Mounier-Kuhn syndrome: A rare cause of severe bronchial dilatation with normal pulmonary function test: A case report

Summary Tracheobronchomegaly (TBM) (Mounier-Kuhn syndrome) is dilatation of the trachea and major bronchi because of atrophy or absence of elastic fibers and smooth muscle cells. We present a case of TBM with normal pulmonary function test (PFT). The patient was a 37-year-old man with increasing pro...

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Bibliographic Details
Published in:Respiratory medicine 2007-08, Vol.101 (8), p.1836-1839
Main Authors: Ghanei, Mostafa, Peyman, Mohammadreza, Aslani, Jafar, Zamel, Noe
Format: Article
Language:English
Subjects:
Adult
Biological and medical sciences
Bronchography
Humans
Lungs
Male
Medical sciences
Mounier-Kuhn syndrome
Pneumology
Pulmonary function test
Pulmonary/Respiratory
Respiratory Function Tests
Respiratory system : syndromes and miscellaneous diseases
Smooth muscle
Studies
Tomography, Spiral Computed
Tracheobronchomegaly
Tracheobronchomegaly - diagnosis
Tracheobronchomegaly - diagnostic imaging
Tracheobronchomegaly - physiopathology
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Summary:Summary Tracheobronchomegaly (TBM) (Mounier-Kuhn syndrome) is dilatation of the trachea and major bronchi because of atrophy or absence of elastic fibers and smooth muscle cells. We present a case of TBM with normal pulmonary function test (PFT). The patient was a 37-year-old man with increasing productive cough and without fever, wheezes, chest pain, weight loss or any respiratory disease. Chest helical computed tomography (CT) scan showed tracheomegaly with transversal diameters of the trachea of 44 mm. CT scan showed collapse of the trachea. Few large diverticular out-pouching and openings in the trachea was seen in bronchoscopy. PFT results were normal. PFT in large airway disorders may be normal while abnormalities may indicate underlying small airway disorder. An underlying small airway disorders is responsible for abnormal reports in PFT of these patients. We may need to re-evaluate the role of PFT within follow-up of patients with large airway disorder.
ISSN:0954-6111
1532-3064
DOI:10.1016/j.rmed.2007.02.005