Congenital lobar emphysema

August 12, 2017 17:51 | Genetic Diseases

Congenital lobar emphysema refers to malformations that occur mainly in the early childhood.This defect is characterized by stretching the fabric of an entire lobe of the lung (less segment) due to partial impaired patency of a bronchus.In lobar emphysema is based on the absence or underdevelopment of the affected bronchus cartilage share.It is also the cause of lobar emphysema may be a partial bronchial obstruction due to proliferation of the bronchial mucosa with the formation of folds of mucous plugs, as well as due to compression of the bronchus outside bronchogenic cysts, abnormally positioned vessels.Partial bronchial obstruction to the development of the valve mechanism causes delay in the air you exhale, increased intra-pulmonary pressure, followed by overstretching of the fabric of the affected lobe.

There are several options lobar emphysema.In some cases, may increase the number of alveoli in proportion with their normal size (polialveolyarnaya emphysema).In other cases, including the normal

alveolar distension occurs lung tissue.Lobar emphysema may be associated with other malformations.We know its combination with cardiovascular abnormalities, congenital funnel chest deformity.The characteristic localization of congenital lobar emphysema is the upper lobe of the left lung.

main clinical manifestation of the disease is respiratory distress, the severity of which depends on the degree of lightness of the affected lobe.Depending on the severity of clinical manifestations distinguish decompensated, subcompensated and compensated form of pathology.

most severe lobar emphysema appear in the first days of life.In these patients, there is a severe respiratory distress syndrome.Observed bouts of breathlessness, accompanied by loss of consciousness, seizures.Above the affected lung lobes breath sharply weakened or never heard.Mediastinum shifted sharply in the opposite direction.This form of lobar emphysema without emergency surgery in the short term leads to the death of a child as a result of cardiopulmonary diseases.

Less severe forms manifest themselves later in life, often after infection layers.Patients concerned about a cough, slight breathlessness on exertion.There bulging chest on the side of evil.When listening to a stethoscope breath sounds are difficult to hear, with chest percussion (percussion) sounds over the affected lung lobes has boxed shade (resembles that when tapped with the tip of your finger on an empty cardboard box), which indicates an increased amount of air in the lungs.Heart and mediastinal organs are moved in the opposite direction with respect to the affected side of the chest.

With a small degree of light airiness clinical symptoms may be so slight that the disease is detected late in school-age children or adults.Sometimes lobar emphysema is a casual radiological finding.The degree of lobar emphysema may vary.Fluctuations in violation of patency of the bronchi create a picture of intermittent lobar emphysema.Increase airiness of lung tissue may increase in respiratory infections and decrease as it subsided.

In recognition lobar emphysema crucial chest X-ray.On radiographs and tomograms is determined by the increased transparency of the affected part of the lung, t. E. Pulmonary lesion looks in comparison with a healthy lighter, due to the high content of air in it.Mediastinal organs are biased towards a healthy lung.Affected share sometimes forms a hernia of mediastinum.Lately, in the diagnosis of lobar emphysema study used state of the pulmonary vessels, which allows not only to clarify the location of the lesion, but also evaluate the condition of blood flow in the pulmonary circulation.

bronchoscopy examinations in this pathology has a great diagnostic value, and most importantly, safe for patients, as it can lead to rupture of the walls of the lung inflated and the development of pneumothorax (appearance of air in the pleural cavity), mediastinal emphysema.

radical methods of treatment of congenital lobar emphysema is a surgical removal of the affected part of the lung.