AJR Radiologic Signs in Thoracic Imaging 2008

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1.

5 CME AJR Integrative Imaging


1.0 SAM
LIFELONG LEARNING
FOR RADIOLOGY
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Radiologic Signs in Thoracic Imaging:


Case-Based Review and Self-Assessment Module
Mark S. Parker1, Marvin H. Chasen2, Narinder Paul3

ABSTRACT B. Exercise, self assess, and improve his or her understanding


of the underlying cause for these particular imaging signs.
Objective
Chest imaging remains one of the most complicated sub- REQUIRED ACTIVITIES
specialties of diagnostic radiology. The successful interpre- 1. Six interactive case scenarios presented in this article.
tation of thoracic imaging studies requires the recognition
and understanding of the radiologic signs that are charac- RECOMMENDED READING
teristic of many complex disease processes. 1. Woodring JH, Reed JC. Radiographic manifestations
of lobar atelectasis. J Thorac Imaging 1996; 11:109–144
Conclusion 2. Catalano O. The incomplete border sign. Radiology 2002;
The educational objectives for this case-based self-assess- 225:129–130
ment module are for the participant to exercise, self-assess, 3. Chung M, Edinburgh K, Webb E, McCowin M, Webb W.
and improve his or her understanding of important thorac- Mixed infiltrative and obstructive disease on high-resolu-
ic radiologic signs that are useful in establishing the diagno- tion CT: differential diagnosis and functional correlates in
sis of particular diseases of the chest. a consecutive series. J Thorac Imaging 2001; 16:69–75
4. Whitten CR, Khan S, Munneke GJ, Grubnic S. A diag-
INTRODUCTION nostic approach to mediastinal abnormalities. Radio-
This self-assessment module on several radiologic signs Graphics 2007; 27:657–671
used in thoracic imaging to assist radiologists in establishing 5. Ferguson EC, Krishnamurthy R, Oldham SA. Classic
a particular diagnosis of pathologic processes affecting the imaging signs of congenital cardiovascular abnormali-
chest has a self-assessment component and an educational ties. RadioGraphics 2007; 27:1323–1334
component. The self-assessment component consists of six 6. Marshall GB, Farnquist BA, MacGregor JH, Burrowes
previously unpublished case-based studies with accompany- PW. Signs in thoracic imaging. J Thorac Imaging 2006;
ing clinical histories and radiologic images. These cases have 21:76–89
been selected to illustrate specific radiologic imaging signs. A
series of multiple-choice questions follows each case, with so- INSTRUCTIONS
lutions and a discussion of that particular radiologic sign and 1. Complete the educational and self-assessment components
its cause. The educational component consists of suggested included in this issue.
readings or references that accompany each case that the par- 2. Visit www.arrs.org
ticipant should review. To claim CME and SAM credit, each 3. Order the online SAM as directed. (The SAM must be
participant must log on to the ARRS Website (www.arrs.org) ordered to be accessed even though the activity is free to
and enter his or her responses to the questions online. ARRS members.)
4. The SAM can be accessed at www.arrs.org under the
EDUCATIONAL OBJECTIVES Lifelong Learning link.
By completing this educational activity, the participant will: 5. Answer the questions online to obtain SAM credit.
A. Exercise, self assess, and improve his or her understanding
of selected radiologic signs useful in establishing a particu-
lar diagnosis of pathologic processes affecting the chest.

Keywords: “head cheese sign,” “hilum convergence sign,” “hilum overlay sign,” “hogs head cheese sign,” “incomplete border sign,” luftsichel sign, thoracic imaging, “walking
man sign,” “water bottle sign”
DOI:10.2214/AJR.07.7081
Received March 9, 2008; accepted after revision July 11, 2008.
M. S. Parker is a design consultant for Worldwide Innovations and Technologies, Inc., Overland Park, KS.
1
Department of Radiology, Medical College of Virginia Hospitals–VCU Health System, Main Hospital, 3rd Floor, 1250 E Marshall St., PO Box 980615, Richmond, VA 23298-0615. Address
correspondence to M. S. Parker (msparker@vcu.edu).
Department of Diagnostic Radiology, The University of Texas M. D. Anderson Cancer Center, Houston, TX.
2

Department of Medical Imaging, Thoracic Imaging, University of Toronto, Toronto, ON, Canada.
3

AJR 2009;192:S34–S48 0361–803X/09/1923–S34 © American Roentgen Ray Society

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Scenario 1 There is ipsilateral cephalad hilar displacement and broncho-


vascular reorientation as well as leftward displacement of the
Clinical History tracheal air column. The left thorax appears smaller in vol-
A 52-year-old woman presented to her primary care phy- ume than the right. The left diaphragm is elevated and shows
sician with a several-week history of nonproductive cough, a juxtaphrenic peak. The left apex is hyperlucent, as is the left
mild dyspnea, chest tightness, and wheezing (Fig. 1). paramediastinal border. Note the luftsichel sign.
Description of Images
Frontal chest radiography (Fig. 1A) shows an ill-defined left Diagnosis
perihilar opacity partially silhouetting the left heart border. The diagnosis is luftsichel sign of left upper lobe collapse
secondary to an obstructing endobronchial carcinoid tumor
(Kulchitsky cell type I).
QUESTION 1 Solution to Question 1
What is the MOST LIKELY diagnosis? The radiographic features of pulmonary edema may in-
clude an increased cardiothoracic ratio, widening of the vas-
A. Pulmonary edema. cular pedicle, vascular redistribution or engorgement, dis-
B. Pneumonia. crepant arterial-to-bronchial ratios, interstitial Kerley lines,
C. Atelectasis. and possibly pleural effusions [1, 2]. Option A is not the best
D. Mediastinal mass. response because none of these signs is present. Pure pneu-
E. Left apical pneumothorax. monia is an air-space-replacing process characterized by an
equal exchange of air in the alveoli for pus and thus preserva-
tion of lung volume [3, 4]. Therefore, Option B is not the best
response. Option D is not the best response because the loca-
tion and morphology of the perihilar opacity support a pa-
renchyma-based, not a mediastinum-based, lesion.
Option C, atelectasis, and left upper lobe atelectasis in par-
ticular, is the best response. This case illustrates both direct and
many indirect signs of volume loss. More important, the case
shows the luftsichel sign. “Luftsichel,” which is German for
“air crescent,” is an indirect sign of overinflation characterized
by hyperexpansion of the superior segment of the left lower
lobe and its insinuation between the collapsed upper lobe and
the mediastinum. This particular imaging sign is seen in the
setting of left upper lobe atelectasis and is a manifestation of
compensatory overinflation in response to the upper lobe vol-
ume loss [5]. Because of the absence of a horizontal fissure in
the left thorax, as the upper lobe loses volume, the oblique fis-
sure becomes vertically oriented in a plane roughly parallel to
the anterior chest wall. The oblique fissure continues to shift
further anteriorly and medially, with progressive volume loss
until the atelectatic upper lobe is contiguous with the left heart
border and partially silhouetting its border (i.e., the silhouette
sign) and creating an ill-defined parahilar haze (i.e., the “veil
sign”) on the frontal examination [5–8].
As the apical segment of the collapsing upper lobe moves
anteromedially, the superior segment of the left lower lobe
A overinflates and fills in the vacated apex with aerated lung
Fig. 1—52-year-old woman with several-week history of nonproductive cough, that can mimic an apical pneumothorax; option E is not the
mild dyspnea, chest tightness, and wheezing. best response. However, the observation of additional indirect
A, Posteroanterior chest radiograph shows ill-defined left perihilar opacity signs of volume loss, such as the juxtaphrenic peak, diaphrag-
partially silhouetting left heart border. Note ipsilateral cephalad hilar displace-
ment and bronchovascular reorientation as well as leftward displacement of matic elevation, and the partial loss of definition of the left
tracheal air column. Left thorax appears smaller in volume than right. Left heart border, allow the appropriate differentiation. Addition-
diaphragm is elevated and shows juxtaphrenic peak. Left apex is hyperlucent, as
is left paramediastinal border. ally, a true pneumothorax can be confidently diagnosed by
(Fig. 1 continues on next page) the recognition of a white visceral pleural line as opposed to a

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delineating the collapsed upper lobe from the overinflated


lower lobe [5–8]. However, observing additional indirect
signs of volume loss (e.g., ipsilateral mediastinal shift, hilar
elevation, diaphragmatic elevation, juxtaphrenic peak, rib
approximation, and so forth) will enable the radiologist to
confidently make the best diagnosis, even in the absence of
a lateral chest radiograph.
The luftsichel sign is a classic and helpful imaging finding
on frontal chest radiography. Once lobar atelectasis has
been identified, the cause (e.g., mucus plug, aspirated for-
eign body, primary or secondary obstructing endobronchial
tumors, and so forth) must be determined either through
bronchoscopy or CT evaluation.
Endobronchial carcinoids are rare neuroendocrine tumors,
accounting for approximately 2% of all lung neoplasms and
12–15% of all carcinoid tumors [12, 13]. These tumors origi-
nate from bronchial mucosa neurosecretory cells and are
classified as low-grade malignant neoplasms because of their
potential for local invasion, local recurrence, and occasional
metastasis [12, 13]. Endobronchial carcinoids have the po-
tential to synthesize and secrete various peptide hormones
and neuroamines (e.g., adrenocorticotropic hormone, sero-
tonin, somatostatin, and bradykinin). These tumors are not
associated with smoking [12–14]. Histologically, carcinoid
tumors are categorized as either Kulchitsky cell carcinoma
B
(KCC) type I (i.e., typical carcinoid) or KCC type II (i.e.,
atypical carcinoid). KCC type I is the classic endobronchial
Fig. 1 (continued)—52-year-old woman with several-week history of
nonproductive cough, mild dyspnea, chest tightness, and wheezing.
carcinoid and is the least aggressive [12, 13]. These lesions are
B, Lateral chest radiograph shows retrosternal sigmoid-shaped band of usually well defined, are smaller than 2.5 cm in diameter, are
increased opacity representing anteriorly displaced oblique fissure secondary located centrally in the mainstem bronchi, and affect rela-
to complete collapse of left upper lobe.
tively young patients, with a marked female predilection
(10:1, females to males). Only 3% of typical carcinoid tu-
black edge due to a Mach effect [9, 10]. Such a white visceral mors metastasize to sites other than regional lymph nodes.
pleural reflection is not present in this patient. Furthermore, The prognosis is excellent, with a 5-year survival rate of 92%
a true visceral pleural reflection will also follow the contour of [12–14]. KCC type II is the atypical carcinoid tumor and is
the lung periphery and not simply fade imperceptibly with responsible for 25% of pulmonary carcinoid tumors [12, 13].
the lung parenchyma, as was also seen in our patient [9, 10]. These latter lesions tend to behave more aggressively, are
However, radiologists should be cautioned about the un- larger, occur in peripheral locations, and usually affect older
common relationship between lobar atelectasis and pneu- patients, with a male preponderance. Regional lymph node
mothorax. A localized pneumothorax may occur adjacent metastases are more common, occurring in up to 50% of pa-
to an atelectatic lobe and has been described as a sign of tients. Distant metastases to the liver, bone, and CNS occur
bronchial obstruction that is referred to as “pneumothorax in one third of patients [12–14]. The prognosis is less favor-
ex vacuo.” In such cases, treatment should be directed to able, with a 5-year survival rate of 69%. Surgical excision is
the underlying bronchus and not to the pleural space [11]. the preferred treatment, typically lobectomy or pneumonec-
The overinflated superior segment of the lower lobe may tomy. Tracheobronchial sleeve resection may be used for cen-
insinuate itself between the collapsed upper lobe and the tral carcinoid lesions with normal distal lung parenchyma.
transverse aorta, creating a sharp crisp crescent or paraaor- CT can prospectively evaluate the likelihood of tumor resec-
tic radiolucency referred to as the luftsichel sign. The diag- tion and is valuable in monitoring patients postoperatively
nosis of left upper lobe collapse on single anteroposterior or for potential recurrence [12–14].
posteroanterior chest radiographs can sometimes be diffi-
cult. When a lateral chest radiograph is available (Fig. 1B), Treatment
the diagnosis of left upper lobe collapse is more easily made The treatment is left upper lobectomy. The patient has
by noting a retro­sternal sigmoid-shaped band of increased since relocated and is now being followed up at another in-
opacity representing the anteriorly displaced oblique fissure stitution. The current disease status is otherwise unknown.

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Scenario 2 margin of this mass is well delineated; however, the supe-


rior margin is ill-defined or incomplete. On lateral chest
Clinical History radiography (Fig. 2B), the lesion appears better defined
A 68-year-old asymptomatic nonsmoking woman under- and is lentiform in morphology. The long axis of this mass
went preoperative screening chest radiography in prepara- parallels the long axis of the right oblique fissure. The an-
tion for a total knee arthroplasty. The radiographic findings terior, superior, and inferior borders appear better defined
prompted subsequent chest CT (Fig. 2). than the posterior border. Chest CT using the mediastinal
window setting (Fig. 2C) reveals a large, slightly lobulated
Description of Images homogeneous mass. Two subtle punctuate hypervascular
A frontal chest radiograph (Fig. 2A) reveals a vague foci are seen in the periphery of the lesion. Lung window
opacity in the right midthorax overlying the fourth and settings (Figs. 2D and 2E) confirm the lesion is localized to
fifth anterior ribs. The ribs appear intact. The inferior the right oblique fissure.

Question 2 Question 3
Where is this lesion MOST LIKELY located? What is the MOST LIKELY diagnosis?
A. Lung parenchyma. A. Primary lung cancer.
B. Mediastinum. B. Chest wall chondrosarcoma.
C. Pleura. C. Pseudotumor or vanishing tumor of the pleura.
D. Chest wall. D. Localized fibrous tumor of the pleura.

A B
Fig. 2—68-year-old asymptomatic nonsmoking woman who underwent preoperative screening chest radiography in preparation for total knee arthroplasty.
Radiographic findings prompted subsequent chest CT.
A, Frontal chest radiograph reveals vague opacity in right midthorax overlying fourth and fifth anterior ribs. Ribs appear intact. Inferior margin of this mass is well
delineated; however, superior margin is ill-defined or incomplete.
B, On lateral chest radiograph, lesion appears better defined and is lentiform in morphology. Long axis of this mass parallels long axis of right oblique fissure.
Anterior, superior, and inferior borders appear better defined than posterior border.
(Fig. 2 continues on next page)

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Diagnosis lesions. Option A is not the best response. Appropriate lo-


The diagnosis in this patient is localized fibrous tumor of calization is necessary before the proper differential diagno-
the pleura, as indicated by the incomplete border sign. sis can be determined.
Extrapulmonary masses often exhibit tapered or ovoid su-
Solution to Question 2 perior and inferior borders and are convex toward the lung
Extrapulmonary masses, when projected en face to the [15]. The overlying pleura of extrapulmonary lesions
x-ray beam, can simulate the presence of an intraparenchy- smoothes out surface irregularities which, combined with the
mal lesion [15]. The incomplete border sign illustrated in interface of the mass with lung air, give it a relatively sharply
this patient is useful in distinguishing between extrapulmo- defined appearance [15]. However, this otherwise sharp bor-
nary (options B, C, and D) and intrapulmonary (option A) der may be lost where the mass becomes continuous with the

C D

Fig. 2 (continued)—68-year-old asymptomatic nonsmoking woman who


underwent preoperative screening chest radiography in preparation for total
knee arthroplasty. Radiographic findings prompted subsequent chest CT.
C, Chest CT scan at mediastinal window setting reveals large, slightly
lobulated homogeneous mass. Two subtle punctuate hypervascular foci are
seen in periphery of lesion.
D and E, CT images at lung window settings confirm lesion is localized to right
oblique fissure.
E

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pleura of the chest wall, thus forming an incompletely visu- fluid collections is usually oriented along the long axis of
alized border on radiography (Figs. 2A and 2B) and creating the interlobar fissure [21]. Fluid has a tendency to accumu-
the so-called incomplete border sign [15–17]. late in the interlobar fissure in the setting of cardiac decom-
Mediastinum- and chest wall–based masses may also pensation and to localize in the horizontal fissure in partic-
show an incomplete border sign [15]. However, the location ular [21]. The fluid collections tend to be spontaneously
of this lesion on the frontal radiograph (Fig. 2A) would ar- absorbed when the heart failure has been relieved and are
gue against a mediastinal or chest wall cause. Options B therefore referred to as either pseudotumors or vanishing
and D are not the best responses. Localization of the lesion tumors of the pleura [21]. Option C is not the best response.
to the oblique fissure on the lateral chest radiograph (Fig. Invariably, concomitant radiographic evidence of cardiac
2B) supports a pleural cause, which is subsequently con- decompensation is seen or an ipsilateral pleural effusion is
firmed on CT (Figs. 2C–2E). Option C is the best response. present [21].
The most common extrapulmonary lesions include, but Localized or solitary fibrous tumor of the pleura is a rare
are not limited to, loculated pleural effusions, various rib pleural neoplasm but is the second most common primary
lesions (e.g., fractures, primary and secondary tumors), pleural neoplasm after malignant mesothelioma [22–24].
mesenchymal tumors, neural tumors, hematomas, lipomas, Option D is the best response. Although most of these tu-
and various cutaneous lesions (e.g., neurofibromas) [15]. mors are related to the pleura, they have also been described
in other intra- and extrathoracic locations. These tumors
Solution to Question 3 typically occur in adult men and women in the fifth through
The incomplete border sign supports a conclusion that eighth decades of life. Many patients are asymptomatic
the lesion in question is extrapulmonary. Option A is not and are diagnosed incidentally because of abnormal chest
the best response. radiographic findings, as in our patient (Figs. 2A and 2B).
Although chest wall metastases are the most common ma- Symptoms typically relate to tumor size and include cough,
lignant chest wall neoplasm in adults, chondrosarcoma is the chest pain, and dyspnea. Hypertrophic pulmonary osteoar-
most common primary malignant tumor of the adult chest thropathy is seen in 20–25% of patients. Symptomatic hy-
wall [18–20]. Chondrosarcomas are malignant neoplasms poglycemia occurs in less than 5% of patients [22–24]. Ra-
with cartilaginous differentiation. Option B is not the best diographically, localized fibrous tumors of the pleura
response. This neoplasm typically arises in the anterior chest present as well-defined, variably sized, lobular extrapulmo-
wall and involves the sternum or costochondral cartilages. nary nodules or masses (i.e., incomplete border sign) and
Less frequently, chondrosarcomas arise in the ribs (17%) and typically abut the pleura [23, 24] (Figs. 2A and 2B). CT re-
scapulae [18–20]. Chondrosarcomas occur across a wide age veals a noninvasive lobular soft-tissue mass of variable size
range but typically affect patients between the ages of 30 that abuts at least one pleural surface or may exhibit an
and 60 years. Most tumors manifest as palpable chest wall interlobar fissure location (Figs. 2C–2E). Smaller lesions are
masses that may grow rapidly and become painful [18–20]. more homogeneous in attenuation, whereas larger lesions
Males are affected slightly more frequently than females, in a may appear heterogeneous. Foci of calcification and en-
ratio of 1.3:1.0 [18–20]. On radiologic imaging, variable hancing vessels may be observed in the lesion [23, 24] (Fig.
intratumoral calcifications (e.g., rings, arches, flocculent, or 2C). These tumors are not related to mesothelioma, asbes-
stippled) can be identified, and osseous destruction is often tos exposure, or tobacco abuse. Benign and malignant vari-
present [18–20]. Surgical resection is the treatment of choice. ants have been described. Prognosis is related more to resec-
The 5-year survival rate is more than 60% and may approach tability than to histologic features [22–24].
80% in patients without metastases. Poor prognosis is associ-
ated with incomplete tumor resection, metastases, local re- Treatment
currence, and patient age older than 50 years [18–20]. The tumor was successfully resected in its entirety at
Interlobar pleural fluid collections are typically ovoid or open thoracotomy. The patient remained disease-free at the
lentiform when viewed in tangent and may simulate a mass time of the last follow-up CT examination 12 months before
on conventional radiography [21]. The long axis of such this writing.

Scenario 3 of community-acquired pneumonia and began taking


levofloxacin. Over the next 3 days, he developed progres-
Clinical History sive hypoxia and was subsequently transferred to the in-
A 33-year-old man presented with a 3- to 4-day history tensive care unit for mechanical ventilation and nitric ox-
of dyspnea and a nonproductive cough. A chest radiograph ide therapy. Follow-up chest radiography (not shown)
(not shown) revealed bilateral perihilar air-space opacities before intubation revealed progressive bilateral perihilar
with intervening normal aerated lung. He was admitted to air-space disease. Subsequent chest CT pulmonary angi-
the general medicine ward with a presumptive diagnosis ography on the same day did not show a pulmonary em-

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A B
Fig. 3—33-year-old man with 3- to 4-day history of dyspnea and nonproductive
cough. Chest radiograph (not shown) revealed bilateral perihilar air-space opaci-
ties with intervening normal aerated lung. Patient was admitted to general
medicine ward with presumptive diagnosis of community-acquired pneumonia
and began taking levofloxacin. Over next 3 days he developed progressive
hypoxia and was subsequently transferred to intensive care unit for mechanical
ventilation and nitric oxide therapy. Follow-up chest radiograph (not shown)
before intubation revealed progressive bilateral perihilar air-space disease.
A–C, Selected chest CT pulmonary angiography images using lung window
setting through upper (A), mid (B), and lower (C) lung zones reveal patchy
pattern of variable attenuation characterized by combination of ground-glass
opacities, consolidations, reduced lung attenuation resulting from mosaic
perfusion, and intervening normal lung.

bolus but did reveal an interesting pattern of air-space attenuation characterized by a combination of ground-
disease (Fig. 3). glass opacities, consolidations, reduced lung attenuation
resulting from mosaic perfusion, and intervening normal
Description of Images lung. Note the “head cheese sign.”
The selected CT images (Fig. 3) through the upper, mid,
and lower lung zones reveal a patchy pattern of variable Diagnosis
The diagnosis is Mycoplasma pneumonia with associated
bronchiolitis, as indicated by the head cheese sign.
Question 4 Solution to Question 4
Which diagnosis would be LEAST LIKELY? The combination of mixed densities in the lung parenchy-
ma created by ground-glass opacities, air-space consolida-
A. Sarcoidosis. tions, reduced lung attenuation from mosaic perfusion, and
B. Atypical infection with associated bronchiolitis. intervening normal lung give the lung a geographic appear-
C. Hypersensitivity pneumonitis. ance on CT [25, 26] (Fig. 3). This pattern of mixed parenchy-
D. Multiple septic pulmonary emboli. mal lung densities has been likened to the morphologic ap-
pearance of the mixture of boiled pork scraps and pigs’ feet

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in a gelatinous background known and marketed as head ple septic pulmonary emboli, would be the least likely diag-
cheese or hog’s head cheese and is therefore known as the nosis; therefore, option D is the best response.
“head cheese sign” or “hog’s head cheese sign” [25, 26]. How Mycoplasmas are bacteria that lack a cell wall, adhere to
does this particular imaging sign help radiologists in their di- ciliated respiratory epithelium, and produce hydrogen per-
agnostic interpretation? CT must clearly show areas of oxide, which damages epithelial cells and interferes with
ground-glass opacity and consolidation with concomitant ciliary function. M. pneumoniae are one of three human
mosaic perfusion (rather than one or the other) (Fig. 3). When pathogenic Mycoplasma species [29]. Bacteria are transmit-
these findings are present, they indicate a mixed infiltrative ted from person to person thorough aerosolized droplets.
disease characterized by ground-glass or consolidation and Infection may occur the year round but usually occurs dur-
an obstructive disease (usually associated with bronchiolitis) ing fall and winter [29, 30]. Patients may present with fever,
characterized by mosaic perfusion, with a decrease in vessel chills, malaise, anorexia, sore throat, dry cough, and head-
caliber and side branches in the hypoattenuating regions of ache. As the disease progresses, nearly all patients develop
lung parenchyma. The latter will often reveal air trapping on an intractable hacking cough, but only 3% of such patients
expiratory images [25–27]. The most common clinical causes develop pneumonia. Extrapulmonary features are common
of this CT pattern of disease include hypersensitivity pneu- and may include cervical lymphadenopathy, skin rash,
monitis, sarcoidosis, atypical infections (e.g., those caused by aseptic meningitis, nausea, vomiting, and diarrhea. Rarely,
Mycoplasma pneumoniae) with associated bronchiolitis, and patients present with or develop acute respiratory distress
acute interstitial pneumonia [25–27]. Options A, B, and C, syndrome [29–31]. Such patients have higher morbidity
which are likely diagnoses, are not the best responses. Addi- and mortality rates. In these latter cases, supportive me-
tional clinical and laboratory data would be necessary to fur- chanical ventilation is necessary in addition to corticoster-
ther narrow the differential diagnosis. In this particular case, oids and antibiotic therapy (e.g., erythromycin, azithromy-
the diagnosis was an atypical infection with associated bron- cin, tetracycline, clarithromycin, and so forth) [29–31].
chiolitis secondary to Mycoplasma pneumonia. After infection, patients may fully recover; or interstitial
The CT pattern of multiple septic pulmonary emboli is fibrosis, bronchiectasis, Swyer-James syndrome, and im-
much different. The latter disease process may be charac- paired pulmonary function may develop as sequelae of the
terized by unilateral or bilateral areas of juxtapleural and infection [29, 30].
wedge-shaped consolidation; diffuse, often angiocentric,
nodules ranging from 0.5 to 3.5 cm in diameter, many of Treatment
which show various stages of cavitation; and a peripheral Treatment is support with a mechanical ventilator and
rimlike pattern of enhancement after the administration of nitric oxide therapy, corticosteroids, and clarithromycin.
IV contrast media. Pleural effusions may also be identified The patient was maintained on mechanical ventilation
in two thirds of patients, and 27% have identifiable hilar or for 5 days and then was successfully extubated. Eight
mediastinal lymphadenopathy [28]. On the basis of the days later, he was discharged and was subsequently lost
clinical presentation and the CT findings, Option D, multi- to follow-up.

Scenario 4
Clinical History
A 35-year-old woman presented with fatigue, chest pain,
and weight loss over the past several months (Fig. 4).
Description of Images
A posteroanterior (Fig. 4A) chest radiograph shows a mas-
sively enlarged cardiomediastinal silhouette. Lateral chest
radiography (not shown) showed complete obliteration of
the retrosternal clear space. On closer inspection, the normal
right and left pulmonary arteries and their respective interlo-

Fig. 4—35-year-old woman with fatigue, chest pain, and weight loss over past
several months.
A, Posteroanterior chest radiograph shows massively enlarged cardiomediasti-
nal silhouette. Lateral chest radiograph (not shown) showed complete obliteration
of retrosternal clear space. On closer inspection, normal right and left pulmonary
arteries and their respective interlobar divisions can be identified well in what
appears to be lateral or peripheral margin of cardiomediastinal silhouette.
A (Fig. 4 continues on next page)

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bar divisions can be identified well in what appears to be the the myocardium (Fig. 4C) show a large aggressive anterior
lateral or peripheral margin of the cardiomediastinal silhou- mediastinal mass intimately related to the ascending aorta
ette. Contrast-enhanced CT images (mediastinal window set- and main pulmonary artery with invasion of the myocardi-
ting) through the main pulmonary artery level (Fig. 4B) and um proper. A small pericardial effusion is present.

Diagnosis
Question 5 The diagnosis is malignant peripheral nerve sheath tu-
mor of the vagus nerve with mediastinal invasion, the hi-
What is the MOST LIKELY diagnosis? lum overlay sign.
A. Cardiomyopathy.
B. Pericardial effusion. Solution to Question 5
C. Anterior mediastinal mass. The proximal segment of the visible left or right pulmo-
D. Pleural effusion. nary artery lies laterally to the cardiac silhouette or just
within its edge on normal frontal chest radiography. As
Question 6 the myocardium enlarges in the setting of a cardio-
All of the following are TRUE statements myopathy or cardiomegaly or as the pericardial sac dis-
regarding the “hilum convergence sign” EXCEPT: tends with fluid from pericardial effusion, the pulmonary
artery segments are simply displaced outward but con-
A. It differentiates a potential hilar mass from an tinue this same relationship to the cardiac silhouette [32,
enlarged pulmonary artery. 33]. Options A and B are not the best responses. Alterna-
B. If the pulmonary arteries converge into the lateral tively, if either the left or right pulmonary artery can be
border of the apparent hilar mass, the mass seen 1.0 cm or more within the lateral edge of an opacity
represents an enlarged pulmonary artery. that appears to represent the cardiac silhouette, that opac-
C. If the pulmonary arteries converge behind the ity does not represent the cardiac silhouette and therefore
apparent hilar mass, the mass represents an cannot be the result of cardiomyopathy, cardiomegaly, or
enlarged pulmonary artery. pericardial effusion, but is related instead to the presence
D. If the convergence of the pulmonary arteries of an anterior mediastinal mass [32, 33]. Option C is the
arises from the cardiac silhouette, a mediastinal best response. This is referred to as the hilum overlay sign,
which can be used to determine that a lesion is extracar-
mass is likely present.
diac and localized to the anterior mediastinal compart-

B C
Fig. 4 (continued)—35-year-old woman with fatigue, chest pain, and weight loss over past several months.
B and C, Contrast-enhanced chest CT scans using mediastinal window setting through main pulmonary artery level (B) and myocardium proper (C) show large
aggressive anterior mediastinal mass intimately related to ascending aorta and main pulmonary artery as well as invasion of myocardium proper. Small pericardial
effusion is present. DA = descending thoracic aorta, LLPA = left lower lobe pulmonary artery, PA = pulmonary artery, PV = pulmonary vein, RLPA = right lower lobe
pulmonary artery, RA = right atrium, RV = right ventricle.

S42 AJR:192, March 2009


Thoracic Imaging
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ment, thus steering the differential diagnosis in the appro- Solution to Question 6
priate direction [32, 33]. Because the lesion of concern is Felson’s hilum convergence sign should be differentiated
mediastinum-based, option D, pleural effusion, is not the from the hilum overlay sign discussed in the solution to ques-
best response. It likewise would be quite unusual for pleu- tion 5 [32]. The hilum convergence sign is useful in distinguish-
ral effusion to present as perihilar ground-glass opacity. ing between a large pulmonary artery and a hilar mass [32].
Additionally, the costophrenic angles are preserved. The Because the pulmonary artery branches arise from the main
mediastinal mass in this particular patient proved to be a pulmonary artery trunk, an enlarged pulmonary artery will
malignant peripheral nerve sheath tumor (MPNST) aris- have branches that arise from its lateral margin, and its vessels
ing from the intrathoracic vagus nerve in the mediasti- will appear to converge toward the main pulmonary artery
num. MPNSTs are rare but aggressive sarcomas that arise [32]. Option B is the best response. A true hilar mass may have
from the nerve sheath or show features of nerve sheath the appearance of an enlarged pulmonary artery, but the ves-
differentiation; they more often involve the extremities, sels will not arise from its lateral margin but rather seem to
the head, and the neck. Intrathoracic MPNST is uncom- pass through the margin as they converge on the true pulmo-
mon [34]. Although such tumors may occur in patients nary artery [32]. Therefore, if the convergence of the pulmo-
with neurofibromatosis 1 (von Recklinghausen’s disease) nary arteries appears behind the apparent hilar mass or ap-
with an incidence of 0.16%, these tumors may also occur in pears to arise from the heart, a mediastinal mass is more likely
patients with no signs of such (incidence of 0.001%), as in [32]. Options A, C, and D are not the best responses.
our patient [34–36]. Successful treatment requires complete
surgical excision of the MPNST. Radiotherapy may delay Treatment
recurrence but has little impact on patient survival. Con- The treatment, which was unsuccessful, was surgical de-
ventional advanced soft-tissue sarcoma single-agent chemo- bulking of the tumor and palliative radiotherapy. The pa-
therapy with doxorubicin has a poor response rate [34]. tient died over the next several weeks.

Scenario 5 pleural effusion or interstitial edema is present. A con-


trast-enhanced coronal maximum-intensity-projection
Clinical History chest CT scan (Fig. 5C) shows separation of the visceral
A 47-year-old man presented with chronic renal failure and parietal pericardial layers by a large fluid collection
and dyspnea (Fig. 5). surrounding the myocardium.
Description of Images Diagnosis
A posteroanterior chest radiograph (Fig. 5A) shows The diagnosis is uremic pericardial effusion, the water
globular enlargement of the cardiomediastinal silhou- bottle sign.
ette. There is an increase in transverse diameter of the
cardiomediastinal silhouette but no increase in its height. Solution to Question 7
The proximal segments of the visible left and right pul- A frontal chest radiograph (Fig. 5A) shows globular en-
monary artery lie laterally to the cardiac silhouette. A largement of the cardiomediastinal silhouette with an in-
coned-down lateral chest radiograph (Fig. 5B) reveals crease in its transverse diameter but no increase in its height.
separation of the black retrosternal fat stripe from the As a result, the superior mediastinal borders appear straight-
black epicardial fat stripe by an opaque interface. No ened, giving the cardiomediastinal silhouette a morphology
that has been likened to that of a water bottle, hence the
designation “water bottle sign” [37, 38]. Applying the hi-
lum overlay sign discussed in scenario 4, the proximal seg-
Question 7 ment of the visible left and right pulmonary artery contin-
What is the MOST LIKELY diagnosis? ues to lie laterally to the enlarged cardiac silhouette (i.e.,
negative hilum overlay), thus eliminating anterior mediasti-
A. Lobar pneumonia. nal mass from diagnostic consideration. Option D is not the
B. Primary lung cancer. best response.
C. Acute heart failure. The pericardium consists of two layers. The visceral peri-
D. Anterior mediastinal mass. cardium is attached to the surface of the myocardium and
E. Pericardial effusion. the proximal great vessels. The parietal pericardium forms
the free wall of the pericardial sac [37–40]. The pericardial

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A B
Fig. 5—47-year-old man with chronic renal failure and dyspnea.
A, Posteroanterior chest radiograph shows globular enlargement of
cardiomediastinal silhouette. Note increase in transverse diameter of
cardiomediastinal silhouette but no increase in its height. Proximal segment of
visible left and right pulmonary artery lies lateral to cardiac silhouette.
B, Coned-down lateral chest radiograph reveals separation of black retrosternal
fat stripe (single arrow) from black epicardial fat stripe (double arrows) by
opaque interface. No pleural effusion or interstitial edema is present.
C, Contrast-enhanced coronal maximum-intensity-projection chest CT scan at
mediastinal window setting shows separation of visceral and parietal
pericardial layers by large fluid collection surrounding myocardium.

sac itself normally contains 20–50 mL of fluid [37]. An ex- 250 mL [37–39]. The cardiomediastinal silhouette may then
cess of pericardial fluid may accumulate in a number of set- show symmetric enlargement and preservation of the nor-
tings. Option E is the best response. The most common cause mal hilar relationships, resulting in the water bottle–shaped
is myocardial infarction with left ventricular failure [37]. morphology [37–39] (Fig. 5A). A well-penetrating lateral
Fifty percent of patients with chronic renal failure develop chest radiograph is even more sensitive in the early detec-
uremic pericardial effusions [37]. Other causes of peri- tion of pericardial effusion and may reveal separation of
cardial effusion may include hypoalbuminemia, myxedema, the retrosternal and epicardial fat stripe by more than 2
infection, drug reactions, trauma, neoplasia, and autoim- mm, which is sometimes referred to as the “Oreo [Nabisco]
mune disease [37]. The chest radiograph may appear rela- cookie sign,” “sandwich sign,” or “bun sign” [37–39] (Fig.
tively normal until the volume of pericardial fluid exceeds 5B). The black epicardial and retrosternal fat stripes consti-

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tute the outer dark cookie layers or slices of bread in the propriate considerations in this patient. Options A and B
sandwich or bun, and the opaque intervening pericardial are not the best responses.
fluid, the white fluff of the cookie or the meat in the sand- Uremic pericardial effusion commonly improves with inten-
wich or bun. Although the cardiac silhouette is enlarged, sified or increased frequency of peritoneal or hemodialysis
the pulmonary vasculature appears normal, and signs of [41]. More aggressive management may be needed if the peri-
heart failure (e.g., vascular redistribution, Kerley B lines, cardial effusion is larger than 250 mL, if it continues to in-
and so forth) are absent (Fig. 5A). Option C is not the best crease despite intensive dialysis for 10–14 days, or if the pa-
response. CT is more sensitive in the detection of small peri- tient develops tamponade [41, 42]. In these latter instances,
cardial effusions. Small effusions first collect dorsally to the pericardiocentesis, pericardial window, subxiphoid pericar-
left ventricle and along the left atrium. Larger effusions col- diotomy, or pericardiectomy may be necessary [41, 42].
lect ventrally and laterally to the right ventricle. Very large
effusions may envelop the myocardium, forming the “halo Treatment
sign” [37, 40] (Fig. 5C). Lobar pneumonia and primary lung The patient was successfully treated with a combination
cancer are both air-space disease processes and are not ap- of increased hemodialysis and subxiphoid pericardiotomy.

Scenario 6 put. A convex bulge is present along the left heart border re-
lated to left atrial chamber enlargement. Midline median ster-
Clinical History notomy wires can be delineated. A lateral chest radiograph
A 62-year-old woman presented with a long-standing his- (Fig. 6B) reveals a convex bulge in the superoposterior cardiac
tory of chronic atrial fibrillation; she had undergone cardiac border below the carina, with posterior displacement of the
surgery 7 days earlier (Fig. 6). left lower lobe bronchus and opacification of the retrocardiac
Description of Images clear space. Right ventricular enlargement encroaches on the
A posteroanterior chest radiograph (Fig. 6A) shows an in- retrosternal clear space. Foci of residual pneumomediastinum
creased cardiothoracic ratio. The pulmonary artery segment and a retained subxiphoid pacer lead can be seen in the retro­
is enlarged, suggesting precapillary pulmonary hypertension. sternum. A newly placed mitral valve prosthesis is present.
The aorta appears small from decreased forward cardiac out- Thin curvilinear calcifications can also be seen paralleling the

A B
Fig. 6—62-year-old woman with long-standing history of chronic atrial fibrillation. Patient had undergone cardiac surgery 7 days earlier.
A, Posteroanterior chest radiograph shows increased cardiothoracic ratio. Pulmonary artery segment is enlarged, suggesting precapillary pulmonary hyperten-
sion. Aorta appears small from decreased forward cardiac output. Convex bulge is present along left heart border related to left atrial chamber enlargement.
Midline median sternotomy wires can be delineated.
B, Lateral chest radiograph shows relative posterior displacement of left upper and lower lobe bronchus relative to right (arrows), forming “walking man sign.”
Right ventricular enlargement encroaches on retrosternal clear space. Foci of residual pneumomediastinum and retained subxiphoid pacer lead can be seen in
retrosternum. Newly placed mitral valve prosthesis is present. Thin curvilinear calcifications can also be seen paralleling posterior wall of enlarged left atrium. No
radiographic evidence of acute cardiac decompensation is seen.

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sociated with cardiac dysrhythmias, enlargement of the left


Question 8 atrium (Fig. 6) is uncommon except in cases of prolapse com-
plicated by severe mitral regurgitation [45, 46]. Option D is
What is the MOST LIKELY diagnosis? not the best response.
A. Acute heart failure. Mitral stenosis is characterized by narrowing of the inlet
B. Ebstein anomaly. valve orifice of the left ventricular chamber, which interferes
with normal opening during diastole [37, 47–49]. Option C is
C. Mitral stenosis.
the best response. Affected patients typically have thickened
D. Mitral valve prolapse.
valve leaflets, fused commissures, or thickened and shortened
Question 9 chordae tendineae. The normal mitral valve orifice area is 4–6
cm2 [47–49]. In early diastole, a small pressure gradient exists
Which imaging sign is demonstrated on the lateral between the atrium and the ventricle, but during most of di-
chest radiograph (Fig. 6B)? astole the pressures in these two chambers are relatively simi-
lar. When the mitral valve area narrows to less than 2.5 cm2,
A. Coeur en sabot sign.
blood flow is impeded, which causes an increase in left atrial
B. Walking man sign.
pressure. Critical mitral stenosis occurs when the area is re-
C. Double density sign. duced to 1 cm2 [47–49]. When this occurs, a left atrial pressure
D. Doughnut sign. of at least 25 mm Hg is necessary to maintain normal cardiac
output [47–49]. The increase in left atrial pressure enlarges
Question 10
the left atrium and increases pulmonary venous and capillary
What is the principal cause of acquired mitral pressures, resulting in pulmonary venous congestion and re-
stenosis? duced cardiac output. This scenario can mimic left ventricular
failure, but left ventricular contractility is usually normal.
A. Rheumatic heart disease. Chronic atrial fibrillation commonly ensues as the left atrium
B. Left atrial myxoma. enlarges [50]. Chronic elevation of left atrial pressures leads to
C. Left atrial thrombus. pulmonary artery hypertension, tricuspid and pulmonary
D. Maternal ingestion of lithium in the first trimester valve incompetence, right ventricular hypertrophy (Fig. 6)
of pregnancy. and, eventually, right heart failure [37, 47–49]. In cases of
long-standing mitral stenosis, the left atrial wall may rarely
posterior wall of the enlarged left atrium. No radiographic calcify (Fig. 6B). Such calcification is more common in pa-
evidence of acute cardiac decompensation is seen. tients with endocarditis resulting from rheumatic heart dis-
ease; most affected patients also have heart failure and chron-
Diagnosis ic atrial fibrillation [51]. Calcification of the mitral valve itself
The diagnosis is mitral stenosis and left atrial calcifica- occurs in approximately 10% of affected patients. This should
tion, as indicated by the walking man sign. not be confused with mitral annulus calcification. Calcifica-
tion of the mitral valve annulus does not indicate underlying
Solution to Question 8 mitral stenosis and is often a finding of senescence [37, 49].
Although the cardiothoracic ratio is enlarged, the vascular
pedicle is not widened and there is no radiographic evidence Solution to Question 9
of vascular redistribution or septal lines to support a diagno- The term “coeur en sabot” refers to a heart that has a boot-
sis of acute heart failure (Fig. 6). Option A is not the best shaped morphology as a result of uplifting of the cardiac
response. Ebstein anomaly is a congenital heart malforma- apex because of right ventricular hypertrophy and the ab-
tion manifested by apical displacement of the septal and pos- sence of a normal main pulmonary artery segment [44]. This
terior tricuspid valve leaflets, leading to atrialization of the is a feature on frontal chest radiographs most often associated
right ventricle and a variable degree of malformation and with tetralogy of Fallot. Additional radiologic features of this
displacement of the anterior leaflet [43]. Although chest ra- congenital cardiac malformation include decreased pulmo-
diography may be normal in patients with Ebstein anomaly, nary vascularity; a normal-sized heart because of the lack of
characteristic radiologic features include right atrial enlarge- pulmonary blood flow and heart failure; right atrial enlarge-
ment, which may occasionally be quite severe; inferior vena ment; and, in approximately 20–25% of affected patients, a
cava and azygos dilatation secondary to tricuspid regurgita- right-sided aortic arch [44]. Option A is not the best response.
tion; hypoplasia of the aorta and main pulmonary artery; The “double density sign” is an early radiologic feature of left
and a normal-sized left atrium [44]. These radiographic fea- atrial enlargement seen on frontal, not lateral, chest radio-
tures are not present in this patient (Fig. 6). Option B is not graphs [52]. Option C is not the best response. This imaging
the best response. Although mitral valve prolapse may be as- sign manifests as an interface projecting over the right retro-

S46 AJR:192, March 2009


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cardiac region (Fig. 6A). The interface represents the inferior Balloon valvotomy is usually the initial procedure of choice
margin of the enlarged left atrium as it pushes into the adja- for symptomatic patients with moderate to severe mitral
cent lung [52]. On frontal radiographs of adult patients, a left stenosis. Valvotomy can double the mean valve area, with a
atrial dimension—defined as the distance from the midpoint 50–60% decrease in the transmitral gradient, which provides
of the double density to the inner margin of the left main- symptomatic improvement. Surgical commissurotomy has a
stem bronchus—greater than 7 cm suggests left atrial en- similar efficacy to that of balloon valvotomy but is usually
largement is present [52]. However, this sign and these dimen- reserved for patients with left atrial thrombus despite antico-
sions are not reliable in pediatric patients [52]. The double agulation or a nonpliable or calcified valve. Mitral valve re-
density sign is often associated with splaying of the normal placement is reserved for patients who are not candidates for
carinal angle of 60–90° and divergence of the caudal main- either percutaneous balloon mitral valvotomy or surgical
stem bronchi, creating a somewhat “wishbone” morphology commissurotomy [58, 59]. Lifelong endocarditis antibiotic
in severe cases [52]. These latter two signs are best appreciat- prophylaxis is recommended for procedures that may be as-
ed on well-exposed or well-penetrating frontal examinations. sociated with transient bacteremia (e.g., dental procedures,
The “doughnut sign” is an imaging sign seen on lateral chest bronchoscopy, colonoscopy, cystoscopy, and so forth) [58–60].
radiography; however, it suggests the presence of mediastinal
Treatment
lymphadenopathy, not cardiac valvular disease [53, 54]. Op-
The treatment is mitral valve replacement. The patient
tion D is not the best response. On normal chest radiography,
was discharged on postoperative day 7. We have no addi-
the aortic arch and the right and left pulmonary arteries cre-
tional information on her current clinical status.
ate an inverted horseshoe appearance [53, 54]. Subcarinal
lymphadenopathy obliterates the notch in the horseshoe, Conclusion
forming a rounded circle likened to the morphology of a bagel This case-based self-assessment module describes several
or doughnut [53, 54]. important radiologic signs that are useful in diagnosing vari-
The normal trachea, right and left upper lobe bronchi, and ous diseases affecting the chest, including the luftsichel sign of
lower lobe bronchi are vertically aligned on a normal lateral left upper lobe collapse; the incomplete border sign of pleural
chest radiograph. The walking man sign is a lateral chest radi- and chest wall-based lesions; the head cheese sign or hog’s head
ography sign that is seen with posterior displacement of the cheese sign of atypical infection with associated bronchiolitis,
left upper or lower lobe bronchus relative to the right bronchi, sarcoidosis, acute interstitial pneumonitis, and hypersensitivi-
so that the bronchial relationship resembles the legs of a man ty pneumonitis; the hilum overlay sign, which is useful in local-
in midstride [55]. Option B is the best response. Posterior dis- izing lesions to the anterior mediastinum; the hilum conver-
placement of the left bronchi is typically the result of mass gence sign that distinguishes an enlarged pulmonary artery
effect on the bronchi by a markedly enlarged left atrium but is from a true hilar mass; the water bottle sign and the Oreo
not pathognomic of an enlarged atrium [55]. The walking man cookie sign of pericardial effusion; and the walking man sign
sign may also occur in the setting of subcarinal lymphadenop- of left atrial enlargement. Future articles will continue this
athy, mediastinal masses, left lower lobe volume loss, large hi- discussion of additional useful signs in thoracic imaging that
atal hernias, and thoracolumbar scoliosis. can be applied to assist the radiologist in establishing the cor-
rect diagnosis or differential diagnosis in applicable cases.
Solution to Question 10
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