Abstract
Major congenital anomalies are known to play a role in the management and prognosis of airway obstruction. Most studies assess acquired forms of airway obstruction. Data on congenital or otherwise non-acquired forms of airway obstruction is sparse. In this retrospective, single-institution cohort study, we sought to evaluate and compare the patterns of airway obstruction in children with and without major congenital anomalies, and to assess the impact of management and outcome, irrespective of aetiology. Fifty-five patients were included, 23 with and 32 without underlying major congenital anomalies. Multilevel airway obstruction (usually affecting the nasopharynx, oropharynx, and the trachea) was more common in children with congenital anomalies (91% vs. 41%, p < .001). Consequently, these children required more frequent and earlier surgical management, especially tracheostomy and adenotonsillar surgery.Conclusions: Major congenital anomalies are associated with multilevel airway obstruction and poor functional prognosis. A simple clinical definition considering impact of major congenital anomalies on development and growth may help guide management plans following endoscopic evaluation of the entire airway and flanked by multidisciplinary discussions.What is Known:• Children with major comorbidities display increased disease severity and more prevalent multilevel airway obstruction• Previous studies include both children with acquired and non-acquired forms of airway obstruction; therefore, the actual impact major comorbidities in children with non-acquired causes of airway obstruction remain unclear.What is New:• A total of 42% children in this study population had major comorbidities with and impact on growth and/or psychomotor development, with a higher prevalence of multilevel airway obstruction and worse rates of functional improvement/recovery.• Children with major comorbidities require tracheostomy more often and earlier than those without major comorbidities, and remain tracheostomy-dependent for a longer time.
Highlights
Airway obstruction features reduced airflow through one or several airway levels, originating from an anatomical and/or functional size reduction of the airway lumen
Major congenital anomalies are associated with multilevel airway obstruction and poor functional prognosis
The presence of anomalies is a key element to consider in children with upper airway obstruction
Summary
Airway obstruction features reduced airflow through one or several airway levels, originating from an anatomical and/or functional size reduction of the airway lumen. When significant enough to create a turbulent airflow, airway obstruction usually manifests with respiratory noises (e.g. stridor, stertor, wheezing), as well as variable degrees of respiratory distress. The most common diagnoses in children with congenital upper airway obstruction include laryngomalacia, vocal cord paralysis, and subglottic stenosis, while subglottic haemangiomas, glottic webs, saccular cysts, and laryngotracheal clefts are less frequently encountered [1]. The presence of anomalies is a key element to consider in children with upper airway obstruction. Neurological and cardiological comorbidities and synchronous airway lesions (especially subglottic stenosis and tracheomalacia) may be associated with less favourable outcomes following supraglottoplasty among patients with severe laryngomalacia [7, 8]. Underlying neurodevelopmental problems, prematurity, cardiac, and other congenital anomalies are in turn associated with multilevel airway lesions [12, 13]
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