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Published on: June 23, 2023
Airway obstruction and inflammation on combined bronchoscopy in children with Down syndrome
Monica Vielkind1, Kristine Wolter-Warmerdam2, Arwen Jackson3
1Division of Pulmonary Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
Insights
Children with Down syndrome often have airway abnormalities, including tracheomalacia and subglottic stenosis. Combined flexible bronchoscopy and microlaryngoscopy improve diagnosis of these respiratory issues.
Area of Science:
- Pediatric Pulmonology
- Genetics
- Otolaryngology
Background:
- Children with Down syndrome frequently experience chronic respiratory symptoms.
- Airway abnormalities are a common concern in this population.
Purpose of the Study:
- To characterize upper and lower airway findings in children with Down syndrome and chronic respiratory symptoms.
- To evaluate the diagnostic yield of combined flexible bronchoscopy (FB) and microlaryngoscopy with bronchoscopy (MLB).
Main Methods:
- Retrospective review of 168 children with Down syndrome (age 1 month-17 years) who underwent both FB and MLB.
- Analysis of anatomical airway findings, bronchoalveolar lavage fluid (BALF) culture results, cell differential, and cytopathology.
- Comparison of BALF results between children with and without dysphagia.
Main Results:
- 96% of patients had at least one abnormal airway finding; 46% had at least three.
- Common findings included tracheomalacia (39% FB; 37% MLB), subglottic stenosis (35% MLB), pharyngomalacia (32% FB), and laryngomalacia (16% FB; 30% MLB).
- Children with dysphagia showed more frequent positive BALF cultures for mixed flora (76% vs. 47%) and higher neutrophil percentages (20% vs. 7%).
Conclusions:
- Abnormal airway findings are highly prevalent in children with Down syndrome presenting with chronic respiratory symptoms.
- Performing flexible bronchoscopy and microlaryngoscopy with bronchoscopy together enhances diagnostic yield for these patients.
Objective:
To characterize the upper and lower airway findings in children with Down syndrome and chronic respiratory symptoms, based on evaluation by flexible bronchoscopy (FB) with bronchoalveolar lavage and microlaryngoscopy with bronchoscopy (MLB).
Study Design:
A retrospective review was conducted of children with Down syndrome aged 1 month to 17 years, who underwent both FB and MLB within a 1-year timeframe between 2010 and 2019 at Children's Hospital Colorado. Anatomic airway findings are reported as frequencies within the cohort. Bronchoalveolar lavage fluid (BALF) culture results, cell differential, and cytopathology are reported as frequencies or mean ± standard deviation. BALF results were compared between children with and without dysphagia documented on a recent swallow evaluation.
Results:
Overall, 168 children with Down syndrome were included, with median age of 2.1 years (interquartile range: 0.9-5.1 years). At least one abnormal airway finding was recorded in 96% of patients and 46% had at least three abnormal findings. The most common findings included tracheomalacia (39% FB; 37% MLB), subglottic stenosis (35% MLB), pharyngomalacia (32% FB), and laryngomalacia (16% FB; 30% MLB). Comparison of BALF based on dysphagia status showed that children with dysphagia had more frequent cultures positive for mixed upper respiratory flora (76% vs. 47%, p = 0.004) and a higher percentage of neutrophils (20% vs. 7%, p = 0.006).
Conclusion:
Abnormal findings for FB and MLB are common in children with Down syndrome and chronic respiratory symptoms, and performing the procedures together may increase the diagnostic yield.
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