Tracheobronchiomegaly (Mounier-Kuhn Syndrome)

Mc, Beachley; Gg, Ghahremani

doi:10.1097/00007611-197609000-00038

Cited by 2 publications

(1 citation statement)

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“…9 In addition recurrent episodes of pneumonia lead to a progressive loss of pulmonary function. 10 But there is no definite description of PFT due to Mounier-Kuhn syndrome while there are some various reports in type and pattern of this test.…”

Section: Introductionmentioning

confidence: 99%

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

Ghanei

Peyman

Aslani

et al. 2007

Respiratory Medicine

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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 44mm. 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.

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Section: Introductionmentioning

confidence: 99%