-
BMJ Case Reports Jan 2021
Topics: Bronchoscopy; Humans; Respiratory Tract Infections; Tomography, X-Ray Computed; Tracheobronchomegaly
PubMed: 33509891
DOI: 10.1136/bcr-2020-239876 -
Atencion Primaria Feb 2020
Topics: Adult; Female; Humans; Radiography, Thoracic; Tomography, X-Ray Computed; Tracheobronchomegaly
PubMed: 31076098
DOI: 10.1016/j.aprim.2019.02.011 -
Respiratory Medicine Dec 2013Mounier-Kuhn syndrome or congenital tracheobronchomegaly is a chronic airway condition which for currently unknown reasons mostly affects males. It is commonly... (Review)
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.
Topics: Adult; Aged; Aged, 80 and over; Anesthesia; Contraindications; Diagnosis, Differential; Dilatation, Pathologic; Dyspnea; Female; Humans; Infant; Male; Middle Aged; Pulmonary Disease, Chronic Obstructive; Recurrence; Respiratory Tract Infections; Tracheobronchomegaly
PubMed: 24070565
DOI: 10.1016/j.rmed.2013.08.042 -
Thorax May 1968Two cases of the rare condition of tracheobronchomegaly are reported. They occurred in people of completely different racial origin and residence. They showed the...
Two cases of the rare condition of tracheobronchomegaly are reported. They occurred in people of completely different racial origin and residence. They showed the characteristic features of this condition—loud, rasping, prolonged, remarkably ineffective cough, abnormally wide trachea and major bronchi, laxity of the cartilaginous rings and membranous part of these airways demonstrable on straight chest radiographs and bronchoscopy and confirmed at bronchography. Evidence is submitted of congenital aetiology.
Topics: Adult; Anthropometry; Bronchi; Bronchography; Bronchoscopy; Humans; Male; Trachea
PubMed: 5656767
DOI: 10.1136/thx.23.3.320 -
Radiology Case Reports Sep 2021Mounier 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...
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.
PubMed: 34276851
DOI: 10.1016/j.radcr.2021.06.021 -
Radiology Case Reports Oct 2022Tracheobronchomegaly, or Mounier-Kuhn syndrome, is a clinical and radiological entity characterized by marked dilatation of the trachea and bronchi as a result of severe...
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.
PubMed: 35923343
DOI: 10.1016/j.radcr.2022.06.077 -
ERJ Open Research Apr 2020https://bit.ly/3azhDjr.
https://bit.ly/3azhDjr.
PubMed: 32613017
DOI: 10.1183/23120541.00138-2020 -
Acta Otorrinolaringologica Espanola 2013
Topics: Humans; Male; Middle Aged; Tracheobronchomegaly
PubMed: 22626013
DOI: 10.1016/j.otorri.2012.02.013 -
Respiratory Medicine Case Reports 2023Homocystinuria (HCU) is a rare autosomal recessive inherited disorder usually diagnosed in childhood. It is characterized by a deficiency of the enzyme that converts...
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.
PubMed: 36655006
DOI: 10.1016/j.rmcr.2023.101808 -
Chest Feb 2018We 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...
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.
Topics: Adult; Diagnosis, Differential; Humans; Lung; Lymphangioleiomyomatosis; Male; Parenchymal Tissue; Tomography, X-Ray Computed; Tracheobronchomegaly
PubMed: 29406234
DOI: 10.1016/j.chest.2017.10.024