Mounier Kuhn Syndrome Presenting with Recurrent Atelectasis

Context: Mounier Kuhn syndrome is usually diagnosed in adulthood, and only a few cases have been described in childhood.
Case report: We present the case of a seven-year old boy suffering from recurrent pneumonia and atelectasis. Previously performed chest X-rays showed bilateral hyperinflation and tracheobronchomegaly. Chest computed tomography (CT) confirmed the presence of distal enlargement of trachea and bronchi. Tracheobronchomegaly associated with recurrent respiratory tract infections is consistent with Mounier Kuhn syndrome. Pseudomonas aeruginosa was isolated from the sputum of the patient. He was then treated according to the guidelines for Pseudomonas aeruginosa management in cystic fibrosis patients considering the similarities in clinical presentations and pathophysiology of both diseases. Antibiotic treatment resulted in a remarkable reduction of events of pulmonary exacerbation and hospitalizations. There are no specific guidelines for treatment options in case of pulmonary exacerbation of Mounier Kuhn syndrome. Case reports discussing the choice and efficiency of antibiotic treatment are random.
Conclusion: We share our experience of treating pulmonary exacerbation caused by Pseudomonas aeruginosa in a patient with Mounier Kuhn syndrome suggesting a possible treatment option of pseudomonas infections in this syndrome.


Christine Quentin, Nicolas Lefevre, Eddy Bodart and Laurence Hanssens

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