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. Mounierkuhn syndrome is most frequently seen in middle age men before the age of 50 years 1,6. Mounier kuhn syndrome is characterized by tracheobronchial dilation due to atrophy of muscular and elastic tissues in trachea and main bronchial walls. Mounierkuhn syndrome, or tracheobronchomegaly, is a rare clinical and radiologic condition. 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. The mounierkuhn syndrome mks or tracheobronchomegaly tbm is a rare condition of unknown frequency, up to now about 100 cases have been reported. The mounier kuhn syndrome is a congenital condition characterized by a distinct tracheobronchial dilatation due to the muscular and elastic tissue atrophy in trachea and in the bronchial walls, which presents more frequently in males, and it is usually diagnosed in the third and fourth life decade.
It is characterized by distinct dilation of the trachea and bronchi and by recurrent lower respiratory tract infections lrtis. Tracheobronchomegalymounierkuhn syndrome is dilatation of the trachea and. Tracheobronchomegaly presents when the defect extends to the central bronchi. The condition can be diagnosed by lung function tests, bronchoscopy, and a chest ct scan. The anatomical and physiological changes present in the airways predispose to stagnation within enlarged portions of the. Mks can be diagnosed in adult women when the transverse and sagittal diameters of the trachea, right mainstem. Mounierkuhn syndrome, or tracheobronchomegaly, is a rare clinical entity. Burcin celik, md, salih bilgin, md, and canan yuksel, md. A 58yearold chronic smoker with history of recurrent pneumonia and bronchiectasis presented for septoplasty. Tracheobronchomegalymounierkuhn syndrome is dilatation of the trachea and major bronchi because of atrophy or absence of elastic fibers and smooth. Mounierkuhn syndrome is a rare disease clinically characterized by recurrent respiratory infections. Mounier kuhn syndrome or tracheobronchomegaly is a rare clinical and radiologic condition which is characterized by distinct dilatation of the trachea and bronchi and associated with recurrent lower respiratory tract infections ltris. Mounier kuhn syndrome is a rare clinical and radiologic entity characterized by the pathologic dilatation of the trachea and bronchi.
It usually presents with recurrent pneumonias, fibrosis and other respiratory complications of pooledstagnant secretions in dilated airways. Mounier kuhn syndrome is another rare cause for a long standing dyspnea a 63 y old male with long standing history of dyspnea, and recurrent cough and expectoration was initially thought to be suffering. Tracheobronchomegaly is a very rare congenital disorder of the lung primarily characterized by an abnormal widening of the upper airways. The features therefore fit with the diagnosis of mounier kuhn syndrome.
Coneddown pa and lateral chest radiographs demonstrate enlargement of the tracheal lumen and bilateral central thinwalled pulmonary cystic lesions. Mounier kuhn syndrome, or congenital tracheobronchomegaly, is an under diagnosed clinical entity with peculiar anatomical and physiological features making anesthetic care challenging. Mounier kuhn syndrome is characterized by tracheobronchial dilation secondary to atrophy of the muscular and elastic tissues in the trachea and main bronchial walls. Mounier kuhn syndrome is another rare cause for a long. The cause of mounierkuhn syndrome is unknown, although cigarette smoke and air pollutants may act as irritating. Sorry, we are unable to provide the full text but you may find it at the following locations. Most cases of tracheobronchomegaly are probably under diagnosed. Conclusion mounier kuhn syndrome or tracheobronchomegaly is a very rare condition whos congenital or acquired origin is still controversial. Mounierkuhn syndrome results from the atrophy of elastic fibers in the trachea and.
The chest ct scan confirmed the abnormally tracheobronchomegaly which was consistent with the diagnosis of the rare mounierkuhn syndrome of an apparently. People with this condition develop frequent respiratory tract infections and recurrent cough. Mounier kuhn syndrome mks is a condition characterized by tracheobronchomegaly resulting from the loss or atrophy of musculoelastic fibers within the airway wall. Mounier kuhn syndrome mks, or tracheobronchomegaly, is a rare clinical and radiologic condition characterized by pronounced tracheobronchial dilation and recurrent lower respiratory tract infections. Mounierkuhn syndrome, or tracheobronchomegaly, is a rare clinical and radiologic. Tracheobronchomegaly or mounierkuhn syndrome is a rare disorder characterized. On reformat ted image of the chest at the level of the trachea and bronchi, lung window setting d, dilated as pect of the trachea and main bronchi is seen. The present report describes a case of this disease with.
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, although rare, constitutes a possible diagnosis in these patients and must be considered. It is more common in men, especially african americans. Most of the cases are sporadic, but familial cases have been described with a possible recessive inheritance. The clinical features consist of expiratory central airway collapse, airflow limitation, and clinical presentation similar to chronic bronchitis.
Mounier kuhn syndrome is another rare cause for a long standing. Mounier kuhn syndrome, one of who unfortunately died in the postoperative period. Sa premiere description endoscopique et radiologique a ete faite en 1932 par mounier kuhn 3. Mounierkuhn syndrome is a lung disorder that causes the respiratory tract to dilate or enlarge. 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 in an elderly female with pulmonary. Mounierkuhn syndrome is a somewhat controversial entity and used synonymously with tracheobronchomegaly by most authors 79. The association of mounierkuhn syndrome and pulmonary. A rare case of mounierkuhn syndrome with bronchial asthma. Mounierkuhn syndrome, or tracheobronchomegaly, is a rare clinical entity characterized by abnormal dilation of the trachea and main bronchi. Mounier kuhn syndrome is a congenital abnormality of the trachea and main bronchi characterized by atrophy or absence of elastic fibers and thinning of muscle, which allows the trachea and main. Service dimagerie medicale, chu tahersfar, 5100 mahdia, tunisie.
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