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Pleural Disease

A. Keith Rastogi and Jeffrey P. Kanne


6

Abstract
The pleural space, which normally contains approximately 10 mL of
fluid on either side, can be a host to a variety of pathologic conditions.
The most common process to affect this space is increased fluid volume,
known as a pleural effusion. An effusion may have a variety of causes,
and both imaging and laboratory evaluation are used to distinguish
among these etiologies. Other collections in the pleural space include
chylothorax, hemothorax, or pneumothorax, and these are also important
to consider on radiologic and clinical workup. Primary pleural malig-
nancies, while rare, can be life threatening, and imaging can play a large
part in the diagnosis and management of these entities. The most
common malignancy in the pleural space is metastatic disease. The most
common primary pleural malignancy is mesothelioma, a deadly
neoplasm that is most often associated with asbestos exposure. Other
pleural neoplasms, including solitary fibrous tumor of the pleura, are
even rarer. This chapter highlights the key concepts of pleural disease,
illustrates radiologic examples of these conditions, and provides a
background into each disease process.

Keywords
Pleural effusion 
Chylothorax 
Hemothorax 
Pneumothorax 
 
Fibrothorax Mesothelioma Solitary fibrous tumor of the pleura (SFTP)

A. K. Rastogi (&)
Department of Radiology, University of Wisconsin
Pleural Disease
Hospital and Clinics, 600 Highland Ave, MC
3252 Madison, WI 53792-3252, USA Two layers of pleura surround the lung.
e-mail: arastogi@unwhealth.org The visceral pleura is tightly adhered to the lung
J. P. Kanne surface while the parietal pleural lines the tho-
Department of Radiology, University of Wisconsin racic cavity. Between these two layers is the
School of Medicine and Public Health,
pleural cavity. This space normally contains
600 Highland Ave, MC 3252 Madison,
WI 53792-3252, USA approximately 10 mL of fluid on either side.
e-mail: jkanne@uwhealth.org This fluid serves as a lubricant for the lung
J. P. Kanne (ed.), Clinically Oriented Pulmonary Imaging, 77
Respiratory Medicine, DOI: 10.1007/978-1-61779-542-8_6,
 Humana Press, a part of Springer Science+Business Media, LLC 2012
78 A. K. Rastogi and J. P. Kanne

Fig. 6.1 Partially loculated pleural effusion. a PA loculation. b Right lateral decubitus radiograph shows
radiograph shows a moderate right pleural effusion, that most of the effusion layers dependently (arrow-
which tracks into the major fissure (arrow). The heads) with a small amount of fluid loculated in the
lobulated contours (arrowheads) suggest partial fissure (arrow)

during normal respiratory motion. Processes


affecting either fluid production or removal may Diagnostic Workup: Radiologic
alter the state of this space and lead to an
increase in pleural fluid volume. This is known Frontal and lateral chest radiographs are gener-
as a pleural effusion. ally obtained as the screening test of choice
when a pleural effusion is suspected. Pleural
effusions (Fig. 6.1) obscure the costophrenic
Pleural Effusions sulcus and are best seen on the lateral view.
As pleural fluid volume increases, the entire
Pleural effusion can occur in numerous scenarios. ipsilateral hemidiaphragm may be obscured
These classically include elevated hydrostatic (Fig. 6.2). Lateral decubitus radiographs can be
pressure as occurs in congestive heart failure, obtained to evaluate for the presence of very
increased capillary permeability as occurs in the small effusions if a lateral view is not feasible or
setting of infection or neoplasm, and decreased to assess whether or not a pleural effusion is
colloid osmotic pressure as occurs in cirrhosis loculated.
and nephrotic syndrome. Decreased pleural fluid Ultrasound can be performed to further
absorption can result from any cause of lym- characterize and localize pleural fluid collec-
phatic obstruction. tions. Commonly, ultrasound is used to find a
Pleural effusions are classified into two broad large pocket of fluid and suitable skin entry site
categories, transudative or exudative, as origi- in order to facilitate drainage via thoracentesis
nally described in 1972 by Light et al. [1]. or pleural drain placement. If additional evalu-
Transudative effusions are generally the result of ation is desired, a chest CT (Fig. 6.3) can be
a systemic process, with common causes includ- performed for a detailed evaluation of the
ing cirrhosis, congestive heart failure, nephrotic pleural surfaces and lung parenchyma.
syndrome, or other causes of hypoalbuminemia. An infected pleural effusion, also known as
Exudative effusions, in contrast, are usually an empyema (Figs. 6.4, 6.5), can be suggested
associated with local disease, such as infection, by certain CT findings, including enhancing,
malignancy, or pulmonary embolism [2]. thick pleural surfaces, or the ‘‘split pleura sign’’,
6 Pleural Disease 79

Fig. 6.2 Subpulmonic pleural effusion. a PA radiograph sulcus. b Contrast-enhanced CT image shows a large
shows a dense, well-demarcated opacity (arrow) obscuring homogeneous pleural effusion (arrowhead) compressing
the hemidiaphragm and blunting the right costophrenic the right lower lobe (arrow)

which is characterized by thickened visceral and


parietal pleural surfaces separated by pleural
fluid [3]. In fact, parietal pleural enhancement
has been shown to be highly sensitive and spe-
cific in identifying patients with complicated
parapneumonic effusions or empyemas versus
those with transudative effusions [4]. Increased
CT attenuation of extrapleural fat is also sug-
gestive of empyema. Direct fluid sampling, most
commonly via thoracentesis, is required to con-
firm empyema.

Diagnostic Workup: Laboratory


Analysis

If direct fluid sampling is desired for determining


the etiology of the accumulation, thoracentesis
can be performed. Numerous laboratory tests can
be performed on a pleural fluid sample for further
characterization in order to help determine its
etiology. Classically, a protein level and lactate
dehydrogenase (LDH) level are used to assess
Fig. 6.3 Simple pleural effusion. Unenhanced CT image whether a pleural effusion is transudative or
shows a moderate layering right pleural effusion (arrow) exudative.
80 A. K. Rastogi and J. P. Kanne

Fig. 6.4 Empyema. Contrast-enhanced CT image shows


a heterogeneous left pleural collection (arrow) containing
pockets of gas (arrowheads). In the absence of instru-
mentation, gas collections such as these are presumably
from gas-forming organisms Fig. 6.5 Empyema. Contrast-enhanced CT image shows
thickening and enhancement of the visceral (arrowhead)
and parietal (straight arrow) pleura separated by an
effusion (‘‘split pleura’’) sign. Note infiltration of the
As initially described by Light et al. [1], an extrapleural fat (curved arrow)
exudative effusion meets any of the following
criteria:
• ratio of pleural fluid protein to serum protein
greater than 0.5 Other Pleural Collections
• ratio of pleural fluid LDH to serum LDH
greater than 0.6
• pleural fluid LDH greater than two-thirds the Chylothorax
upper limit of normal for serum LDH
If none of these criteria is met, then the A chylothorax forms as a consequence of injury to
effusion is classified as transudative. or disruption of the thoracic duct. Potential causes
The serum-effusion albumin gradient (serum include trauma, tumor, and venous thrombosis.
albumin concentration minus effusion albumin Lymphangioleiomyomatosis, a rare disease
concentration) can be used as a more specific characterized by lung cysts and renal angiomyo-
criterion in the diagnosis of an exudative effu- lipomas, can also cause chylothorax. Measuring a
sion [5]. A value below 1.2 g/dL has been pro- triglyceride level in pleural fluid can assist in this
posed to define an exudate. This information diagnosis. A triglyceride level greater than
should be used with the criteria mentioned above 110 mg/dL confirms the diagnosis, and a level
for thorough evaluation (see Table 6.1). less than 40 mg/dL excludes the diagnosis [2].
6 Pleural Disease 81

Table 6.1 Differentiating transudative and exudative pleural effusions


Transudate Exudate
Specific gravity \1.012 [1.020
Fluid protein: serum protein \0.5 [0.5
Fluid LDH: serum LDH \0.6 [0.6
Serum effusion albumin gradient (g/dL) [1.2 \1.2
Cholesterol content (mg/dL) \45 [45

Fig. 6.7 Traumatic pneumothorax from collision in a


soccer game. Coned-down view of a PA radiograph
shows a sharp, thin visceral pleural line denoting the
small pneumothorax
Fig. 6.6 Hemothorax. Unenhanced CT image shows a
high attenuation right pleural collection (black arrow)
similar in attenuation to skeletal muscle. A large anterior
mediastinal hematoma (white arrow) is also present 30–70 Hounsfield units (HU). In contrast,
a simple pleural effusion measures \20 HU.
Furthermore, if the hematocrit level in the pleural
fluid is greater than 50% of the patient’s serum
Hemothorax hematocrit level, a hemothorax is present.

Hemothorax refers to an accumulation of blood in


the pleural space. This condition is most often the
result of chest trauma. Non-traumatic etiologies Pneumothorax
are rare but include underlying malignancy or
pulmonary embolism. On imaging, a hemothorax Air within the pleural space is referred to as a
should be suspected if the attenuation of pleural pneumothorax. Causes include trauma, iatrogenic
fluid on CT imaging (Fig. 6.6) is approximately causes (surgery, barotrauma), chronic obstructive
82 A. K. Rastogi and J. P. Kanne

Fig. 6.8 Spontaneous pneumothorax. a PA radiograph adhesion (arrow) is apparent in the pneumothorax,
shows a moderate right pneumothorax (arrowheads) and a presumably from previous pneumothorax. c CT image at
large right apical bleb (arrow). b CT image confirms the the level of the diaphragm shows several pockets of pleural
large bleb, which has a thin wall (arrowheads). An air (arrowheads) and small pleural adhesions (arrow)

Fig. 6.9 Pneumothorax and deep sulcus sign. Supine


AP radiograph shows hyperlucency in the inferolateral
aspect of the left hemithorax and inferior displacement of
the costophrenic sulcus (arrow). Note the sharpness of
the left inferior mediastinal and diaphragmatic borders

pulmonary disease (often from rupture of a


peripheral bleb into the pleural space), infection,
and malignancy. Pneumothoraces can be small
and produce no symptoms or can be life threat-
ening.
Fig. 6.10 Hydropneumothorax. a Supine AP radiograph
On an upright chest radiograph, a pneumo- shows diffuse hazy opacity in the right hemithorax. Note the
thorax is seen as a well-defined visceral pleural sharp outline of the right heart and mediastinal borders.
line usually located along the apical and lateral b Semiupright AP radiograph obtained shortly after A clearly
shows a sharp visceral pleural line (arrowheads) from pneu-
margins of the affected lung (Figs. 6.7, 6.8). mothorax. Blunting of the right costophrenic sulcus (arrow)
Pulmonary vessels are usually not seen reflects the liquid component of the hydropneumothorax
6 Pleural Disease 83

Fig. 6.11 Pneumothorax and deep sulcus sign. a Supine b Right lateral decubitus radiograph shows a sharp
AP radiograph shows inferior displacement of the left visceral pleural line (arrowheads) and the basal pneu-
costophrenic sulcus (arrowheads) with left basal hyper- mothorax (arrow)
lucency and sharpness of the inferior left heart border.

more inferiorly located costophrenic sulcus


(deep sulcus sign) (Fig. 6.9), basilar hyperlu-
cency, and sharply defined hemidiaphragm and
cardiac margins on the affected side. These
findings reflect that air will collect anteriorly
and basally in a supine patient (Fig. 6.10).
If pneumothorax is suspected in a patient who is
unable to stand or sit upright, a lateral decubitus
radiograph (with the suspected side up) can be
useful for further evaluation (Fig. 6.11).
A tension pneumothorax (Figs. 6.12, 6.13) is a
life-threatening situation in which the intrapleural
pressure rises, causing contralateral mediastinal
shift. Prompt identification and decompression are
imperative. Tension pneumothorax can occur
when there is free inflow of air into the pleural
space but limited outflow such as in trauma.
Fig. 6.12 Tension pneumothorax. Supine AP radiograph
shows a sharp left visceral pleural line (white arrows) and
large left pneumothorax (asterisk). The heart and trachea
(arrowheads) are displaced to the right, indicating tension.
A small right pleural effusion (black arrow) is also present
Fibrothorax

Fibrothorax describes fibrosis of the pleural


space secondary to a fibrous peel forming over
peripheral to the pleural line. In contrast to an the pleura. It can result from a number of pre-
upright radiograph, pneumothoraces may be ceding incidents that cause undrained pleural
extremely subtle or occult on a supine chest fluid collections including empyema, inflamma-
radiograph. tory pleural disease, or hemothorax [6, 7].
Findings suggesting pneumothorax on a Specific causes include tuberculosis, empyema,
supine radiograph include a very discrete and asbestosis-related pleural disease, and
84 A. K. Rastogi and J. P. Kanne

Fig. 6.14 Fibrothorax. PA radiograph shows diffuse,


coarse left pleural calcification (arrows) resulting from
remote tuberculous empyema
Fig. 6.13 Tension pneumothorax. Supine AP radiograph
shows complete right lung collapse (arrowheads), right
hemithorax hyperlucency and inferior displacement of the
right hemidiaphragm (white arrow). The mediastinum
(black arrow) is shifted to the left

hemothorax. Fibrothorax restricts lung motion


during respiration, impairing lung function. It
manifests radiographically as volume loss in the
affected hemithorax with relatively smooth
pleural thickening. Pleural calcification may be
present (Figs. 6.14, 6.15) and can be quite
extensive, especially with previous hemothorax
or tuberculous empyema. Treatment often
requires surgical decortication. Fig. 6.15 Fibrothorax. Contrast-enhanced CT scan
shows smooth left pleural thickening and calcification
(arrows). Note the marked leftward mediastinal rotation
and shift
Pleural Malignancies

gastrointestinal tract malignancies. These tumors


Metastases most commonly metastasize to the pleural sur-
face via hematogenous spread. Lymphoma can
The most common pleural malignancy is meta- arise primarily in the pleura or involve the pleura
static disease. Specifically, lung cancer is the with widespread thoracic or systemic disease.
most common primary malignancy to affect the Patients with pleural metastases may present
pleura with direct pleural invasion being the most with non-specific symptoms including pleuritic
common route of spread. Other tumors that chest pain and dyspnea. A pleural effusion is often
frequently metastasize to the pleura include present (Fig. 6.16), and there may be smooth or
breast carcinoma, ovarian carcinoma, and nodular pleural thickening (Fig. 6.17).
6 Pleural Disease 85

Fig. 6.17 Metastatic renal cell carcinoma. Contrast-


enhanced CT image shows circumferential enhancing
right pleural soft tissue thickening (arrows)

Fig. 6.16 Metastatic breast carcinoma. Unenhanced CT


image shows a small right pleural effusion and nodular foci
of pleural thickening (arrowheads). The presence of
Pleural effusions are common, seen in
mediastinal pleural thickening (arrows) is highly suggestive
of malignancy but can occur in tuberculous pleural disease 80–95% of patients, and may be the only
radiographic finding [11]. Other findings of
mesothelioma include diffuse, nodular pleural
Mesothelioma thickening and a contracted ipsilateral hemi-
thorax (Figs. 6.18, 6.19) [12]. Although cross-
The most common primary pleural malignancy is sectional imaging may suggest this diagnosis,
mesothelioma, which has an incidence of tissue sampling is required for confirmation.
approximately 2,500 cases per year in the US [8]. The most common sites of metastases include
Asbestos exposure remains the number one risk lymph nodes, bone, liver, and lung. Currently,
factor for the development of mesothelioma, and imaging is generally used to assess disease
exposure can occur in mining of asbestos fibers, as extent, specifically evaluating for surgical
well as other occupations that involve work with resectability and any evidence of metastatic
asbestos containing products such as ceiling and disease. Imaging can also assist in guiding
pool tiles or automobile brake lining [9]. Most biopsy and can aid in following disease course
patients are middle-aged men as the latency from with treatment.
asbestos exposure to clinical disease is often Unfortunately, prognosis remains poor in
several decades [10]. However, mesothelioma these patients, given the often-advanced disease
may occasionally be seen in younger patients. at time of presentation. Median survival is
Patients often present with non-specific symp- approximately 4–13 months in untreated
toms, including chest wall pain and dyspnea. patients and 6–18 months with treatment [10].
86 A. K. Rastogi and J. P. Kanne

Fig. 6.18 Mesothelioma. a PA radiograph shows cir- circumferential pleural soft tissue thickening (arrows).
cumferential, nodular left pleural thickening (arrowheads) A small effusion (asterisk) is present, and tumor (arrow-
and a slightly contracted left hemithorax. b–c Contrast- heads) has extended into the mediastinal fat
enhanced CT images show extensive nodular and

Fig. 6.19 Mesothelioma. a PA radiograph shows cir- enhanced CT image shows extensive left pleural tumor
cumferential left pleural thickening (arrowheads) and a with focal rib invasion (thin arrow) and mediastinal
large left pleural mass (white arrow). A small right (arrowheads) and left axillary (wide arrow) lymph node
pleural effusion (black arrow) is also present. b Contrast- metastases

are extremely rare malignant soft-tissue tumors.


Other Pleural Neoplasms In contrast to lipomas, liposarcomas tend to be
heterogeneous with varying degrees of contrast
Other pleural tumors are rare. Pleural lipomas enhancement and non-fatty soft tissue.
(Fig. 6.20), which rarely cause symptoms, are Solitary fibrous tumor of the pleura (SFTP) is
benign homogeneous fatty tumors that are often an uncommon neoplasm that arises from the
detected incidentally on imaging examinations submesothelial mesenchymal layer. This tumor
obtained for other reasons. Pleural liposarcomas has previously been called by many names,
6 Pleural Disease 87

Fig. 6.20 Pleural lipoma. a PA radiograph shows a consist entirely of homogeneous fat, consistent with a
large, homogeneous mass (arrow) in the left hemithorax. pleural lipoma
b Unenhanced CT image shows the mass (arrow) to

Fig. 6.21 Solitary fibrous tumor of the pleura. a PA CT image shows a large, somewhat heterogeneous mass
radiograph shows a large mass (arrow). The left heart containing calcification (arrowhead) and low attenuation
border and portions of the left hemidiaphragm remain foci (arrow)
visible, suggesting a posterior location. b Unenhanced

including benign pleural fibroma, which is mis-


leading, as it does not always have a benign diameter (Fig. 6.21) [3]. They often arise from a
course. Given its origins from pluripotent cells, stalk and may change configuration as patient
SFTP can have a variety of appearances. These position changes (Fig. 6.22). Some patients with
tumors tend to be solitary, lobulated, and het- SFTP may present with symptomatic hypogly-
erogeneous masses ranging from 2 to 30 cm in cemia or digital clubbing.
88 A. K. Rastogi and J. P. Kanne

Fig. 6.22 Solitary fibrous tumor of the pleura. PA a and CT image shows a large mass (arrow) in the lower right
lateral b radiographs show a lobulated mass (arrows) in hemithorax abutting the paraspinal region. The apparent
the lower right hemithorax. The posteromedial margins slight change in shape reflects differences in patient
are not well defined. c Coronal reformatted unenhanced position between radiography (upright) and CT (supine)

Radiologic Evaluation of Pleural References


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