Congenital malformations of the bronchopulmonary system

Clinically diagnosed malformations of the bronchopulmonary system are detected in 10% of patients with chronic lung diseases.

Agenesis, aplasia, hypoplasia of the lung. Clinically, these defects are characterized by chest deformation - depression or flattening on the side of the defect. Percussion sound over this area is shortened, respiratory sounds are absent or greatly weakened. The heart is displaced toward the underdeveloped lung.

Typical radiographic signs are a decrease in the volume of the chest on the side of the defect, intense darkening in this area, prolapse of the healthy lung through the anterior mediastinum into the other half of the chest. The bronchographic picture allows us to judge the degree of lung underdevelopment.

Polycystic lung disease (cystic hypoplasia) is one of the most common defects. Infected polycystic lung disease has a fairly vivid clinical picture. Patients are bothered by coughing with the separation of a large amount of sputum, often hemoptysis. Crackling moist rales ("drum roll") are heard over the lungs, in the presence of large cavities - amphoric breathing. Oral crepitation is noted. Patients lag behind in physical development, have signs of chronic hypoxia.

Chest X-rays reveal cellular formations, while bronchograms or computed tomography scans reveal round cavities.

Congenital lobar emphysema. This defect is characterized by underdevelopment or absence of bronchial cartilages in the affected lobe, which causes air retention during exhalation with subsequent increase in intrapulmonary pressure and overstretching of the parenchyma in the affected lobe. The main clinical manifestation is respiratory failure, the severity of which depends on the degree of swelling (hyperinflation) of the lobe.

Tracheobronchomegaly (Mounier-Kuhn syndrome) is a pronounced widening of the trachea and main bronchi, leading to chronic respiratory infection. Characteristic features include a loud, vibrating cough of a specific timbre, reminiscent of a goat's bleating, hoarseness, abundant purulent sputum, and possibly hemoptysis. There are characteristic bronchoscopic signs. X-ray examination of children with this defect reveals a trachea diameter equal to or greater than the diameter of the thoracic vertebrae. A characteristic feature is the shape of the forced expiratory curve during the study of the function of external respiration: a curve with a break or a typical notch.

Tracheobronchomalacia is a congenital defect associated with increased softness of the cartilages of the trachea and large bronchi. It manifests itself as a syndrome of stenosis of the trachea and main bronchi: stridor, "sawing" breathing, barking cough, attacks of suffocation that are not relieved by antispasmodics.

A low degree of tracheobronchomalacia in early childhood is manifested by stinky breathing, recurrent obstructive bronchitis, the frequency of which decreases in the second year of life.

Williams-Campbell syndrome is a deficiency of cartilaginous rings in the bronchial wall, starting from the III-IV and up to the VI-VIII order. The disease manifests itself as slowly progressing pneumosclerosis, pulmonary emphysema, bronchiolitis, sometimes with obliteration of the bronchioles. Sick children lag behind in physical development, have a barrel-shaped chest, signs of chronic hypoxia. Radiographically, the stretched bronchi look like cavities, sometimes with a fluid level. The diagnosis is confirmed by bronchoscopy and bronchography.

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