title: Mounier-Kuhn syndrome: a rare cause of severe bronchial dilatation with normal pulmonary function test: a case report.
abstract: 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.
authors.affiliation: Pulmonologist, Baqiyatallah University of Medical Sciences, Mollasadra Street, Tehran, Iran. mghanei@excite.com
authors.forename: Mostafa
authors.initials: M
authors.lastname: Ghanei
authors.forename: Mohammadreza
authors.initials: M
authors.lastname: Peyman
authors.forename: Jafar
authors.initials: J
authors.lastname: Aslani
authors.forename: Noe
authors.initials: N
authors.lastname: Zamel
mesh_terms: D000328:Adult; D001995:Bronchography; D006801:Humans; D008297:Male; D012129:Respiratory Function Tests; D036542:Tomography, Spiral Computed; D014137:Tracheobronchomegaly / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000503:physiopathology