R e s i d e n t s ’ S e c t i o n • S t r u c t u r e d R ev i ew A r t i c l e
Chung et al.
CT of the Trachea and Central Airways
Residents’ Section
Structured Review Article
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Residents
inRadiology CT of Diffuse Tracheal Diseases
Jonathan H. Chung1
R
adiologists can be instrumental
Jeffrey P. Kanne2 in the diagnosis of tracheal ab-
Matthew D. Gilman1 normalities. Patients are often
initially misdiagnosed with asth-
Chung JH, Kanne JP, Gilman MD ma or other obstructive lung disease because
there is considerable overlap among the clin-
ical presentation of these ailments and dif-
fuse tracheal diseases. Although the trachea
can be imaged with chest radiography or flu-
oroscopy, CT is the imaging test of choice to
evaluate the trachea and central airways.
MDCT provides high-resolution images that
can be used to generate multiplanar reforma-
tions, minimum intensity projections, 3D
volume-rendered images, and virtual bron-
choscopic images. In this article, we will il-
lustrate CT findings of diffuse nonmalignant
tracheal diseases, emphasizing key differ-
ences that aid in diagnosis (Table 1).
Key Points
1. CT is currently the primary noninvasive ex-
Keywords: Mounier-Kuhn syndrome, saber-sheath
amination to evaluate the trachea because
trachea, trachea, tracheal stenosis, tracheomalacia,
Wegener granulomatosis it offers multiplanar capabilities, evaluates
the morphology of the tracheal wall and lu-
DOI:10.2214/AJR.09.4146 men, and can be acquired in seconds.
2. The trachea is supported by C-shaped
Received December 23, 2009; accepted after revision
February 14, 2010.
cartilaginous rings anteriorly; the poste-
rior trachea is primarily supported by the
1
Department of Radiology, Massachusetts General thin trachealis muscle.
Hospital, 55 Fruit St., FND-202, Boston, MA 02114. 3. The lunate-shaped trachea on inspiration
Address correspondence to M. D. Gilman is highly suggestive of tracheomalacia.
(mgilman@partners.org).
4. Traditionally, collapse of greater than
CME 50% of the trachea during expiration was
This article is available for CME credit. defined as tracheomalacia; however, re-
See www.arrs.org for more information. cent evidence suggests that greater than
50% dynamic expiratory collapse can be
WEB
This is a Web exclusive article.
seen in healthy patients.
5. In saber-sheath trachea, only the coronal
AJR 2011; 196:W240–W246 diameter of the intrathoracic trachea is
narrowed; primarily men with chronic ob-
0361–803X/11/1963–W240
structive pulmonary disease are affected.
© American Roentgen Ray Society 6. The posterior wall of the trachea and the
central bronchi are classically spared in
W240 AJR:196, March 2011
both relapsing polychondritis and tra-
cheobronchopathia osteochondroplasti-
ca. However, the presence of focal coarse
calcification and ossification is highly
suggestive of tracheobronchopathia os-
teochondroplastica rather than relapsing
polychondritis.
7. Nodular calcification of the trachea is
common in tracheobronchopathia osteo-
chondroplastica and amyloidosis. How-
ever, amyloidosis tends to involve the
airway concentrically, as opposed to tra-
cheobronchopathia osteochondroplastica
which spares the posterior wall.
8. Wegener granulomatosis most often af-
fects the subglottic trachea but can be dif-
fuse or multifocal.
9. Mounier-Kuhn syndrome is unique among
the diffuse tracheal diseases in that it re-
sults in diffuse airway dilatation. Diver-
ticula project between the cartilaginous
rings giving the trachea and proximal
bronchi a corrugated appearance.
Normal Anatomy
The trachea is composed of approximately
16–22 C-shaped anterior cartilaginous rings
that support the trachea during expiration.
An inner mucosal layer, a submucosal layer,
cartilage and muscle, and an adventitial lay-
er comprise the tracheal wall. The posterior
wall is formed primarily by the thin trache-
alis muscle, which lacks cartilaginous sup-
port (Fig. 1).
The coronal diameter of the normal trachea
ranges from 13 to 25 mm in men and from 10
to 21 mm in women, and the sagittal diame-
ter of the normal trachea ranges from 13 to 27
mm in men and from 10 to 23 mm in wom-
en [1]. At end expiration, the trachea normal-
ly decreases in cross-sectional area, primarily
from anterior movement of the posterior wall;
there is wide variation in the degree of trache-
al expiratory collapse [2, 3]. The arterial supply
 CT of the Trachea and Central Airways

TABLE 1:  Imaging Findings of Various Diffuse Tracheal Diseases

Tracheal Wall Wall
Tracheal Disease CT Appearance Narrowing Thickened Calcification Useful Differentiators
Tracheomalacia Severe expiratory collapse of the Yes No No Tracheal collapse; history of predisposing condition
tracheal lumen; lunate-shaped or trauma
trachea
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Saber-sheath trachea Decreased coronal diameter with Yes No No Characteristic bullet-shaped trachea on axial CT;
concomitant increase in sagittal history or imaging findings suggesting chronic
diameter obstructive pulmonary disease
Relapsing polychondritis Thickening of the cartilaginous Yes Yes Sometimes Sparing of the posterior trachea; cartilaginous
trachea with sparing of the abnormalities of the ears or nose
posterior membrane
Tracheobronchopathia Calcified or ossified nodules in the Yes Yes Always Nodular calcified or nodular ossified tracheal wall
osteochondroplastica cartilaginous trachea with sparing thickening sparing the posterior tracheal
of the posterior membrane membrane and superior trachea
Amyloidosis Calcified or ossified nodular or Yes Yes Often present Concentric calcified or ossified nodular or
concentric tracheal wall thickening concentric wall thickening without posterior
sparing; may involve the larynx and upper trachea
Wegener granulomatosis Circumferential tracheal wall Yes Yes No Subglottic narrowing when focal; history of sinus or
thickening or cartilage defects; renal disease, pulmonary cavitary nodules, or
subglottic region most often pulmonary hemorrhage
involved
Mounier-Kuhn syndrome Tracheal diameter > 3 cm; main No No No Only diffuse tracheal abnormality resulting in both
bronchial diameter > 2.4 cm; anteroposterior and lateral tracheal dilatation
scalloping of tracheal wall and
diverticula
Sarcoidosis Secondary tracheal narrowing from Yes Yes Yes Hilar and mediastinal lymphadenopathy; perilym-
lymphadenopathy or primary phatic pulmonary nodules
tracheal narrowing from tracheal
noncaseating granulomas
Inflammatory bowel disease Diffuse or focal tracheal narrowing Yes Yes No History of inflammatory bowel disease or chronic
abdominal pain

of the trachea is derived from a number of
sources including the inferior thyroid artery, the
bronchial arteries, and the intercostal arteries.
Nonpathologic senescent tracheal calcifica-
tion is more common in women and tends to
be diffuse; calcification occurs along the nor-
mal contour of the trachea. Nodular focal cal-
cification suggests underlying disease.
Tracheomalacia
Tracheomalacia arises from weakness of the
tracheobronchial walls and hypotonia in the
myoelastic elements. In patients with trache-
omalacia, collapse of the intrathoracic trachea
secondary to increased intrathoracic pressure
during expiration is characteristic and is ac-
centuated during forced expiration [1]. Causes
of tracheomalacia include long-term intuba-
tion or other trauma, congenital abnormalities,
chronic extrinsic compression (vascular ring or
sling), chronic obstructive pulmonary disease,
chronic inflammation, and infection [1].
Traditionally, collapse of greater than
50% of the trachea on CT during expiration
was defined as tracheomalacia [1] (Fig. 2).
Characteristically, the posterior membra-
nous trachea bows anteriorly, producing an
upside down U-shaped air column on trans-
verse CT termed the “frown” sign [4] (Fig.
2). However, recent evidence suggests that
tracheal collapse of greater than 50% during
dynamic expiration can be seen in up to 78%
of healthy patients [3].
In a minority of patients, the trachea may
have a lunate configuration during inspira-
tion—that is, the trachea is characterized by
a coronal diameter that is greater than the
sagittal diameter. This finding is highly sug-
gestive of tracheomalacia and can be corrob-
orated with dynamic expiratory CT [4] (Fig.
3). In cases not resulting from another dif-
fuse tracheal disease such as relapsing poly-
chondritis or Wegener granulomatosis, the
tracheal wall usually has a normal thickness,
thereby helping differentiate tracheomalacia
from other diffuse tracheal diseases [1].
Saber-Sheath Trachea
Saber-sheath trachea is a common trache-
al abnormality occurring almost exclusively
in men with chronic obstructive pulmonary
disease [1]. The extrathoracic trachea has
a normal shape. However, the intrathoracic
trachea shows a marked decrease in coronal
diameter and an associated increase in sag-
ittal diameter without tracheal wall thicken-
ing (Fig. 4). This deformity of the trachea is
believed to result from repeated injury to the
intrathoracic trachea from chronic cough-
ing. Initially, changes are noted at only the
thoracic inlet but can involve the entire in-
trathoracic trachea over time. Other smok-
ing-related conditions may be present such
as emphysema, respiratory bronchiolitis, and
lung cancer.
Relapsing Polychondritis
Relapsing polychondritis (RPC) is an au-
toimmune disease that affects cartilaginous
structures such as the nose, ear, and laryngot-
racheobronchial tree. Airway involvement oc-
curs in up to half of patients, with pneumonia
being the most common cause of death [5].
On CT, RPC is characterized by increased at-
tenuation and thickening of the anterior and

AJR:196, March 2011 W241

 Chung et al.

Fig. 1—Normal trachea.

A, Diagram of normal
trachea in axial plane
shows discontinuity
of posterior aspect
of cartilaginous ring.
Posterior wall of
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trachea is supported by
trachealis muscle.
B, Axial CT image of
55-year-old woman
with normal trachea
shows typical inverted
U-shaped appearance of
trachea.

A B

Fig. 2—29-year-old man

with neck pain after self-
inflicted head trauma.
A, Unenhanced CT image
shows normal tracheal
caliber in extrathoracic
upper chest.
B, CT image shows
severe collapse of
intrathoracic trachea on
expiration; this finding
is highly suggestive of
tracheomalacia.
C, Difference in caliber
of intrathoracic (thick
white arrow) and
extrathoracic (thin
white arrow) trachea
is illustrated on
sagittal reformation.
Incidental note is made
of ossification (black
arrows) of posterior
longitudinal ligament.
A B C

lateral walls of the large airways and concom-

itant destruction of the cartilaginous trache-
obronchial rings with sparing of the posteri-
or wall [6] (Fig. 5). Tracheomalacia and large
airways stenosis may also be present.

Tracheobronchopathia
Osteochondroplastica
Tracheobronchopathia osteochondroplastica
is characterized by the development of osseous
and cartilaginous nodules in the submucosa of
the lower two thirds of the trachea and central
bronchi. On CT, nodules typically measuring
1–3 mm arise from the cartilaginous rings and
protrude into the airway lumen; the location of
A B these nodules is sometimes better depicted with
virtual bronchoscopy. Tracheomalacia does
Fig. 3—45-year-old man with history of wheezing and shortness of breath.
A and B, Contrast-enhanced chest CT images show increased ratio of coronal-to-sagittal tracheal diameters not occur, although an association with saber-
(arrows). This finding is characteristic of lunate trachea in this patient with tracheomalacia. sheath trachea has been described [7]. Similar

W242 AJR:196, March 2011


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Fig. 4—64-year-old man with long history of
smoking and chronic cough. Contrast-enhanced CT
image shows increased anteroposterior-to-lateral
dimensions of trachea (thick arrow) from smoking-
related chronic obstructive pulmonary disease. This
finding is characteristic of saber-sheath trachea. Left
upper lobe bronchogenic carcinoma (thin arrow) is
present.
to RPC, the posterior walls of the trachea and
bronchi are spared. However, the presence of
focal coarse calcification and ossification (Fig.
6) is more typical of tracheobronchopathia os-
teochondroplastica than RPC.
Amyloidosis
Tracheobronchial amyloidosis is the most
common subtype of thoracic amyloidosis
(Fig. 7). CT shows diffuse nodular thicken-
ing of the trachea and main bronchi, often
involving the subglottic trachea. Bronchial
stenosis or occlusion may result in lobar or
segmental atelectasis [8]. Nodular calcified
regions within the trachea are common, as in
A B
Fig. 6—47-year-old woman with chronic cough.
A–C, Unenhanced CT images show nodular, partially calcified, irregular thickening of trachea and main bronchi (arrows) with posterior sparing; these findings are
consistent with tracheobronchopathia osteochondroplastica.
AJR:196, March 2011 W243
CT of the Trachea and Central Airways
A B
Fig. 5—60-year-old man with history of asthma
presents with shortness of breath and cough.
A–C, Contrast-enhanced CT images of chest
show smooth thickening (white arrows) of
trachea with posterior sparing (black arrows);
these findings are most consistent with relapsing
polychondritis.
tracheobronchopathia osteochondroplastica.
Amyloidosis typically involves the airway
concentrically, whereas tracheobroncho-
pathia osteochondroplastica and RPC char-
acteristically spare the posterior tracheal
and bronchial walls. In addition, amyloidosis
may affect the larynx, pharynx, and superior
trachea in contrast to tracheobronchopathia
osteochondroplastica [9]. C
Wegener Granulomatosis and kidneys are involved in most patients
Wegener granulomatosis is an idiopath- (90% and 80%, respectively) [10]. Eleva-
ic necrotizing granulomatous vasculitis that tion of cytoplasmic antineutrophil antibod-
can affect patients of any age. The upper air- ies against protease 3 in cytoplasmic granule
ways are almost always affected; the lungs titers is common and reflects disease activity.
C
 Chung et al.

Fig. 7—54-year-old woman with history of tracheal

amyloidosis.
A and B, Axial (A) and coronal (B) contrast-enhanced
CT images of chest show diffuse circumferential
thickening (arrows) of trachea.
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A B

A B C
Fig. 8—49-year-old man with chest pain and cough.
A, Contrast-enhanced CT image of lower neck shows circumferential soft-tissue thickening and smooth narrowing of subglottic trachea (arrow).
B, Three-dimensional reconstruction of trachea and proximal airways shows smooth narrowing of subglottic trachea (arrow).
C, CT image obtained using lung window settings shows cavitary lesion in right upper lobe (arrow) in this patient with Wegener granulomatosis.

On CT, the trachea and main bronchi may be
diffusely or focally circumferentially thick-
ened; subglottic stenosis occurs in approxi-
mately 25% of patients and bronchial steno-
sis in about 18% of patients [11] (Fig. 8).
Peripheral bronchial narrowing, lobar and
segmental atelectasis, or bronchiectasis may
also be present. Coexistent pulmonary nod-
ules and masses, which are sometimes cavi-
tary; consolidation; and ground-glass opaci-
ties may suggest Wegener granulomatosis.
Mounier-Kuhn Syndrome
Mounier-Kuhn syndrome (tracheobron-
chomegaly) is unique among the diffuse tra-
cheal diseases in that it is characterized by
diffuse airway dilatation. Tracheobroncho-
megaly arises from either a congenital abnor-
mality or atrophy of the connective tissue of
the trachea and central bronchi. A tracheal
diameter of greater than 3 cm and mainstem
bronchi diameters of greater than 2.4 cm are
highly suggestive of Mounier-Kuhn syn-
drome [12, 13]. Broad diverticula may project
between the cartilaginous rings, giving the
trachea and proximal bronchi a corrugated
appearance (Fig. 9). Mild tracheal dilatation

W244 AJR:196, March 2011

 CT of the Trachea and Central Airways
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A B C
Fig. 9—70-year-old man with history of melanoma.
A, Contrast-enhanced CT image of chest shows diffuse dilatation of trachea.
B, Coronal reformation shows diffuse central bronchiectasis.
C, Sagittal reformation shows tracheal corrugation (arrows). Overall, findings are consistent with Mounier-Kuhn syndrome.

Fig. 10—61-year-old man with shortness of breath

and history of sarcoidosis.
A and B, Contrast-enhanced CT images of chest
show partially calcified, irregular, nodular thickening
of trachea (arrows). Tracheal lumen is narrow and
abnormal in shape.
A B

may also occur in the setting of chronic ob-
structive pulmonary disease, although differ-
entiation from Mounier-Kuhn syndrome is
usually readily made.
Sarcoidosis
Sarcoidosis is an idiopathic systemic dis-
ease characterized by the formation of nonca-
seating hyalinizing granulomas in a variety of
tissues including the lungs and airways. Hilar
and mediastinal lymphadenopathy, lung nod-
ules in a perilymphatic distribution, broncho-
vascular bundle thickening with or without fi-
brosis, and ground-glass opacities are common
CT manifestations of sarcoidosis. Large air-
way involvement, on the other hand, is very
uncommon. Airway abnormalities may result
from extrinsic compression by adjacent dis-
eased lymph nodes or from primary infiltration
of the airway walls with noncaseating granulo-
mas [6] (Fig. 10). Upper tracheal and laryngeal
involvement is more common than distal cen-
tral airway involvement [6].
Inflammatory Bowel Disease
Tracheal or bronchial involvement in in-
flammatory bowel disease is uncommon, of-
ten occurring late in the course of disease.
Pulmonary manifestations include bron-
chiectasis, chronic bronchitis, obliterative

AJR:196, March 2011 W245


Fig. 11—29-year-old woman with history of Crohn
disease.
A and B, Unenhanced CT images of chest show
circumferential, irregular thickening of trachea
(arrows). Mid and lower tracheal lumen is mildly
narrowed.
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A B
bronchiolitis, interstitial fibrosis, and large
airway disease [14]. Involvement of the large
airways manifests on CT as diffuse or focal
large airway narrowing. The tracheal wall
may be smoothly or irregularly thickened
(Fig. 11). This diagnosis should be consid-
ered in those presenting with asthma-like
symptoms with a history of inflammatory
bowel disease or with chronic gastrointesti-
nal complaints.
Conclusions
Traditionally, the trachea has been an af-
terthought on noninvasive imaging studies.
MDCT is the imaging test of choice to evalu-
ate the trachea and central airways because
of its high spatial resolution and ability to
produce a variety of reformations. Further-
F O R YO U R I N F O R M AT I O N
This article is available for CME credit. See www.arrs.org for more information.
W246 AJR:196, March 2011
Chung et al.
more, CT can show abnormalities of the tra-
cheal lumen, tracheal walls, and immediate
surrounding tissues. Additionally, tracheal
diseases may be misdiagnosed as asthma by
the clinician. The radiologist is often the first
to suggest the diagnosis of a diffuse tracheal
disease and can often suggest a single diag-
nosis. In cases when the diagnosis is unclear,
CT can serve as a guide for bronchoscopic
evaluation and transbronchial biopsy.
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