Hypoplasia of the lungs

Congenital absence or underdevelopment of the lung is the most severe malformation of the respiratory system. Under agenesis understand the absence of the lung along with the main bronchus. Aplasia is characterized by a lack of lung in the presence of rudimentary main bronchus. At a hypoplasia there is a main and share bronchi which come to an end with functionally imperfect rudiment, a pulmonary tissue is underdeveloped.

Currently, isolated simple and cystic hypoplasia. Different degrees of lung underdevelopment are considered a violation of the development of this body in the early stages of intrauterine development( embryogenesis), most often at the 6th week after ovulation.

Hypoplasia of the lung occurs more often. There are a number of basic factors leading to the development of hypoplasia. The most important of these is the reduction in the volume of the chest cavity in the fetus, which occurs with diaphragmatic hernia, chest deformities, spinal changes, and swelling in the pleural cavity of edematous fetuses. As one of the possible causes of hypoplasia, there is a violation of the patency of the fetal airways and anomalies in the development of pulmonary vessels. The relationship of lung hypoplasia with abnormalities of the kidneys and urinary tracts is emphasized. Family cases of lung hypoplasia are described.

The clinical picture of agenesis, aplasia and hypoplasia of the lung is determined by both the defect itself and the layered infection. From the first years of life the child has repeated pneumonia and bronchitis, cough, as a rule, moist, dyspnea occurs. Children lag behind in physical development. Attention is drawn to the characteristic deformation of the thorax - withering or flattening on the side of the defect. In children with lung hypoplasia, sometimes there is a keeled bulging in the sternum( "chicken breast"), which probably can be the result of swelling of the unaffected lung, which appears as a compensation to maintain the normal supply of the body with oxygen.

With percussion( percussion) of the lungs, the sound on the side of the defect is shortened, reminiscent of that when tapping the wooden tabletop with a fingertip. Normally, when you tap the unchanged tissue of a healthy lung, you can hear a clear sound, which is called pulmonary. The shortening of sound during percussion can speak either of the compaction of the lung tissue( as noted in pneumonia) or the absence of pulmonary tissue in this place( which is precisely what is noted in the underdevelopment and congenital absence of the lung).When listening to a healthy person with a stethoscope, you can hear the special noises that occur when breathing( breathing noises).In hereditary diseases of the lungs, these noises are either absent or sharply weakened, which also indicates the underdevelopment or absence of pulmonary tissue. A person between the lungs in the middle of the body is located a number of organs that combine into one structure called "mediastinum."The organs of the mediastinum include the heart, trachea, esophagus, various large vessels and nerves. In the case of underdevelopment or congenital absence of the lung, the mediastinum organs are biased towards the vicious. This is due to the fact that in that half of the chest there is free space, and a healthy lung "displaces" the mediastinal organs into this space. In the event that the right lung is affected, the heart shifts to the right, which can lead to an erroneous diagnosis of the Kartagener syndrome. As a result of displacement and rotation( rotation) of the heart with right-sided localization of the defect, cardiac activity is heard to the right of the sternum, and not to the left, as in healthy people.

X-ray examination of chest organs reveals typical for this disease signs. These include, first of all, a decrease in the volume of the chest on the side of the disease, the affected half of the chest looks compared to a healthy, darker one( as a result of the absence of air in this place, which gives a healthy light lighter color), the diaphragm is also high due to lack ofpulmonary tissue. The heart and other organs of the mediastinum are shifted to the affected side, which is clearly visible in the picture. Also in the picture you can clearly see the displacement of a healthy lung in the opposite direction of the chest( "pulmonary hernia").

In the diagnosis of agenesis, aplasia and lung hypoplasia, bronchial examination is crucial. Bronchoscopy( a method of examining the bronchial tree, in which a special instrument with a video surveillance system - a bronchoscope) is introduced into the respiratory tract, reveals the absence or rudiment of the main bronchus, the narrowing of the lobar bronchi. On bronchograms with lung agenesis, the absence of the main bronchus is noted, with aplasia( underdevelopment), there is an underdeveloped( rudimentary) bronchus. In case of hypoplasia, large bronchi are filled with contrast medium, and there are no small bronchial branches. When the lung portion is underdeveloped, its volume decreases, bronchial branches in this region are absent or narrowed and deformed. In the diagnosis of agenesis, aplasia and hypoplasia of the lung, an algopulse monography is used, ie, a study of the lung vessels. This method gives an idea of ​​the state of the branches of the pulmonary artery, displacement and rotation of the heart, as well as large vessels.

When examining children with aplasia or hypoplasia of the lung( or its proportion), a radionuclide method for assessing blood flow in the lung tissue is also used. In this case, the radioisotope accumulates in the lung tissue, and in the underdeveloped part of the lung, the absence of its accumulation is recorded.

Agentsia( aplasia, hypoplasia) of the lung is often combined with other developmental anomalies. Describe the heart and vascular defects, skeletal anomalies, gastrointestinal tract, diaphragmatic hernia quite often.

Hypoplasia of the lung in some cases is one of the manifestations of genetically determined syndromes. So, among congenital and hereditary diseases the syndrome is described, which includes finger contractures, multiple joint deformations, facial anomalies and lung hypoplasia.

There are assumptions about the autosomal recessive type of inheritance of this syndrome. Close to the described above syndrome is the Potter tetrad, which includes a combination of lung hypoplasia with anomalies of the structure of the face, clubfoot, changes in hands and lagging behind in physical development.

The combination of lung hypoplasia( or its proportion) with the anomaly of the development of pulmonary veins is known as "yatagan syndrome".A combination of this syndrome with other malformations of the lungs is described. These changes are known to be hereditary.

In the treatment of patients with agenesis and aplasia of the lung, the choice is conservative therapy aimed at suppressing the infection of the respiratory tract, maintaining its normal function. With lung hypoplasia, underdevelopment of the shares, preference is given to surgical treatment. The question of the expediency and the possibility of surgical intervention is solved taking into account the volume of lung tissue that is to be removed, as well as the functional state of the remaining segments of the lung.