Congenital malformations of the bronchopulmonary system

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

Aging, aplasia, hypoplasia of the lung. Clinically, these deformities are characterized by a deformity of the thorax - an occlusion or a flattening on the side of the defect. The percussion sound over this area is shortened, the respiratory noises are either absent or sharply attenuated. The heart is shifted towards the underdeveloped lung.

Typical radiologic signs are a decrease in the volume of the chest on the side of the defect, intense darkening in this region, proliferation of the healthy lung through the anterior mediastinum to the other half of the thorax. Bronchographic picture allows you to judge the degree of underdevelopment of the lung.

Polycystic lung disease (cystic hypoplasia) is one of the most common defects. Infected polycystic pulmonary disease has a fairly vivid clinical picture. Patients are concerned about coughing with the separation of large amounts of sputum, often hemoptysis. Above the lungs are heard rattling wet rales ("drum roll"), in the presence of large cavities - amphoric breathing. There is an oral crepitation. Patients lag behind in physical development, have signs of chronic hypoxia.

Chest radiographs show cellular formations, bronchograms or CT scans - rounded cavities.

Congenital lobar emphysema. In this defect, there is an underdevelopment or absence of cartilage of the bronchus of the affected lobe, which causes an air retention on expiration with a subsequent increase in intrapulmonary pressure and a hyperextension of the parenchyma of 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 enlargement of the trachea and major bronchi leading to chronic respiratory infection. Characterized by a loud vibrating specific timbre cough, reminiscent of bleating goats, hoarseness of the voice, profuse purulent sputum, possibly hemoptysis. There are characteristic bronchoscopic signs. In the X-ray study of children with this defect, the diameter of the trachea is equal to or greater than the diameter of the bodies of the thoracic vertebrae. A characteristic feature is the shape of the forced exhalation curve in the study of the function of external respiration: a curve with a fracture or a typical notch.

Tracheobronchomalacia is a congenital malformation associated with increased softness of the cartilages of the trachea and large bronchi. It is manifested by a syndrome of stenosis of the trachea and the main bronchi: stridor, "sawing" the breath, barking cough, attacks of suffocation that are not stopped by spasmolytics.

A small degree of tracheobronchomalacia in early childhood is manifested by stigmoid respiration, relapsing obstructive bronchitis, whose frequency decreases in the second year of life.

Williams-Campbell Syndrome - a deficit of cartilaginous rings in the bronchial wall, beginning with III-IV and up to VI-VIII order. The disease manifests itself slowly progressive pneumosclerosis, emphysema of the lungs, bronchiolitis, sometimes with bronchioles obliteration. Sick children lag behind in physical development, have a barrel chest, signs of chronic hypoxia. Radiographically stretched bronchi look like cavities, sometimes with a liquid level. Diagnosis is confirmed by bronchoscopy and bronchography

[1], [2]