Mounierkuhn syndrome europe pmc article europe pmc. Mounier kuhn syndrome, or congenital tracheobronchomegaly, is an under diagnosed clinical entity with peculiar anatomical and physiological features making anesthetic care challenging. The diagnosis is usually made when the tracheal diameter is greater than 3 cm on a ct chest measured 2 cm above the aortic arch 2. Des malformations sternocostales ont ete egalement signalees. Mounierkuhn syndrome, or tracheobronchomegaly, is a rare clinical and radiologic condition characterized by marked tracheobronchial dilation and recurrent lower respiratory tract infections. Tracheobronchomegaly or mounierkuhn syndrome is a rare condition also known as tracheal diverticulosis and tracheobronchiectasis.
Mounierkuhn syndrome is a rare clinical and radiologic entity characterized by the pathologic dilatation of the trachea and bronchi. Residente medicina interna, universidad del rosario. In 1988, the first visualizations of mks in computed tomography ct were described 2, 3, and although numerous case reports are published every year, there have been. The syndrome was first described by mounierkuhn in 1932. Mounier kuhn syndrome presenting with recurrent atelectasis. Aug 15, 2019 the syndrome was first described by p. Mounierkuhn syndrome or tracheobronchomegaly is a rare disorder characterized by marked dilatation of the trachea and main bronchi, bronchiectasis, and recurrent respiratory tract infections. Mounierkuhn syndrome or congenital tracheobronchomegaly. Service dimagerie medicale, chu tahersfar, 5100 mahdia, tunisie. Tracheobronchomegaly a condition characterized by bronchiectasis, cough, recurrent infections. Most of the cases are sporadic, but familial cases have been described with a possible recessive inheritance. Thoracic imaging revealed a dilated trachea and main bronchi, tracheal and bronchial diverticuli.
It is commonly overlooked on conventional chest xrays, and is considered to be rare, but the prevalence might. Introduction enlargement of the proximal airways, mainly trachea and large bronchi, was first described in 1932 by mounier kuhn 1. Tracheobronchomegaly is a very rare congenital disorder of the lung primarily characterized by an abnormal widening of the upper airways. Mounierkuhn syndrome, or tracheobronchomegaly, is a rare clinical entity characterized by abnormal dilation of the trachea and main bronchi. Other diagnostic criteria include a mainstem bronchial diameter of 2024 mm right and 1523 mm left 3. Computed tomography typically suggests the diagnosis 6 that can be confirmed by fiberoptic bronchoscopy which typically reveals an expiratory collapse of the intrathoracic.
Sa premiere description endoscopique et radiologique a ete faite en 1932 par mounierkuhn 3. Mounierkuhn syndrome is most frequently seen in middle age men before the age of. Mounier kuhn syndrome is a rare disorder characterized by marked dilatation of the trachea and main bronchi, sometimes with tracheal diverticulosis, bronchiectasis, and recurrent lower respiratory. Mounierkuhn syndrome or congenital tracheobronchomegaly is a rare clinical and radiological condition. Irritants like cigarette smoke and air pollution could act as irritating factors. Congenital tracheobronchomegaly mounier kuhn syndrome, mks is a rare idiopathic disorder characterized by dilation of the central airways, including the trachea and first through fourth order bronchi. Conclusion mounierkuhn syndrome or tracheobronchomegaly is a very rare condition whos congenital or acquired origin is still controversial. Mounierkuhn syndrome is a rare disorder characterized by marked dilatation of the trachea and main bronchi, sometimes with tracheal diverticulosis, bronchiectasis, and. Thoracic imaging revealed a dilated trachea and main bronchi, tracheal and. The condition can be diagnosed by lung function tests, bronchoscopy, and a chest ct scan.
This is the oldest case of mounier kuhn syndrome at presentation and highlights the importance of appropriate history taking and investigation of older people. The present report describes a case of this disease with analysis of chest radiography and high resolution computed tomography showing increased caliber of the trachea, main bronchi and central bronchiectasis. Mounier kuhn syndrome is most frequently seen in middle age men before the age of. Jan 01, 2008 a ct of thorax for suspected bronchiectasis revealed tracheobroncomegaly mounier kuhn syndrome. The diagnosis is made through the synthesis of clinical and radiological data. Kuhn syndrome mks, the first case of which was described in 1937 1, is a rare, most likely congenital syndrome, whose main characteristic is an enlarged trachea and main bronchi. Mks disproportionately affects men and results in chronic respiratory tract infections. Mounierkuhn syndrome or congenital tracheobronchomegaly is a chronic airway condition which for currently unknown reasons mostly affects males.
Mounierkuhn syndrome definition of mounierkuhn syndrome. It is characterized by a tracheal and bronchial dilation. A 58yearold chronic smoker with history of recurrent pneumonia and bronchiectasis presented for septoplasty. It clinically presents as chronic bronchitis or bronchiectasis, hence under diagnosed, and less than 100 cases are. Mounier kuhn syndrome is characterized by tracheobronchial dilation secondary to atrophy of the muscular and elastic tissues in the trachea and main bronchial walls. Mounierkuhn syndrome is a lung disorder that causes the respiratory tract to dilate or enlarge. Mounier kuhn syndrome or congenital tracheobronchomegaly is a rare clinical and radiological condition. It is more common in men, especially african americans. The disease commonly affects males and presents in adult life. Williamscampbell syndrome chronic airway inflammationinfection with tracheobronchomalacia allergic bronchopulmonary fungal disease. Kuhn syndrome is a rare disease with abnormal enlargement of major airways, but epidemiological studies are lacking, and currently the most available data about it come from case reports, making it difficult to collate changes in a particular patient to those in previously published cases. Mounierkuhn syndrome is a somewhat controversial entity and used synonymously with tracheobronchomegaly by most authors 79.
Mounierkuhn syndrome results from the atrophy of elastic fibers in the trachea and main bronchi which lead to thinning of the smooth muscle layer and subsequent tracheobroncheal flaccidity, dilatation and collapse. Mounierkuhn syndrome is a rare disorder characterized by marked dilatation of the trachea and main bronchi, sometimes with tracheal diverticulosis, bronchiectasis, and recurrent lower respiratory. Mounierkuhn syndrome, one of who unfortunately died in the postoperative period. Mounierkuhn syndrome genetic and rare diseases information. It is characterized by distinct dilation of the trachea and bronchi and by recurrent lower respiratory tract infections lrtis. Diagnosis is typically accomplished with the use of computed tomography and bronchoscopy, as well as pulmonary function testing. Mounierkuhn syndrome, or tracheobronchomegaly, is a rare clinical and radiologic condition. This patient had lung transplantation later in their clinical course. The cause of mounierkuhn syndrome is unknown, although cigarette smoke. Mounier kuhn syndrome is a rare congenital disorder. Mounierkuhn syndrome acta otorrinolaringologica espanola. A ct of thorax for suspected bronchiectasis revealed tracheobroncomegaly mounierkuhn syndrome. Mounierkuhn syndrome might cause bronchiectasis, study.
A case of tracheobronchomegaly mounier kuhn syndrome diagnosed via flexible bronchoscopy. Mounierkuhn syndrome is defined by a marked dilatation of the trachea and the main bronchi, often responsible for a tracheobronchomalacia that may occasionally be severe. Sa premiere description endoscopique et radiologique a ete faite en 1932 par mounier kuhn 3. This association makes the understanding of the mechanism of the occurrence of meunier kuhn syndrome more complicated. Mounierkuhn syndrome might cause bronchiectasis, study finds. Congenital tracheobronchomegaly mounierkuhn syndrome in. Mounierkuhn syndrome or congenital tracheobronchomegaly is characterized by atrophy or absence of elastic fibers and smooth muscle cells leading to a markedly dilated trachea and bronchi associated with chronic pulmonary suppuration. Mounier kuhn syndrome is a rare clinical and radiologic entity characterized by the pathologic dilatation of the trachea and bronchi. Epidemiology mounierkuhn syndrome is most frequently seen in middle age men before the age of 50 years 1,6. Mounier kuhn syndrome mks is a unique condition characterized by dilation of the trachea and major bronchi and recurrent pneumonias. Apr 06, 2016 mounier kuhn syndrome, also known as tracheobronchomegaly, is a condition characterized by a dilated windpipe and main bronchi associated with frequent infections of the lower respiratory tract. Mounierkuhn syndrome mks is a unique condition characterized by dilation of the trachea and major bronchi and recurrent pneumonias.
Endoscopic successful management of tracheobronchomalacia. We present the case of a man with mounierkuhn 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. Mounierkuhn syndrome mimicking lymphangioleiomyomatosis. Tracheobronchiomegaly, also known as mounierkuhn syndrome mks, is a rare condition, characterised by pathological dilation of the trachea and major bronchi with recurrent chest infections 15. Mounierkuhn syndrome is a rare disease clinically characterized by recurrent respiratory infections. Mounier kuhn syndrome is defined by a marked dilatation of the trachea and the main bronchi, often responsible for a tracheobronchomalacia that may occasionally be severe.
This is the oldest case of mounierkuhn syndrome at presentation and highlights the importance of appropriate history taking and investigation of older people. The condition arises due to the absence of elastic fibers and smooth muscle cells in these parts of the airways. Tracheobronchiomegaly, also known as mounier kuhn syndrome mks, is a rare condition, characterised by pathological dilation of the trachea and major bronchi with recurrent chest infections 15. Mounier kuhn syndrome is a somewhat controversial entity and used synonymously with tracheobronchomegaly by most authors 79. Mounierkuhn syndrome, also known as tracheobronchomegaly, is a condition characterized by a dilated windpipe and main bronchi associated with frequent infections of the lower respiratory tract. People with this condition develop frequent respiratory tract infections and recurrent cough. Arranz caso ja, fernandez frances j, jimenez jurado d, manzano espinosa l. Mounierkuhn syndrome is characterized by tracheobronchial dilation secondary to atrophy of the muscular and elastic tissues in the trachea and main bronchial walls. The abnormally widened trachea and mainstem bronchi are associated with recurrent lower respiratory tract infection and copious purulent sputum production, eventually leading to bronchiectasis and other respiratory complications. The patient was evaluated using ongoing protocols and provided written informed consent.