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.

Pulmonary Function and Flow-Volume Loop Patterns in Patients With Tracheobronchomalacia: Is There an Independent Effect?

“…[1][2][3][4] Furthermore, several patients with substantial tracheobronchomalacia from MounierKuhn syndrome, but normal lung parenchyma, are reported to have normal PFT results. 5 Flow-volume loops in COPD and asthma can also be characterized by low maximum forced expiratory flow and biphasic morphology. Could this suggest differential patterns of airways obstruction and in turn convey differential responses to interventions intended to address tracheobronchomalacia?…”

Development of Tracheobronchomegaly during Mechanical Ventilation: An Unusual Case Report / Mekanik Ventilasyon Sırasında Gelişen Trakeobronkomegali: Ender Bir Olgu Sunumu

“…First, it is thought to be the first reported case of tracheobronchomegaly that developed and was diagnosed during mechanical ventilation. A few cases of tracheobronchomegaly associated with endotracheal intubation have been reported in the literature, (8,11,13,14), but these patients were sometimes diagnosed after discharge and presented with respiratory complaints.…”

Case Report: Mounier–Kuhn Syndrome

“…Although the aetiology is uncertain, it is believed to be due to the lack of smooth muscles and elastic connective tissue in the trachea and main bronchi, leading to sacculations and formation of diverticulae between the cartilaginous rings [6,7].…”