Thoracic Computed Tomography Scan and Bronchoscopy Appearance of Mounier-Kuhn Syndrome: A Case Report.

Mounier-Kuhn syndrome (MKS) is a rare congenital disease with an autosomal recessive inheritance pattern, characterized by an enlargement of the trachea and bronchi. MKS is secondary to a thinning of the muscular mucosa and atrophy of the longitudinal muscle and elastic fibers of the tracheobronchial tree. As a consequence, tracheal diverticulosis and dilatations in the posterior membranous wall appear, along with bronchiectasis that tend to be cystic in appearance. Overall, there is an impairment of mucocilliary clearance, with an ineffective cough, which predisposes the patient to recurrent lower respiratory tract infections. Clinical manifestations vary from asymptomatic to respiratory failure and death, most patients being diagnosed between the third and fourth decades of life. It is an often undiagnosed disease, with a diagnostic algorithm that includes the use of radiological techniques, alone or in combination with bronchoscopy. Specific diagnostic criteria have been developed, based on patients' tracheal and main bronchi diameter on chest X-ray and thoracic computed tomography scan. We present the case of a 45-year-old African American man who presented with a history of multiples episodes of pneumonia that required management in the intensive care unit, on whom MKS was diagnosed.

A case of tracheobronchomegaly.

Tracheobronchomegaly (Mounier-Kuhn syndrome) is a rare condition characterized by an abnormally enlarged trachea and main bronchi. Herein, we present a case of 79-year-old male with idiopathic pulmonary fibrosis and acute hypoxemic respiratory failure due to multilobar pneumonia. Computed tomography of the chest demonstrated a markedly dilated trachea, with the transverse diameter of 31mm and the sagittal diameter of 30mm. The clinical manifestations as well as its diagnosis, classification, and treatment are discussed.

A grossly abnormal trachea- severe tracheal diverticulosis and Mounier-Kuhn syndrome.

A 53-year-old smoker presented with a history of recurrent lower respiratory tract infections. A diagnosis of Tracheal Diverticulosis due to Tracheobronchomegaly (Mounier-Kuhn Syndrome) was made. The clinical history, diagnosis and treatment options are discussed.

Mounier-Kuhn syndrome.

Mounier-Kuhn syndrome or congenital tracheobronchomegaly is a rare clinical and radiologic condition. It is characterized by a tracheal and bronchial dilation. Fewer than 100 cases have been reported in the medical literature since the original description in 1932. The first utilization of bronchoscopy for diagnosis of this condition was recorded by Lemoine only in 1949. The cause of this condition is not clearly understood; however, histopathologic findings of atrophy of smooth muscles and elastic tissue in the trachea and main bronchi have been observed. Tracheobronchomegaly can be associated with tracheal and bronchial diverticuli.

Airway stenting and tracheobronchoplasty improve respiratory symptoms in Mounier-Kuhn syndrome.

BACKGROUND: Mounier-Kuhn syndrome (MKS) is a condition characterized by tracheobronchomegaly resulting from the loss or atrophy of musculoelastic fibers within the airway wall. Concomitant tracheobronchomalacia is seen in most patients with MKS, often leading to significant respiratory compromise due to bronchiectasis, increased dead space, and impaired secretion clearance. METHODS: We report a series of 12 patients with MKS and tracheobronchomalacia who were evaluated at our institution for significant respiratory problems. Stent trials were conducted in 10 patients, with seven proceeding to operative tracheobronchoplasty (TBP) and one continuing with long-term stent placement. One patient underwent TBP without prior stent placement. Of the remaining three patients, two had no improvement with trials of stent placement, and a stent could not be placed in the third because of a large tracheal diameter. RESULTS: Compared with baseline values, clinically significant improvements in health-related quality-of-life measures and pulmonary function testing were seen in patients who underwent central airway stabilization (n = 9). Complications of both stent placement and TBP were generally mild. However, one death was reported in the surgical group secondary to an exacerbation of preexisting interstitial pneumonia. CONCLUSIONS: An aggressive approach that targets central airway stabilization may improve outcomes for patients with MKS. TRIAL REGISTRY: ClinicalTrials.gov; No.: NCT00550602; URL: www.clinicaltrials.gov.

Mounier-Kuhn syndrome: a rare cause of bronchial dilation.

Mounier-Kuhn syndrome, or tracheobronchomegaly, is a rare clinical and radiologic condition characterized by marked tracheobronchial dilation and recurrent lower respiratory tract infections. Diagnosis is typically accomplished with the use of computed tomography and bronchoscopy, as well as pulmonary function testing. Patients may be asymptomatic; however, symptoms can range from minimal with preserved lung function to severe respiratory failure. Therapy, if any, is supportive but minimal. Surgery rarely has a place in the treatment of Mounier-Kuhn syndrome.Herein, we report the case of a 58-year-old man with chronic obstructive pulmonary disease who had a chronic cough, increased sputum production, and chest pain. Thoracic computed tomography showed tracheal dilation (diameter, 34 mm) and multiple diverticula in the posterior region of the trachea. Fiberoptic bronchoscopy revealed enlarged main bronchi, the dilated trachea, and prominent tracheal diverticula. Pulmonary function testing disclosed impaired respiratory function. Histopathologic examination of biopsy specimens from the bronchi and the tracheal wall supported the diagnosis of Mounier-Kuhn syndrome. The patient was released from the hospital and his condition was monitored for 2 years, during which time he developed no lower respiratory tract infections.Regardless of radiologic findings that suggest recurrent lower respiratory tract infection, we recommend that Mounier-Kuhn syndrome be considered in the differential diagnosis.

[An unusual association: tracheobronchomegaly with a normal pulmonary function test]. / Trachéobronchomégalie avec exploration fonctionnelle respiratoire normale: une association rare.

Tracheobronchomegaly is a rare condition characterised by marked dilation of the trachea and the main bronchi. The clinical presentation of this disease is nonspecific and the diagnosis is based on the radiological features, especially computed tomography of chest. Pulmonary function tests are often abnormal showing airflow limitation with increased residual volume. The authors report a rare case of a 31-year-old man presenting tracheobronchomegaly is normal pulmonary function test.

Mounier-Kuhn syndrome: report of 8 cases of tracheobronchomegaly with associated complications.

Mounier-Kuhn syndrome is a rare congenital abnormality characterized by atrophy or absence of elastic fibers and thinning of smooth muscle layer in the trachea and main bronchi. These airways are thus flaccid and markedly dilated on inspiration and collapsed on expiration. First- to fourth-order bronchi are affected. There is an increase in dead space, tidal volume and diminished clearing of secretions. The usual presentation is recurrent respiratory tract infections with a broad spectrum of functional impairment ranging from minimal disease with preservation of lung function to severe disease in the form of bronchiectasis, emphysema and pulmonary fibrosis, ultimately culminating in respiratory failure and death. A congenital connective tissue weakness, in combination with inhalation of irritants like cigarette smoke and air pollution, are raised as possible factors in the development of this syndrome. Eight cases of tracheobronchomegaly with its associated complications are reported. Computed tomography scan of the chest was used for the diagnosis of tracheobronchomegaly. Treatment is mainly supportive with chest physiotherapy and antibiotics; however, there are a few reported cases where insertion of a tracheal stent resulted in some success.

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

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.

[Specific notch in early expiration region of a flow-volume curve in a case of Mounier-Kuhn syndrome].

A 75-year-old man presented at our hospital for evaluation of a chronic cough and sputa. Radiographic examination showed enlargement of the trachea and main bronchi. On chest radiography, the transverse diameter of the trachea was 39 mm at the level of the third thoracic spine. On bronchoscopy, the trachea and main bronchi were dilated on inspiration and were completely collapsed on expiration. A 3-D CT examination showed the trachea and main bronchi dilated, and the cartilage of the trachea and bronchi distorted. In pulmonary function testing, this disorder is characterized by the appearance of a specific notch in the early phase of expiration on the flow-volume curve.

[Treatment of tracheobronchomegaly using the Freitag prosthesis]. / Traitement de la trachéobronchomégalie par prothèse de Freitag.

Tracheobronchomegaly (TBM) is a rare disorder. It is characterised by a dilatation of the trachea and subsequent bronchial divisions associated with a dynamic pathology, in particular a cough which explains the symptomatology of the patients. We report the observation of a patient suffering from TBM whose clinical progress rapidly improved after the insertion of a Freitag prosthesis. This 60-year-old male presented with a year's history of chronic cough which was painful, cavernous and had been incapacitating associated with moderate dyspnoea of effort. Bronchial endoscopy showed dyskinesia extending almost totally throughout the tracheobronchial tree with complete expiratory collapse. The FEV1, the Vital Capacity and the TLC were subnormal and the PEF was 57% of the predicted. The total airways resistance (RAW) was elevated (306% of the predicted), the arterial blood gases were normal. A CT scan showed a deformed trachea with increased diameter. A tracheobronchial prosthesis of Freitag or Dynamic stent (Rüsch) was inserted with the help of a rigid bronchoscope. One month later the cough had regressed and the effort dyspnoea had disappeared. One year later the clinical improvement persisted with very good tolerance of the prosthesis with normal spirometry despite the persistence of an elevated RAW. Despite a recoil which is still imperfect the tracheobronchial endoprosthesis seems to be a useful treatment for forms of TBM which are potentially progressive.

Hernia of the lung repaired by VATS: a case report.

Hernia of the lung is an uncommon clinical entity. The majority of reported hernias are acquired traumatic thoracic hernias. A case of an acquired spontaneous hernia occurring through an old anterior thoracotomy scar is presented. We believe pathogenesis of this hernia was the result of increased intrathoracic pressure secondary to tracheobronchomegaly and anterior-posterior collapse of the trachea during expiration. The hernia was successfully repaired by a video-assisted thoracic surgical (VATS) technique using prolene mesh and a hernia stapler similar to the technique used in repair of an inguinal hernia.

Neonatal tracheobronchomegaly.

Tracheobronchomegaly is rarely reported in neonates. We present five cases of tracheobronchomegaly occurring in neonates receiving intensive ventilatory and oxygen support. Barotrauma is speculated to be the primary pathophysiologic factor in these cases.