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Mounier-Kuhn Syndrome
Tracheobronchomegaly

 

 

 

  • Congenital abnormality of the trachea and main bronchi\
  • Characterized by cystic dilatation of the tracheobronchial tree
  • Pathologic findings
    • Atrophy or absence of elastic fibers and thinning of muscle of the trachea and central bronchi
    • Rapid change to normal caliber at the 4th-5th order of bronchi
  • More common in the males
    • Especially African Americans
    • Only 5% of patients with the disease are female
  • Occurs most often during the third and fourth decades of life
  • Has an autosomal recessive type of inheritance
  • Allows trachea and main bronchi to become markedly dilated on inspiration with narrowing or collapse on expiration
  • Abnormal airway dynamics and pooling of secretions in broad outpouchings predispose to the development of
    • Pulmonary fibrosis
    • Emphysema
    • Bronchiectasis
    • Chronic pulmonary suppuration
  • Clinical findings
    • Recurrent pneumonia
    • Dyspnea
    • Cough
    • Hoarseness
    • Production of copious amounts of purulent sputum consistent with bronchiectasis
  • Signs and symptom range from minimal disease with preservation of pulmonary function to progressive disease leading to respiratory failure and death

  • Imaging Findings
    • Tracheobronchomegaly (see below)
    • Tracheal size increases with Valsalva and narrows with Muller maneuver
    • Saclike outpouchings between tracheal cartilages
    • Pulmonary fibrosis
    • Bulla
    • Bronchiectasis
  • Has been associated with Ehlers-Danlos syndrome

 

 

Normal Dimensions of Trachea and Bronchi

 

Trachea

Main Bronchi

 

Transverse

Sagittal

Right

Left

Men

>25mm

>27mm

>21.1mm

>18.4mm

Women

>21mm

>23mm

>19.8mm

>17.4mm

 

  • The diagnosis can be confirmed by computed tomography

 

Munier-Kuhn Syndrome image .jpg

Mounier-Kuhn Syndrome. Two axial CT images of the thorax demonstrate marked dilatation of the trachea (T)
and right (R) and left (L) main bronchi in this patient with Mounier-Kuhn syndrome. Notice the bronchiectasis
(red arrows and red circle) in which the bronchi are larger than their accompanying blood vessel
and there is tram=tracking of thickened bronchial walls seen in profile.
For the same photo without the arrows, click here



eMedicine   Carol A Boles, MD Wrist, Scaphoid Fractures and Complications