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Thursday, 19.10.2017, 19:22
Main » Pediatrics » Malformations of the wall of the trachea and bronchi 
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Malformations of the wall of the trachea and bronchi


Malformations of the wall of the trachea and bronchi

Malformations of the structural elements of the wall of the trachea, bronchi and bronchioles are morphologically related to the absence, shortage or disruption of the cartilage or elastic and muscle tissue, they share a similar pathological mechanism - the weakness of the bronchial wall.


Malformations of the wall of the trachea and bronchi.
A. Common malformations of the tracheal wall and bronchi:
  • traheobronhomegaliya
  • traheobronhomalyatsiya
  • Williams syndrome, Campbell
  • bronhomalyatsiya
  • bronhiolektaticheskaya emphysema
B. Restricted malformations wall of the trachea and bronchi:
  • Congenital tracheal stenosis
  • Congenital lobar emphysema
  • diverticulum of the trachea and bronchi
  • traheobronhopischevodnye fistula

Traheobronhomegaliya (Mounier-Kuhn syndrome) - reveals the excessive expansion of the trachea and main bronchi are sometimes due to congenital anomalies of elastic and muscle tissue. Clinically may not be apparent for several years. Clinical manifestations are usually observed in the presence of underlying chronic bronchopulmonary lesions. Do patients have cough with purulent sputum. Frequent symptoms of chronic intoxication and hypoxemia, retarded physical development, the deformation of fingers on the type of drum sticks.

Traheomalyatsiya - congenital weakness of the tracheal wall and major bronchi associated with abnormal softness of cartilage skeleton. Distinguish between diffuse and localized forms. In the local form observed functional stenosis of the trachea, diffuse forms do not have a clear clinics in severe cases can be observed symptoms of tracheal stenosis, the main bronchi.

Williams syndrome, Campbell - the weakness of bronchial wall leads to severe bronchial dyskinesia, which manifests itself more than an extension of their lumen during inhalation and exhalation restriction, these changes lead to a breach of ventilation and cleaning function of bronchi, which leads to stagnation, infection of bronchial secretions and Development chronic bronchitis. The latter contributes to the appearance of emphysema, atelectasis, and chronic pneumonia.

Bronhomalyatsiya - increased bronchial wall compliance associated with the unusual softness of cartilaginous rings. It differs from Williams-Campbell syndrome, a lower degree of bronchial dyskinesia.

Bronhiolektaticheskaya emphysema - is characterized by lesions of the peripheral parts of the bronchial tree with the development of bronhiolektazov and emphysema. The lungs are auscultated constantly moist rales, observed deformation of fingers on the type of drum sticks. The disease occurs with frequent exacerbations, constant wet cough and severe respiratory failure.

Congenital tracheal stenosis may be associated with birth defects of the wall and the external compression (abnormally located vessels, double aortic arch, etc.). The clinical picture in the foreground expiratory stridor, noisy breathing. The disease is complicated by attacks of breathlessness, which resembles a picture of false croup.

Lobar emphysema - bronchial malformation of the cartilage, leading to hyperventilation, the relevant department of the lung.

Diverticulum of the trachea and bronchi - protrusion of the wall of the trachea or bronchi. Diverticula of congenital origin are rudimentary bronchi additional, clinically undetected, and are a random finding.

Traheobronhopishevodnye fistula - this defect is manifested at the first feeding, severe attack of breathlessness, cough, cyanosis. In what quickly joined aspiration pneumonia with severe and usually fatal.



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