pulmonary hypoplasia

August 12, 2017 17:51 | Genetic Diseases

Congenital absence or hypoplasia of the lung - the most severe malformations of the respiratory system.Under agenesis understand the absence of light, along with the main bronchus.Aplasia characterized by the absence of light in the presence of rudimentary main bronchus.When hypoplasia are the main and lobar bronchi, which end functional rudiment imperfect, underdeveloped lung tissue.

Now allocate a simple and cystic hypoplasia.Different degrees of immature lungs are considered a violation of this body in the early stages of fetal development (embryogenesis), often at the 6th week after ovulation.

pulmonary hypoplasia more common.There are a number of key factors leading to the development of hypoplasia.Foremost among them is the reduction of the volume of the chest cavity of the fetus, which is the case with diaphragmatic hernia, thoracic deformities, spinal changes, effusion in the pleural cavity in the swollen fruit.As one of the possible causes of hypoplastic considered a violation of patency of

the fetal airway and pulmonary vascular malformations.It emphasizes the relationship of lung hypoplasia with abnormalities of the kidneys and urinary tract.Described familial pulmonary hypoplasia.

clinical picture agenesis, aplasia and hypoplasia of the lung is determined both by the defect, and laminating infection.From the first years of life the child has observed the repeated pneumonia and bronchitis, cough, usually wet, there is shortness of breath.Children lag behind in physical development.Noteworthy is the characteristic deformity of the chest - retraction or flattening on the side of evil.In children with pulmonary hypoplasia sometimes found keeled bulging in the sternum ( "chicken breast"), which could probably be due to swelling of the unaffected lung, which occurs as a compensation to maintain normal oxygen supply of the organism.

When percussion (percussion) lung sounds on the side of vice shortened, resembles that when tapped a fingertip on a wooden tabletop.Normally, you can hear clear sound with percussion over the unmodified tissue healthy lung, which is called pulmonary.Shortening sound with percussion can say anything about the seal lung tissue (as seen with pneumonia), or in the absence of this point of the lung tissue (which just celebrated with hypoplasia and congenital absence of light).When listening to a stethoscope a healthy person can hear specific sounds that occur during breathing (breath sounds).In hereditary diseases of the lungs, these noises are absent or greatly weakened, which also points to the absence or underdevelopment of the lung tissue.In humans, a number of bodies located between the lungs in the middle of the body, which are combined into a single entity called "mediastinum".For mediastinal organs include the heart, trachea, esophagus, various large vessels and nerves.In the event of a defect or congenital absence of lung mediastinal organs are biased towards evil.This is due to the fact that to the side of the chest there is free space and healthy lung "displace" in this space mediastinal organs.In the event that affected the right lung, heart, it moves to the right, which can lead to misdiagnosis Kartagener syndrome.As a result of displacement and rotation (rotation) of the heart at the right-sided heart defect activity is listened to the right of the sternum, and not the left, as in healthy people.

X-ray examination of the chest revealed typical signs of the disease.These primarily include the reduction in volume of the chest on the side of the disease, affected half chest looks compared with healthy darker (due to the absence in the place of air which imparts a healthy lung lighter in color), the aperture is high and due to the absencelung tissue.The heart and other organs mediastinal shift to the affected side, which is clearly visible in the picture.Also in the image can be clearly seen healthy lung displacement in the opposite direction of the chest ( "pneumocele").

The diagnosis agenesis, aplasia and hypoplasia of the lung is crucial bronchoscopy examination.Bronchoscopy (method of investigation of the bronchial tree, in which the airways introduced a special tool with CCTV - bronchoscope) reveals the absence or vestige of the main bronchus, narrowing of bronchi equity.On bronchograms with agenesis of light there is a lack of the main bronchus, with aplasia (hypoplasia) has undeveloped (rudimentary) bronchus.In the case of hypoplasia of contrast agent filled in the large bronchi and smaller bronchial branching absent.If underdevelopment lobe revealed a decrease in its volume, bronchial branching in this area are not available or are narrowed and deformed.In the diagnosis of agenesis, aplasia and hypoplasia of the lung is used algiopulmonografiya, t. E. Research vessels of the lungs.This method gives an idea about the state of branches of the pulmonary artery, the displacement and rotation of the heart and major blood vessels.

In a study of children with aplasia or hypoplasia of the lung (or fraction) is also used radionuclide blood flow valuation method in the lung tissue.In this radioisotope is accumulated in the lung tissue and in lung undeveloped part no accumulation registers.

agenesis (aplasia, hypoplasia) light is often associated with other malformations.Described defects of the heart and blood vessels, skeletal abnormalities of the gastrointestinal tract, are quite common diaphragmatic hernia.

lung hypoplasia in some cases is one of the manifestations of genetically determined syndromes.Thus, among the congenital and hereditary diseases described a syndrome that involves contracture of the fingers, multiple joint deformities, facial anomalies and pulmonary hypoplasia.

There is speculation about an autosomal recessive type of inheritance of this syndrome.Close to the described above syndrome is a tetralogy of Potter, which includes a combination of lung hypoplasia with abnormalities in facial structure, clubfoot, change hands and retarded physical development.

combination of pulmonary hypoplasia (or share) with an anomaly of the pulmonary veins is known as the "Scimitar Syndrome".Described combination of the syndrome associated with other malformations of lung development.These changes are known to be hereditary.

the treatment of patients with agenesis and aplasia easy method of choice is conservative therapy aimed at the suppression of respiratory infections (breathing) tract, maintaining its normal functions.In hypoplastic lung hypoplasia share of preferred surgical treatment.The question of the desirability and feasibility of surgical intervention is decided by the volume of lung tissue that is to be removed, as well as on the functional status of the remaining lung segments.