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Mounier-Kuhn syndrome, also known as tracheobronchomegaly, is a rare clinical and radiological condition characterized by tracheobronchial dilatation and recurrent respiratory infections. Patients may be asymptomatic or present with severe respiratory illnesses. A CT scan is sufficient to confirm the diagnosis. Treatment is symptomatic, and surgery is rarely indicated. We present the case of a 55-year-old patient with a 30-year history of chronic dyspnea, who presented with worsening shortness of breath, a productive cough, and episodes of bronchial superinfection. A chest CT scan, performed before and after contrast injection during both inspiratory and expiratory phases, confirmed the diagnosis of Mounier-Kuhn syndrome. This case aims to raise awareness of this rare condition and highlights a presentation where dyspnea is the primary symptom.

We report a coexistence of Mounir-Kuhn syndrome and aspergilloma in a 69-year-old male presented with hemoptysis, cough, shortness of breath and fever. This patient has a history of recurrent hospital admissions for infective exacerbations of chronic obstructive pulmonary disease. Diagnostic imaging revealed the presence of aspergilloma and marked dilatation of the trachea and main bronchi, leading to a diagnosis of Mounier-Kuhn syndrome. Given the frequent association of Mounier-Kuhn syndrome with recurrent respiratory infections, we recommend that clinicians consider this syndrome when evaluating patients with recurrent pneumonia.

Mounier-Kuhn syndrome is a rare airway disease characterized by tracheal and bronchial dilatation, primarily affecting middle-aged men. We present a case of Mounier-Kuhn syndrome in a 40-year-old man with a history of recurrent respiratory infections since adolescence. The diagnostic journey involved a multidisciplinary approach incorporating clinical evaluation, radiological imaging, and bronchoscopy. Computed tomography findings, including maximum intensity projection reconstructions and 3D rendering, facilitated the diagnosis by revealing significant airway dilation and associated abnormalities. Treatment primarily focused on supportive measures, including antibiotic therapy and respiratory physiotherapy.This case underscores the importance of considering Mounier-Kuhn syndrome in patients with recurrent respiratory infections and highlights the role of advanced imaging techniques in diagnosis.

Tracheobronchomegaly, or Mounier-Kuhn syndrome, is a clinical and radiological entity characterized by marked dilatation of the trachea and bronchi as a result of severe atrophy of the elastic fibers, with thinning of the muscularis, and the formation of diverticula between the cartilaginous rings. The etiopathogenesis is uncertain and may be congenital or acquired. The clinical signs are not specific and are frequently revealed by recurrent respiratory infections and chronic cough. The diagnosis of Mounier-Kuhn syndrome is based on well-documented measurements of the trachea and main bronchi performed on a chest computed tomography scan. The management of patients is based on symptomatic treatment and may require, in severe cases, the use of endoscopic treatment by stent placement or surgical tracheobronchoplasty. We present a case of a 59yearold patient with recurrent respiratory infections that required several hospitalizations. Diagnosed with Mounier Kuhn syndrome, the thoracic computed tomography scan demonstrated a dilated trachea until the bifurcation and focal points of bronchial dilatation. Bronchoscopic examination showed a dilated and deformed trachea with the presence of diverticula on the tracheal anterior wall. The diameter of the trachea was reduced by more than 50% during expiration and coughing. For this reason, Mounier-Kuhn syndrome should be considered in cases of recurrent respiratory infection or persistent respiratory symptoms.

Homocystinuria (HCU) is a rare autosomal recessive inherited disorder usually diagnosed in childhood. It is characterized by a deficiency of the enzyme that converts homocysteine to cystathionine. The accumulation of homocysteine leads to abnormalities in the ocular, skeletal, cardiovascular, and central nervous systems. HCU shares several clinical features with Marfan syndrome; however, respiratory system involvement in HCU is uncommon and rarely reported. Bronchiectasis has been previously reported in a few cases of HCU, and it was attributed mainly to fibrillin deficiency. This case describes a young girl diagnosed with classical HCU since childhood who presented with a chronic productive cough and was initially misdiagnosed as bronchial Asthma. However, upon further evaluation, she was eventually diagnosed with tracheobronchomegaly (TBM), or Mounier-Kuhn Syndrome, and bronchiectasis based on the computed tomography (CT) scan of chest findings. To our knowledge, this is the first reported case of TBM and bronchiectasis in HCU. We believe that fibrillin degeneration may be the key to understanding this unusual association in HCU.

Mounier Kuhn Syndrome or tracheobronchomegaly is a rare disease, characterized by dilatation of the trachea and the main bronchi. Our study concerns a case of 67-years old male patient, smoker, with a chronic cough. Chest scan was requested; it shows an enlargement of the tracheal clarity and the two main bronchi. Meanwhile, the Functional respiratory exploration was normal. The treatment includes mucolytics and pulmonary physiotherapy. Mounier-Kuhn syndrome is rare and the clinical signs are not specific, the positive diagnosis is purely radiological. The treatment is about to free the airways to prevent infection.

Mounier-Kuhn syndrome or tracheobronchomegaly is a rare disease characterized by marked dilation of the trachea and proximal bronchi with recurrent lower tract respiratory infections. Computed tomography and bronchoscopy are the key tools to accomplish the diagnosis. This is a condition with a clinical polymorphism, symptoms vary from minor with preserved respiratory function, to very severe with life threatening exacerbations leading to respiratory failure and premature death. The treatment is mainly symptomatic, stenting or surgery are reserved to extreme cases.Herein, we report two cases of the same condition with different clinical signs and diverse outcome.

A síndroma de Mounier-Kuhn é uma doença rara, de etiologia desconhecida e que se presume subdiagnosticada. Cursa frequentemente com infecções respiratórias de repetição e bronquiectasias. A clínica inespecífica, semelhante a outras patologias supurativas brônquicas crónicas, dificulta o diagnóstico, muitas vezes efectuado vários anos após o início dos sintomas. Os autores apresentam uma revisão da literatura a propósito de dois casos clínicos que ilustram a heterogeneidade e as dificuldades diagnósticas desta entidade nosológica. Um dos doentes encontrava-se em idade pediátrica na altura do diagnóstico e outro na quarta década de vida.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.

Tracheomegaly is defined as an abnormally dilated trachea and is seen in patients requiring long-standing mechanical ventilation and/or significant hyperinflation of their endotracheal or tracheostomy tube cuffs can occur in adults with severe COVID-19. Tracheomegaly is linked with inadequate nutrition, pneumonia, diabetes, hypotension, steroids, and protracted mechanical ventilation, which are common in COVID-19 patients. Findings include cuff leaks that necessitate cuff overinflation to maintain adequate tidal volumes. Tracheomegaly can be diagnosed with chest radiographs, chest computed tomography, bronchoscopy, or diagnostic laryngoscopy or tracheoscopy. This condition leads to a concern for obstruction, airway collapse, aspiration pneumonia, and iatrogenic tracheal injuries., (© 2022 Elsevier Inc. All rights reserved.)

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., (Copyright © 2017. Published by Elsevier Inc.)

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., (Copyright © 2013 Elsevier Ltd. All rights reserved.)