An unusual cause of recurrent spontaneous pneumothorax: the Mounier-Kuhn syndrome.

We present a case of 63-year-old man who was referred to the emergency department with a right-sided pneumothorax. He had a history of spontaneous pneumothorax for 2 times. The chest computed tomographic scan showed tracheobronchomegaly with an increase in the diameter of the trachea and right and left main bronchus. Fiberoptic bronchoscopy revealed enlarged trachea and both main bronchus with diverticulas. These findings are consistent with a diagnosis of Mounier-Kuhn syndrome. Mounier-Kuhn syndrome is a rare clinical and radiologic condition. It is characterized by a tracheal and bronchial dilation. Diagnosis is made by computed tomography and bronchoscopy. Mounier-Kuhn syndrome should be kept in mind in the differential diagnosis of recurrent spontaneous pneumothorax.

[Tracheobronchoplasty for Severe Diffuse Tracheomalacia]. / Tracheobronchoplastie bei schwerer diffuser Tracheobronchomalazie.

Patients with diffuse airway instability due to tracheobronchomalacia or excessive dynamic airway collapse are typically highly symptomatic, with marked dyspnoea, recurrent bronchopulmonary infections and excruciating intractable cough. Silicone stents achieve immediate symptom control, but are - due to the typical complications associated with stent treatment - usually not an option for long-term treatment. The aim of surgical intervention is definitive stabilisation of the trachea and of both main bronchi by posterior splinting of the Paries membranaceus with a polypropylene mesh. This operation is an appropriate treatment option for patients with documented severe tracheobronchomalacia or excessive dynamic airway collapse and is ultimately the only therapy that can achieve permanent symptom control. The success of the operation, however, depends on many factors and requires close interdisciplinary collaboration.

Coincidence of Tracheobronchomegaly (Mounier-Kuhn Syndrome) and Juvenile Idiopathic Arthritis.

Mounier-Kuhn syndrome (MKS) or tracheobronchomegaly includes clinical and radiographic findings of tracheobronchial dilatation and recurrent respiratory infections. MKS is a very rare pathology, especially in the paediatric age group which makes it a diagnostic challenge. A 4-year-old girl suffered from dyspnea, recurrent respiratory infections and joint pain. Chest radiography detected peribronchial reinforcement and CT-scan revealed extended tracheal dilatation and bronchiectasis. In addition to MKS our patient was diagnosed with juvenile idiopathic arthritis (JIA) and scleroderma. MKS can be caused by congenital disorder or acquired aetiology. Several connective tissue diseases have been associated with MKS but no cases of JIA or scleroderma are described previously. Our case illustrates that patients who suffer from recurrent respiratory infections with unsatisfactory evolution and unspecific chest X-ray alteration, MKS always has to be considered in the differential diagnosis particularly in patients who suffer from connective tissue diseases.

A 54-Year-Old Man with Tracheomegaly, Tracheal Diverticulas and Bronchiectasis--Mounier-Kuhn Syndrome.

Mounier-Kuhn syndrome is a rare idiopathic clinical, radiological and bronchoscopic disorder characterised by abnormal dilatation of the tracheo-bronchial tree. The usual presentation is with recurrent lower respiratory tract infections. Herein, we report the case of an adult male who was diagnosed to have Mounier-Kuhn syndrome based on radiographic finding of a tracheal diameter of 45.5 mm on computed tomography and dynamic complete collapse of the tracheo-bronchial tree on forced expiration, observed during ronchoscopy.

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.

[Dyspnea and chronic recurrent respiratory tract infections]. / Dyspnoe und rezidivierende Atemwegsinfekte.

A 48-year-old man presented with recurrent bronchopulmonary infections of many years' standing. The diagnosis of tracheobronchomegaly (Mounier-Kuhn syndrome) was based on CT examination and subsequent bronchoscopy. Since lung transplantation was not considered advisable, a biluminal stent was inserted to prevent bronchial collapse.

Congenital tracheobronchomegaly (Mounier-Kuhn syndrome) in a 28-year-old Zambian male: a case report.

Congenital tracheobronchomegaly, also known as Mounier Kuhn Syndrome (MKS) is a rare respiratory disorder characterized by dilatation of the trachea and bronchi. We report a case of a 28-year-old male of African descent in Zambia, who presented with a history of chronic productive cough and repeated chest infections since childhood. He had been treated numerous times for lower respiratory tract infections, and had received empiric tuberculosis (TB) treatment based on chest radiograph findings, despite negative sputum microscopy and molecular tests for TB. Investigations revealed normal baseline blood results and sputum results. He however, had markedly increased levels of serum immunoglobulin E, and spirometry showed an obstructive pattern with significant post bronchodilator improvement. High-resolution computed tomography scan revealed tracheal dilatation, extensive bilateral bronchiectasis and tracheal and bronchial diverticula. The latter were also seen on bronchoscopy, confirming the diagnosis of Mounier-Kuhn syndrome. The patient was treated with combined inhaled corticosteroids and bronchodilators, as well as chest physiotherapy for mucus clearance, which led to improvement in his symptoms. Our case highlights how in low-resource settings, chronic lung diseases, particularly bronchiectasis, are often clinically and radiologically mistaken for and presumptively treated as TB (or its sequelae). Mounier-Kuhn syndrome, albeit rare, should be considered in the differential diagnosis of patients with recurrent lower respiratory tract infections or bronchiectasis. Multidisciplinary team meetings can help in the diagnosis of rare lung diseases.

A curious case of cough: Mounier-Kuhn syndrome in a Namibian female patient.

We report a case of a 43-year-old female who presented with a history of recurrent productive cough, since her teenage years. Her associated symptoms included dyspnoea, occasional pleuritic chest pain and rarely, constitutional symptoms. Treated numerous times for lower respiratory tract infections, her symptoms would improve after antimicrobial therapy, but always recurred. She had a background of HIV infection and was virologically suppressed on antiretroviral therapy for nine years. Investigations revealed an active pseudomonas infection and high-resolution computed tomography scan (HRCT) and bronchoscopy confirmed features of Mounier-Kuhn syndrome. The patient was treated accordingly with positive airway pressure, mucolytic agents and chest physiotherapy aimed at aiding mucus clearance and received pneumococcal and influenza vaccines. Mounier-Kuhn syndrome, though rare, should be considered in the differential diagnosis of patients with recurrent lower respiratory tract infections. In Africa, more cases may be identified and treated appropriately with timely investigation and treatment.

[Evaluation with chest CT scan of CPAP as a treatment for tracheobronchomalacia in the frame of a Mounier-Kuhn syndrome]. / Évaluation par tomodensitométrie thoracique d'un traitement de la trachéobronchomalacie par pression positive continue dans le cadre d'un syndrome de Mounier­Kuhn.

INTRODUCTION: Tracheobronchomegaly disease is often associated with a tracheobronchomalacia which is responsible for recurrent lower respiratory tract infections. Currently there is no evidence to support any specific treatment for the condition. CASE REPORT: We report the case of a 79 years old patient presenting with tracheobronchomegaly in the context of Mounier-Kuhn syndrome complicated by a tracheobronchomalacia responsible for her symptomatology. The diagnosis of tracheobronchomalacia had been confirmed by high-resolution chest computed tomography (CT) with expiratory slices and virtual bronchoscopy. Treatment with continuous positive airway pressure (CPAP) was proposed, and we confirmed its efficacy using high-resolution chest CT, which showed a decrease in tracheobronchial collapse and a reduction in air trapping. CONCLUSIONS: Three-dimensional virtual bronchoscopy is an interesting tool and a noninvasive method to diagnose tracheobronchomegaly for patients who are at a high anesthetic risk. It is also possible to use it demonstrate the effect of CPAP in tracheobronchomalacia.

Mounier-Kuhn syndrome in an older patient.

Respiratory problems in older adults are frequently labelled as being due to chronic obstructive airways disease (COAD). However where the presentation may be suggestive of another pathology due to the clinical history or type of pathogen isolated from the sputum, then consideration should be given to appropriate imaging. We describe a case of an older lady labelled for many years as COAD but who was a lifelong non-smoker and had Pseudomonas in her sputum. A CT of thorax for suspected bronchiectasis revealed tracheobroncomegaly (Mounier-Kuhn syndrome). This is the oldest case of Mounier-Kuhn syndrome at presentation and highlights the importance of appropriate history taking and investigation of older people.

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 Mimicking Lymphangioleiomyomatosis.

We present the case of a man with Mounier-Kuhn syndrome (MKS), or tracheobronchomegaly, who was referred to the National Institutes of Health Clinical Research Center because of a potential diagnosis of lymphangioleiomyomatosis (LAM), a rare condition in men. The patient was evaluated using ongoing protocols and provided written informed consent. The case demonstrates the presence of chronic inflammation surrounding the dilated airways and histologic changes of the lung parenchyma with emphysematouslike disruption in areas adjacent to the dilated airways. This finding suggests that damage to the lung parenchyma is an ongoing phenomenon in MKS. Moreover, our analysis of CT images indicates similar abnormalities in areas remote from the dilated airways. Finally, because of increased anatomic dead space, calculation of lung diffusion capacity by the single-breath method yielded abnormally low values that required making a correction for the large anatomic dead space, which can be measured by the single-breath nitrogen washout test.

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.

An Atypical Case of Mounier-Kuhn Syndrome: Time to Change the Diagnostic Approach?

Mounier-Kuhn syndrome (MKS) is a rare disease of unknown etiology characterized by abnormal pathologic dilatation of tracheabronchial tree. The diagnosis of MKS is normally made on CT scan of chest on the basis of enlarged diameters of trachea and main stem bronchi. We are presenting histologically confirmed case of MKS, where the diameter of right main bronchus is below minimum diameter (mean+3 SD) required for the diagnosis. We suggest that the diagnosis of MKS should not be solely based on fixed criteria such as the diameter of airways, but on the basis of the overall clinical, pathologic, and radiologic profile.