Bullous Lung Disease (includes HIV-related emphysema)
Aug 30, · Bullous lung disease is an uncommon cause of respiratory distress. In patients with severe emphysema, discrete emphysematous bullae have been shown to functionally impair pulmonary mechanics and result in diminished exercise capacity and even acute respiratory distress. Bullous lung disease is characterized by the development of bullae within the lung parenchyma. A bulla is a permanent, air-filled space within the lung parenchyma that is at least 1 cm in size and has a thin or poorly defined wall; it is bordered only by remnants of alveolar septae and/or pleura. Bullae are to be distinguished from other air-filled spaces within the lung.
Bullae of Lung also known by the name of Giant Bulla is a common pathological condition found in the lungs which is usually caused by emphysema. Bullae of Lung develops when the emphysema damages the lungs to an extent that air pockets develop.
These air pockets tend to grow and put pressure on the lungs since kungs starts occupying the space within the chest cavity resulting in symptoms of chest soreness, pressure in the chest, and in some cases problems breathing. If Bullae of Lung ruptures then it may lead to a serious complication called pneumothorax. There may be severe lung dysfunction as a result of Bullae of Lung.
As stated, emphysema is the major cause of Bullae of Lung. Emphysema is a progressive medical condition in which there is irritation of the alveoli or the air sacs present in the lungs which results in shortness of breath among dosease symptoms.
Apart from emphysema there are certain other causes of Bullae of Lung or Giant Bulla. These causes are:. Radiological tests in the form of an how to hack into wireless router or advanced imaging in lunggs form of Whxt or MRI scan of the chest is good enough to diagnose Bullae of Lung.
Once Bullae of Lung is identified, in order to look for more information on the type of the bulla and the extent to which the lung has been damaged the physician may go for inserting a needle in the bulla and analyzing the cells and fluid within the bulla. A biopsy may also be performed to look at the status of the Ot of Lung.
For almost all of the cases of Bullae of Lung, the patient is treated with a surgical procedure called bullectomy in which the Bullae of Lung is completely removed. Before embarking on the removal of the Bullae of Lung, the patient will first be categorized based on the nature and size of the Bullae of Lung or Giant Bulla into one of the four categories. In the first category, patients tend to have a single bulla but have normal lung function.
In the second category, there are multiple Bullae of Lung but the lung function is not impaired. In the third category, there are multiple bullae with the lung thw being impaired by the underlying emphysema. In the fourth category, there are multiple bullae and the function of the lung is impaired by other medical conditions. Unfortunately, for people falling in the wwhat and fourth category do how to do a flowchart in word have the same outcome and in cases where there is massive destruction of the lungs due to emphysema or other medical conditions then the chances of complete recovery is very minimal and the patient ultimately is advised to undergo lung transplantation for treatment of Bullae of Lung or Giant Bulla.
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Feb 07, · Bullous lung disease, or bullous emphysema, is a long-term progressive disease of the lungs characterized by single or multiple bullae. A bulla is a blister of more than 5 mm (about 3/16 inch) in diameter with thin walls that is full of fluid or air. Bullae on the pleura, the membrane that covers the lung, are also called blebs. The term bullous disease of the lungs indicates the presence of bullae, which are abnormal airspaces resulting from the destruction of normal airspace wall tissue and are best described as thin. Feb 15, · Bullous emphysema is a medical condition in which spherical air sacs in the lungs become severely enlarged and eventually rupture and deteriorate. Individuals with progressive bullous emphysema often experience chest pain, difficulty breathing, chronic coughing, and other debilitating symptoms related to a lack of oxygen in the blood.
A bulla is an air-containing space within the lung parenchyma that arises from destruction, dilatation, and confluence of airspaces distal to terminal bronchioles and is larger than 1 cm in diameter Fig.
Figure Blebs and bullae. Development of a bleb. A bleb is an accumulation of air within the pleura that is not confined by connective tissue septa within the lung. Air that escapes within the substance of the lungs makes its way to the surface, separating the internal from the external elastic layers on the visceral pleura.
Different types of bullae. In contrast to a bleb, a bulla is confined by connective tissue septa of the lung and is deep to the internal elastic layer of the visceral pleura.
Three different types of bullae are shown arising from a lung that has been removed from within the chest wall. The short dark lines denote connective tissue septa. Adapted with permission from Reid L. The Pathology of Emphysema.
Chicago: Year Book; — Distinctions are drawn between bullae, blebs, and cysts Table Cysts are epithelial-lined cavities that may resemble bullae on radiographs. The term bullous disease is reserved for multiple bullae in lungs that are otherwise normal. Confusion occasionally arises between the two entities because some pathologists regard bullous disease as a subset of panacinar emphysema. Bullae may occur not only as part of obstructive lung disease, but also as a complication of fibrotic lung disease Table Bullae are classified anatomically into three main types Fig.
This type of bulla may be caused by overinflation of a volume of flawed lung tissue. The walls of type I bullae are thin, and their interiors are empty.
Type I bullae are usually found at the lung apices and along the edges of the lingula and middle lobes. They often occur in association with paraseptal emphysema.
Scanning electron microscopy has demonstrated that the thin neck is a consistent feature and that pleural mesothelial cells on the external surface are either reduced in number or completely absent; bundles of collagen fibers lie naked and separated from one other by small pores or crevices. Type II bullae arise from the subpleural parenchyma and are characterized by a neck of panacinar emphysematous lung tissue.
The interior of these airspaces consists of emphysematous lung in which blood vessels are still present. In contrast to type I bullae, the outer wall is formed by pleura covered with intact mesothelial cells. Although connective tissue septae are present within the bullae, they are not found in the wall.
Type II bullae may occur anywhere in the lung, but they are most frequent in the upper lobe, at the anterior surface of the middle lobe, and over the diaphragm. Type III bullae consist of slightly hyperinflated lung connected to the rest of the lung by a broad base extending deep into the parenchyma. This type is believed to represent an atrophic form of emphysema. Several hypotheses have been proposed over the years for how bullae develop, although none have been proved.
This theory underscores the proclivity of bullae for the upper lobes and stresses the influence of mechanical forces acting upon flawed tissue. Of all the hypotheses, that of underlying paraseptal emphysema is the most popular.
The pattern relates to the fact that capillaries in alveolar walls that abut connective tissue septa are less numerous than elsewhere because of a sparse network of arterioles and arteries in peripheral alveoli.
Consequently, these regions of the acinus have less vascularity and greater compliance. Dynamic computerized tomography and intrabulla pressure measurements have raised questions about the theory that bullae are formed by positive pressure within the airspace. The pressure within a giant bulla has been found to be the same as pleural pressure. Therefore, when a bulla and its surrounding lung are exposed to the same negative pleural pressure, the bulla fills preferentially and completely like an inflated paper bag, prior to the surrounding lung inflating.
Further inspiration increases the elastic recoil pressure, thereby exerting a greater retractive force on the lung parenchyma and enlarging the airspace. Nevertheless, bullae can be removed from within the lung while still maintaining their volume, indicating a positive intrabulla pressure. Bullae within the intact chest are molded and compressed to fit adjacent anatomic configurations.
However, if the lung is released from these constraints e. Within the thoracic cavity, large bullae cause crowding of adjacent lung parenchyma, and structures such as bronchi are displaced, stretched, and narrowed over the bullae surfaces. Very large airspaces can expand across the midline or even extend into the neck. Bullae represent more than just overexpanded alveoli, because the remnants of bronchioles and their accompanying vessels sometimes persist as trabeculae within the bullae.
Interlobular septae can be incorporated into the wall as the airspace expands from within the secondary lobule. Figure A. Surgically resected specimen with a bulla projecting from the lung surface. A bulla is shown projecting through a previous chest tube insertion site onto the surface of the skin.
Many patients with bullous emphysema are cigarette smokers, and most bullous lesions are associated with paraseptal or centriacinar emphysema. A hereditary predisposition to bullous emphysema is also suggested by its association with a variety of rare familial disorders, including Fabry disease, Salla disease, cutis laxa, Ehlers—Danlos syndrome, and Marfan syndrome.
The tight skin mouse, which has a dominant mutation for the elastase gene and is characterized by multiple connective tissue abnormalities, serves as a unique model for bullous emphysema.
As noted previously, the tendency for bullae to occur in the upper lobes is usually attributed to the greater mechanical stresses imposed on the lung apices than bases. Because intrapleural pressure near the lung apices is more negative than at the bases, apical alveoli are subjected to greater expanding stresses than are basal alveoli. Radioactive gas studies and in situ freezing techniques have demonstrated that alveoli in the upper lung zones are considerably larger than those in the lower zones.
Gravity also plays a role, as the upright lung behaves like a coiled spring, which, when allowed to dangle in the upright position, shows larger gaps between coils at the top than the bottom.
Engineering techniques used to study the distribution of stresses in aircraft have been applied to the analysis of stresses on the lung.
The stresses tend to increase with expansion of the lung, but they are present also when the lung volume decreases below functional residual capacity FRC. The increase in apical stress at low lung volumes has been attributed to an increase in the rigidity of the lungs as residual volume is approached. In asymptomatic individuals, bullae may be detected in the course of routine chest radiography.
Small bullae rarely become visible on the chest radiograph but are usually easily visible by computed tomography CT.
As a rule, small bullae usually produce no symptoms, signs, or discernible alterations in pulmonary function. In some patients bullae give rise to progressive dyspnea or chest pain Fig. In patients with known bullous disease infection in a bulla can occur see below. Radiographically, infection is usually identified by the appearance of an air—fluid level Fig.
Only infrequently do giant bullae reach a size sufficient to cause a localized decrease in regional air entry, with absent breath sounds and increased resonance to percussion. Chest radiograph demonstrating a right lower lobe bulla.
CT image of the lungs demonstrating the right lower lobe bulla with compressed lung arrow. Perfusion scintigraphy demonstrating decreased perfusion to the right lower lung zone. CT image of the lung following surgical resection of the right lower lobe bulla.
Chest radiograph PA view showing bilateral upper zonal areas of increased radiolucency and an air-fluid level in the left upper lobe bulla. Chest CT scan axial view demonstrating bilateral upper lobe bullae. Chest CT scan coronal view demonstrating bilateral upper lobe bullae.
Routine laboratory testing in the evaluation of bullae includes the measurement of hemoglobin and hematocrit to identify if anemia is contributing to respiratory symptoms and to assess for possible secondary polycythemia due to chronic hypoxemia.
Imaging techniques used in the evaluation of bullous lung disease include chest radiography, computed tomography, and nuclear medicine-based studies. Distinction between hairline shadows produced by a bulla, and thicker, sometimes irregular, walls of a cavity is usually not difficult. More difficult is distinguishing bullae from cysts. The presence of other radiologic signs of emphysema or fibrotic lung disorders suggests that the cystic structure is a bulla.
A chest radiograph obtained after forced expiration is sometimes helpful in demonstrating the presence of bullae: air trapping during the expiratory maneuver accentuates their outline by preventing a decrease in their size as the surrounding lung empties.
CT provides valuable anatomic information about the size, number, and relationships of bullae, as well as crowding of adjacent lung and disposition of the pulmonary vasculature Fig. High-resolution computed tomography HRCT shows that large bullae are frequently associated not only with distal acinar paraseptal emphysema, but also with centriacinar emphysema—the type of emphysema usually associated with cigarette smoking.
These observations are consistent with the hypothesis that peripheral airspaces in paraseptal emphysema may coalesce to form larger bullae that may crowd normal adjacent lung. In addition, CT has shown that when bullae occur in the context of generalized emphysema, the extent of bullous emphysema correlates poorly with measurements of pulmonary function, and that the main determinant of respiratory function is the severity of emphysema in the bullous-free parts of the lung.
On chest CT, giant bullae are predominantly located in the upper lobes and are generally subpleural. Approximately half of the patients have bilateral bullae Fig. Chest CT axial view showing bilateral upper lobe bullae. Chest CT axial view demonstrating larger left upper lobe compared to right upper bulla. Chest CT coronal view; three-dimensional reconstruction demonstrating bilateral upper lobe bullae arrows. Lung scanning using radionuclide-based techniques may provide useful preoperative information in evaluating patients with bullous lung disease.
A single-breath scan using xenon often fails to demonstrate ventilation of a bulla, whereas a continuous ventilation scan often shows slow filling and emptying of the structure. Clinical evaluation of bullous lung disease is aided by assessment of pulmonary function, pulmonary mechanics, exercise performance, and the pulmonary circulation.
Pulmonary function tests have considerable practical value in distinguishing between individuals with localized bullae in whom intervening lung is normal bullous disease , and those in whom localized bullae are part of obstructive airways disease bullous emphysema Table Bullous Disease of the Lung. Only gold members can continue reading. Log In or Register to continue.
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