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[Mounier-Kuhn syndrome].

Dragica Pesut1, Ruza Stević, Jelica Milosavljević

  • 1Univerzitet u Beogradu, Medicinski fakultet, Beograd, Srbija. dragica.pesut@gmail.com

Vojnosanitetski Pregled
|February 23, 2012
PubMed
Summary

Mounier-Kuhn syndrome, a rare condition causing enlarged trachea and bronchi, can mimic other diseases. Advanced imaging now aids in its diagnosis, crucial for effective cardio-pulmonary management.

Area of Science:

  • Pulmonology
  • Radiology
  • Rare Diseases

Background:

  • Mounier-Kuhn syndrome (MKS), also known as tracheobronchomegaly, is a rare congenital disorder.
  • Characterized by significant dilatation of the trachea and main bronchi, often accompanied by bronchiectasis and recurrent respiratory infections.
  • MKS presents with varied symptoms, potentially mimicking other cardiopulmonary conditions.

Observation:

  • A 43-year-old non-smoking female presented with intermittent dyspnea.
  • Recurrent lower respiratory tract infections prompted a computed tomography (CT) scan.
  • CT revealed a transversal tracheal diameter of 2.8 cm, meeting diagnostic criteria for MKS.

Findings:

  • The patient's lung function and cardiac evaluations were normal.

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  • The diagnosis of Mounier-Kuhn syndrome was confirmed via CT imaging.
  • This case was sporadic, with no identified secondary causes of tracheobronchomegaly.

    • Implications:

    • Mounier-Kuhn syndrome is an important differential diagnosis in cardio-pulmonary medicine due to its diverse clinical presentations.
    • Modern imaging techniques, particularly CT, facilitate easier and more accurate diagnosis of MKS.
    • Early diagnosis of MKS is vital for appropriate patient management and to prevent complications.