Mounier-Kuhn Syndrome. Imaging and Bronchoscopic Findings

El‐Kersh, Karim; Yasin, Muhammad; Cavallazzi, Rodrigo; Perez, Rafael L.

doi:10.1164/rccm.201307-1306im

Cited by 6 publications

(6 citation statements)

References 2 publications

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“…Involvement can occur at different levels, from the trachea to the 4th bronchial branch, despite the fact that its etiology is not fully understood, tracheobronchomegaly is thought to be associated with a familial susceptibility and possibly inherited through an autosomal recessive mechanism [7]. Mounier-Kuhn syndrome includes 3 subtypes; in type 1, there is a slight symmetrical enlargement of the trachea and the main bronchi, type 2 has a more evident dilation with diverticula that are clearly distinct and in type 3, the diverticular and saccular structures reach the distal bronchi [8].…”

Section: Discussionmentioning

confidence: 99%

CT scan Diagnosis of Mounier-Kuhn Syndrome in A Patient with Recurrent Respiratory Infections: About A Rare Case Report

Benfaddoul¹

2022

J Clin Stud Rev Rep

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Tracheobronchomegaly or Mounier-Kuhn syndrome is a very rare condition characterized by dilatation of the trachea and main bronchi, bronchiectasis and recurrent respiratory tract infections. The causality of this syndrome is unclear and the clinical representation is variable. The diagnosis is mostly established on the basis of characteristic CT scan findings. We report the case of a 65-year-old female with a history of recurrent lower respiratory tract infections, that was not initially diagnosed by repeated chest radiographs, but was later identified by CT scan

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Section: Discussionmentioning

confidence: 99%

CT scan Diagnosis of Mounier-Kuhn Syndrome in A Patient with Recurrent Respiratory Infections: About A Rare Case Report

Benfaddoul¹

2022

J Clin Stud Rev Rep

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“…The etiology of TBM is not clear, and it may be a connective tissue-related disease or an autosomal recessive genetic disease, but most cases had not been found; therefore, the etiology theory of TBM requires further study [1]. In TBM patients, the respiratory tract is softened due to congenital dysplasia of smooth muscle and elastic fiber in the trachea and main bronchi, which affects the movement of cilia and sputum discharge, resulting in repeated infections due to the accumulation of pathogens and foreign bodies, eventually leading to bronchiectasis, emphysema, bullae, and other conditions [2][3][4]. The clinical manifestations of TBM lack specificity; furthermore, cough, expectoration, and progressive dyspnea are common symptoms; in addition, some patients could be asymptomatic [5].…”

Section: Discussionmentioning

confidence: 99%

An Accidental Discovery of Tracheobronchomegaly: a Case Report

Liu

et al. 2020

Indian J Surg

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Tracheobronchomegaly is a rare disease with congenital abnormal change in respiratory tract; its image features are also very special. In this case, we described a 57-year-old male with cough, expectoration, chest pain, and dyspnea. In our institution, the result of chest CT scan is highly extraordinary, which showed obvious dilation of the trachea and main bronchi, emphysema, and a number of pulmonary bullae, and there was a big bulla with air-fluid level on the lower lobe of the right lung. Fortunately, after wedge resection for the big bulla on the lower lobe of right lung under video-assisted thoracoscopic surgery, this patient’s symptoms were significantly relieved. The clinical manifestations of tracheobronchomegaly lack specificity; this disease has freakish image features. At present, there are no effective treatments for tracheobronchomegaly, which just was an accidental discovery in this patient; we just mainly take surgical measures to treat the big bulla for relieving symptoms.

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“…Other potential etiologies of Mounier-Kuhn syndrome have been proposed including the disappearance of the connective tissue network of the airway wall [5], a chronic inflammatory state leading to greater matrix metalloproteinase activity [6], and atrophy of elastic tissues in the trachea and main bronchial walls [7,8]. Moreover, several reports have described an association between Mounier-Kuhn syndrome and connective tissue diseases such as Ehlers-Danlos syndrome, Marfan syndrome, and cutis laxa [9][10][11], but an exact genetic etiology remains unknown.…”

Section: Discussionmentioning

confidence: 99%