Kuhn Syndrome Research Articles

Incidental Finding of Mounier-Kuhn Syndrome

Tracheobronchomegaly (TBM) or Mounier - Kuhn syndrome is a rare disorder of uncertain etiology characterized by marked dilatation of the trachea and bronchi and recurrent lower respiratory tract infection. We report the case of a 56-year-old male presenting with a chronic cough.

A Case of Mounier Kuhn Syndrome with Bronchial Asthma

Tracheobronchomegaly (Mounier–Kuhn syndrome) is a rare disease characterized by dilatation of the trachea and major bronchi because of atrophy or absence of elastic fibers and smooth muscle cells. The clinical signs are diverse and not very specific. Imaging provides a positive diagnosis. We present a case of 40 year old male who presented with intermittent episodes of cough with productive sputum, wheezing and breathing difficulty since last 35 years and he was treated for Bronchial Asthma since last 25 years. He was later diagnosed with Mounier–Kuhn syndrome with Bronchial Asthma by clinical history supported by Radiological evidences.

Volume Measurement in the Diagnosis of Mounier Kuhn Syndrome and an Unknown Accompanying Pathology: Pulmonary Artery Enlargement.

Mounier Kuhn Syndrome (MKS) is a rare congenital anomaly characterized by abnormal dilatation of the trachea and main bronchi. The aim of this study is to discuss tracheal volume measurement in MKS, and the pathologies accompanying MKS, especially pulmonary artery enlargement. 38 patients, 18 of whom were diagnosed with MKS and 20 as control group, were included in the study. Trachea volume and pulmonary artery diameter were measured through thorax-computed tomography (CT) images of the patients. Accompanying pathologies were recorded. In the measurements done through the CT scans, the trachea volume was found to be 25.45 cm3 in the control group and 44.17 cm3 in the patient group. The most frequent accompanying pathologies were tracheal diverticulum, bronchiectasis and pulmonary artery enlargement. In patients with MKS, there is a significant difference in volume calculation as in trachea diameter. Though bronchiectasis and tracheal diverticulum are known as pathologies most frequently accompanying MKS, to the knowledge of the researchers, pulmonary artery enlargement due to the increase in pulmonary truncus diameter was first emphasized in this article.

Mounier kuhn syndrome is another rare cause for a long standing dyspnea

A 63 y old male with long standing history of dyspnea, and recurrent cough and expectoration was initially thought to be suffering from recurrent lower respiratory tract infection (LRTI) and fibrosing alveolitis. The latest plain chest radiograph showed dilated trachea and bronchi in addition to bilateral diffuse interstitial lung disease. The chest CT scan confirmed the abnormally tracheobronchomegaly which was consistent with the diagnosis of the rare Mounier-Kuhn syndrome of an apparently a sporadic form. Such diagnosis should be considered in the differential diagnosis of settings with radiological findings suggestive of LRTI. The case also emphasizes the importance of the size of air passages on the plain chest radiograph.

The Association of Mounier-Kuhn Syndrome and Pulmonary Aspergillomas: A Case Report

A 60-year-old woman was admitted to our hospital with dyspnea, a cough and hemoptysis. She had a history of long-term treatment of asthma and recurrent lower respiratory tract infections. She had been hospitalized twice in the previous 3 years to treat non-massive hemoptysis caused by cystic bronchiectasis, but without pulmonary aspergillomas. Chest computed tomography (CT) revealed tracheobronchomegaly involving the entire trachea and main bronchi. The transverse and sagittal diameters of the trachea were 36.5 and 29.9 mm, respectively. Diverticula were evident in the posterolateral proximal tracheal wall and cystic bronchiectasis with pulmonary aspergillomas was detected in both lower lung lobes (Figure 1). Fiberoptic bronchoscopy revealed a dilated trachea, diverticula in the posterolateral region of the proximal tracheal wall, and enlargement of both main bronchi. The cartilage rings of the trachea and main bronchi were notably prominent. Mucosal atrophy of both the trachea and bronchi was also evident (Figure 2). Pathological examination of bronchial lavage specimens revealed septate hyphae typical of Aspergillus spp.; the septae branched at a 45° angle. In the absence of any secondary cause of the tracheobronchial dilation, we diagnosed Mounier–Kuhn syndrome (MKS) with tracheal diverticula and pulmonary aspergillomas. Oral itraconazole and tranexamic acid were started. Although we advised surgery to counter the probably life-threatening massive hemoptysis, the patient refused. She recovered rapidly from the hemoptysis, which did not recur. The patient continues on oral itraconazole and has been followed without complications for two months.

Mounier Kuhn Syndrome Presenting with Recurrent Atelectasis

Context: Mounier Kuhn syndrome is usually diagnosed in adulthood, and only a few cases have been described in childhood. Case report: We present the case of a seven-year old boy suffering from recurrent pneumonia and atelectasis. Previously performed chest X-rays showed bilateral hyperinflation and tracheobronchomegaly. Chest computed tomography (CT) confirmed the presence of distal enlargement of trachea and bronchi. Tracheobronchomegaly associated with recurrent respiratory tract infections is consistent with Mounier Kuhn syndrome. Pseudomonas aeruginosa was isolated from the sputum of the patient. He was then treated according to the guidelines for Pseudomonas aeruginosa management in cystic fibrosis patients considering the similarities in clinical presentations and pathophysiology of both diseases. Antibiotic treatment resulted in a remarkable reduction of events of pulmonary exacerbation and hospitalizations. There are no specific guidelines for treatment options in case of pulmonary exacerbation of Mounier Kuhn syndrome. Case reports discussing the choice and efficiency of antibiotic treatment are random. Conclusion: We share our experience of treating pulmonary exacerbation caused by Pseudomonas aeruginosa in a patient with Mounier Kuhn syndrome suggesting a possible treatment option of pseudomonas infections in this syndrome.

English

Mounier Kuhn syndrome, also known as tracheobronchomegaly is a rare idiopathic 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. The diagnosis is easily missed as tracheobronchomegaly is overlooked on plain chest x-ray. Here we present an interesting report of our patient who presented with recurrent cough and shortness of breath. A diagnosis of bronchiectasis was first thought of. Computed tomography scan revealed a final diagnosis of this rare disorder. Management of the disease is only supportive and depends on the severity of the condition.

INTERACTIVE CASE REPORT: Mounier- Kuhn Syndrome Accompanied by Cases: A Distinguishing Diagnosis of Treatment Resistant Bronchospasm

INTERACTIVE CASE REPORT: Mounier- Kuhn Syndrome Accompanied by Cases: A Distinguishing Diagnosis of Treatment Resistant Bronchospasm

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

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.

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: Anesthetic Experience

Mounier Kuhn syndrome, or congenital tracheobronchomegaly, is an under diagnosed clinical entity with peculiar anatomical and physiological features making anesthetic care challenging. A 58-year-old chronic smoker with history of recurrent pneumonia and bronchiectasis presented for septoplasty. Thoracic imaging revealed a dilated trachea and main bronchi, tracheal and bronchial diverticuli, and chronic bronchiectasis with mediastinal lymphadenopathy. An 8.5 cuffed endotracheal tube (ETT) proved too big for his glottic aperture. An 8.0 cuffed ETT with wet gauze packing yielding an adequate seal. Postoperative continuous positive airway pressure to prevent airway collapse followed awake extubation. Anesthetic concerns include grossly enlarged and weakened airways, inefficient cough mechanisms, presence of tracheal diverticuli, and post operative tracheal collapse. Anesthetic planning includes management of endotracheal cuff size. Small size yields air leak and ineffective ventilation. Large size may lead to mucosal damage. Tube dislodgement, copious secretions, chance of expiratory collapse due to the abnormally dilated and thin airways, and post operative monitoring all must be considered.

One-lung ventilation in a patient with tracheobronchomegaly: a case report and literature review

One-lung ventilation in a patient with tracheobronchomegaly: a case report and literature review

Mounier–Kuhn syndrome: A rare cause of bronchial dilatation: 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 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.

Síndroma de Mounier-Kuhn: Uma causa rara de infecções respiratórias de repetição

The incidence of the syndrome may be greater than is suspected. Patients present with recurrent respiratory infections and bronchiectasis. The symptoms are non-specific and indistinguishable from those of other chronic respiratory diseases. The diagnosis is often made several years after the first clinical complaints. The authors perform a brief review of the literature and report two cases of Mounier--Kuhn syndrome. One of the patients was diagnosed in childhood and the other in the fourth decade of life.

Double Lung Transplantation in a Patient with Tracheobronchomegaly (Mounier-Kuhn Syndrome)

Mounier-Kuhn syndrome is a rare condition characterized by marked dilation of the trachea and main bronchi resulting in bronchiectasis and emphysema. We report a case in which a patient underwent successful double lung transplantation for COPD that was found on pathologic examination of the explanted lungs to be Mounier Kuhn syndrome. To our knowledge this is the first case reporting lung transplantation in this syndrome.

Tracheal dilatation followed by stenosis in Mounier–Kuhn syndrome

A patient with previously undiagnosed Mounier-Kuhn syndrome (tracheobronchomegaly) was admitted with a head injury after a fall. The trachea was intubated with an oral tracheal tube with high-volume low-pressure cuff. The intracuff pressure was within the normal safe range recommended by the manufacturer. However, the patient developed tracheal dilatation on the second day after intubation. The trachea was extubated on the 15th day, and it was noticed 48 hours later that the patient was developing a tracheal stenosis at the site of the previous dilatation. The stenosis was so severe that the patient underwent resection-anastomosis surgery of his stenotic tracheal segment 2 months after extubation. It may be preferable in patients with Mounier-Kuhn syndrome who require mechanical ventilation to intubate the trachea with an uncuffed tube and to pack the throat to decrease the chances of gas leak and inhalation.