Congenital lobar emphysema

Congenital lobar emphysema refers to malformations that occur primarily in early childhood. This defect is characterized by the stretching of the tissue of an entire lobe of the lung( less often the segment) due to a partial disruption of the patency of any bronchus. At the heart of lobar emphysema is the underdevelopment or absence of cartilage of the bronchus of the affected lobe. Also, the cause of lobar emphysema may be a partial violation of bronchial patency due to the proliferation of the bronchial mucosa with the formation of folds, mucous plugs, as well as the compression of the bronchus from the outside by bronchogenic cysts, abnormally located vessels. Partial violation of bronchial patency with the development of the valve mechanism causes an air retention on the exhalation, an increase in intra-pulmonary pressure with subsequent overstretch of the tissue of the affected lobe.

There are several variants of lobar emphysema. In some cases, it is possible to increase the number of alveoli within the lobe at normal size( polyalveolar emphysema).In other cases, with a normal number of alveoli, stretching of the lung tissue takes place. Lobar emphysema can be combined with other developmental malformations. It is known for its combination with cardiovascular anomalies, with congenital funnel-like deformation of the thorax. The characteristic localization of congenital lobar emphysema is the upper lobe of the left lung.

The main clinical manifestation of the disease is respiratory failure, the severity of which depends on the degree of airiness of the affected lobe. Depending on the severity of clinical manifestations, decompensated, subcompensated and compensated forms of pathology are distinguished.

The most severe forms of lobar emphysema appear in the first days of a child's life. These patients have a severe respiratory distress syndrome. Attacks of absence of breath, accompanied by loss of consciousness, convulsions are observed. Over the affected part of the lungs, the breath is sharply weakened or not at all audible. Mediastinum sharply displaced in the opposite direction. This form of lobar emphysema without emergency surgery in a short time leads to the death of the child as a result of pulmonary heart failure.

Less severe forms appear later in life, often after infection. Patients are worried about cough, a slight shortness of breath while exercising. There is a swelling of the chest on the side of the blemish. When listening to a stethoscope, breathing noises are audible with difficulty, when tapping the chest( percussion), the sound over the affected lobe of the lung has a boxy tint( reminiscent of that when tapping the empty cardboard box), which indicates an increased amount of air in the lungs. The heart and organs of the mediastinum are shifted in the opposite direction from the affected side of the chest.

With a slight degree of lightness of the lung, clinical symptoms may be so small that the disease is detected late in school-age children or adults. Sometimes lobar emphysema is a random radiographic finding. The degree of lobar emphysema may vary. Oscillations in the violation of bronchial patency create a picture of intermittent lobar emphysema. Increase in airiness of lung tissue can increase with respiratory infection and decrease as it subsides.

In the recognition of lobar emphysema, chest X-ray is crucial. On the roentgenogram and tomograms the increased transparency of the affected part of the lung is determined, i.e. the affected lung looks in comparison with the healthy lighter, which is due to the high content of air in it. The organs of the mediastinum are biased towards the healthy lung. The affected portion sometimes forms a hernia of the mediastinum. Recently, the diagnosis of lobar emphysema is used to study the state of the lung vessels, which allows not only to clarify the localization of the lesion, but also to assess the blood flow in the system of the small circle of the circulation.

Bronchological examination in this pathology is not of great diagnostic significance, and most importantly, it is unsafe for patients, as it can lead to rupture of the walls of the inflated part of the lung and the development of pneumothorax( the appearance of air in the pleural cavity), emphysema of the mediastinum.

Radical method of treatment with congenital lobar emphysema is the operative removal of the affected part of the lung.