Infecciones respiratorias recurrentes en paciente escolar, asociadas a Síndrome de Mounier-Khün: reporte de caso / Recurrent respiratory infections associated with Mounier-Khün Syndrome in a school patient: clinical case report / Infecções respiratórias recorrentes associadas à Síndrome de Mounier-Khün num paciente escolar: relato do caso

El síndrome de Mounier Kühn es una patología infrecuente de la vía aérea, caracterizada por una dilatación anormal de tráquea y bronquios. Se debe sospechar ante la presencia de infecciones broncopulmonares recurrentes e irritación traqueobronquial. El diagnóstico se lleva a cabo a través de la medición del diámetro traqueal en tres segmentos de su anatomía, a través de tomografía torácica. Se presenta el caso de un paciente masculino escolar de 10 años de edad, con antecedentes de infecciones respiratorias recurrentes, hospitalizado por un cuadro neumónico; los hallazgos reportados en la tomografía de tórax corresponden a traqueobroncomegalia además de incremento del diámetro esofágico. El tratamiento de esta entidad es sintomático con medidas de sostén y fisioterapia pulmonar.

Mounier Kühn syndrome is a rare airway pathology characterized by abnormal dilatation of the trachea and bronchi. It should be suspected in the presence of recurrent bronchopulmonary infections and tracheobronchial irritation. The diagnosis is made by measuring the tracheal diameter in three segments of its anatomy, through thoracic tomography. We present the case of a 10 year-old male school boy with a history of recurrent respiratory infections, hospitalized for a pneumonic condition; the findings reported in the thoracic tomography correspond to a tracheobronchomegaly in addition to an increase of the esophageal diameter. The treatment of this entity is symptomatic with supportive measures and pulmonary physical therapy.

A síndrome de Mounier Kühn é uma patologia incomumdas vias aéreas, caracterizada por dilatação anormal da traqueia e brônquios. Devese suspeitar na presença de infecções broncopulmonares recorrentes e irritação traqueobrônquica. O diagnóstico é realizado através da medida do diâmetro traqueal em três segmentos de sua anatomia, através da tomografia de tórax. Apresentamos o caso de um paciente escolar de 10 anos de idade, do sexo masculino, com história de infecções respiratórias de repetição, internado por sintomas pneumônicos; os achados relatados na tomografia de tórax correspondem a uma traqueobroncomegalia, além de aumento do diâmetro esofágico. O tratamento dessa entidade é sintomático com medidas de suporte e fisioterapia pulmonar.

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.

Flexible bronchoscopy and mechanical ventilation in managing Mounier-Kuhn syndrome: a case report / Broncoscopia flexível e ventilação mecânica no tratamento da síndrome de Mounier-Kuhn: relato de caso

ABSTRACT CONTEXT: Mounier-Kuhn syndrome is a rare congenital condition with distinct dilatation and diverticulation of the tracheal wall. The symptoms may vary and the treatment usually consists of support. CASE REPORT: The patient was a 60-year-old male with recurrent hospital admission. He was admitted in this case due to dyspnea, cough and sputum production. An arterial blood sample revealed decompensated respiratory acidosis with moderate hypoxemia. A chest computed tomography (CT) scan showed dilatation of the trachea and bronchi, tracheal diverticula and bronchiectasis. Flexible bronchoscopy was performed, which revealed enlarged airways with expiratory collapse. Furthermore, orifices of tracheal diverticulosis were also detected. Non-invasive positive pressure ventilation (NPPV) was added, along with long-term oxygen therapy. At control visits, the patient's clinical and laboratory findings were found to have improved. CONCLUSION: Flexible bronchoscopy can be advocated for establishing the diagnosis and non-invasive mechanical ventilation can be used with a high success rate, for clinical wellbeing in Mounier-Kuhn syndrome.

RESUMO CONTEXTO: A síndrome de Mounier-Kuhn é uma condição congênita rara com dilatação e diverticulação distintas da parede traqueal. Os sintomas podem ser variáveis ​e o tratamento geralmente é de suporte. RELATO DE CASO: Paciente do sexo masculino, de 60 anos, com internação hospitalar recorrente, foi internado neste caso devido a dispneia, tosse e produção de expectoração. A amostra de sangue arterial revelou acidose respiratória descompensada, com hipoxemia moderada. A tomografia computadorizada de tórax mostrou dilatação da traqueia e brônquios, divertículos traqueais e bronquiectasias. Realizou-se broncoscopia flexível, que revelou aumento das vias aéreas com colapso expiratório. Além disso, também foram detectados orifícios de diverticulose traqueal. Foi adicionada ventilação com pressão positiva não invasiva (NPPV) juntamente com a oxigenoterapia a longo prazo. Foram verificadas melhoras dos resultados clínicos e laboratoriais do doente nas visitas de controle. CONCLUSÃO: A broncoscopia flexível pode ser defendida para estabelecer o diagnóstico, e a ventilação mecânica não invasiva pode ser utilizada com alta taxa de sucesso, para bem-estar clínico, na síndrome de Mounier-Kuhn.

Flexible bronchoscopy and mechanical ventilation in managing Mounier-Kuhn syndrome: a case report.

CONTEXT: Mounier-Kuhn syndrome is a rare congenital condition with distinct dilatation and diverticulation of the tracheal wall. The symptoms may vary and the treatment usually consists of support. CASE REPORT: The patient was a 60-year-old male with recurrent hospital admission. He was admitted in this case due to dyspnea, cough and sputum production. An arterial blood sample revealed decompensated respiratory acidosis with moderate hypoxemia. A chest computed tomography (CT) scan showed dilatation of the trachea and bronchi, tracheal diverticula and bronchiectasis. Flexible bronchoscopy was performed, which revealed enlarged airways with expiratory collapse. Furthermore, orifices of tracheal diverticulosis were also detected. Non-invasive positive pressure ventilation (NPPV) was added, along with long-term oxygen therapy. At control visits, the patient's clinical and laboratory findings were found to have improved. CONCLUSION: Flexible bronchoscopy can be advocated for establishing the diagnosis and non-invasive mechanical ventilation can be used with a high success rate, for clinical wellbeing in Mounier-Kuhn syndrome.

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 or congenital tracheobronchomegaly: a literature review.

Mounier-Kuhn syndrome or congenital tracheobronchomegaly is a chronic airway condition which for currently unknown reasons mostly affects males. It is commonly overlooked on conventional chest X-rays, and is considered to be rare, but the prevalence might be higher as commonly assumed. The hallmark of it is a dilatation of the main airways which frequently, but not always, causes marked, mainly respiratory, symptoms, and patients usually present with varying degrees of recurrent infections, breathlessness, haemoptysis, dyspnoea. Although at least 200 case reports have been published, there have been only a few attempts to review them, and none in the last 20 years. Due to the lack of clinical trials and wide variability of case-report format, a systematic review was deemed not feasible, therefore PubMed and Medline databases were searched using terms "Mounier-Kuhn syndrome", "tracheobronchomegaly", "tracheomegaly", and "bronchomegaly", without any time restrictions, to summarize currently known facts about the syndrome. To the authors' best knowledge, the result is currently the most comprehensive review of previously published literature about the congenital tracheobronchomegaly, and summarizes what's known about symptoms, prevalence, disease associations, and treatment options for this syndrome.

[Tracheobronchomegaly: a report of 3 cases and literature review].

OBJECTIVE: To analyze the clinical, radiological, and pathological characteristics of tracheobronchomegaly (TBM, Mounier-Kuhn syndrome). METHODS: The clinical, radiological and pathological characteristics of 3 cases of TBM were analyzed, and the literatures were reviewed. RESULTS: All 3 patients were men, between the age of 58 - 71 years old. From the onset to diagnosis, the shortest time was 2 months, and the longest 43 years. The most usual presentations included recurrent cough and sputum, and occasional haemoptysis. In the advanced stage of the disease, patients would present shortness of breath and the symptoms associated with respiratory failure because of the reduction in pulmonary function. All the diagnoses were confirmed by X-ray and CT of the chest finding that the trachea and the main bronchi dilated markedly. After anti-infection treatment, all patients recovered. Mounier-Kuhn syndrome was 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 were thus flaccid and markedly dilated on inspiration and collapsed on expiration. The usual presentation was 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. Computed tomography scan of the chest was used for the diagnosis. Treatment was mainly supportive with chest physiotherapy and antibiotics. CONCLUSIONS: Mounier-Kunh syndrome should be suspected in patient with recurrent respiratory infections and chronic sputum production. A careful analysis of the central airways at the chest radiograph of these patients is required.

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.

[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: a case report.

Mounier-Kuhn syndrome is a rare clinical entity. It was described in 1932 to refer to the presence of tracheobronchomegaly. The clinical presentation is variable and it can occur in several different age groups. We present the case of a young man who was admitted to the hospital for an asthma exacerbation and was found to have tracheobronchomegaly on his CT scan. A review of the literature for this rare, but interesting, phenomenon is included.

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.

Tracheobronchomegaly-Mounier-Kuhn syndrome.

Tracheobronchomegaly is a rare disorder of uncertain etiology, characterized by marked dilatation of the trachea and major bronchi, associated with tracheal diverticulosis, bronchiectasis and recurrent respiratory tract infection. We are reporting a 60-year-old man from the Kingdom of Saudi Arabia with this condition and review the literature for such a rare entity.

Rapidly progressing case of Mounier-Kuhn Syndrome in early childhood.

Reports of Mounier-Kuhn Syndrome in childhood are extremely rare, as it usually presents in the third or fourth decades. In the only other report of a case diagnosed in early childhood, the child was well at the time of publication. We report on a 15-month-old boy who presented with his first respiratory illness at age 3 months. His disease course was more severe than previously reported. Diagnosis was characteristically delayed until acute respiratory failure complicated a respiratory tract infection at 13 months, prompting high-resolution computerized tomography (HRCT) of the chest. He gradually deteriorated, eventually requiring ventilatory support; death occurred at age 15 months. This report illustrates the clinical heterogeneity of the syndrome. We review the theories about etiology and the recognized clinical findings in adults. Incidence may be higher than previously estimated, and tracheobronchomegaly (TBM) should be considered as a cause of respiratory failure and recurrent pneumonia in children where other investigations, including chest X-ray (CXR), are normal. A chest HRCT (cHRCT) scan and bronchoscopy are usually diagnostic.

[Treatment of tracheobronchomegaly with an Ultraflex prosthesis. A case report]. / Traitement de la trachéobronchomégalie par la mise en place d'une prothèse Ultraflex.

Tracheobronchomegaly is defined as a dilatation of the trachea and the large bronchi. It may occur as a familial condition or in association with a connective tissue disease, e.g. Ehlers-Danlos syndrome. Tracheobronchomegaly occurs late in adults. The predominant symptoms are bronchial irritation and recurrent bronchopulmonary infections (because of ineffective cough). Diagnosis is provided by thoracic imaging, particularly computed tomography that enables measuring the precise diameter of the trachea. We report the case of one patient with tracheobronchomegaly who was greatly improved after implantation of Ultraflex tracheobronchial prostheses.