Tracheobronchomegaly

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Tracheobronchomegaly
Other namesMounier-Kühn syndrome [1]
Specialty Pulmonology   OOjs UI icon edit-ltr-progressive.svg
Complications Recurrent pulmonary infections
Causesatrophy of elastic fibers in the trachea and main bronchi, leading to thinning of the smooth muscle layer
Diagnostic method CT Chest. Tracheobroncheal flaccidity, dilatation, and/or collapse.
Frequency300 cases have been reported to date

Tracheobronchomegaly is a very rare congenital disorder of the lung primarily characterized by an abnormal widening of the upper airways. [2] 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. [3]

Contents

Diagnosis

Woodring et al. (1991) suggested the following diagnostic criteria for tracheomegaly in adults based on chest radiography: [4]

History

The term "Mounier-Kuhn syndrome" derives from the characterization of the condition by Prof. Pierre-Louis Mounier-Kuhn in 1932, while the name "tracheobronchomegaly" was introduced by Katz et al. in 1962. [5] [6] [7]

Related Research Articles

<span class="mw-page-title-main">Trachea</span> Cartilaginous tube that connects the pharynx and larynx to the lungs

The trachea, also known as the windpipe, is a cartilaginous tube that connects the larynx to the bronchi of the lungs, allowing the passage of air, and so is present in almost all animals with lungs. The trachea extends from the larynx and branches into the two primary bronchi. At the top of the trachea the cricoid cartilage attaches it to the larynx. The trachea is formed by a number of horseshoe-shaped rings, joined together vertically by overlying ligaments, and by the trachealis muscle at their ends. The epiglottis closes the opening to the larynx during swallowing.

<span class="mw-page-title-main">Tracheal intubation</span> Placement of a tube into the trachea

Tracheal intubation, usually simply referred to as intubation, is the placement of a flexible plastic tube into the trachea (windpipe) to maintain an open airway or to serve as a conduit through which to administer certain drugs. It is frequently performed in critically injured, ill, or anesthetized patients to facilitate ventilation of the lungs, including mechanical ventilation, and to prevent the possibility of asphyxiation or airway obstruction.

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Mechanical ventilation, assisted ventilation or intermittent mandatory ventilation (IMV) is the medical term for using a machine called a ventilator to fully or partially provide artificial ventilation. Mechanical ventilation helps move air into and out of the lungs, with the main goal of helping the delivery of oxygen and removal of carbon dioxide. Mechanical ventilation is used for many reasons, including to protect the airway due to mechanical or neurologic cause, to ensure adequate oxygenation, or to remove excess carbon dioxide from the lungs. Various healthcare providers are involved with the use of mechanical ventilation and people who require ventilators are typically monitored in an intensive care unit.

<span class="mw-page-title-main">Bronchiectasis</span> Disease of the lungs

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<span class="mw-page-title-main">Laryngotracheal stenosis</span> Medical condition

Laryngotracheal stenosis refers to abnormal narrowing of the central air passageways. This can occur at the level of the larynx, trachea, carina or main bronchi. In a small number of patients narrowing may be present in more than one anatomical location.

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Tracheobronchial injury is damage to the tracheobronchial tree. It can result from blunt or penetrating trauma to the neck or chest, inhalation of harmful fumes or smoke, or aspiration of liquids or objects.

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References

  1. RESERVED, INSERM US14-- ALL RIGHTS. "Orphanet: Mounier Kühn syndrome". www.orpha.net. Retrieved 26 May 2019.
  2. Schwartz, W; Rossoff L (Nov 1994). "Tracheobronchomegaly". Chest. American College of Chest Physicians. 106 (5): 1589–1590. doi:10.1378/chest.106.5.1589. PMID   7956426. Archived from the original (PDF) on 2020-01-09. Retrieved 2008-02-27.
  3. Menon B, Aggarwal B, Iqbal A (2008). "Mounier-Kuhn syndrome: report of 8 cases of tracheobronchomegaly with associated complications". South Med J. 101 (1): 83–87. doi:10.1097/SMJ.0b013e31815d4259. PMID   18176298.
  4. Woodring J, et al. (1999). "Congenital tracheobronchomegaly (Mounier-Kuhn syndrome)". J Thorac Imaging. 6 (1).
  5. Smith DL, Withers N, Holloway B, Collins JV (August 1994). "Tracheobronchomegaly: an unusual presentation of a rare condition". Thorax. 49 (8): 840–1. doi:10.1136/thx.49.8.840. PMC   475137 . PMID   8091335.
  6. KATZ I, LEVINE M, HERMAN P (December 1962). "Tracheobronchiomegaly. The Mounier-Kuhn syndrome". Am J Roentgenol Radium Ther Nucl Med. 88: 1084–94. PMID   13958486.
  7. Mounier-Kuhn P. Dilatation de la trachée: constatations radiographiques etbronchoscopiques. Lyon Med. 1932;150:106-9.