Bullous emphysema of a lung are the local changes of pulmonary fabric which are characterized by destruction of alveolar partitions and formation of air cysts with a diameter more than 1 cm of (bulls). At the uncomplicated course of bullous emphysema of lungs symptoms can be absent up to developing of spontaneous pheumothorax. Diagnostic confirmation of bullous emphysema of lungs is reached by means of a X-ray analysis, KT of high resolution, a stsintigrafiya, a torakoskopiya. At an asymptomatic form perhaps dynamic observation; in case of the progressing or complicated course of a bullous pulmonary disease surgical treatment (a bullektomiya, a segmentektomiya, lobectomy) is carried out.
Bullous emphysema of a lung
Bullous emphysema of a lung – limited emphysema which morphological basis is made by air cavities (bulls) in a lung parenchyma. In foreign pulmonology it is accepted to distinguish bleba (English "blebs" - bubbles) - the air cavities less than 1 cm in size located in an interstition and subplevralno and bulls - air educations with a diameter more than 1 cm which walls are covered by an alveolar epithelium. Exact prevalence of bullous emphysema of a lung is not defined, however it is known that this disease serves as the reason of spontaneous pheumothorax in 70–80% of cases. In literature bullous emphysema of lungs can be met under the names "bullous disease", "bullous lung", "false/alveolar cyst", "syndrome of a disappearing lung", etc.
Today there is a number of the theories explaining genesis of a bullous disease (mechanical, vascular, infectious, obstructive, genetic, enzymatic). Adherents of the mechanical theory suggest that the horizontal arrangement of the I-II edges at a part of people leads to a lung top travmatization, causing development of apikalny bullous emphysema. Also there is an opinion that bulls are a consequence of pulmonary ischemia, i.e. the vascular component participates in development of a bullous disease.
The infectious theory connects an origin of bullous emphysema of lungs with nonspecific inflammatory processes, mainly, viral infections of airways. In this case local bullous changes are a direct consequence of the obstructive bronkhiolit which is followed by restretching of sites of a lung. This concept is confirmed by the fact that often a recurrence of spontaneous pheumothorax happens during the periods of flu epidemics and an adenoviral infection. Developing of local bullous emphysema in the field of a lung top after the postponed tuberculosis is possible. On the basis of observations the theory about genetic conditionality of bullous emphysema of lungs is put forward. Families in which this disease was traced at representatives of several generations are described.
Morphological changes in lungs (bull) can have either the congenital, or acquired origin. Congenital bulls are formed at deficiency of inhibitor of an elastaza - a1-anti-trypsin, enzymatic destruction of pulmonary fabric is a consequence of what. The high probability of development of bullous emphysema of lungs is noted at Marfan's syndrome, Elersa-Danlos's syndrome and other forms of a soyedinitelnotkanny dysplasia.
The acquired bulls in most cases develop against the background of the available emphysematous changes of lungs and a pneumosclerosis. At 90% of patients with bullous emphysema of lungs in the anamnesis the long experience of smoking (is traced 10-20 years at daily smoking more than 20 cigarettes). It is proved that even passive smoking increases probability of development of a bullous disease for 10–43%. Act as other known risk factors air pollution by aerogenic pollyutant, combustion gases, volatile chemical compounds and so forth; frequent respiratory diseases, hyperreactivity of bronchial tubes, violations of the immune status, male and so forth.
Process of formation of bulls takes place two consecutive stages. At the first stage the bronkhoobstruktion, limited cicatricial and sclerous processes and pleural unions create the valvate mechanism increasing pressure in small bronchial tubes and promoting formation of air bubbles with preservation of interalveolar partitions. At the second stage there is a progressing stretching of air cavities. At deficiency of a1-anti-trypsin activity of the neytrofilny elastaza causing splitting of elastichesky fibers and destruction of alveolar fabric increases. Further expansion of air cavities and the account of the mechanism of collateral breath leads to an expiratory collapse of bronchial tubes. The area of a respiratory surface is reduced, develops respiratory insufficiency.
In relation to a parenchyma of a lung distinguish bulls of three types:
- 1 – bulls are located ekstraparenkhimatozno and connected with a lung by means of a narrow leg;
- 2 - bulls are located on the surface of a lung and connected with it the wide basis;
- 3 – bulls are located intraparenkhimatozno, in the thickness of pulmonary fabric.
Besides, bulls can be solitary and multiple, one – and bilateral, intense and not intense. On prevalence in a lung differentiate localized (within 1-2 segments) and generalized (with defeat more than 2 segments) bullous emphysema. Depending on the size of the bull can be small (with a diameter up to 1 cm), average (1-5 cm), large (5-10 cm) and huge (10-15 cm in the diameter). Bulls can settle down both in not changed lung, and in the lungs affected with diffusion emphysema.
By a clinical current classify bullous emphysema of lungs:
- asymptomatic (clinical manifestations are absent)
- with clinical manifestations (short wind, cough, thorax pain)
- complicated (recurrent pheumothorax, hydropheumothorax, haemo pheumothorax, pulmonary and pleural fistula, a blood spitting, a rigidny lung, sredosteniye emphysema, chronic respiratory insufficiency).
Patients with a bullous pulmonary disease often have the asthenic constitution, vegeto-vascular disorders, a backbone curvature, deformation of a thorax, a hypotrophy of muscles. The clinical picture of bullous emphysema of lungs is defined, mainly, by its complications therefore the long period of time a disease does not prove in any way. In spite of the fact that bullezno the changed sites of pulmonary fabric do not participate in gas exchange, compensatory opportunities of lungs long remain at the high level. If bulls reach the huge sizes, they can squeeze the functioning sites of a lung, causing breath malfunction. Signs of respiratory insufficiency can decide at patients on multiple, bilateral bulls, and also the bullous disease proceeding against the background of diffusion emphysema of lungs.
The most frequent complication of a bullous disease is recurrent pheumothorax. The mechanism of its emergence is most often caused by increase in intra pulmonary pressure in bulls owing to the physical tension, a raising of weights, cough, a natuzhivaniye. It leads to a rupture of a thin wall of an air cavity with an air outlet in a pleural cavity and development of a collapse of a lung. Sharp thorax pains with irradiation in a neck, a clavicle, a hand are signs of spontaneous pheumothorax; short wind, impossibility to make a deep breath, pristupoobrazny cough, the compelled situation. Objective inspection reveals , tachycardia, expansion of intercostal intervals, restriction of respiratory excursions. Developing of hypodermic emphysema with distribution on a face, a neck, a trunk, a scrotum is possible.
Diagnosis of bullous emphysema of lungs is based on clinical, functional and radiological data. Kuration of the patient is carried out by the pulmonologist, and at development of complications – the thoracic surgeon. The X-ray analysis of lungs is not always effective in detection of bullous emphysema of lungs. At the same time, possibilities of radiodiagnosis are expanded significantly by introduction in practice of KT of high resolution. On tomograms of the bull decide as thin-walled cavities on accurate and equal contours. At the doubtful diagnosis the diagnostic torakoskopiya allows to make sure available bulls.
Ventilating the stsintigrafiya of lungs allows to estimate a ratio of the pulmonary fabric functioning and switched off from ventilation that is extremely important for planning of surgical intervention. For the purpose of definition of degree of pulmonary insufficiency function of external breath is investigated. As criterion of emphysematous changes serves decrease in OFV1, Tiffno's test and ZhYoL; increase in total amount of lungs and FRC (functional residual capacity).
Treatment of bullous emphysema of lungs
Patients with the asymptomatic course of bullous emphysema of lungs and the first episode of spontaneous pheumothorax are subject to observation. They are recommended to avoid the physical tension, infectious diseases. Methods of physical rehabilitation, metabolic therapy, physical therapy allow to prevent progressing of bullous emphysema of lungs. At the developed spontaneous pheumothorax immediate performance of a pleural puncture or drainage of a pleural cavity for the purpose of a lung raspravleniye is shown.
In case of increase of signs of respiratory insufficiency, increase in the sizes of a cavity (at a control X-ray analysis or KT of lungs), emergence of a recurrence of pheumothorax, inefficiency of the draining procedures for a lung raspravleniye, the question of expeditious treatment of bullous emphysema is raised. Depending on expressiveness of changes, localization and the sizes of bulls their removal it can be made by a bullektomiya, anatomic resections. Various operations for a bullous disease can be carried out in the open way or with use of video endoscopic technologies (a torakoskopichesky resection of a lung). On purpose the prevention of a recurrence of spontaneous pheumothorax can be carried out (processing of a pleural cavity by the iodated talc, laser or a diatermokoagulyation) or a plevrektomiya.
Forecast and prevention
Without expeditious treatment the bullous disease is followed by retsidiviruyushcha pheumothoraxes that limits household and professional activity of the patient. After the postponed surgical treatment all displays of a disease usually disappear. Prevention of a bullous disease in general is similar to actions for the prevention of emphysema of lungs. The unconditional exception of smoking (including impacts of tobacco smoke on children and non-smoking people), contact with harmful production and environmental factors, the prevention of respiratory infections is necessary. Patients with the diagnosed bullous emphysema of lungs should avoid situations, a provocative rupture of bulls.