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[The Mounier-Kuhn syndrome]
Rade Milić1, Goran Plavec, Marko Stojisavljević
1Vojnomedicinska akademija, Klinika za plućne bolesti, Beograd, Srbija. rademilic@ptt.rs
Vojnosanitetski Pregled
|November 12, 2010
Summary
Mounier-Kuhn syndrome (MKS), a rare tracheobronchomegaly, causes airway dilation and recurrent infections. Multislice computed tomography (MSCT) is key for diagnosing MKS, often missed due to similar symptoms to COPD.
Area of Science:
- Pulmonology
- Radiology
- Rare Diseases
Background:
- Mounier-Kuhn syndrome (MKS), also known as tracheobronchomegaly (TBM), is a rare condition characterized by significant dilation of the trachea and major bronchi.
- It often leads to recurrent respiratory infections, bronchiectasis, and lung parenchymal scarring. Associated colonic enlargement may also occur.
Observation:
- Two patients, aged 77 and 72, presented with typical MKS symptoms and enlarged upper airways.
- Diagnosis was confirmed via chest multislice computed tomography (MSCT), revealing tracheal diameters of 30 mm and 33 mm.
- Observed complications included tracheal diverticulosis, pulmonary fibrosis, emphysema, and bronchiectasis.
Findings:
- Lung function tests indicated mixed ventilation disorders.
- Arterial blood gas analysis showed disturbances in respiratory gas values.
- MSCT is the gold standard for diagnosing MKS, which is frequently misdiagnosed as COPD or bronchiectasis.
Implications:
- Early and accurate diagnosis of MKS is crucial, despite its rarity and often missed diagnosis.
- MSCT imaging is essential for definitive diagnosis of tracheobronchomegaly.
- Current therapeutic approaches for MKS are primarily supportive.
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