Pneumothorax



Pneumothorax





A pneumothorax is air in the pleural space, that is, air between the lung and the chest wall. Pneumothoraces can be divided into spontaneous pneumothoraces, which occur without antecedent trauma or other obvious cause, and traumatic pneumothoraces, which occur from direct or indirect trauma to the chest. A subcat-egory of traumatic pneumothorax is iatrogenic pneumothorax, which occurs as an intended or inadvertent consequence of a diagnostic or therapeutic maneuver. Spontaneous pneumothoraces are further divided into primary and secondary spontaneous pneumothoraces. Primary spontaneous pneumothoraces occur in otherwise healthy individuals, whereas secondary spontaneous pneumothoraces occur as a complication of underlying lung disease, most commonly chronic obstructive pulmonary disease (COPD).


PRIMARY SPONTANEOUS PNEUMOTHORAX


Incidence

The most complete figures on the incidence of primary spontaneous pneumothorax probably come from a study of the residents of Olmsted County, Minnesota, where complete medical records are kept on all residents. Between 1959 and 1978, 77 cases of primary pneumothorax occurred among the county’s population that averaged 60,000 over this period. The age-adjusted incidence of primary spontaneous pneumothorax was 7.4/100,000/year for men and 1.2/100,000/year for women (1). If these figures are extrapolated to the entire population of 250 million in the United States, one can anticipate approximately 10,000 new cases of primary spontaneous pneumothorax per annum. In a more recent study from the United Kingdom, the incidence of spontaneous pneumothorax in males and females aged 15 to 34 was 37.0 and 15.4/100,000/year, respectively. Since most patients in this age range have primary spontaneous pneumothorax, it appears that the incidence in the United Kingdom is greater than that previously reported in the United States (2).


Etiologic Factors

The general consensus is that primary spontaneous pneumothorax results from rupture of subpleural emphysematous blebs that are usually located in the apices of the lung (3,4). In one older study, Gobbel et al. operated on 31 patients with primary spontaneous pneumothorax and found subpleural blebs or bullae in each patient (3). In a more recent study, Lesur et al. (4) obtained computed tomography (CT) scans on 20 young (mean age 27) patients with spontaneous pneumothorax and could demonstrate apical subpleural emphysematous lesions in 16 of the 20 patients (80%). In another study, Bense et al. obtained CT scans on 27 nonsmoking patients with primary spontaneous pneumothorax and reported that 22 (81%) had emphysema-like changes, mostly in the upper lobes (5). It appears that the apical blebs present on direct visualization and the emphysema-like changes seen on CT scan represent the same abnormality. It should be noted, however, that there is some controversy concerning the significance of the subpleural blebs. Noppen et al. have identified abnormal regions of the visceral pleura by fluoresceinenhanced autofluorescence thoracoscopy and suggest that leakage of air through these areas, rather than rupture of blebs, may be responsible for primary spontaneous pneumothorax (6). I believe that most primary spontaneous pneumothoraces are due to
rupture of a bleb. The reason that I believe this is that the symptoms of primary spontaneous pneumothorax start suddenly. If the pneumothorax were due to slow leakage through the visceral pleura, the symptoms should not start abruptly.

The pathogenesis of these subpleural blebs is probably related to airway inflammation. Respiratory bronchiolitis was found in 70 of 79 patients (89%) who underwent a surgical procedure for recurrence or persistence of primary spontaneous pneumothorax (7). All the patients in this study were smokers, and cigarette smoking can certainly produce airway inflammation. Cigarette smoking is known to be strongly associated with the development of primary spontaneous pneumothorax. When the smoking habits of 505 patients from four separate studies were analyzed (8,9,10,11), 461 of the patients (91%) were smokers. Furthermore, the occurrence of a spontaneous pneumothorax appears to be related to the level of cigarette smoking. Compared with nonsmokers, the relative risk of a pneumothorax in men is seven times higher in light smokers (1-12 cigarettes/day), 21 times higher in moderate smokers (13-22 cigarettes/ day), and 102 times higher in heavy smokers (>22 cigarettes/day). For women, the relative risk is 4, 14, and 68 times higher in light, moderate, and heavy smokers than in nonsmokers, respectively (11). Disease of the small airways related to smoking probably contributes to the development of the subpleural blebs (12). Interestingly, the prevalence of smoking in Chinese patients with primary spontaneous pneumothorax is only about 50% (13,14).

Two studies concluded that spontaneous pneumothoraces were more likely to develop following days when there are broad swings in the atmospheric pressure (15,16). It was postulated that the air in the apical blebs was not in free communication with the airways. Therefore, when the atmospheric pressure falls, the distending pressure of the bleb may increase and could result in its rupture (15). It should be noted that three other studies found no relationship between change in the atmospheric pressure and the occurrence of a spontaneous pneumothorax (17,18,19). In one study, however, there was a significant relationship between thunderstorms and the occurrence of pneumothoraces (17). Noppen et al. described the development of five episodes of primary spontaneous pneumothorax in four patients upon exposure to loud music (20).

Patients with primary spontaneous pneumothorax are usually taller and thinner than control patients. In a study of military recruits with pneumothorax, Withers et al. (21) found that those with pneumothoraces were 2 in. taller and 25 lb lighter than the average military recruit (21). Because the gradient in pleural pressure is greater from the lung base to the lung apex in taller individuals (see Chapter 2), the alveoli at the lung apex are subjected to a greater mean distending pressure in taller individuals. Over a long period, this higher distending pressure could lead to the formation of subpleural blebs in taller individuals who are genetically predisposed to bleb formation.

The tendency to develop a spontaneous pneumothorax may be genetically determined (22). There is a high incidence of pneumothorax in patients with the Birt-Hogg-Dubé syndrome. This syndrome is autosomal dominant (23) and is characterized by an increased incidence of spontaneous pneumothorax, benign skin tumors, and renal tumors (23,24). The gene has been mapped to chromosome 17p11.2 (23). Mutations in the folliculin (FLCN) gene are responsible for the Birt-Hogg-Dubé syndrome (25). At least 53 different germline mutations and 31 SNPs have been identified in patients with the Burt-Hogg-Dubé syndrome (26). Pneumothorax occurred in 25 of 111 patients (22.5%) in one study (27). Radiographically, 15% to 83% have pulmonary cysts and/or bullae (28). When these patients are explored surgically, they are found to have apical blebs (27). Microscopic examination of the resected lung tissue reveals cysts comprising intraparenchymal collections of air surrounded by normal parenchyma or a thin fibrous wall or blebs consisting of collections of air within the pleura (28).

There have been other reports of a familial tendency for the development of primary spontaneous pneumothorax. In one study of primary spontaneous pneumothorax in the Israeli Defense Forces, 11.5% of 286 patients with spontaneous pneumothorax had a positive family history for primary spontaneous pneumothorax (29). A more in-depth analysis of 15 families suggested that the mode of inheritance for the tendency for pneumothorax was either autosomal dominant with incomplete penetrance or X-linked recessive (30). These reports were written before the Birt-Hogg-Dubé syndrome was described and the patients may have had this syndrome. In another report of patients with familial pneumothorax, individuals with human leukocyte antigen (HLA) haplotype A2, B40 were found to be much more likely to have a pneumothorax (31). Other studies of familial pneumothorax have been unable to document any association with the HLA haplotypes (32).
Well-known inherited diseases associated with pneumothorax include Marfan’s syndrome, homocystinuria, Ehlers-Danlos syndrome, and α1-antitrypsin deficiency (24).

There is a very high prevalence of bronchial abnormalities in nonsmoking patients with spontaneous pneumothorax. Bense et al. (33) performed fiberoptic bronchoscopy on 26 people who had never smoked but had a history of spontaneous pneumothorax. They reported that 25 of 26 (96%) of the patients had bronchial abnormalities bilaterally. In comparison, only 1 of 41 control patients had such abnormalities (33). The bronchial abnormalities included disproportionate bronchial anatomy (smaller than normal dimensions and deviating anatomic arrangements of the airways at various locations), an accessory bronchus, or a missing bronchus. The most common abnormality was the disproportionate bronchial anatomy (33).


Pathophysiologic Features

When resected specimens from the lungs of patients with spontaneous pneumothorax are examined, there is frequently an eosinophilic pleuritis (34). In addition, some patients have mild pulmonary vascular and perivascular eosinophilia (34). Many patients also have pulmonary artery intimal fibrosis and pulmonary vein intimal fibrosis (35). The eosinophilic pleuritis is probably directly related to the air in the pleural space, and the presence of abnormalities in the pulmonary vessels should not serve as an indication to investigate the patient for pulmonary vascular disease.

The physiological consequences of a pneumothorax are discussed in Chapter 3.


Clinical Manifestations

The peak age for the occurrence of a primary spontaneous pneumothorax is the early 20s, and primary spontaneous pneumothorax rarely occurs after age 40. The main symptoms associated with the development of primary spontaneous pneumothorax are chest pain and dyspnea. In a series of 39 patients reported by Vail et al. (36), every patient had chest pain or dyspnea, and both symptoms were present in 25 of the 39 patients (64%). Seremetis (10) reported chest pain in 140 of 155 patients (90%). The chest pain usually has an acute onset and is localized to the side of the pneumothorax. On rare occasions, the patient has neither chest pain nor dyspnea. The pneumothorax is bilateral in less than 2% of patients (13). In the series of Seremetis (10), five patients (3%) complained only of generalized malaise. On rare occasions, the pneumothorax is discovered on a routine chest radiograph (37). Horner’s syndrome has been reported as a rare complication of spontaneous pneumothorax and is thought to be due to traction on the sympathetic ganglion produced by shift of the mediastinum (38).

Primary spontaneous pneumothorax usually develops while the patient is at rest. In the series of 219 patients of Bense et al. (39), 87% were at rest at the onset of symptoms and none were exerting themselves heavily when symptoms began. Other series have reported comparable findings (8,10).

Many patients with spontaneous pneumothorax do not seek medical attention immediately after the development of the symptoms. Eighteen percent of the patients in one series had symptoms for more than a week before seeking medical attention (10), whereas 46% in a second series waited more than 2 days before seeing a physician (8). Patients with symptoms for more than 3 days should not have negative pressure applied to their chest tubes in view of the higher incidence of reexpansion pulmonary edema with prolonged pneumothorax (see Chapter 28).


Changes on Physical Examination

Physical examination of patients with primary spontaneous pneumothorax reveals vital signs that are usually normal, with the exception of a moderate tachycardia. If the pulse rate exceeds 140 or if hypotension, cyanosis, or electromechanical dissociation is present, a tension pneumothorax should be suspected (see the section later in this chapter on tension pneumothorax). Examination of the chest reveals that the side with pneumothorax is larger than the contralateral side and moves less during the respiratory cycle. Tactile fremitus is absent, the percussion note is hyperresonant, and the breath sounds are absent or reduced on the affected side. The trachea may be shifted toward the contralateral side. With right-sided pneumothoraces, the lower edge of the liver may be shifted inferiorly.


Electrocardiographic Changes

Patients with spontaneous pneumothorax may show electrocardiographic changes due to the pneumothorax (40). In a study of seven patients with spontaneous left pneumothorax, Walston et al. (41) found that a rightward shift of the frontal QRS axis, a diminution of precordial R voltage, a decrease in QRS amplitude,
and precordial T-wave inversion could all occur. A different study (40) reports that the V2-6 amplitude was decreased with left-sided pneumothorax. In a study (42) of patients with right-sided pneumothorax, prominent R-wave voltage in lead V2 with loss of S-wave voltage, mimicking posterior myocardial infarction, and reversible reduced QRS voltage were reported. In another study (40), the QRS amplitude was increased in V5-6 with right-sided pneumothorax (40). In addition, marked PR-segment elevation in the inferior leads with reciprocal PR-segment depression in aVR has been reported (43). These changes should not be mistaken for an acute non-Q wave myocardial infarction. There has also been a report of a patient with a tension pneumothorax who developed pronounced ST-segment elevation in II, III, a VF, and V4-6 (44). When a chest tube was inserted, the ST changes resolved and studies of myocardial enzymes were negative (44).



Quantitation

One should estimate the amount of lung collapse when treating a patient with a pneumothorax. The volume of the lung and the hemithorax are roughly proportional to the cube of their diameters. Therefore, one can estimate the degree of collapse by measuring an average diameter of the lung and the hemithorax, cubing these diameters, and finding the ratios.

Mathematically,


For example, in Figure 6.12, the average diameter of the hemithorax is approximately 10 cm, and the distance between the lung and chest wall is 4 cm. Therefore, the ratio of the diameters cubed 63:103 equals 22% and approximately a 80% pneumothorax is present, although it appears substantially smaller at first glance. This method of estimating the size of primary spontaneous pneumothorax has been called the Light index (55). Noppen et al. have demonstrated that there is a close correlation between the Light index and the actual amount of air in the pleural space, as quantitated by manual aspiration (55).

Collins et al. (56) have described an alternate method for estimating the percentage of collapse. With their method, the distance between the apex of the partially collapsed lung and the apex of the thoracic cavity (distance A), and the midpoints of the upper (distance B) and lower (distance C) halves of the collapsed lung and the lateral chest wall were measured in centimeters. They found that the percentage pneumothorax size could be calculated by the formula,

% pneumothorax = 4.2 + [4.7 × (A + B + C)]

When the volume calculated from a helical CT scan was compared with the volume measured with this formula, the correlation coefficient in 20 patients was 0.98 (56). Even though the correlation coefficient
was very high, improvements can be made in the preceding formula because it does not take into account the patient’s size. Obviously, a very large person will have a smaller pneumothorax in relation to the overall size of the lung than a small person with identical distances between the lung and the chest wall.

A third method for estimating the size of a pneumothorax, the Rhea method, uses a nomogram that relates the average intrapleural distance to the pneumothorax size (57). On this nomogram, there is a 10% pneumothorax for every centimeter of intrapleural distance. A recent article found that there was a close correlation when the Collins method and the Rhea method were used to calculate pneumothorax percentage (58). However, there is no close agreement between the Collins method and the Light index (59). In general, the Rhea method or the Collins method is recommended.

The size of a pneumothorax can also be calculated from a CT scan of the thorax (60). Cai et al. (60) demonstrated that was very close agreement for the volume of a pneumothorax calculated via a computer from the chest CT scan and the volume of air aspirated from the hemithorax.

Position papers have used much simpler schemes for semiquantitating the size of pneumothoraces. In the British Thoracic Society’s (BTS) guidelines for the management of spontaneous pneumothorax, small pneumothoraces were defined as those in which the rim of air between the pleura and the chest wall at the level of the hilum was less than 2 cm and large as greater than 2 cm (61). In their consensus statement of the management of spontaneous pneumothorax, the American College of Chest Physicians defined a small pneumothorax as one in which the apex-to-cupola distance was less than 3 cm whereas a large pneumothorax was one in which this distance was greater than 3 cm (62). Collins method and Rhea method are preferred to measuring just the apex-to-cupola distance because they give a more precise estimate of the size of the pneumothorax.


Recurrence Rates

A patient who has had a primary spontaneous pneumothorax is at risk of having a recurrence. Sadikot et al. (63) followed up 153 patients with primary spontaneous pneumothorax for a mean of 54 months and found that the recurrence rate was 54.2%. In this study, the recurrence rates were less in men (46%) than in women (71%) and were less in individuals who stopped smoking (40%) than in those who continued smoking (70%). There was no significant relationship between the size of the original pneumothorax or the treatment of the original pneumothorax and the recurrence rates. Twenty-four of their patients (16%) had a pneumothorax on the contralateral side; in only one patient did the pneumothoraces occur simultaneously (63). Gobbel et al. (3) followed a group of 119 patients with spontaneous pneumothorax for a mean of 6 years. These investigators found that, of the 110 patients who did not have a thoracotomy at the time of their initial pneumothorax, 57 (52%) had an ipsilateral recurrence. Once a patient had second and third pneumothoraces without thoracotomy, the incidence of subsequent recurrence was 62% and 83%, respectively.

Older studies suggested that there is substantial risk of recurrence over many years. In the series of Gobbel et al. (3), the average interval between the first and the second pneumothorax was 2.3 years, although the average interval for recurrence in the series of Seremetis was 17 months (10). However, more recent studies have suggested that most recurrences occur within the first year (49,64,65).

Attempts have been made to predict which patients with a primary spontaneous pneumothorax are more likely to have recurrence. If one could predict which patients are more likely to have a recurrence, then those patients could be treated more aggressively to prevent a recurrent pneumothorax at the time of their first pneumothorax. The presence of blebs or bullae on chest CT scan does not predict whether the patient will develop a recurrent pneumothorax (49,51). Abolnik et al. (29) did report that taller, thinner individuals were more likely to have a recurrence. Guo et al. (66), using a multivariate analysis of 138 patients, demonstrated that recurrences were more frequent in taller patients and patients with lower weights (66).

In a recent study, Ganesalingam et al. (67) carefully studied the chest radiographs taken on the initial presentation of 100 patients for spontaneous pneumothorax for pleural thickening, blebs/bullae, pleural irregularities, and pleural adhesions. Over a mean follow-up period of 57 months, 52% of the patients had a recurrence. Patients having one, two, and three or more abnormalities were 3.0, 5.3, and 12.6 times more likely to develop a recurrence, respectively (67). They recommended that surgical treatment be offered to patients in whom two or more radiological abnormalities were identified (67).




SECONDARY SPONTANEOUS PNEUMOTHORAX

Secondary spontaneous pneumothoraces are more serious than primary spontaneous pneumothoraces because they decrease the pulmonary function of a patient with already compromised pulmonary function. The secondary spontaneous pneumothoraces that occur in patients with the acquired immunodeficiency syndrome (AIDS), cystic fibrosis, tuberculosis, lymphangioleiomyomatosis (LAM), and Langerhans cell histiocytosis are discussed in separate sections.



Incidence

The incidence of secondary spontaneous pneumothorax is similar to that of primary spontaneous pneumothorax. In the study from Olmsted County, Minnesota, the incidence was 6.3 and 2.0/100,000/year for men and women, respectively (1). If these figures are extrapolated to the entire population of the United States, approximately 10,000 new cases of secondary spontaneous pneumothorax will be seen each year. In a more recent study from the United Kingdom, the incidence of spontaneous pneumothorax for males and females above 55 was 32.4 and 10.9/100,000/year, respectively (2). Interestingly, the incidence in men kept increasing as the age increased (2).


Etiologic Factors

Most secondary spontaneous pneumothoraces are due to COPD, although almost every lung disease has been reported to be associated with secondary spontaneous pneumothorax. In one series of 505 patients from Israel with secondary spontaneous pneumothorax, the etiologies were as follows: COPD, 348; tumor, 93; sarcoidosis, 26; tuberculosis, 9; other pulmonary infections, 16; and miscellaneous, 13 (154).

There appears to be a tendency for patients with more severe COPD to develop spontaneous pneumothorax. In the VA cooperative study, which included 171 patients with secondary spontaneous pneumothorax, 51 of the patients (30%) had a forced expiratory volume1 (FEV1) <1,000 mL and 56 of the patients (33%) had an FEV1/forced vital capacity (FVC) <0.40 (65).


Clinical Manifestations

In general, the clinical symptoms associated with secondary spontaneous pneumothorax are more severe than those associated with primary spontaneous pneumothorax. Most patients with secondary spontaneous pneumothorax have dyspnea (155,156), which frequently seems out of proportion to the size of the pneumothorax (157). In one series of 57 patients with COPD, all complained of shortness of breath, whereas 42 (74%) had chest pain on the side of the pneumothorax (155). In addition, five patients were cyanotic and four patients were hypotensive.

The occurrence of a pneumothorax in a patient with underlying lung disease is a serious event. Because the pulmonary reserve of these patients is already diminished, the partial or total loss of the function of a lung can be life threatening. In one series of 18 patients in whom arterial blood gases were obtained at the time of admission, the mean Pao2 was 48 mm Hg and the mean Paco2 was 58 mm Hg (155). In the VA cooperative study, the Pao2 was below 55 mm Hg in 20 of 118 (17%) and was below 45 mm Hg in 5 of 118 (4%). The Paco2 exceeded 50 mm Hg in 19 of 118 (16%) and exceeded 60 mm Hg in 5 of 118 (4%) (65).

A substantial mortality rate is associated with secondary spontaneous pneumothorax. When three older series totaling 120 patients are combined, the mortality rate was 16% (155,157,158). Causes of death included sudden death before chest tubes could be inserted in three patients, respiratory failure within the first 24 hours of treatment in three patients, late respiratory failure in three patients, and massive gastrointestinal bleeding in three patients. However, in the VA cooperative study, none of the 185 patients with secondary spontaneous pneumothorax died from a recurrent ipsilateral pneumothorax. However, the overall mortality rate in the 5-year follow-up period was 43% (65). The high mortality rate probably reflects the severity of the underlying disease. The leading causes of death were COPD, lung cancer, pneumonia, and heart disease (65).

The physical examination of patients with secondary spontaneous pneumothorax is less helpful than it is in primary spontaneous pneumothorax. These patients already have hyperexpanded lungs, decreased tactile fremitus, hyperresonant percussion notes, and distant breath sounds over both lung fields. Accordingly, when a pneumothorax develops, side-to-side differences in the physical examination may not be apparent. The possibility of a pneumothorax should be considered in any patient with COPD who has increasing shortness of breath, particularly if chest pain is also present.



Recurrence Rates

The recurrence rates for secondary spontaneous pneumothorax appear to be somewhat higher than those for primary spontaneous pneumothorax (64,65,163). Videm et al. (163) followed a total of 303 patients for a median period of 5.5 years and reported that 24 of the 54 patients (44%) with COPD had a recurrence. In patients without COPD, 96 of 249 (39%) had a recurrence (163). In the VA cooperative study, 92 patients with secondary spontaneous pneumothorax were treated with chest tubes without pleural sclerosis and the recurrence rate was 47% with a median follow-up of 3 years (65). In this study, the recurrence rate with primary spontaneous pneumothorax was 32% (65). Guo et al. in a multivariate analysis of the factors related to recurrent pneumothorax found that patients with secondary spontaneous pneumothoraces were significantly (p < 0.007) more likely than patients with primary spontaneous pneumothorax to have a recurrence (66).

Jun 19, 2016 | Posted by in RESPIRATORY | Comments Off on Pneumothorax

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