Tracheobronchiomegaly

Tracheobronchomegaly, or Mounier-Kuhn syndrome, is a clinical and radiological entity characterized by marked dilatation of the trachea and bronchi as a result of severe atrophy of the elastic fibers, with thinning of the muscularis, and the formation of diverticula between the cartilaginous rings. The etiopathogenesis is uncertain and may be congenital or acquired. The clinical signs are not specific and are frequently revealed by recurrent respiratory infections and chronic cough. The diagnosis of Mounier-Kuhn syndrome is based on well-documented measurements of the trachea and main bronchi performed on a chest computed tomography scan. The management of patients is based on symptomatic treatment and may require, in severe cases, the use of endoscopic treatment by stent placement or surgical tracheobronchoplasty. We present a case of a 59yearold patient with recurrent respiratory infections that required several hospitalizations. Diagnosed with Mounier Kuhn syndrome, the thoracic computed tomography scan demonstrated a dilated trachea until the bifurcation and focal points of bronchial dilatation. Bronchoscopic examination showed a dilated and deformed trachea with the presence of diverticula on the tracheal anterior wall. The diameter of the trachea was reduced by more than 50% during expiration and coughing. For this reason, Mounier-Kuhn syndrome should be considered in cases of recurrent respiratory infection or persistent respiratory symptoms.

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 refractory lower respiratory infection and haemoptysis.

The patient was a 53-year-old man with recurrent haemoptysis 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 50 mm. A bronchoscopy was performed and revealed markedly enlarged airways and traces of bleeding arising from the right airways. The haemoptysis was stopped only after bronchial artery embolization.

Mounier-Kuhn syndrome (MKS) or tracheobronchomegaly, and tracheopathia osteoplastica (TO) are rare diseases. MKS is a clinical entity characterized by abnormal dilatation of the trachea and main bronchi. TO is a clinical and pathologic condition characterized by multiple submucosal cartilaginous and osseous nodules lining the upper respiratory tract. Some people with MKS manifest recurrent pneumonia or chronic cough with sputum production, hemoptysis or dyspnea. Occasionally, it is found in asymptomatic individuals. CT scan imaging is the key tool for the diagnosis when showing a dilatation of the trachea and main bronchi. TO is usually benign and asymptomatic, frequently diagnosed incidentally during intubation. Flexible fiberoptic bronchoscopy and laryngoscopy are the key diagnostic tools which typically demonstrate irregular spicules of submucosal bone and cartilage projecting into the tracheobronchial lumen and causing various degrees of airway obstruction. When this aspect is found bronchial biopsy is not mandatory. We report the case of a 26-year-old man with features of both MKS and TO who presented a previous history of productive cough, recurrent pneumonia and dyspnea; admitted for pulmonary infection resulting in a respiratory insufficiency. MKS was diagnosed by CT scan and TO by fiberoptic bronchoscopy. To our knowledge, this is the first time these pathologies are reported in the same patient, and our patient is the second one having MKS who presented with respiratory insufficiency.

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.