Mounier Kuhn syndrome presenting with recurrent atelectasis.

Area of Science:

• Pediatric Pulmonology
• Rare Diseases
• Respiratory Medicine

Background:

• Mounier Kuhn syndrome (tracheobronchomegaly) is typically diagnosed in adults, with limited pediatric case reports.
• Recurrent respiratory infections are a common presentation in affected children.

Observation:

• A seven-year-old boy presented with recurrent pneumonia and atelectasis.
• Imaging revealed tracheobronchomegaly, consistent with Mounier Kuhn syndrome.
• Pseudomonas aeruginosa was identified in the patient's sputum.

Findings:

• The patient received antibiotic treatment guided by cystic fibrosis protocols for Pseudomonas aeruginosa.
• This therapeutic approach led to a significant decrease in pulmonary exacerbations and hospitalizations.
• This case suggests a potential treatment strategy for Pseudomonas aeruginosa infections in Mounier Kuhn syndrome.

Implications:

• Highlights the importance of considering Mounier Kuhn syndrome in children with recurrent respiratory issues.
• Demonstrates the efficacy of adapting cystic fibrosis treatment guidelines for Pseudomonas aeruginosa in Mounier Kuhn syndrome.
• Suggests a viable therapeutic option for managing pulmonary exacerbations in this rare condition.