Pediatrics, Division of Pediatric Pulmonology, Rotterdam, the Netherlands. r.boogaard@erasmusmc.nl
Insights
Congenital airway malacia, a cause of irreversible airway obstruction in children, occurs in at least 1 in 2,100. Clinical diagnosis is challenging due to overlapping symptoms with asthma, necessitating bronchoscopy for definitive diagnosis.
Area of Science:
- Pediatric Pulmonology
- Respiratory Medicine
- Pediatric Airway Disorders
Background:
- Congenital airway malacia causes irreversible airway obstruction in children.
- The incidence and clinical features of primary airway malacia are not well-established.
- Early diagnosis is crucial, especially for cases not associated with syndromes.
Purpose of the Study:
- To determine the incidence of primary airway malacia in children.
- To describe the clinical features of children with primary airway malacia.
- To evaluate the accuracy of clinical diagnosis before flexible bronchoscopy.
Main Methods:
- Analysis of flexible bronchoscopies performed between 1997 and 2004.
- Summarization of clinical features in children diagnosed with primary airway malacia.
- Estimation of primary airway malacia incidence and predictive value of clinical diagnosis.
Main Results:
- Airway malacia was diagnosed in 160 of 512 children, with 136 classified as primary.
- The incidence of primary airway malacia was estimated at a minimum of 1 in 2,100 children.
- Clinical diagnosis was correct in 74% of suspected cases, but often not suspected prior to bronchoscopy (52%).
- Clinical features were variable and overlapped with allergic asthma; peak expiratory flow was more reduced than FEV1.
Conclusions:
- Primary airway malacia is relatively common, with an incidence of at least 1 in 2,100.
- Clinical recognition is difficult due to overlapping symptoms with common respiratory conditions.
- Bronchoscopy is recommended for children with unexplained exercise intolerance, recurrent infections, or atypical asthma to rule out airway malacia.
Objective:
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.
Methods:
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.
Results:
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).
Conclusion:
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.
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