Mounier-Kuhn Syndrome Research Articles

Mounier-Kuhn syndrome: a systematic analysis of 128 cases published within last 25 years.

Mounier-Kuhn syndrome is a rare disease with abnormal enlargement of major airways, but epidemiological studies are lacking, and currently the most available data about it come from case reports, making it difficult to collate changes in a particular patient to those in previously published cases. The aim of this work is to systematically review cases published in the last 25 years and to use descriptive statistics to summarize the patient demographic and clinical information therein in order to acquire details about patient clinical characteristics. Cases published in world literature between 1987 and 2013 were sought and reviewed according to PRISMA guidelines. Cases were included only if patient's age was at least 18 years, and a computed tomography scan with tracheal measurements was available. An 8:1 male predominance was found in 89 identified reports (128 cases). Mean age was 53.9 years, and average tracheal diameter was 36.1 mm. No correlation between increasing age and increasing tracheal diameter was found. Bronchiectasis, tracheal diverticulosis and tracheobronchial dyskinesia were common (49.2%, 33.6% and 28.9%, respectively). Cough, dyspnea and recurrent respiratory infections (71.1%, 51.6% and 50.8%, respectively) were the most common complaints. The data confirm that syndrome mostly manifests with nonspecific respiratory symptoms and is significantly more common in males. Importantly, there was no connection between age and airway diameter, a fact that could mean that the enlargement does not progress over time, and its severity depends on some other yet undetermined factors.

The Man with the Large Trachea: Mounier-Kuhn Syndrome

The Man with the Large Trachea: Mounier-Kuhn Syndrome

Mounier-Kuhn Syndrome. Imaging and Bronchoscopic Findings

Mounier-Kuhn Syndrome. Imaging and Bronchoscopic Findings

Das Mounier-Kuhn-Syndrom – eine seltene Ursache rezidivierender Bronchitiden

A 43-year-old man presented at our emergency room with progressive dyspnea and productive cough. He was in moderate respiratory distress. Symptomatic treatment had so far not led to clinical improvement. He had suffered from similar episodes since adolescence. An intensive diagnostic investigation was started to evaluate the source of infection and dyspnea. CRP levels were elevated to 26 mg/l. Arterial blood gas analysis showed a moderate hypoxemia with a pO2 of 8.35kPa (63 mmHg) and a pCO2 of 3.84kPa (29 mmHg). Computed tomography of the chest revealed the diagnosis of trachebobronchomegaly. This rare cause of recurrent bronchitis is named after Mounier-Kuhn who established this diagnosis for the first time. The patient improved under a course with antibiotics and inhaled bronchodilators. In patients with recurrent bronchitis, an underlying disease of the tracheobronchial system has to be suspected. The Mounier-Kuhn syndrome is only rarely diagnosed because the diagnosis cannot be established by conventional chest x-ray. Computed tomography is the gold standard for diagnosis. Mounier-Kuhn syndrome is likely to lead to severe venilatory failure and death, if untreated.

Anesthetic Considerations for Bilateral Lung Transplantation in Mounier-Kuhn Syndrome

OUNIER-KUHN SYNDROME (MKS), or tracheobronchomegaly, is a rare disorder of unknown etiology that is characterized by marked dilation of the trachea and major bronchi, due to severe atrophy and thinning, as well as absence of elastic fibers and smooth muscular fibers of the large airways. 1 This syndrome commonly presents in men in the third or fourth decade of life. It is marked by weakness of the membranous and cartilaginous parts of the trachea and main bronchi leading to an insufficient cough, mucous retention, and tracheobronchial wall collapse during respiration. Symptoms can range from well-preserved lung function to refractory respiratory failure. There are fewer than 100 case reports in the literature with only 2 reports of successful lung transplantation for patients with this syndrome. 1–3 Herein, the authors present a case of a patient diagnosed with MKS presenting for bilateral lung transplantation and describe the one-lung ventilation (OLV) strategies that were attempted and utilized.

Tracheomalacia treatment using a large-diameter, custom-made airway stent in a case with mounier-kuhn syndrome.

Mounier-Kuhn Syndrome (MKS) is a rare congenital disease that presents with abnormal enlargement in the central airways. In MKS, tracheomegaly is accompanied by difficulty in expelling recurrent lung infections and bronchiectasia. We presented a patient with MKS where commercially made stents were inadequate for stabilization and a custom-made, self-expandable metallic stent with a diameter of 28 mm and length of 100 mm was used. Chest pain that was thought to develop due to the stent and that disappeared after stent removal may be considered the main complication leading to stent removal. Continuous positive airway pressure therapy (CPAP) therapy was planned for the control of symptoms, which re-emerged after stent removal. This case is presented as an example that complications developing due to the stent as well as patient noncompliance may lead to stent removal, even when useful results are obtained from treatment of MKS.

Mounier-Kuhn syndrome: a rare and often overlooked cause of bronchial dilation and recurrent respiratory tract infections

It should be noted that one rare, often overlooked and underrecognized, cause of respiratory tract infections and bronchial dilation is Mounier-Kuhn syndrome (MKS). The primary feature of MKS, also known as “tracheobronchopathia malacia”, is congenital tracheal dilation accompanied by dilation of the main bronchi.

Mounier-Kuhn syndrome masquerading pulmonary thromboembolism in an elderly male

Mounier-Kuhn syndrome, also referred to as tracheobronchomegaly, is a rare idiopathic clinical and radiologic disorder characterized by significant tracheobronchial dilation. It results in recurrent lower respiratory tract infections and bronchiectasis. In severe cases, patients may present with acute respiratory distress requiring hospital admission and ventilatory support. Clinical examination and chest radiography may be misleading in these patients as tracheobronchomegaly is easily overlooked on radiographs. We present an interesting report of our patient who presented with acute shortness of breath. A diagnosis of acute pulmonary thromboembolism was suggested by initial evaluation. Computed tomography pulmonary angiography was negative for thromboembolism; however, it revealed diagnosis of this rare disorder.

Mounier-Kuhn syndrome: A rare cause of recurrent respiratory tract infections

Mounier-Kuhn syndrome: A rare cause of recurrent respiratory tract infections

Case Report: Mounier–Kuhn Syndrome

Tracheobronchomegaly (TBM) or Mounier - Kuhn syndrome is a rare disorder of uncertain aetiology characterized by marked dilatation of trachea and bronchi and recurrent lower respiratory tract infection. We report a case in a 20 year old male presenting with recurrent lower respiratory tract infection.

Recurrent pneumonia as a result of Mounier–Kuhn syndrome

British Journal of Hospital MedicineVol. 75, No. 1 Case ReportsRecurrent pneumonia as a result of Mounier–Kuhn syndromeMichael Desiderio, Jade Fettig, Vijaiganesh NagarajanMichael DesiderioSearch for more papers by this author, Jade FettigSearch for more papers by this author, Vijaiganesh NagarajanSearch for more papers by this authorMichael Desiderio; Jade Fettig; Vijaiganesh NagarajanPublished Online:28 Mar 2014https://doi.org/10.12968/hmed.2014.75.1.52AboutSectionsView articleView Full TextPDF/EPUB ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareShare onFacebookTwitterLinked InEmail View article References Bourne TM, Raphael JH, Tordoff SG (1995) Anaesthesia for a patient with tracheobronchomegaly (Mounier-Kuhn syndrome). Anaesthesia 50: 545–6 Crossref, Medline, Google ScholarCelik B, Bilgin S, Yuksel C (2011) Mounier-Kuhn syndrome: a rare cause of bronchial dilation. Tex Heart Inst J 38: 194–6 Medline, Google ScholarMeyer E, Dinkel E, Nilles A (1990) Tracheobronchomegaly: clinical aspects and radiological features. Eur J Radiol 10: 126–9 Crossref, Medline, Google ScholarNoori F, Abduljawad S, Suffin DMet al. (2010) Mounier-Kuhn syndrome: a case report. Lung 188: 353–4 Crossref, Medline, Google Scholar FiguresReferencesRelatedDetails 2 January 2014Volume 75Issue 1ISSN (print): 1750-8460ISSN (online): 1759-7390 Metrics History Published online 28 March 2014 Published in print 2 January 2014 Information© MA Healthcare LimitedPDF download

Long-Term Success after Bilateral Lung Transplantation for Mounier-Kuhn Syndrome: A Physiological Description

Long-Term Success after Bilateral Lung Transplantation for Mounier-Kuhn Syndrome: A Physiological Description

Mounier–Kuhn syndrome

a Lecturer, Department of Medical Oncology, Regional Cancer Centre, Thiruvananthapuram, India b Additional Professor, Department of Medical Oncology, Regional Cancer Centre, Thiruvananthapuram, India c Professor & HOD, Department of Medical Oncology, Regional Cancer Centre, Thiruvananthapuram, India loaded from http://zl.elsevier.es, day 16/05/2016. This copy is for personal use. Any transmission of this document by any media or format is strictly prohibited.

Mounier-Kuhn syndrome: an unusual underlying cause for chronic coughs and recurrent pneumonias

Mounier-Kuhn syndrome: an unusual underlying cause for chronic coughs and recurrent pneumonias

Mounier-Kuhn syndrome: a rare and dangerous condition

Mounier-Kuhn syndrome: a rare and dangerous condition

Management of a patient with Mounier-Kuhn syndrome undergoing repeated general anesthetics

Management of a patient with Mounier-Kuhn syndrome undergoing repeated general anesthetics

Infección respiratoria causada por Alcaligenes xylosoxidans en un paciente con síndrome de Mounier-Kuhn

Mounier-Kuhn syndrome is a rare entity characterized by abnormal dilatation of the trachea and main bronchi (tracheobronchomegaly). Alcaligenes xylosoxidans is a non fermenting gram-negative pathogen common in extra-and intra-hospital environment, which may be related to immunosuppression states. We describe the case of a 75 years old male, ex-smoker with moderate functional obstruction, chronic respiratory failure and chronic colonization by Pseudomonas aeuriginosa. He had an infectious exacerbation of his disease, reason that previously required several hospital admissions. The patient was treated with antibiotics and his evolution was favourable with negativization in cultures of the pathogen. This is the first description of the isolation of Alcaligenes xylosoxidans as a cause of respiratory infection in a patient with Mounier-Kuhn syndrome.

A child with progressive multiple tracheal diverticulae: A variation of the Mounier–Kuhn syndrome

Mounier-Kuhn syndrome is a rare disorder characterized by tracheobronchomegaly. Most commonly presenting in adults, a broad spectrum of clinical abnormalities has been described. We report a young woman followed since 4 years of age for respiratory symptoms who was eventually found to have tracheobronchomegaly and multiple tracheal diverticulae.

Mounier-Kuhn Syndrome and Bilateral Vocal Cord Paralysis

Mounier-Kuhn syndrome is a rare disorder of unknown cause that is characterized by atrophy of the elastic and smooth muscle of the tracheobronchial tree leading to tracheobronchomegaly and bronchiectasis. The syndrome is likely underdiagnosed, because the patients usually present with common respiratory symptoms such as productive cough and usually labeled as chronic obstructive pulmonary disease. Diagnosis is established on the basis of radiologic findings. Association with bilateral vocal cord paralysis has not been described. Treatment is mainly supportive. Symptomatic patients may require endobronchial stenting if airway collapse is encountered. Here, we described a patient who presented with hoarseness and pneumonia. Further studies confirmed the diagnosis of Mounier-Kuhn syndrome with bilateral vocal cord paralysis.

Central airway obstruction due to malignant fibrous histiocytoma metastasis in a case with Mounier-Kuhn syndrome

Malign fibrous histiocytoma is one of the most observed soft tissue sarcomas seen in the adults. The most common metastasis region is the lung and metastasis. Mounier-Kuhn syndrome is characterized by the highly dilatation of the trachea and bronchi. We may encounter with the major airway obstruction in the endoluminal or extraluminal lung and mediastinal masses or those with both components together. In this article, we would like to highlight the occurrence of a rare seen clinical situation secondary to the giant mediastinal malign fibrous histiocytoma metastasis and the clinical difficulties experienced in resolving of the main airway obstruction caused by the mass. Since the lack of the similar studies conducted previously, we found the case worth presenting.