Mounier-Kuhn Syndrome (tracheobronchomegaly) is a rare disorder that affects the trachea and main bronchi.
Pathogenesis: atrophy of muscular and elastic tissues in the tracheal and main bronchial wall.
Inheritance: may be familial, often sporadic
Diagnosis usually is made in the third or fourth decade.
Clinical features:
(1) tracheobronchial dilatation with or without diverticula
(2) variable changes in pulmonary function, from minimal to severe
(3) recurrent lower respiratory tract infections (LRTIs) with or without bronchiectasis
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Location
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Cutoff on CT scan
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trachea
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> 30 mm
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right main stem bronchus
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> 20 mm
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left main stem bronchus
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> 18 mm
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Differential diagnosis:
(1) ataxia-telangiectasia
(2) Ehlers-Danlos syndrome
(3) Marfan's syndrome
(4) Kenny-Caffey syndrome
(5) Brachmann-de Lange syndrome
(6) cutis laxa)
