2009, Vol.47, Issues 2, Imaging of Airway Diseases

Imaging of Airway Diseases
Preface
Philippe A. Grenier, MD
Guest Editor
In the past 20 years, remarkable technologic response to therapy. Recent improvement in image
advances in CT imaging have revolutionized analysis techniques has made possible accurate
noninvasive imaging of all thoracic structures, and reproducible quantitative analysis of airway
including the airways. CT has assumed a central wall and lumen areas, as well as lung volume and
position in the modern management of both focal attenuation, leading to better insights in physiopa-
and diffuse airway diseases. The combination of thology of obstructive lung disease, particularly
thin collimation and helical acquisition during chronic obstructive pulmonary disease and asthma.
a single breath-hold at full inspiration multidetector The authors of this issue of Radiologic Clinics of
CT (MDCT) provides high-resolution volumetric North America were chosen for their focused
data sets that allow the generation of high-quality expertise in airway imaging. I thank those chest
multiplanar and three-dimensional images of the radiologists for sharing their experience and
airways. Accurate assessment and anatomical insights to provide a comprehensive update on
display of proximal and distal airways are routinely practical imaging for airway disease. While high-
obtained. In addition, dynamic acquisition during resolution CT and MDCT have tremendously
a forced expiratory maneuver is highly appreciated improved our ability to assess large- and small-
to detect and assess obstruction on small airways airway diseases, I anticipate even more develop-
and abnormal collapse of large airways. ments in the future. Rapid-volume scanning and
Nowadays, MDCT is used not only to detect new postprocessing techniques may promote
neoplastic and non-neoplastic endotracheal and sophisticated functional imaging and advanced
endobronchial lesions and to assess the extent interventions. MR imaging in this respect may
of tracheobronchial stenosis for planning treat- become an additional modality to MDCT.
ment and follow-up, but also to diagnose and
assess the extent of bronchiectasis and small- Philippe A. Grenier, MD
airway disease, and, in addition, to detect bron- Service de Radiologie Polyvalente
chial fistula, cysts, or dehiscences. Diagnostique et Interventionnelle
In parallel, there have also been important Hopital Pitie-Salpetriere
advances in diagnostic and interventional bronchos- 47-83, boulevard de I’Hopital
copy and surgery. In this respect, the information 75651 Paris cedex 13, France
provided by CT has become increasingly essential
for establishing accurate diagnoses, for guiding E-mail address:
and planning procedures, and for assessing [email protected] (P.A. Grenier)
radiologic.theclinics.com
Radiol Clin N Am 47 (2009) xi

doi:10.1016/j.rcl.2009.01.007
0033-8389/09/$ – see front matter ª 2009 Elsevier Inc. All rights reserved.
MDC T of the Air ways :
Technique and Normal
Results
Catherine Beigelman-Aubry, MDa, Pierre-Yves Brillet, MD, PhDb,
Philippe A. Grenier, MDa,*
KEYWORDS
Trachea anatomy Bronchi anatomy
Airway dimensions Airway MDCT technique
Previously, the trachea and main bronchi were as- expiratory maneuver may be requested to assess
sessed with a variable slice thickness up to 5 mm the degree of tracheobronchomalacia and the
with sequential or volumetric CT, and small extent of air trapping.
airways diseases were explored with high-resolu-
tion CT (HRCT), based on a 1.5-mm slice at IMAGE ACQUISITION AND RECONSTRUCTION
10-mm intervals. Currently, the new generation of
multidetector CT (MDCT) by combining volumetric Because the lung parenchyma offers a unique
CT acquisition and thin collimation during a single natural contrast, low radiation dose may be used
breathhold provides an accurate continuous without significant loss of information (100–120
assessment from the trachea to the most distal kV, 60–160 mAs). Using a detector size of 0.625
airway visible. Isotropic voxels allow image recon- mm with MDCT, images are reconstructed with
structions in which the z dimension is equivalent to a slice thickness of approximately 1 mm and over-
the x and y (in plane) resolution.1 This approach lapped with a reconstruction interval of approxi-
creates multiplanar reformations of high quality mately one-half slice thickness. This produces
along the long axis of the airways2 and three- a resolution voxel of almost cubic dimensions of
dimensional volume rendering, including extrac- approximately 0.4 mm in each direction by using
tion of the airway and virtual endoscopy without a spatial resolution algorithm. Experts recommend
any distortion in any orientation. Whatever their using a 512 or even a 768 matrix, which permits
nature and severity, excellent assessments of fields of view of 265 mm and 400 mm, respec-
stenoses may be obtained by a combination of tively. The pixel size at the workstation, which is
various reconstructions, especially the determina- defined as the ratio between the field of view and
tion of the morphology, including the identification the matrix, has to be lower than the intrinsic reso-
of horizontal webs and the length and exact loca- lution in the plane of image to benefit from the
tion from the vocal cords and carina.3,4 Airway intrinsic resolution capabilities of the equipment.5
stents and extrinsic airway compression are also A rotation time of approximately 500 msec
assessed perfectly. Preprocedural planning before allows an important decrease in cardiac pulsation
stent placement or surgery3 and posttherapeutic artifacts and allows a good analysis of all bronchi,
aspects also benefit from the same techniques. including the paracardiac areas. Breathholding for
Despite images usually being obtained during acquisition of the entire chest lasts approximately
suspended inspiration for analysis of airways, 6 to 8 seconds using a 40 or 64 detector row CT
a complementary acquisition during forced scanner, which avoids respiratory motion artifacts
a
Service de Radiologie, Hôpital Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (APHP), Université
Pierre et Marie Curie, 47/83 boulevard de l’Hôpital, 75651 Paris cedex 13, France
b
Service de Radiologie, Hôpital Avicenne, Assistance Publique-Hôpitaux de Paris (APHP), Université Paris XIII,
UPRES EA 2363, Hôpital Avicenne, 125, route de Stalingrad, 93009 Bobigny, France
* Corresponding author.
E-mail address: [email protected] (P.A. Grenier).
Radiol Clin N Am 47 (2009) 185–201

doi:10.1016/j.rcl.2009.01.001
186 Beigelman-Aubry et al
in most cases. The use of cardiac gating is not rec- tracheobronchial tree, the airway walls, and the
ommended because of the higher radiation dose spurs at the same time. Moving up and down
delivered and short rotation time available with through the volume at the monitor has become
the last generations of MDCT. a useful alternative to film-based review. This
viewing technique helps indicate the exact loca-
tion of any airway lesion and may serve as a road-
READING AND POSTPROCESSING TOOLS
map for the endoscopist. Reading of chest MDCT
Cine Viewing
goes actually far beyond the standard assessment
Visualization of overlapped thin axial images of axial slices, because multiplanar reformats are
sequentially in a cine mode allows analysis of easily performed in real time in all directions6 and
bronchial divisions from the segmental origin slabs with various rendering modes. Once any
down to the smallest bronchi that can be identified abnormality has been detected, an oblique refor-
on thin section images. Particular attention must mat plane may be chosen with the swivel mode
be paid to the analysis of the lumen of the by focusing a rotation center on the abnormality
Fig. 1. (A) Down and backward 1.41-mm oblique reformat allows visualization of the trachea, carina, main
bronchi, and some segmental and subsegmental bronchi. Progressive thickening of the slabs—17 mm (B) and
54 mm (C)—allows reproduction of previous tomographic aspects with better understanding of the underlying
pathology, especially for the airways. (D) Slab average of 180 mm thickness allows reproduction of the aspect
of the frontal chest radiograph. The right tracheal stripe is clearly explained by the correlation on (A).
MDCT of the Airways: Technique and Normal Results 187
found. A combination of slabs of various thick- surrounding pulmonary parenchyma

nesses with minimum intensity projection (mIP) (Fig. 2), with a difference of density between
or maximum intensity projection (MIP) or both 50 and 150 HU.9 The overall morphology of
usually is obtained. the tracheobronchial tree is particularly well
displayed on longitudinal views combined
Two-Dimensional Reformats and Multiplanar with a multiplanar volume reformation mIP
Volume Rendering Slabs technique. Three- to 7-mm slabs are partic-
Reformations and reconstructions are easy to ularly adapted for the assessment of central
generate and may be interactively performed in airways stenosis, but the slab thickness
real-time at the console or workstation. Multipla- may be chosen according to the complexity
nar reformation images are single-voxel sections and morphology of the abnormality and
with a 0.6- to 0.8-mm displayed image. They are may be increased up to several centime-
the easiest reconstructions to generate and ters. Abnormal lucencies, including bron-
permit creation of images oriented in any plane, chial wall diverticula observed in patients
especially along the long axis of any airway (eg, who have chronic obstructive pulmonary
in a coronal oblique orientation for the trachea disease and bronchial anastomosis, dehis-
and the carina). On the other hand, multiplanar cence, or fistula during or after lung trans-
volume reformation consists of a slab of adjacent plantation, may be assessed using the
thin slices of various thicknesses that may be same technique. Multiplanar volume refor-
combined with the use of intensity projection mation mIP is also used for a systematic
techniques. The reformation plane may be analysis of the parietal wall and lumen of
selected by focusing a rotation center on the the bronchi. This analysis also includes the
abnormality and using the swivel mode or using assessment of peribronchial thickening
a three-dimensional reconstructed image of the encountered in case of lung diseases with
airways.7 A significant decrease in the number a perilymphatic distribution. In chronic
of slices to be analyzed is achieved by analysis bronchial disease, bronchial wall thickening
of longitudinal reformats compared with the axial is often irregular and associated with thick-
images with a complementary role of both ening of the spurs and irregularities in the
viewing techniques. morphology and caliber of the bronchi.
Analysis of various large and small airway This technique may help plan the correct
diseases may be enhanced with this technique. bronchoscopic pathway toward a distal
In fact, multiplanar volume reformation images lesion for biopsy. Postexpiratory mIP
combine the excellent spatial resolution of multi-
planar reformats images with the anatomic display
of thick slices8 and the possibility of using various
rendering tools:
Average: the mean attenuation value of the
voxels in every view throughout the volume
explored is projected on a two-dimensional
image. A less noisy image may be obtained
de facto. Tomographic equivalent images
may be obtained by thickening the slabs
with equivalent of plain films in the coronal
and lateral views with the thickest slabs
(Fig. 1).
mIP imaging is a simple form of volume
rendering (sliding thin slab or multiplanar
volume reformation mIP technique) that is
able to project the tracheobronchial air
column onto a viewing plane by projecting
the pixels with the lowest attenuation value.
This technique enhances the visibility of the
airways within lung parenchyma below the
subsubsegmental level because of lower
attenuation of air contained within the Fig. 2. Coronal mIP 60-mm slab allows display of the
tracheobronchial tree compared with the normal bronchial tree to the subsegmental level.
Fig. 3. Single (A) and 14-mm slab MIP (B) coronal reformats in a patient suffering from infectious bronchiolitis. (A)
Patchy ground-glass bronchoalveolar nodules with bronchial wall thickening (white arrow). (B) Diffuse tree in
bud aspects (black and white arrows) difficult to assess in (A) are obvious with MIP.
images may be useful for detecting and as- This technique has proved to be of particular
sessing the extent of air trapping. interest in diagnosing mild changes in airway
MIP consists of projecting the voxel with the caliber and understanding complex tracheobron-
highest attenuation value in every view chial abnormalities.14 When correlating bronchos-
through the volume explored.10,11 It displays copy and three-dimensional reconstructions,
10% of the data set, as does the mIP tech- Kauczor and colleagues15 observed no
nique. Centrilobular nodules related to
inflammatory or infectious changes in the
small airways are easily recognized with
respect to landmarks of the secondary
pulmonary lobule (Fig. 3).12 A rapid assess-
ment of the regional distribution in the cra-
niocaudal and axial dimensions is obtained
at the same time. MIP of variable thickness
also provides an excellent assessment of
the location and size of vessels. In this
way, mosaic perfusion pattern is diagnosed
by combination of mIP and MIP and is easily
differentiated from mosaic attenuation
pattern caused by infiltrative lung disease.
External Three-Dimensional
Rendering Technique
The volume-rendering technique applied at the
level of the airways ensures a three-dimensional
reconstruction of the airways to the subsegmental
level by depicting the inner surface of the airway
with a specific color and opacity (Fig. 4). It has
capabilities of visualization in semi-transparent
mode similar to conventional bronchograms.13
For this reason, this technique has been referred
as CT bronchography. Three-dimensional segmen-
tation techniques provide an anatomic map of the
airways and easily may demonstrate changes
between inspiration and expiration in the case of Fig. 4. Three-dimensional volume rendering of
tracheomalacia. a normal tracheobronchial tree.
discrepancies concerning the location and severity for three-dimensional reconstruction of the
of central stenoses. Three-dimensional helical CT tracheobronchial tree based on bronchial lumen
provided an accurate road map for the central detection within the thoracic volume data set ob-
airways. Clinical relevance in patients with tumors tained from thin MDCT acquisition. It provides
resulted from severe stenoses or occlusions that a specific visualization modality that relies on
lead to dyspnea and stridor. When bronchoscopy energy-based three-dimensional reconstruction
revealed severe stenosis or total occlusion, the of the bronchial tree up to the sixth or seventh
patency of distal bronchi, tumor involvement, or order subdivisions with a semi-transparent volume
collapse could not be assessed bronchoscopically. rendering technique.17 Automatic delimitation and
In comparison, three-dimensional helical CT was indexation of anatomic segments make local and
superior at showing the residual lumen and length reproducible analysis possible at any level of the
of the stenoses, spatial orientation, branching bronchial tree. Automatic extraction of the central
angles, and patency of distal air-filled bronchi. axis of the bronchial tree allows interactivity during
These complementary details were important for navigation within CT bronchography or virtual
possible endobronchial procedures such as laser endoscopy modes.
ablation, stent placement, and transbronchial
radiotherapy. This approach facilitated the choice
Internal Rendering Technique or Virtual
of endobronchial procedures, and the size of the
Bronchoscopy
stent could be determined accurately.
Using the same concept, Fetita and Virtual bronchoscopy by combining helical CT
colleagues16 developed a fully automatic method data and virtual reality computing techniques13
Fig. 5. Virtual endoscopy at the level of the middle trachea (A) and the carina (B).
provides an internal rendering of the tracheobron-

chial walls and lumen by using reconstruction with
soft kernel. An endoscopist’s view of the internal
surface of the airways is simulated with a perspec-
tive-rendering algorithm (Fig. 5). The observer may
interactively move through the airway at a rate of
15 to 25 images/second down to the subsegmen-
tal bronchi. Potential applications include the
assessment of airway stenoses and guidance of
transbronchial biopsy procedures.18 The tech-
nique allows accurate reproduction of major endo-
luminal abnormalities and has excellent correlation
with fiberoptic bronchoscopy results regarding the
location, shape, and severity of airway narrowing.
Virtual bronchoscopy is also able to evaluate the
airways distal to a high-grade stenosis, beyond
which a conventional bronchoscope cannot
pass.19 It is also possible to perform retroscopy
when looking back toward the distal part of the
stenosis. Virtual endoscopy may be considered
a substitute for repeated bronchoscopies when
performed in the follow-up of interventional proce-
dures.20 Despite its potential, virtual endoscopy is
unable to identify the causes of bronchial obstruc-
tion21 or detect mild stenosis, submucosal infiltra-
tion, and superficial spreading tumors.4
Fig. 6. Patient suffering relapsing polychondritis. (A)
Normal aspect on inspiration is visualized on a volume
LEVEL OF BREATHING from the vocal cords to the lower pulmonary veins. (B)
Multifocal air trapping is assessed on 20-mm slab
All CT examinations of the airways are first per- coronal mIP reformat despite the fact that the acqui-
formed at suspended full inspiration. In case of sition was performed with only 15 mAs.
suspicion of mosaic attenuation or bronchomala-
cia, expiration has to be performed on a dynamic
mode. In fact, this technique has proved to opti- airway lumen and airway walls when they are not
mize the detection of air trapping, which is an perpendicular to the scanning plane may lead to
indirect sign of small airway disease,22 and trache- significant errors related to an overestimation of
obronchomalacia.23 The advantage of volumetric airway wall area, as in the case of oblique bronchi.
acquisition in this setting is use of mIP that The larger the angle and field of view and the
enhances the visual detectability of air trapping thicker the collimation, the greater the overestima-
and may increase the conspicuity of this finding,24 tion of airway wall area. Accurate measurements
even using low-dose CT (Fig. 6).25 of airway lumen and wall area have to be restricted
to airways that appear rounded (ie, cut in cross-
QUANTITATIVE CT ASSESSMENT OF AIRWAYS section). The new generation of multislice CT
scanner allows segmentation of bronchial lumens
Airway lumen and airway wall areas may be as- with three-dimensional reconstruction of the
sessed quantitatively on CT images by using airways, extraction of the central axis of the
specific techniques that must be reproducible airways, and reconstruction of the airway cross-
and accurate to compare the airways before and section in a plane perpendicular to this axis
after therapy and carry out longitudinal studies of (Fig. 7).26
airway remodeling. Because airway lumen and Numerous techniques have been reported for
wall areas measured on axial images depend on measuring airway diameter using CT. They have
lung volume, volumetric acquisition at controlled been validated using data from phantom studies
lung volume is required to precisely match the and excised animal lungs or by developing realistic
airways of an individual on repeated studies. modeling of airways and pulmonary arteries
Lumen and wall areas measured on axial images included in CT scans of animal lungs obtained in
also depend on the angle between the airway vivo.27–32 These techniques have proved to be
central axis and the plane of section. Measuring more accurate than those obtained with manual
Fig. 7. Segmentation of bronchial lumens with three-dimensional reconstruction of the airways, extraction of the
central axis of the airways, and reconstruction of a subsegmental branch of the posterior basal segmental bron-
chus of the left lower lobe (LLL) cross-section in a plane perpendicular to this axis.
methods. In fact, manual tracing of the inner and the cricoid cartilage at the level of the sixth cervical
outer contours of the airway cross-section on axial vertebra to the carina at the level of the fifth
CT images is a time-consuming technique that thoracic vertebra, has an oblique course down-
suffers from large intra- and interobserver vari- wards and backwards. Its length on inspiration
ability in measurement of airway wall and lumen has a value of 10 to 12 cm in adults, including
areas.29,33–35 Their accuracy in measuring the the extrathoracic (2–4 cm) and intrathoracic
airway lumen27–29,31 and wall28,30 areas was portions (6–9 cm).42,43 Changes in position may
good only for bronchi that measured at least be observed between inspiration and expiration,44
2 mm in diameter. These techniques have been up to 3 cm, and with neck flexion and extension.
used to quantify the magnitude and distribution Adjacent structures directly related to the
of airway narrowing in excised lung animals and trachea are the thyroid gland, which is anterior
animal lungs in vivo and in normal patients and and lateral to the cervical trachea, vessels anterior
patients who have asthma.27,29,36–38 Although the to the intrathoracic trachea (supra-aortic vessels,
techniques have been used almost exclusively aortic arch, pulmonary arteries), systemic veins
for research purposes, with continued refinements (superior vena cava, azygos vein), which are ante-
they eventually will be beneficial in the clinical rolateral or lateral to the trachea, lymph nodes, the
practice of radiology.39–41 esophagus, which is posterior or lateral to the
trachea, and the left recurrent nerve. The trachea
NORMAL ANATOMY AND NORMAL CT FINDINGS
is often displaced slightly to the right at the level
Trachea
of the aortic arch, which may be accentuated in
The conducting airways that distribute air to the older patients with tortuous atherosclerotic aorta.
gas-exchanging units begin with the trachea. The The right or posterior tracheal wall contacts the
trachea, which originates at the inferior margin of right lung at a variable extent.42
The tracheal wall is comprised of several layers, value of approximately 1.42,43,47 A ‘‘saber sheath
including an inner mucosa layer, a submucosa, trachea’’ is characterized by a marked coronal
cartilage or muscle, and an outer adventicia layer. narrowing with a tracheal index of less than 0.5.
The anterior walls of the trachea and main bronchi This finding is suggestive of chronic obstructive
are formed with U-shaped rings of hyaline carti- lung disease with emphysema. Conversely,
lage—16 to 22 for the trachea—that open dorsally. a ‘‘lunate’’ configuration with a ratio of more than
These rings are linked longitudinally by annular 1 suggests tracheomalacia related to excessive
ligaments of fibrous and connective tissue and expiratory collapsibility of the airway lumen.50
help support the tracheal wall and maintain an
adequate tracheal lumen during forced expira-
Main, Lobar, Segmental, Subsegmental
tion.43 The flat dorsal wall consists of a thin fibro-
Bronchi and Small Airways
muscular membrane that includes the trachealis
muscle, which is composed by transversely The trachea gives rise to the right and left main
disposed smooth muscle.45 bronchi with an asymmetrical branching. The left
The cross-sectional appearance of the trachea is main bronchus is narrower than the right one,
most commonly rounded, oval, or horseshoe with a length of approximately 5 cm51 and a typical
shape on inspiration, with a posterior wall that is elliptical shape and branches off at a greater angle
typically flat or convex posteriorly. Several shapes than the right.52 The left main bronchus branches
may be encountered at different levels.42 The right into the left upper and lower lobe bronchi. The right
wall of the inferior aspect of the trachea is in contact bronchus is shorter than the left one and extends
with air within the medial aspect of the right upper for 1 to 2 cm before dividing into the right upper
lobe, which results in the right paratracheal stripe lobe bronchus and the bronchus intermedius.
seen in routine posteroanterior chest radiographs Within the lung, the bronchi branch dichotomously
with a normal size less than 4 mm. The tracheal and give rise to progressively smaller airways.
wall appears as a 1- to 3-mm soft-tissue stripe Branching is asymmetrical, taking into account
between the air-filled tracheal lumen and the lateral that the two daughters of a given branching may
fat density of the mediastinum.42,46,47 Normal carti- differ in diameter, length, and angle. The number
laginous rings may appear slightly denser than of generations from the main bronchus to the
surrounding soft tissue and fat. Calcification of acini varies from as few as 8 to as many as 25, de-
the cartilage is commonly seen in older patients, pending on the region of the lung supplied.52 The
particularly women, at the level of the trachea and lobar bronchi branch off to segmental bronchi.
more distal bronchi (Fig. 8). These calcifications Several systems for labeling segmental anatomy
are usually discontinuous.42 have been proposed, mainly the Jackson and
The normal transverse diameter of the trachea in Huber and the Boyden classifications.53,54
men and women is 13 to 25 mm and 10 to 21 mm, Segmental bronchi are designated by ‘‘B’’ fol-
respectively, and the normal anteroposterior diam- lowed by a number, and subsegmental bronchi
eter in men and women is 13 to 27 mm and 10 to are indicated by the segmental number followed
23 mm, respectively. The tracheal diameter aver- by a lower case letter. The numbering of the
ages 19.5 mm in men and 17.5 mm in segmental bronchi corresponds to their order of
women.43,46,48 A mean decrease in the transverse origin from the airway. Although the segmental
diameter of approximately 15%, in the anteropos- bronchial anatomy varies, the right lung contains
terior diameter of approximately 30%, and in the ten segmental bronchi and the left contains eight
cross-sectional area of the trachea of approxi- in most patients. The first Boyden classification,
mately 35% is observed on forced expiration, which initially designated the anterior segment as
mainly related to invagination of the posterior B2 and the posterior segment as B3, is used in
tracheal membrane. On expiration, the posterior this article (Figs. 9–12). Identification of the origin
tracheal membrane appears convex anteriorly; of most bronchi depends on recognizing the spurs
the horseshoe shape ensures actual patient expi- that separate them. Depending on its angle rela-
ration. Tracheomegaly is defined in men as tive to the CT plane, a spur appears either as
a tracheal diameter of more than 25 mm in the a triangular wedge of soft tissue or, when
transverse diameter and more than 27 mm in the sectioned along its length, as a linear septum
anteroposterior dimension. In women it is defined that separates adjacent airways or faint curvilinear
as tracheal diameter of more than 21 mm in the densities.45
transverse diameter and more than 23 mm in the After branching off the pulmonary artery below
anteroposterior dimension.49 the aortic arch, the right pulmonary artery enters
The tracheal index is obtained by dividing the the lung anterior to the right main bronchus; the
coronal diameter by the sagittal one, with a normal left pulmonary artery passes above the main
Fig. 8. Single (A), 3-mm (B), and 11-mm (C) slabs average coronal reformats perfectly demonstrate the calcified
tracheal rings in a normal older woman. Single (D) and 62-mm (E) slab average sagittal reformats. The lack of
cartilage at the level of the posterior wall of the trachea is well seen (arrows). Conversely, the ring cartilages
that are partially calcified are obvious at the level of the anterior and lateral part of the trachea (arrows).
194
Fig. 9. Normal anatomy of the bronchi on the right side. The first Boyden’s classification is used with successive
1-mm slices. (A) The arrow shows the division of the apical bronchus of the right upper lobe. Note the oval shape
of the normal trachea. (B) Right upper lobe bronchus arises from the lateral aspect of the main bronchus, approx-
imately 2 cm distal to the carina (large arrow) and courses horizontally for approximately 1 cm from its origin
before dividing in segmental branches. Subsegmental bronchi of the anterior bronchus of the right upper
lobe are marked by arrows. (C) The bronchus intermedius divides after 3 to 4 cm into the middle lobe and lower
lobe bronchi. (D–F) The middle lobe bronchus arises from the anterolateral wall of the bronchus intermedius and
courses anteriorly and laterally for 1 to 2 cm before dividing into lateral (B4) and medial (B5) branches. This origin
of the middle lobe is almost at the same level as B6, which is the first branch of the short right lower lobar bron-
chus (RLLB). The superior segmental bronchus B6 and its subsegmental bronchi arise from the posterior aspect of
the RLLB just beyond its origin and course posteriorly. The truncus basalis represents a continuation of the lower
lobar bronchus below the origin of B6 and typically appears circular or ovoid. It extends for approximately 1 cm
before dividing in four basal segmental bronchi. The first basal bronchi is the medial basilar segmental bronchus
(B7) that arises anteromedially from the TB. (G–I) Next, there is a successive appearance of the anterior, lateral
(B9), and posterior basilar (B10) bronchi and their respective subsegmental bronchi. Note that B7 and its subseg-
mental bronchus lie typically anterior to the inferior pulmonary vein. (J) 3D volume rendering.
first four to six generations that effectively

provided a circumferential support; the axial
bronchi had only scattered plates for another
four to six generations. Bronchioles are conduct-
ing airways with a wall less than 1 mm in diameter
consisting in smooth muscle enclosed in a thin
connective tissue space without cartilage.
Membranous bronchioles do not contain alveoli,
as opposed to respiratory bronchioles, which are
lined partly by alveoli. The terminal bronchioles
identify the most distal generation of membranous
bronchioles, that is, the parent generation to the
respiratory bronchioles. Two to three additional
generations of respiratory bronchioles are present
after the most proximal branch.
The outer diameter of a bronchus is approxi-
mately equal to that of the adjacent pulmonary
artery. The normal bronchoarterial ratio, defined
as the internal diameter (ie, luminal diameter) of
the bronchus divided by the diameter of the adja-
cent pulmonary artery, generally averages 0.65 to
0.70.56 The presence of a bronchoarterial ratio of
Fig. 9. (continued). more than 1 in normal subjects has been associ-
ated with increased age.56 The measurement has
been found to be influenced by altitude, presum-
ably as a result of the combination of hypoxic
vasoconstriction and bronchodilatation. In one
investigation of 17 normal, nonsmoking individuals
stem bronchus and then over the superior lobar living at 1600 m and 16 individuals living at sea
bronchus, coming to lie posterior to the bronchus. level, the mean bronchoarterial ratio was 0.76 at
Within the lung parenchyma, the bronchi and altitude and 0.62 at sea level.57 The bronchoarterial
pulmonary artery branches are closely associated ratio may also appear to be more than 1 if the
and branch in parallel until they reach the acini. scan traverses an undivided bronchus near its
Their appearance depends on their orientation. branch point and its accompanying artery
When imaged at an angle to their longitudinal already has branched.58
axis, central pulmonary arteries normally appear Anatomically, the walls of large bronchi, outlined
as rounded or elliptic opacities accompanied by by lung on one side and air in the bronchial lumen
uniformly thin-walled bronchi of similar size and on the other, appear smooth and of uniform thick-
shape. Bronchi and pulmonary arteries appear as ness. The normal thickness of an airway wall is
cylindrical structures that taper as they branch related to its diameter, with a normal bronchial
when imaged along their long axes; they appear wall thickness corresponding to approximately
rounded if they lie perpendicular to the plane of 10% to 30% of the bronchial diameter. Lobar to
the CT or elliptical when oriented obliquely. segmental bronchi (second to fourth generation)
Bronchi and arteries are encased by the peribron- have a wall thickness of approximately 1.5 mm
chovascular interstitium, which extends from the and a mean diameter between 5 and 8 mm. Sub-
pulmonary hila into the peripheral lung. The pulmo- segmental (sixth to eighth generation) bronchi
nary veins drain independently from the bronchi have a wall thickness of approximately 0.3 mm
with two trunks that enter the left atrium and mean diameters between 1.5 and 3 mm.
separately. Currently, subsegmental bronchi are routinely
All conducting airways are muscular tubes lined identifiable; more distal airways (eleventh to thir-
by a ciliated epithelium. Bronchi with a diameter of teenth generation) have a wall thickness of 0.1 to
approximately 1 mm or more have walls reinforced 0.15 mm and diameters that measure 0.7 to
by cartilage, which take the form of variably 1 mm.59 The visibility of normal bronchioles
shaped islands that diminish in size and number depends on their wall thickness rather than diam-
progressively with the decreasing caliber of the eter. The smallest airways normally visible using
bronchi. According to Hayward and Reid,55 HRCT have a diameter of approximately 2 mm
a high density of cartilage was observed for the and a wall thickness of 0.2 to 0.3 mm. The value
Fig.10. Anatomic variations of the right bronchial tree. (A–C) Subsuperior segmental bronchus B*, accessory supe-
rior segmental bronchus or subapical bronchus, is a common variation of the right lower lobe originating below
B6 (A) at a variable level from the truncus basalis down to the posterior segmental basilar bronchus. In this case,
the origin is well seen in (B) at the posterior part of the B8 1 9 1 10 trunk (curved arrow), B7 lying medially.
Successive origin of B8 and B9 1 10 in (C). (D–F) Most common variation of the subdivision of medial basilar
segmental bronchus B7. In this case, the medial ramus B7b courses posterior to the inferior pulmonary vein,
unlike the anterior ramus B7a, which remains anterior to the vein.
Fig. 11. (A, B) Subapical bronchus originates from the posterior basilar segmental bronchus of the right lower
lobe. Single reformat (left) and thin mIP (right) allow visualization of the complete course of B6 and the
abnormal bronchi, which have a similar horizontally posterior course.
of the caliber of a bronchiole supplying and cartilage varying at different levels of the
a secondary lobule, a terminal bronchiole, and bronchus, variation within individual airway
a distal respiratory bronchiole is 1 mm, approxi- lumens, and wall area percentage may be based
mately 0.6 mm, and approximately 0.4 mm, on variation of bronchial morphology. Quantita-
respectively, and the thickness of its wall tive evaluation of the degree of heterogeneous
measures approximately 0.15 mm, 0.1 mm, and constrictor responses to bronchial challenge
less than 0.1 mm, respectively.58 As a result, should include consideration of normal variation
normal bronchioles are not visible on CT scans. of airway lumen.
Airways are rarely seen within 1 cm of the pleural Expiratory HRCT may demonstrate lobular
surface in most locations, except adjacent to the areas of air trapping in as many as 60% of normal
mediastinum.57 patients. The prevalence of air trapping increases
CT measurement slightly overestimates wall with age.61-63 The dependent lung, the lung bases,
thickness because it also includes the surrounding and the superior segments of the lower lobes are
peribronchial interstitium. The apparent bronchial most often concerned. The degree of air trapping
wall thickness and diameter of bronchi are mark- that may be identified in normal patients remains
edly influenced by the display parameters. The controversial, however.
window and width levels that provide the most
accurate measurement of bronchial caliber and
wall thickness are 450 to 500 HU and 1000 to SUMMARY
1500 HU, respectively.29,35,60 When an incorrect
parameter is used, the error in estimating the thick- The new generation of MDCT has revolutionized
ness or size of a structure is related to its actual noninvasive imaging of proximal and distal
thickness or size, greater fractional overestimates, airways. Exquisite anatomic details of the airway
or underestimates being made for small structures. lumen and airway wall on axial CT images benefit
In their study, Matsuoka and colleagues56 in routine practice from postprocessing tools in
found variation of airway caliber and wall area adequate orientation, ensuring excellent assess-
percentage within individual bronchi on cross- ment of the morphology and location of any path-
sectional CT sections in asymptomatic subjects ologic condition. This technique may be combined
without cardiopulmonary disease. In 32.7% of with use of low-dose CT. The next challenge for CT
the sites observed in contiguous CT sections, is the functional assessment with accurate quanti-
the airway lumen did not decrease on the periph- fication of caliber and thickness of the whole bron-
eral side, as classically expected. According to chial tree integrated in the more complex
the authors, the proportions of epithelium, evaluation of the lung parenchyma, including
smooth muscle, interstitial connective tissue, hypoattenuated areas.
Fig. 12. Normal anatomy of the bronchi on the left side. The first Boyden’s classification is used with successive
1-mm slices. (A–C) The left upper lobe bronchus bifurcates in most cases in an upper culminal bronchus (CB), which
almost immediately divides into an apicoposterior (B1 1 3) and anterior (B2) segmental bronchus and in a lower divi-
sion, the lingular bronchus. (A) Subsegmental divisions of B1, B2, B3 are shown (arrows). (D–F) The lingular bronchus
(arrow in D) arises from the lower part of the left upper lobar bronchus (LULB), extends anteriorly and inferiorly for 2
to 3 cm, and then bifurcates into the superior (B4) and inferior divisions (B5). The course of B4 tends to be more
lateral and horizontal than B5. Left lower lobar bronchus (LLLB) and B6 are similar to those on the right side. (F)
Note that the rounded lucency corresponds to the medial subsegmental branch of B6 and that the lucency at the
posterior part of the TB (star) corresponds to a subapical branch of the LLLB, more rarely seen on the left side
than on the right side. (G–I) The truncus basalis is longer than on the right side and divides into three basal segmental
bronchi, including anteromedial (B7 1 8), lateral (B9), and posterior (B10). (J) 3D volume rendering.
9. Fraser RS, Muller NL, Colman N, et al. The normal

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14. Remy-Jardin M, Remy J, Artaud D, et al. Volume
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Congenital
Abnormalities of
Intrathoracic Airways
Amandine Desir, MD, Beno^|t Ghaye, MD*
KEYWORDS
Computed tomography (CT) helical Bronchi abnormalities
Bronchi CT Trachea abnormalities Lung anatomy
In the past, congenital tracheobronchial variants or tracheobronchial tree, whereas cartilage, connec-
anomalies were demonstrated in 1% to 12% of tive tissue, and muscle develop from the
patients at bronchography or bronchoscopy.1–3 surrounding splanchnic mesenchyme. By 28 to
Anomalies of the tracheobronchial tree are diag- 30 days, the lung buds have elongated into
nosed with increasing frequency as a result of primary bronchi. The five lobar bronchi have ap-
refinement in contemporary imaging and classifica- peared by 30 to 32 days, and all segmental bronchi
tion. Multidetector CT (MDCT) has broadened the are formed by 36 days. Over the same period, the
potential of imaging of lung anatomy, offering vascular supply to this tissue shifts from branches
different modalities of reformatting which improve of the splenic plexus to define the pulmonary
the understanding of complex tracheobronchial artery (PA) as fusion of the lung bud plexus with
anomalies, even in the pediatric population.4–12 the sixth branchial arches occurs.13
Most congenital tracheobronchial anomalies are Three major hypotheses have been formulated
rare and almost always nonsymptomatic. to explain the pathogenesis of anomalous
Nevertheless, such anomalies are important to tracheobronchial development.14,15 The reduc-
know because some may be confused with or tion theory postulates that the final anatomy is
even responsible for respiratory disease. They the result of shrinkage and finally suppression
may also be associated with cardiovascular and/ of portions of a primitive pattern encompassing
or other organ malformations;13 furthermore, all the components of the extant mammalian
radiologists should be familiar with anatomic vari- bronchial distribution.16 The migration theory
ants and anomalies to guide adequately clinicians considers that bronchial outgrowths have the
or surgeons when bronchoscopy or surgical plan- potential of migrating from their initial locus to
ning is needed. new points of origin of a bronchus or the
trachea. The selection theory advocates local
disturbances in the development during budding
EMBRYOLOGY
of the bronchial buds under the influence of
Normal tracheobronchial development is initiated bronchial mesenchyma.17 It is likely that defects
at 24 to 26 days as a median bulge of the ventral involving supernumerary tracheal bronchi occur
wall of the pharynx which develops at the caudal early in the development around 29 to 30 days
end of the laryngotracheal groove. At 26 to 28 as the lobar bronchi start to differentiate. On
days, it gives rise to right and left lung buds. As the other hand, displaced bronchi would more
the lung buds elongate, the trachea is separ likely occur after 32 days as the bronchi elongate
ated from the esophagus by lateral ingrowth of and branch further.14 Various hypotheses have
the mesoderm forming the tracheoesophageal been proposed to explain anomalous communi-
septum. The endodermal lining of the laryngotra- cations between the esophagus and tracheo-
cheal tube gives rise to the epithelium of the bronchial tree.9,18
Department of Medical Imaging, University Hospital of Liège, B35 Sart Tilman, B-4000 Liège, Belgium
E-mail address: [email protected] (B. Ghaye).
Radiol Clin N Am 47 (2009) 203–225

doi:10.1016/j.rcl.2008.11.009
204 Desir & Ghaye
DEVELOPMENTAL INTERRUPTION asymptomatic, leading to incidental discovery in

adults.19–23 When symptoms are present, respira-
Congenital underdevelopment of the tracheobron- tory distress or recurrent infections are more
chial tree and the lungs, known as the agenesis- frequently reported in agenesis or aplasia of the
hypoplasia complex, has been classified into three right lung, probably due to greater mechanical
groups according to the embryologic stage of compression and distortion of bronchial and
developmental interruption: (1) agenesis (absence vascular structures secondary to an ipsilateral
of bronchus, vessel, and lung parenchyma), (2) rotation of the mediastinum.22,23 Recurrent
aplasia (absence of lung parenchyma with blind infections may be even more frequent in patients
rudimentary bronchus present), and (3) hypoplasia with aplasia of one lung, with the blind bronchus
(decrease of the number and size of bronchi, potentially acting as a reservoir.20,24 Vestigial lung
vessels, and parenchymal structures).19 Diagnosis parenchyma can be found at the end of the aplastic
is easily established when related to a whole lung bronchus.25 Associated malformations are reported
or lobe but can be more difficult when related to in half of patients.19,22,23 Lung hypoplasia is
segmental bronchi (Fig. 1). generally secondary (ie, to a neighboring space-
Tracheal agenesis/aplasia is an exceptional occupying lesion or malformation that stunts lung
malformation due to failure of development of the growth).22,23
lung bud resulting in complete absence of the Lobar and segmental agenesis or aplasia is more
trachea and lungs, not compatible with life. frequently found in the right upper lobe (RUL). When
Pulmonary agenesis and aplasia can affect both isolated, it is usually asymptomatic (see Fig. 1).
lungs with similar frequency and may remain Lobar hypoplasia may be seen in various
Fig. 1. Aplasia of segmental bronchi of the left upper lobe. (A–E) Axial CT slices, coronal MPR, and three-dimen-
sional volume-rendering reformatting show blind anterior and apicoposterior segmental bronchi (arrows) of the
left upper lobe. The left lung is smaller than the right one, and the left major fissure (arrowheads) is displaced
anteriorly due to moderate hypoplasia of the left upper lobe. The apex of the left lung is ventilated by the supe-
rior segmental bronchus of the left lower lobe (long arrow). MPR, multiplanar reformatting.
Congenital Abnormalities of Intrathoracic Airways 205
conditions, including bronchial atresia, bridging the bronchial system and the esophagus and
bronchus (BB), esophageal bronchus, and congen- a systemic vascular supply.31 Another classifica-
ital bronchial stenosis, or in patients with an ipsilat- tion of CBPFMs based on the morphology of the
eral vascular malformation such as in the fistulous tract (neck, diverticulum, cyst) and the
hypogenetic lung syndrome or veno-lobar blood supply has also been proposed.38
syndrome.11,19,22–24 Congenital bronchiectasis is The most common communications are between
a rare disease manifesting as tubular dilatation of the mid- or distal third of the esophagus and the
all bronchi in a lobe or a lung extending up to the right lower lobe (RLL) (43%), left lower lobe (LLL)
pleural surface and associated with focal lung (22%), or right main bronchus (RMB) (10%).39
hypoplasia. It has been hypothesized to be The clinical course of CBPFMs is generally
secondary to incomplete branching of the devel- insidious, including cough, recurrent pneumonia,
oping bronchial tree.26 and hemoptysis owing to chronic bronchopulmo-
nary infection.27,32,39,40 Episodes of choking
COMMUNICATING BRONCHOPULMONARY when swallowing liquids or the presence of food
FOREGUT MALFORMATIONSçESOPHAGEAL particles in sputum are suggestive and reported
BRONCHUS in 65% of patients.32,38–40 Nevertheless, the dia-
gnosis is not made before adulthood in approxi-
Communicating bronchopulmonary foregut mal- mately 75% of cases.39,40 Various hypotheses
formations (CBPFMs) are uncommon anomalies concerning the late diagnosis have been pro-
characterized by a patent congenital communica- posed, including a late onset or intermittence of
tion between a portion of respiratory tract on one symptoms or mild complaints leading to delayed
side and the esophagus or stomach on the other investigation only after the development of compli-
side.27–32 Concomitant congenital tracheobron- cations. The late appearance of symptoms may
chial stenoses have been reported.33,34 CBPFMs result from the presence of a membrane or valve
can also occur in combination with other con- within the fistulous tract that subsequently
genital anomalies involving the pulmonary and ruptures or becomes incompetent,29,39 the oblique
systemic vascular systems, diaphragm, upper upwards course of the fistula,40 or the closure or
gastrointestinal tract, and rib and vertebrae.28,30,35 contraction of the fistula during swallowing.29,40
A classification of CBPFMs into four groups has Diagnosis is usually made by barium esophagog-
been proposed by Srikanth based on anatomic raphy together with bronchial and esophageal
features and how the lung bud joins the esoph- endoscopy.7,30,36,40,41 In addition to the fistulous
agus.31 The first group comprises an esophageal tract, CT may demonstrate bronchiectasis, rudi-
atresia, with the distal gastroesophageal tract mentary lung tissue, and zones of consolidation
originating from the trachea, a condition generally and fibrosis in the area ‘‘ventilated’’ by the anom-
readily diagnosed in neonates.5,30,33 One lung, alous bronchus and may also map vascular supply
lobe, or segment then arises from the distal esoph- and drainage.7,32,42 Furthermore, three-dimen-
agus. The three other groups encompass commu- sional CT esophagography may help to evidence
nicating malformations without esophageal atresia the communication and display complex anatomic
and with variable blood supply. Malformations in features.5,41,43 The differential diagnosis includes
the second and the third groups are the most pulmonary sequestration, congenital cystic ad-
frequent, being reported in 33% and 46% of enomatoid malformation (CCAM), and iatrogenic,
patients presenting with CBPFMS, respectively.31 inflammatory, or neoplastic fistulas.32 Treatment
In the second group, one whole lung originates consists of either resection of the esophageal
from the esophagus and is generally termed an lung, or lobe, or anastomosis of the esophageal
esophageal lung or accessory lung.27,35,36 Ninety- lung with the normal tracheobronchial tree when
five percent of esophageal lungs are right sided.31 there is no severe broncho-parenchymal lesion
The ipsilateral main bronchus is absent, resulting and the vascular supply is normal.36
in complete absence of ventilatory function of the
involved lung that is hypoplastic and atelectated.34
CONGENITAL OBSTRUCTION OR COMPRESSION
Malformations in the third group are characterized
Tracheal Atresia
by a single lobe or segment arising from the
esophagus or the stomach.30 The bronchus origi- Tracheal atresia is an exceptional anomaly in
nating from the gastrointestinal tract is classically which a segment of the trachea, generally prox-
called an ‘‘esophageal bronchus’’ (Fig. 2).32,36 imal, is missing.9 The anomaly is lethal except
Exceptional bilateral esophageal bronchi have when associated with a tracheo-esophageal
been reported.27,37 In the fourth group, there is fistula, allowing esophageal intubation and subse-
a simple communication between a portion of quent surgical reconstruction.9,44 Three types of
206 Desir & Ghaye
Fig. 2. Esophageal bronchus (A–E). Chest X-ray and CT show lung consolidation on the right side. CT shows intra-
pulmonary contrast after esophagram. Esophagram shows aberrant bronchus arising from the esophagus
(arrows). Three-dimensional volume rendered image shows esophageal bronchus (arrow) and esophageal lobe
(arrowheads). (Courtesy of Woo Sun Kim, MD, Seoul, Korea).
tracheal atresia have been described depending lobe.58 On the other hand, when fissures are
on the tracheobronchial segment that communi- incomplete, collateral ventilation may be respon-
cates with esophagus, with the carina being the sible for huge dilatation of lung parenchyma distal
most frequent (60%) site of communication.9,20,45 to the lobar atresia and severe compression of
Prenatal diagnosis of such malformations has normal lung tissue.51 Systemic arterial supply to
virtually never been reported, and CT is helpful in the anomalous area simulating a pulmonary
demonstrating their complex anatomy before sequestration has been reported.59 MR imaging
possible surgery.9 can demonstrate the bronchocele, showing high
signal intensities on T1- and T2-weighted images
in 86% of patients.50 Prenatal identification by
Bronchial Atresia
sonography and MR imaging has been an impor-
First described by Ramsay and Byron in 1953, tant contributor to the marked increase of bron-
bronchial atresia results from a focal and juxtahilar chial atresia in the last decade.51
interruption of a segmental (more rarely lobar or Most patients with bronchial atresia are asymp-
subsegmental) bronchus, associated with normal tomatic, but dyspnea, pain, recurrent pneumonia
development of the distal airways.46–51 The left (up to 20% of patients) or pneumothorax, hemop-
upper lobe (LUL) is most commonly involved (two tysis, and asthma have been reported.47–49,60 The
thirds of patients), particularly the apicoposterior diagnosis is incidental in approximately 50% to
segmental bronchus. The other lobes are more 60% of cases, mostly in men during the second
uncommonly affected, with the RUL, LLL, middle or third decade.6,47,48,50,53 When diagnosed in
lobe (ML), and RLL in descending order.6,47,48,52–54 young children, the clinical presentation may be
Involvement of multiple lung segments has rarely more severe, including cases of respiratory
been reported.48,55 distress.47,51,55 Treatment is usually conservative,
The origin of bronchial atresia is not known, but with surgery only indicated when major clinical
most authors suggest that it is congenital. Two symptoms are present.48–51,55,60
main hypotheses have been proposed. The first The differential diagnosis of the bronchocele-
sug-gests an ischemic insult to a focal portion of hyperlucency syndrome includes an acquired bron-
the bronchial wall, whereas the second suggests a chocele, such as in association with bronchiectasis
disconnection from the bronchial bud of a distal or allergic bronchopulmonary aspergillosis, or may
group of dividing bronchial cells. The separated be secondary to bronchial obstruction by tumor,
cell mass then continues to divide normally, result- foreign body, external compression, or broncholi-
ing in the normal branching pattern distal to thiasis. Hyperlucency can also suggest congenital
atresia.47,48,54,56 lobar emphysema, CCAM, bronchogenic cyst,
The bronchi distal to the site of atresia become pulmonary sequestration, or acquired diseases as
filled with mucus not removable by ciliary action the MacLeod syndrome.25,57 Some of those malfor-
and are progressively dilated, forming a broncho- mations may also be associated with bronchial
cele (Fig. 3). Generally, no connection is found atresia.48,51,54,61 Fiberoptic bronchoscopy is usually
between the bronchocele and the proximal airway, normal in bronchial atresia, although sometimes
but a fibrous strand may sometimes be a blind bronchus may be identified.49,55
present.47,48,57 The alveoli distal to the atresia
are ventilated through collateral pathways and
Tracheal and Bronchial Stenosis
show features of air trapping, resulting in focal
hyperinflation.6,47,50,53,57 Congenital tracheobronchial stenosis corresponds
Radiographic features may be highly suggestive to a more than 50% reduction of the luminal diam-
of the diagnosis, consisting of central opacities eter.44 Primary tracheobronchial stenosis can result
that may show an air-fluid level surrounded by from various anomalies of the tracheobronchial wall
a systematized hyperlucency due to oligemia and or from an intraluminal diaphragm.10,62 Dispropor-
air trapping.47,48,50 Bronchoceles and hyperinfla- tionate development of the cartilage relative to the
tion are seen together only in 57% to 83% of membranous part may result in absent, hypoplastic,
cases.47,48,50,52,54 CT is the most sensitive method near-complete, or complete cartilaginous rings, or
for showing the tubular, ramified, or nodular bron- less often in other complex patterns of cartilaginous
chocele and the distal areas of oligemia and air malformations responsible for stenosis or mala-
trapping, which can be more clearly depicted at cia.44,62,63 Dynamic MDCT performed during inspi-
expiratory CT.22,25,49,50,54 In lobar atresia with ration and expiration is helpful to differentiate
complete fissure, hyperinflation can be missing stenosis and malacia.12 Primary tracheal stenoses
due to the absence of collateral pathways of venti- are divided into three types. The first type (30%)
lation and be replaced by atelectasis or a cystic corresponds to a diffuse stenosis of the trachea,
208 Desir & Ghaye
Fig. 3. Bronchial atresia. (A–E) Asymptomatic atresia of the anterior segmental bronchus of the left upper lobe
(arrowheads). Note the large central bronchocele (arrows) and localized air-trapping distal to occlusion. Fiberop-
tic bronchoscopy was normal. The patient was treated conservatively.
the second type (20%) to a ‘‘funnel-like’’ or progres- ECTOPIC OR SUPERNUMERARY LUNG BUDS
sive stenosis of various lengths and locations, and
the third type (50%) to a segmental stenosis.20,53 Noncommunicating bronchopulmonary foregut
Long-segment tracheobronchial stenosis due to malformations, including among others broncho-
complete cartilaginous rings is commonly found in genic cysts, result from abnormal budding of the
patients with a sling of left PA (SLPA) (ring-sling developing tracheobronchial tree. Pulmonary
complex) and represents a factor of worse prog- sequestration, CCAM, and congenital lobar
nosis even after surgery.64–66 emphysema are diseases of lung tissue.23,25,26
Secondary tracheobronchial stenoses are Those anomalies are beyond the scope of this
related to tracheal compression of various causes, article and are not discussed further herein.
particularly vascular malformations including
vascular rings, a dilated or displaced innominate Tracheobronchial Diverticulum
artery, SLPA, cardiac disease, and mediastinal Congenital diverticula are rare and considered to
masses.10,44,62,67 Tracheobronchial stenoses are be remnants of an aborted division of the primary
also reported in patients with congenital lobar lung bud.69 They are more frequently reported at
emphysema and congenital pulmonary venolobar the level of the trachea than at the bronchi. A
syndrome.23,24 tracheal bronchus (TB) and accessory cardiac
bronchus (ACB) may present as divertic-
Tracheal Dilatation
ulum.3,69,70 Tracheal congenital diverticula usually
Congenital tracheobronchomegaly, also called arise on the right side, 4 or 5 cm below the true
Mounier-Kuhn syndrome or mega trachea, is char- vocal cords or a few centimeters above the carina.
acterized by an intrinsic weakness or flaccidity of They are directed obliquely downward and filled
the wall of the trachea and central bronchi leading with air or mucus (Fig. 4).69,71,72 Histologically,
to tracheobronchial dilatation at inspiration and congenital tracheal diverticula are composed of
collapse at expiration and to formation of normal tracheal elements such as muscle or carti-
diverticula.68 lage.69 Associated congenital tracheo-esophageal
Fig. 4. Congenital diverticulum. (A–C) Blind diverticulum filled with air arising from the posterolateral aspect of
the right tracheal wall and directing obliquely downwards (arrows). According to its location, it may correspond
to a blind tracheal bronchus. (D–F) Blind diverticulum arising from inferior part of the lateral wall of the inter-
mediate bronchus (arrows).
210 Desir & Ghaye
fistula, tracheal stenosis, and CCAM have been pharyngocele, Zenker’s diverticulum, lymphoepi-
reported.69,71,73 thelial cyst, bronchogenic cyst, and apical lung
The main differential diagnoses of congenital hernia or bulla.74,75
tracheobronchial diverticula are acquired diver- Acquired diverticula correspond to simple
ticula of various forms, including a tracheocele evaginations of the mucosa through a weak point
and air-filled hypertrophic adenoid recess (Fig. 5). located laterally between the cartilaginous rings
Paratracheobronchial structures containing air or posterolaterally through the trachealis or bron-
should also be differentiated from laryngocele, chialis muscle. They are devoid of smooth
Fig. 5. Congenital diverticulum, differential diagnosis. (A–C) Tracheocele originating from the posterolateral
aspect of the trachea at the level of T2. Note the small communication between the diverticulum and the trachea
on axial minimum intensity projection reformat (arrowhead). (D) Multiple adenoid recesses filled with air origi-
nating from main, lobar, or segmental bronchi (arrowheads). (E–F) Probably acquired diverticulum originating
from the left lower lobe bronchus (arrow). It mimics a left-sided blind accessory cardiac bronchus, but no cartilage
was demonstrated inside its wall at fiberoptic bronchoscopy. (G) Acquired bronchial fistula due to tuberculosis
lymphadenopathy. Note small irregularities in the wall contour of the fistulous structure.
muscle or cartilage.71,76 When developing post- acquired diverticula.75 When developing laterally,
erolaterally, they are almost invariably located acquired diverticula can occur on both sides,
on the right side at the level of the thoracic inlet mainly at the bronchial level. These 1- to 3-mm
and are termed tracheoceles.74,76,77 Tracheo- outpouchings may be single or multiple, as
celes are generally single and larger than other frequently seen in association with chronic
Fig. 6. Right tracheal bronchi. Volume-rendering CT bronchography showing the three typical locations of
tracheal bronchi (arrows). All tracheal bronchi are displaced and pre-eparterial type. (A) Tracheal bronchus orig-
inating from the right main bronchus, also called double-right superior lobe bronchus in this location. The
tracheal bronchus corresponds to a displaced superior segmental bronchus (B1) of the right upper lobe. (B)
Tracheal bronchus originating from the carina, also corresponding to a displaced B1. (C) Tracheal bronchus orig-
inating from the trachea, also called ‘‘pig bronchus’’ in this location, which is the most caricatural but less
common presentation of a tracheal bronchus. The tracheal bronchus gives B1 (superior segmental bronchus)
and B2 (anterior segmental bronchus). B3 (posterior segmental bronchus) is displaced downward on the interme-
diate bronchus (arrowhead).
212 Desir & Ghaye
obstructive pulmonary disease, or even as compression from a tracheal congenital divertic-

a diverticulosis in Mounier-Kuhn disease.77,78 ulum has been reported.72 Ineffective ventilation
Other acquired diverticula may be secondary to or pneumomediastinum secondary to accidental
traction phenomena, usually in relation with ad- perforation of a diverticulum after tracheal intuba-
enopathy or pulmonary fibrosis. Considering the tion has also been described.69,72,75
shape of the mouth, number, or size of diver-
ticula is not sufficient to differentiate congenital
from acquired forms.72,75,79 The presence of Tracheal Bronchus
cartilage may suggest a congenital origin. Never- The TB was first described by Sandifort in 1785 as
theless, it remains difficult to demonstrate these a RUL bronchus originating from the trachea.3
features with CT.72 Although anatomically incorrect, these defects
Congenital diverticula are generally asymptom- encompassed a variety of bronchial anomalies
atic but sometimes present with nonspecific symp- originating from the trachea or the main bronchi
toms related to any form of inflamed or infected and directed to the upper lobes territory.80 An
diverticula, such as chronic cough, hemoptysis, incidence of 0.1% to 2% for right-sided TB and
dyspnea, and stridor, and as repetitive episodes of 0.3% to 1% for left-sided TB was reported in bron-
bronchopulmonary infections.69,72,75 A case of chographic, bronchoscopic, or CT series.80–82
recurrent laryngeal nerve paralysis secondary to Contrary to chest radiography, which
Fig. 7. Displaced and supernumerary right tracheal bronchi. (A–D) Pulmonary infection due to Streptococcus
pneumonia in the territory ventilated by a supernumerary tracheal bronchus (arrow) originating from the
trachea (pre-eparterial). The right upper lobe bronchus is displaced on the carina and gives the three modal
segmental bronchi. Partial anomalous venous return of right upper pulmonary vein into the superior vena
cava is also present (not shown).
demonstrates TB in less than 20% of the patients, supernumerary.’’70 Bronchial anomalies affecting
chest CT easily diagnoses TB.15,82,83 the upper lobes are seven times more frequent
Because in a series of 35 TBs, only 8 originated on the right side.3 The displaced right pre-eparte-
from the trachea (23%), 3 from the carina (9%), rial bronchus is the most common type (58%). Dis-
and 24 from more distal bronchi (68%), we use placed bronchi ventilate predominantly the apical
a modified nomenclature from Boyden84 and Ku- segment on the right and the apicoposterior
bik3 to clarify the classification of aberrant bronchi segment on the left.15
directed to the upper lobes.15 The normal RUL A true TB should designate any bronchus origi-
bronchus is termed eparterial because it arises nating from the trachea, usually within 2 cm and
cranial to the right PA. The normal LUL bronchus up to 6 cm of the carina.14,82,87 When the entire
is termed hyparterial because it arises below the RUL bronchus is displaced on the trachea, it is
left PA. An anomalous bronchus arising proximal called a ‘‘pig bronchus’’ or ‘‘bronchus suis;’’ it
to the origin of the upper lobe bronchus is termed has a reported frequency of 0.2% (see
pre-eparterial on the right side (Figs. 6–8) and Fig. 6C).1,82,83 The angle range of a TB with the
eparterial or pre-hyparterial on the left side trachea is wide and ranges from 22 to 108 degrees
(Fig. 9). An aberrant bronchus originating distal (mean, 73 degrees).82 Occasionally, a TB may
to the origin of the upper lobe bronchus is termed ventilate an area of pulmonary tissue separated
post-eparterial on the right side and post-hyparte- by its own fissure, which is termed a tracheal
rial on the left side. Double right-sided TBs have lobe. An incomplete TB may present as a divertic-
been reported.80,85 Bilateral TBs are found in 6% ulum at bronchoscopy (see Fig. 4A–C).88 Vascu-
to 9% of cases (Fig. 10).15,82,86 larization is usually normal for the territory
A TB is termed supernumerary when it coexists ventilated by the anomalous bronchus.
with a normal type of branching of the upper Although they are usually asymptomatic, recur-
lobe bronchus (23%) and displaced when, in addi- rent local infections, cough, stridor, acute respir-
tion to the aberrant bronchus, one branch of the atory distress (especially in children), and
upper lobe bronchus is missing (77%) (see hemoptysis can occur if drainage is impaired
Fig. 7). The displaced type is more frequent than (narrowing at the TB origin) or if the TB is asso-
the supernumerary type.3,15 As Foster-Carter ciated with other abnormalities (see Figs. 7 and
stated, ‘‘unless there is a clear evidence that all 8).80,82,83,85,87,89,90 Clinical symptoms are more
the normal bronchi are present, as well as the frequent in a left-sided TB and in supernumerary
abnormal bronchus, it is always possible that the TB.80,81 RUL atelectasis due to endotracheal
latter is merely a normal branch arising in an intubation and even severe hypoxia secondary
abnormal position (displaced) rather than a true to TB intubation during anesthesia have been
Fig. 8. Tuberculosis and displaced right tracheal bronchus. (A–B) Post primary tuberculosis in a territory ventilated
by a right tracheal bronchus (B1) arising from right main bronchus (pre-eparterial). Bronchoalveolar lavage was
directed into the anomalous bronchus and showed multiresistant mycobacterium tuberculosis that required
surgical treatment.
214 Desir & Ghaye
Fig. 9. Left tracheal bronchus. (A–D) Left tracheal bronchus (arrow) corresponding to a displaced apicoposterior
bronchus in an eparterial position. The right major fissure (arrowheads) is located between the anterior and api-
coposterior segments, resulting in inclusion of the latter segment in the left lower lobe. The level of the left
pulmonary artery is indicated by a star.
reported.82,91,92 Bronchiectasis, atelectasis, focal Accessory Cardiac Bronchus

emphysema (particularly of the LUL), and cystic
The ACB was described by Brock in 1946 as the
lung malformations may coexist.13,80,87,88
only true supernumerary anomalous bronchus.94
Despite the lack of evidence that TB is more
The reported incidence of ACBs is 0.07% to
susceptible to malignancy, various types of
0.5%.15,94–96 The ACB originates from the inner
bronchial tumor in a TB have been described
wall of the intermediate bronchus (IB) in 86% of
as case reports.93 Associated anomalies
cases and from the RMB in 14%, usually almost
included supernumerary lobes completely sepa-
opposite to the orifice of the RUL bronchus. Only
rated from adjacent lung by an accessory
one ACB originating from the left side has been re-
fissure, tracheal stenosis, partial anomalous
ported.97 The abnormal bronchus is demarcated
pulmonary venous return that may drain the
by a spur at its origin in more than 80% of cases.
abnormal territory, and, uncommonly, congenital
The bronchus has a diameter of 4 to 13.8 mm
heart or other organ diseases.15,82,87,89
(mean, 8.7 mm) and advances conically for 5 to
Fig. 10. Bilateral ‘‘tracheal’’ bronchi. (A–D) Right (long arrows) and left (arrows) tracheal bronchi originate from
right and left main bronchi, respectively. The right anomalous bronchus is pre-eparterial and ventilates the supe-
rior segment of the right upper lobe (B1). The left is eparterial, as it lies above the arch of the left pulmonary
artery (star), and ventilates the apicoposterior segment of the left upper lobe (B113). The minimum intensity
projection reformat is a frontal anterior view and the volume-rendered reformat is a left posterior oblique view.
25 mm (mean, 12 mm) in a caudal direction toward ACBs are generally asymptomatic and inciden-
the pericardium, hence the cardiac appella- tally discovered, but cough, hemoptysis, recurrent
tion.15,96 It is lined by normal bronchial mucosa infections, empyema, aspergilloma, or even tumor
and has cartilage within its wall, which distin- have been reported.88,95,96,100–102 Surgical resec-
guishes it from an acquired diverticulum, fistula, tion may be indicated in patients with recurrent
or adenoid recess. In normal anatomy, the only or severe symptoms.102 Associated anomalies
bronchus arising from the medial wall of the RMB, include a right- or left-sided TB, coexistence of
IB, or RLL bronchus is the paracardiac segmental two ACBs, and bronchiectasis.15,94,95,100,101
bronchus (B7). ACB is different from this bronchus
which arises from the RLL bronchus and does not
Bridging Bronchus
correspond to a proximal migration of this
structure.98 A BB is a rarely reported anomaly defined as an
Most ACBs are of the diverticulum type with anomalous bronchus crossing the midline of the
a blind extremity (71%), but some develop into mediastinum from one side to the other, hence
a series of small bronchioles which may end in a the term bridging. We found only 25 cases
vestigial or rudimentary bronchiolar parenchymal reported as such in the literature up to
tissue (50%), a ventilated lobulus located in the azy- 2008.11,62,64–67,103–111
goesophageal recess and demarcated from the Two types of BB have been described. In the
RLL by an anomalous fissure (29%), or rarely in classic form, the RLL (and sometimes the ML) is
cystic degeneration (Figs. 11 and 12).95,96,99,100 ventilated by a bronchus which arises from the
An abnormal PA directed to the lobulus has only medial aspect of the left main bronchus (LMB)
been reported once.96 and then ‘‘bridges’’ the mediastinum along its
216 Desir & Ghaye
Fig. 11. Accessory cardiac bronchus (diverticulum type). (A–C) The blind accessory cardiac bronchus (arrow) arises
from the medial aspect of the intermediate bronchus as a large-mouthed diverticulum. Curved MPR reformat
shows absence of vestigial lung tissue distal to the accessory cardiac bronchus. MPR, multiplanar reformatting.
course to the right lung (Fig. 13).103 In a first subtype The differential diagnosis between the bronchial
of classic BB reported by Holinger in 1950, the RMB pattern of a BB and TB may be difficult and remains
is either absent or present only as a short blind questionable in some cases (Fig. 14).112,113 Wells
tracheal diverticulum at the presumed level of the and colleagues65suggest that the pseudocarina
true carina. This pattern is usually associated with (bifurcation between a BB and LMB) has often
tracheal stenosis and right lung hypoplasia, with been misinterpreted as the true carina; therefore,
the right lung being ventilated only by the the RMB is misinterpreted as a TB or pre-eparterial
BB.62,106 In a second subtype of classic BB, the bronchus. Establishing the differential diagnosis
trachea is normal in structure and the true carina between a TB and BB is important because a BB
is located at the normal level, with the RMB venti- is associated with tracheal and bronchial stenoses
lating the RUL (and sometimes the due to cartilaginous anomalies, including complete
ML).11,103,106,107 Some authors give the denomina- rings, and frequent multiorgan malformations.11,104
tion of BB to a bronchus located between the true Typically, in normal anatomy, the carina is located
carina and the pseudocarina.108,110 A second at the level of the sixteenth to twentieth cartilagi-
type of BB described only once in the literature is nous rings or the level of T4–T5, and the normal
called an ‘‘anterior BB.’’ It corresponds to a bron- vertical angles for the RMB and LMB are approxi-
chus originating from the distal anterior aspect of mately 32 and 50 degrees, respectively.65 In
the carina and bridging the mediastinum to the a BB, the carina (uppermost dichotomy site of the
RLL.109 trachea) is located at a normal level, and the
Fig. 12. Accessory cardiac bronchus (with ventilated lobulus). (A–C) The accessory cardiac bronchus (arrow) origi-
nates from the medial wall of the intermediate bronchus and ventilates a small lobulus separated from the right
lower lobe by a fissure (long arrow).
pseudocarina, corresponding to the site of origin of hyperinflation that may cause clinical complica-
the BB, is located at the level of T5–T7. The angle is tions such as pneumothorax.103 In the past, inap-
reduced for the proximal LMB (25 degrees) and propriate surgery on the RUL was performed due
normal for the distal LMB below the pseudocarina. to misknowledge of a BB, because the overinfla-
The course of the BB is more horizontal (65 tion of the RUL may guide the clinician to a wrong
degrees) when compared with the RMB and IB. diagnosis of congenital lobar emphysema, bron-
This difference explains the ‘‘inverted T’’ bronchial chial atresia, or overinflation of any acquired
pattern seen in the majority of patients with BB, cause.105 A BB should also be differentiated from
with the angle between the BB and the distal LMB crossover lung segments and horseshoe
being approximately 115 degrees (see lung.114,115
Fig. 13).64,106,107 Furthermore, the pseudocarina Associated thoracic malformations include,
is situated substantially to the left of the midline of among others, vascular anomalies including partic-
the trachea. ularly SLPA,11,105–108 partial anomalous venous re-
In most cases, major symptoms consist of respi- turn,103,104 a bilobate right lung,103,104 and cardiac
ratory distress and repeated pulmonary infec- or extrathoracic malformations.66,103,104,106,108
tions.103–105,107 Pulmonary lobes ventilated by
a BB can show signs of atelectasis or a cystlike
Other Displaced Bronchi
appearance.103,104 The permanent or iterative
poor aeration of the BB lobes may be due to nar- Minor forms of proximal or distal displacement of
rowing of the trachea, LMB or BB, compression segmental or subsegmental bronchi in the same
of the LMB by an SLPA, or kinking or obstruction lobe are found in approximately 10% of individ-
of the BB secondary to positional chan- uals.83,84,94 More rarely, a displaced segmental
ges.103–105,108,110 The RUL (and ML) may show or subsegmental bronchus can originate from an
218 Desir & Ghaye
Fig. 13. Schematic drawings of classic bridging bronchus. (A–C) Normal anatomy (A), first subtype of bridging
bronchus with right upper lobe bronchus presenting as a diverticulum (B), and second subtype of bridging bron-
chus (C). The level of carina and pseudocarina are indicated. See text for explanations on bronchial angles. (Cour-
tesy of Catherine Beigelman, MD, Hopital la Pitie-Salpetriere, Paris, France.)
adjacent lobe.15 Such variants predominantly the so-called ‘‘axillary area’’ of the RUL
involve the upper lobes and particularly the right (Fig. 16).94,117,118 Less commonly, the posterior
side.116 The area ventilated by the displaced bron- segmental bronchus of the RUL is displaced
chus remains normal, but CT often demonstrates distally on the IB, which corresponds to a post-
a displaced or incomplete fissure between both hyparterial bronchus in the TB classification
territories (Fig. 15). The axillary bronchus is one (see Fig. 15). Occasional displacement or fusion
of the most frequently encountered (5%–16%) of lobar bronchi is also reported.3,84 A displaced
variants and usually corresponds to a displaced bronchus responsible for bronchial tangle or in-
subsegmental bronchus of the posterior or ante- terdigitation has been termed a braided
rior segmental bronchi of the RUL, ventilating bronchus.115
Fig. 14. Pseudo-bridging bronchus. (A–D) This case illustrates the borderline feature between a bridging and
tracheal bronchus. At a first glance, pro arguments for a bridging bronchus include the general morphologic
pattern of the tracheobronchial tree and particularly a stenosis of the ‘‘left main bronchus,’’ which may be found
in association with such anomalous bronchi. Cons are nevertheless numerous and among others include the
finding that the ‘‘carina’’ is located at the level of the aortic arch and ‘‘pseudocarina’’ at the level of T5-T6. The
angle of the pseudocarina is that of the true carina and is furthermore not displaced to the left of the midline
of the trachea; therefore, this case should be considered as a pseudo-bridging bronchus, namely, a real right
tracheal bronchus associated with stenosis of the distal trachea. (Courtesy of Catherine Beigelman, MD, Hôpital
la Pitié-Salpétrière, Paris, France).
MALFORMATIONS ASSOCIATED blood from the pulmonary veins. Situs anomalies

WITH ABNORMALITIES OF SITUS imply a disordered organ arrangement in the chest
and/or abdomen.
Situs solitus is defined as the modal arrangement
of organs and vessels within the body. The right- Reversal Right–Left, Situs Inversus
sided lung is trilobed, and the right atrium is the
Situs inversus is a congenital condition in which the
systemic one receiving blood from the vena
major visceral organs are reversed through the
cava. On the left side, the lung is bilobed, and
medial sagittal plane from their normal position. It
the left atrium is the pulmonary one receiving
is seen in 0.00005% to 0.01%119,120 of the
220 Desir & Ghaye
Fig. 15. Displaced bronchus. (A–B) The posterior segmental bronchus (B3) (arrows) of the right upper lobe origi-
nates from the lower posterior part of the intermediate bronchus. This anomaly corresponds to a post-eparterial
bronchus in the classification of the tracheal bronchi. The right upper lobe bronchus (long arrows) is in its usual
location and gives two segmental bronchi for the anterior (B2) and apical segment (B1). The upper part of the
right major fissure is posteriorly displaced and incomplete medially. The volume-rendering bronchography is
a right anterior oblique view.
population. Kartagener syndrome is found in wide number of possible combinations of malfor-

approximately 20% of patients with situs mations and the overlapping spectrum of findings
inversus.120 commonly seen, an individualized approach is
now preferred describing all malformations present
in a particular patient (ie, heterotaxic syndrome of
Situs Ambiguous, Heterotaxic Syndromes bilateral trilobed lungs, dextrocardia, asplenia,
Situs ambiguous or heterotaxia refers to anatomic and a left-sided inferior vena cava).120 When the
arrangements in which the major organs bronchial tree is involved, the patterns of airways
are dis-tributed abnormally within the chest and/ branching and the pulmonary lobation are identical
or abdomen. Traditionally, it was common to in the two lungs (isomerism), and the structure of
describe heterotaxic syndromes as the syndromes both atria is also similar.120 The thoracic and atrial
of asplenia (right isomerism or Ivemark syndrome) situs are almost invariably associated, which
or polysplenia (left isomerism). Because of the means, the atrial situs can be determined from
Fig.16. Axillary bronchus. (A–B) Trifurcation pattern of the right upper lobe bronchus in the axial plane with the
‘‘axillary bronchus’’ (AB) ventilating the axillary part of the lobe. Note a supernumerary right tracheal bronchus
arising from right main bronchus (arrow). B1, superior segmental bronchus; B2, anterior segmental bronchus; B3,
posterior segmental bronchus.
Fig. 17. Left isomerism. (A–B) The right lung is smaller than the left one and shows a mirror pattern of left bron-
chial division (left isomerism). The ratio of the left main bronchus/right main bronchus is 1.17:1. Right lobar
bronchi are slightly hypoplastic when compared with the left ones. Right lung also showed a pseudo-scimitar
syndrome (meandering vein, arrow).
bronchial anatomy.121,122 According to Partridge in right isomerism. The death rate is lower, being
and colleagues,123 a ratio between the length of approximately 60% in the first year of life. Left
the LMB and RMB less than 1.5:1 strongly suggests isomerism may be an incidental finding in an
isomerism, with the lowest ratio in situs solitus or in- asymptomatic adult, and cases of patients with
versus being 1.7:1. Nevertheless, exceptional bilateral bilobed lungs, normal abdominal situs,
discordances between bronchial and atrial situs and the absence of cardiac abnormality have
have been reported.123 been reported.119,120,125,126 Left isomerism with
In right isomerism or bilateral right-sidedness, bilobed lungs is present in 55% of cases of
the two lungs are trilobed with bilateral minor polysplenia.119
fissures. The main bronchi are short (<1 cm) and
are located superior to the ipsilateral main PA SUMMARY
on each side (eparterial bronchi).121 Both atria
are of the systemic type. A left-sided azygos Recognition of bronchial variants and anomalies by
lobe is suggestive of right isomerism.119,121 Right the radiologist is an important step in reporting on
isomerism is classically associated with asplenia, CT examinations of the chest because it has
bilateral systemic atria, a centrally located liver, numerous clinical implications. Precise description
and a stomach in indeterminate position (Ivemark is necessary for the pneumologist (for fiberoptic
syndrome). Cardiac anomalies are common (99% bronchoscopy, bronchoalveolar lavage, biopsy,
to 100%) and complex, and survival beyond 1 and endobronchial treatment), the chest surgeon
year of life is rare, ranging from less than 5% to (for lung resection and transplantation), and the
20%.119,120 Right isomerism may rarely exist anesthesiologist (for endotracheal tube placement).
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Imaging of Tumors
of the Trachea
and Central Bronchi
G.R. Ferretti, MD, PhDa,b,c,*, C. Bithigoffer, MDb,
C.A. Righini, MD, PhDb,c,d, F. Arbib, MDe,
S. Lantuejoul, MD, PhDb,c,f, A. Jankowski, MDb
KEYWORDS
CT Chest radiography Trachea
Main bronchi Malignant tumor Benign tumor
Primary tumors of the trachea and main bronchi CLINICAL PRESENTATION

are rare, accounting for 1% to 2% of all respiratory
tract tumors.1,2 In adults, most (60%–90%) of Centrally located tumors of the airways present
these tumors are malignant,3,4 whereas benign with a limited number of signs and symptoms
tumors represent the majority of lesions in chil- generally related to the obstruction of the airways
dren. Among those tumors in adults, squamous (eg, dyspnea, acute respiratory failure, wheezing,
cell carcinoma (SCC) and adenoid cystic carci- stridor, recurrent pneumonia, bronchiectasis, atel-
noma (ACC) are the most frequent, representing ectasis), whereas other symptoms are not specific
approximately 80% of all tumors of the trachea (eg, cough, expectoration, hemoptysis).3 Clinical
and main bronchi. Other tumors are less common, findings may erroneously suggest the presence of
arising from epithelial or mesenchymal tissue, and asthma or chronic obstructive lung disease.1 In
constitute a large list of heterogeneous benign and other cases, these tumors may be asymptomatic
malignant tumors (Table 1). Imaging plays a key and the lesions are discovered incidentally. The
role in depicting these tumors and assessing mean duration of symptoms is shorter in patients
tumor extent within the lumen, airway wall, and with malignant tumors (4 months) compared to
surrounding structures before treatment plan- patients with benign neoplasms (8 months), with
ning.2,5 Multidetector computed tomography the exception of patients with ACCs (12 months).3
(MDCT) has increased the quality of noninvasive
imaging with the recent introduction of isotropic MULTIDETECTOR COMPUTED TOMOGRAPHY
resolution and high quality two- and three-dimen- TECHNIQUE
sional postprocessing.6,7 Despite the high quality
of CT, there is considerable overlap in CT appear- MDCT technology has completely modified the
ance of most tumors; histologic evaluation is diagnostic approach and the noninvasive planning
needed in nearly all cases. of treatment in patients who present with central
a
Clinique Universitaire de Radiologie et Imagerie Médicale, CHU Grenoble, 38043 Grenoble cedex, France
b
Université J Fourier, Clinique Universitaire de Radiologie et Imagerie Médicale, CHU Grenoble, 38043 Greno-
ble cedex, France
c
INSERM U 823, Institut A Bonniot, la Tronche, France
d
Clinique ORL, CHU Grenoble, 38043 Grenoble cedex, France
e
Clinique de Pneumologie, CHU Grenoble, 38043 Grenoble cedex, France
f
Pôle de biologie-Département d’Anatomie et Cytologie Pathologiques, CHU Grenoble, 38043 Grenoble
cedex, France
* Corresponding author. Clinique universitaire de radiologie et imagerie médicale, CHU Grenoble, 38043
Grenoble cedex, France.
E-mail address: [email protected] (G.R. Ferretti).
Radiol Clin N Am 47 (2009) 227–241

doi:10.1016/j.rcl.2008.11.010
228 Ferretti et al
Table 1
Classification of tracheal tumors
Epithelial Neoplasms Mesenchymal Neoplasms

Surface epithelium
Malignant Malignant
SCC Soft-tissue sarcomas
Adenocarcinoma Chondrosarcoma
Large cell carcinoma Lymphomas
Neuroendocrine tumors Benign
Carcinoids (typical and atypical) Lipoma
Large cell neuroendocrine tumor Fibroma
Small cell carcinoma Fibromatosis
Benign Histiocytoma
Papilloma Hemangioma
Papillomatosis Hemangiopericytoma
Chemodectoma
Salivary glands Leiomyoma
Malignant Granular cell tumor
Adenoid cystic carcinoma Schwann cell tumors
Mucoepidermoid carcinoma Chondroma
Carcinoma Chondroblastoma
Benign Secondary tumors
Adenoma, pleiomorphic Invasion by adjacent malignancy (esophagus, thyroid,
larynx, lung); hematogenous metastases
Adenoma, mucous gland
Myoepithelioma
Onconcytoma
airway neoplasms.6 Currently, MDCT enables resolution of images in any reformatted plane is
acquisition of overlapped (30%–50%) thin section equivalent to the resolution in the transaxial plane,
(< 1 mm) images with voxels of almost cubic which allows for the creation of excellent two- and
dimensions (isotropic resolution) of the entire three-dimensional reformatted images.6
airways in a single apnea of few seconds. Fast
gantry rotation (< 0.5 seconds) increases the POSTPROCESSING OF CT DATA
temporal resolution of CT images. Contrast media
administration is usually needed to analyze the Postprocessing offers the opportunity to visualize
relationships of tumors of the central airways the trachea and bronchi along their main axis, on
with the surrounding anatomy and evaluate the external three-dimensional views, or on internal
contrast enhancement of the tumor. We typically bronchoscopic images.8–11 Multiplanar reforma-
inject 90 to 110 mL of nonionic contrast medium tions in sagittal, coronal, or oblique planes create
at a rate of 3 mL/s through a peripheral catheter. planar images that eliminate the known limitations
The natural high contrast between the airways of axial images, that is, the partial ability to detect
and their environment allows reduction of radiation subtle airway stenoses, the underestimation of
dose (80–120 kV, 70–160 mAs), particularly in chil- longitudinal extent of narrowing, the inadequate
dren and young adults. Data should be recon- representation of the airways oriented obliquely
structed with a high spatial resolution kernel and to axial plane, and the difficulty to display complex
a soft-tissue resolution kernel. Acquisition is three-dimensional anatomy of the airways.10
usually performed at full inspiration but can be per- Adding high-quality three-dimensional reconstruc-
formed at the end of expiration or during expiration tions—whether the representation of the air cast8
to study the dynamic of the airways and the impact or the endoluminal view using virtual bronchos-
of a tumor on the distal airways and lung paren- copy (VB)7,12—may even enhance the detection
chyma.8 With isotropic images, the spatial of localized or diffuse diseases.
Tumors of Trachea and Central Bronchi 229
Three-dimensional surface rendering selects the natural contrast between luminal air and soft-tissue
surface of the column of air contained in the airways density of lesions. Limitations of MDCT are well
by thresholding. Most initial data are lost in the final known: lesions smaller than 2 to 3 mm are not de-
reconstruction. Shading the surface creates the tected, whereas subtle irregularities of airway walls
impression of depth. Although this technique offers often result from prominent bronchial cartilage or
an overview of the disease and allows for better volume averaging. CT is not able to separate
understanding of extent of airway narrowing, it between mucosal and submucosal lesions.
suffers from limitations, mainly because of the
choice of threshold, which may artificially increase CLASSIFICATION OF MAIN AIRWAYS STENOSES
or decrease the size of airways.6 With volume-
rendering techniques, all the information contained Surgery is the optimal therapy for malignant and
in data acquisition is used in the final three-dimen- benign tumors of central airways.1,3,21 Radiation
sional images. External three-dimensional volume therapy is an option, as is endoluminal therapy.4
rendering of the airways shows the surface of the MDCT is useful for detecting, describing, and
airways and the adjacent anatomy, creating CT grading airway stenosis. Description of the lesion
bronchographic images.13 Such images enhance should mention the precise location of the tumor,
the detection of mild airway stenoses.14 the distance from the cricoid cartilage to the upper
Burke and colleagues15 showed an excellent limit of the tumor, the distance from the lower part
correlation between VB and conventional bronchos- of the lesion to the carina, and the craniocaudal
copy regarding the description of stenotic shape and length and its relationship to surrounding struc-
contour and stenosis-to-lumen ratio. Sensitivity of tures, including mediastinal vessels and esoph-
VB was 100% for detection of obstructive broncho- agus. Using the same acquisition, enlarged
genic carcinoma and 83% for endoluminal non- lymph nodes, pulmonary metastases, and postob-
obstructive neoplasms but 0% for mucosal structive complications can be described.
abnormalities.16 VB allows passing through high- In order to standardize the therapeutic approach
grade airway stenosis to assess distal airways, of airways stenoses, Freitag and colleagues22
which is impossible using classic bronchoscopy.17 proposed a new classification system. This classi-
VB can provide a road map for bronchoscopy and fication identified twp groups of airway stenoses
guide transbronchial biopsy.18 Main limitations of (structural and dynamic); each structural stenosis
VB include false-positive results related to mucus is described according to the following categories:
or coagulated blood pseudotumors and the inability
1. The type of the stenosis. Type I: exophitic intra-
to visualize the mucosa or perform biopsy.
luminal tumor or granulation tissue; type II:
Thin slab maximum intensity projection should
extrinsic compression; type III: distortion, kink-
not be used to detect or characterize airway
ing, bending, or buckling; type IV: scarring or
stenosis because selection of high-density voxels
shrinking.
artificially increases the severity of stenosis in
2. The degree of stenosis (0%, approximately 25%,
eliminating air-containing voxels and may even
approximately 50%, approximately 75%,
create artificial stenosis.8 On the other hand, thin
approximately 90%, complete occlusion).
slab minimum intensity projection may artificially
3. The location of the stenosis. Location I: upper
decrease the size of asymmetrical narrowing by
third of the trachea; location II: middle third of
specifically selecting air-containing voxels. Intralu-
the trachea; location III: lower third of the
minal growth of eccentric tumors is underesti-
trachea; location IV: right main bronchus; loca-
mated and may be completely ignored.8 Viewing
tion V: left main bronchus.
two- and three-dimensional images simulta-
4. The transition zone. The transition zone or the
neously on a workstation is beneficial for radiolo-
abruptness of stenosis is relevant for treatment
gists in increasing their diagnostic ability and
planning.
confidence in their findings.19 Two- and three-
dimensional images facilitate the communication Most tumors of the trachea and main bronchi
of information to colleagues who are not familiar are type 1. Radiologists may use this classification
with axial anatomy and improve preoperative plan- in their reports to simplify communication with in-
ning of surgery and bronchoscopy and postproce- terventional pulmonologists, otorhinolaryngolo-
dural noninvasive evaluation.20 gists, or thoracic surgeons.
CT BRONCHOSCOPIC CORRELATIONS MALIGNANT TUMORS

Lesions larger than 5 mm within the airways are Primary tracheal cancer is rare and its incidence
usually detected using CT because of the excellent is low compared to laryngeal or bronchial cancer.
230 Ferretti et al
It accounts for approximately 0.2% of malignant esophagus results in tracheoesophageal or bron-

neoplasms of the respiratory tract.21 It is more choesophageal fistulization.
common in men in their 60s with a history of
smoking. The prognosis of patients is poor, with ADENOID CYSTIC CARCINOMA
the 5-year survival rate being 5% to 35%.21 The
distribution of histologic types of neoplasms may ACC is a low-grade malignancy formerly named
vary regarding the origin of data (surgical versus cylindroma that is not associated with cigarette
medical series). In surgical series, SCC accounts smoking.21 It occurs in patients in their 40s; there
for approximately 50% of cases, ACC for approxi- is no sex predilection. ACC arises from the epithe-
mately 30%, and carcinoid for approximately lium of the glands lining the mucosa of the airways
10%.3 The national Danish Cancer registry report and is the most common tumor of the mucosal
(1978–1995) showed that SCC accounted for 63% glands. (It is also named sialadenoid tumor.)24 In
of tracheal cancers and ACC for only 7% of cases.23 the central airways, ACC has a propensity to infil-
trate the wall of the airways and spread along
submucosal and perineural planes.25 ACC grows
SQUAMOUS CELL CARCINOMA slowly and is rarely associated with regional lymph
node metastases. Distant metastases are rare and
SCC is the most frequent primary malignancy of often are diagnosed late after the ACC but may
the trachea, affecting predominantly men (sex present an intense fluorodeoxyglucose uptake.26
ratio 4:1) between age 50 and 60. SCC is strongly Despite nonspecific signs or symptoms, early
associated with cigarette smoking and conse- recognition of ACC may improve the surgical
quently with other smoking-related cancers of resectability and the prognosis of patients. Most
the upper and lower respiratory tract.1 Macro- of the tumors arise in the lower trachea or main-
scopically, SCC appears as a large mass within stem bronchi.
the central airways with either exophytic or ulcera- Unfortunately, chest radiography is often
tive component. It can be multifocal in approxi- considered unremarkable; however, an intralumi-
mately 10% of patients. Regional extent into the nal mass with smooth, irregular, or lobulated
esophagus or mainstem bronchi is frequent. The margins can be identified. CT shows a well-limited
tumor often spreads to regional lymph nodes. soft-tissue attenuating intraluminal mass that often
Chest radiography in patients with SCC is often infiltrates the airway wall (Fig. 2) and the
considered unremarkable, but retrospective anal- surrounding mediastinal fat.25 Other presentations
ysis usually shows focalized asymmetrical filling include circumferential wall thickening of the
defect within the tracheal lumen. CT demonstrates trachea creating localized stenosis (Fig. 3) or
a polypoid intraluminal mass of soft tissue density multifocal narrowing.
with irregular, smooth, or lobulated contours
(Fig. 1).2 The relationships of the tumor with the MUCOEPIDERMOID CARCINOMA
tracheal wall vary from localized eccentric pedun-
culated lesions to circumferential invasion. CT This rare tumor originates from the minor salivary
shows the extent to the adjacent anatomy. In glands lining the tracheobronchial tree.27 It usually
some patients, the extent of SCC to the occurs in patients younger than age 40 and affects
Fig.1. Squamous cell carcinoma in a 63-year-old man. (A) Axial CT at the level of the upper trachea shows a lobu-
lated, intraluminal mass extending to the cartilages of the trachea and to the mediastinal fat (arrow). (B) Coronal
reformation of CT data shows the longitudinal extent of the tumor and the severity of the asymmetrical narrow-
ing of the tracheal lumen (arrow).
Fig. 2. Adenoid cystic carcinoma in a 36-year-old patient with clinical history of asthma. (A) Axial CT at the level of
the aortic arch demonstrates severe narrowing of the lower trachea (arrow). (B) Sagittal reformation shows the
large pedicle of the tumor and extraluminal component of the tumor (arrow). (C) Virtual endoscopy demon-
strates the severity of the tracheal stenosis.
mainly the segmental bronchi,28 creating airway appreciated: mild enhancement was reported by
obstruction. It is even rarer than ACC, representing Kim and colleagues,28 whereas marked heteroge-
5% of sialadenoid tumors. Histologicaly, mucoepi- neous contrast enhancement was reported in four
dermoid carcinoma associates variable propor- of five cases in a small series by Ishizumi and
tions of squamous cells, mucus secreting cells, colleagues.29 The presence of abundant micro-
and intermediate cells with variable degree of vessels at histopathology correlated with the CT
mitoses, nuclear pleomorphism, and cellular findings.29 Lymphadenopathy is rare.
necrosis, defining low- or high-grade malignancy
(Fig. 4).28 CARCINOID TUMORS
Mucoepidermoid carcinoma shows the same
CT pattern as bronchial carcinoid tumors. It pres- Carcinoid tumors are rare thoracic neuroendocrine
ents as an endobronchial mass, often smoothly neoplasms that range from low-grade typical
oval or lobulated with its long axis parallel to that tumors to intermediate-grade atypical aggressive
of the airways containing the tumor, but contrast carcinoids and high-grade small cell carcinoma.30
enhancement is usually mild.28 Punctate calcifica- These tumors may secrete peptide hormones and
tions within the tumor were present in 6 of 12 neuroamines such as ACTH, serotonine, somato-
cases.28 CT contrast enhancement of mucoepi- statin, and bradikinin. Both sexes are affected in
dermoid carcinoma has been differently equal proportions, and patients are usually in their
232 Ferretti et al
most cases, carcinoids are endobronchial but

also can be partially encased in the bronchial
wall, creating an iceberg growth pattern, which is
nicely demonstrated using CT and multiplanar
reconstructions. In some cases (up to 60%), CT
demonstrates marked homogeneous early
contrast enhancement of an endobronchial nodule
that reflects the rich vascularity of the carcinoid
tumor (Fig. 5).31 This pattern is highly suggestive
of bronchial carcinoid tumor but is not always
present. Differential diagnosis of endobronchial
tumors with marked contrast enhancement on
early phase dynamic contrast-enhanced CT
detects even rarer neoplasms, such as glomus
tumor32 and hemangioma.33 Intratumoral calcifi-
cations are reported in approximately 25% of
cases (Fig. 6).31 In some cases, bronchial carci-
noid tumors produce complete obstruction of the
bronchial lumen and subsequent atelectasis of
the distal lung. In case of partial obstruction, expi-
ratory air trapping can be demonstrated.
Hilar or mediastinal enlargement of lymph nodes
can be present and is often related to inflammatory
reaction from recurrent pulmonary infection. Lymph
node metastasis of carcinoid tumor also may occur,
more frequently in atypical carcinoid tumors.
OTHER PRIMARY MALIGNANT NEOPLASMS

Other malignant neoplasms are listed in Table 1.
Their imaging presentation is not specific except
for chondrosarcoma, which shows foci of calcifi-
cations within a mass.
Fig. 3. Adenoid cystic carcinoma in a 50-year-old LYMPHOMA

woman. (A) Axial CT after contrast media administra-
tion shows circumferential narrowing caused by Primary malignant lymphoma of the trachea is
circumferential wall thickening of the upper trachea rare. It is usually related to the mucosa-associated
(arrow). (B) Three-dimensional reconstruction of the lymphoid tissue, a low-grade malignancy.34 Clin-
trachea demonstrates the longitudinal extent of the ical presentation is nonspecific. CT can reveal
tumor (arrows). focal tracheal narrowing caused by a solitary
mass (Fig. 7) or polypoid thickening of the tracheo-
40s, whereas carcinoid is the most frequent bron- bronchial wall caused by diffuse infiltration of the
chial tumor in childhood. No relation was found to submucosa.
cigarette smoking. Symptoms related to tracheo-
bronchial obstruction and hemoptysis are by far SECONDARY TRACHEAL MALIGNANCY
more frequent than symptoms caused by ectopic
secretion of hormones. In most cases (80%) these Direct invasion of the central airways by
tumors are centrally located within the airways, neoplasms of the thyroid, esophagus, lung, and
affecting the main, lobar, and segmental bronchi. larynx is much more frequent than hematogenous
Tracheal involvement is exceptional. Carcinoid metastases. In case of massive direct invasion, CT
tumors classically appear as smooth, polypoid, demonstrates the primary neoplasm and its exten-
cherry-red endobronchial masses at bronchos- sion by contiguity to the main airways. The signs
copy. Although histologic diagnosis can be made associated with tracheal invasion are evidence of
with endoscopic biopsies, there is a high risk of an endoluminal mass, the destruction of the carti-
hemoptysis. lage, or a tracheoesophageal or bronchoesopha-
Imaging presentation of centrally located carci- geal fistula (Fig. 8).35 Many cancers have the
noids is similar for typical and atypical cases. In potential to metastasize to the trachea and
Fig. 4. Mucoiepidermoid carcinoma in a 23-year-old man who presented with hemoptysis. (A) Anteroposterior
chest radiograph shows an endoluminal mass within the distal left mainstem bronchus (arrow). (B) Axial CT after
contrast enhancement shows a 12-mm polypoid endobronchial mass slightly enhanced (arrow).
bronchi, such as breast, colorectal, renal, lung, eccentric thickening of the airway wall (Fig. 9) or
ovarian, thyroid, uterine, and testicular and mela- soft-tissue density with contrast enhancement.
nomas and sarcomas. The incidence of tracheal Histopathologic examination of tracheal biopsy
metastases in nonpulmonary malignancies is specimens demonstrates the diagnostic of
highly variable, ranging from 0.44% to 50%, ac- metastasis.
cording to their definition.36 The overall incidence
of tracheal metastasis in surgically resected non– BENIGN TUMORS
small cell lung cancer was 0.44%; it was 0.77%
in SCC and 0.18% in adenocarcinoma.36 Endotra- Benign tumors of the central airways are rare and
cheal metastases of nonpulmonary cancers arise account for less than 2% of all lung neoplasms.4
with a mean recurrence interval of 50.4 to 65.3 They can be of epithelial, mesenchymal, neural,
months37 compared to a mean recurrence interval or composite origin.38 These tumors appear
of 25.8 months in patients who present with lung in clinical practice as the differential diagnosis
malignancies. Endotracheal or endobronchial of malignant lesions that are much more
metastases appear as endotracheal nodules or common. Although fiberoptic bronchoscopy
Fig. 5. Carcinoid tumor in a 14-year-old patient. (A) Unenhanced axial CT shows soft-tissue density (42 UH) nodule
within the B6 bronchus and distal atelectasis (arrow). (B) Contrast-enhanced CT shows marked contrast enhance-
ment of the nodule (174 UH) (arrow).
234 Ferretti et al
composed of a mixture of fat, cartilage, fibrous

tissue, and an epithelial component. Endobron-
chial hamartomas arise more commonly in
segmental bronchi;41 tracheal location is
unusual.42 Radiologically, hamartomas are round,
well-circumscribed lesions ranging from 0.5 to
3 cm in diameter. Demonstration of fat and calcifi-
cations within the lesion, which is considered diag-
nostic when present (Fig. 10),43 is facilitated by
using isotropic MDCT acquisitions with limited
volume averaging effect. The fatty content or the
calcifications may not be identified on CT,
however,42 and hamartoma appears as a nonspe-
cific soft tissue mass. Goodman and colleagues44
Fig. 6. Carcinoid tumor obstructing the right main
described a unique case of peripheral hamartoma
stem bronchus. The tumor contains foci of calcifica-
tions and has an iceberg growth pattern (arrow).
arising from peripheral lung tissue with proximal
extension and subsequent obstruction of the large
airways. Because the tumor is slow growing, it
identifieslesions within the airways, biopsies are may be responsible for bronchial obstruction and
often noncontributive because of severe inflam- irreversible damage of the underlying lung. Hamar-
matory reaction in the periphery of the tumor. tomas that develop within the airways require
Chest radiography is usually unremarkable.5 surgical treatment.
MDCT with thin collimation allows precise analysis
of tissue attenuation of the lesions, enhancing in
some cases the diagnostic capabilities of CT LIPOMA
(Box 1). CT usually shows masses that are
confined within the tracheobronchial lumen Lipomas (0.1% of benign lung tumors) are often
without evidence of invasion of surrounding struc- pedunculated and arise from the submucosal or
tures.39 Ko and colleagues39 recently reported on interstitial adipose tissue of the central airways.
a large series of 17 patients with pathologically Bronchial obstruction is frequent and responsible
proven benign tumors of the central airways. for atelectasis and postobstructive pneumonitis.
As for other slow-growing endobronchial tumors,
HAMARTOMA fiberoptic bronchoscopy is often nondiagnostic
because of the fibrous capsule surrounding the
Hamartomas represent 3% to 10% of intrathoracic lesions that may present with atypical inflamma-
hamartomas.40 These mesenchymal tumors are tory cells, leading to an incorrect diagnosis of
Fig. 7. Endobronchial mucosa-associated lymphoid tissue of the right main bronchus in an 80-year-old patient.
(A) Axial CT after IV contrast media administration shows a 1.6-cm soft-tissue density mass (arrow). (B) Coronal
oblique reformation of the tumor demonstrates the relationship with the bronchial tree.
Fig. 8. Tracheoesophageal fistula in a patient with esophageal cancer extending to the right main bronchus. CT is
acquired after contrast media opacification of the esophagus. (A) Axial CT shows the fistula between the esoph-
agus and the right main bronchus (arrow). (B) Coronal maximum intensity projection provides a CT esophago-
gram, which shows the narrowed esophagus, the fistula, and the dilated esophagus above the cancer.
chronic inflammation or even bronchogenic carci- bronchoscopy is the treatment of choice for endo-
noma.39 In those cases, CT is diagnostic when it luminal lipomas.
shows the fatty density of the lesion without any
calcification (Fig. 11).45,46 A correct preoperative
diagnosis may prevent lobectomy or pneumonec-
tomy, because laser resection by means of GRANULAR CELL TUMORS (ABRIKOSSOFF’S
TUMOR OR MYOBLASTOMA)
Granular cell tumors are uncommon benign
neoplasms of neuroectodermal origin that are
mainly located in the head and neck region.2
They are discovered in the fourth decade of life
and are more common in women. Pathologically,
these tumors show a characteristic appearance;
the cells are polymorphic and are embedded in
various amounts of connective or reticular tissues.
Mitoses are uncommon. The tumors are circum-
scribed but not encapsulated. At the level of
central airways, they appear as polypoid masses
of soft tissue density that may totally obstruct
bronchi. Differentiation of granular cell tumors
Box 1
CT pathologic correlation in central airways
neoplasms
Fat attenuation: lipoma, hamartochondroma
Fig. 9. Tracheobronchial metastasis of breast cancer in Calcification: hamartoma, chondroma, carcinoid
a 65-year-old woman. Coronal reformation shows
soft-tissue density infiltration of the wall of the distal Fat and calcification: hamartoma
trachea extending to the right main bronchus High contrast-enhanced tumor: carcinoid,
(arrows), responsible for a severe narrowing of the hemangioma, glomus tumor, fibroma
right main bronchus lumen.
236 Ferretti et al
Fig.10. Endobronchial hamartoma. (A) Axial CT without contrast shows an endoluminal nodule within the lumen
of left B6 (arrow). (B) Axial CT (mediastinal window) shows fat and small calcifications within the lesion (arrow).
(C) Bronchoscopy confirmed the diagnosis.
from carcinomas on the basis of imaging findings the trachea in approximately 5% of cases.48 Two
is not possible. third of patients are diagnosed before age 5. Papil-
lomas are cauliflower lesions that enlarge around
PAPILLOMAS a central fibrovascular core on the central airway
mucosal surface. These strictly endoluminal
Inflammatory papillomas or polyps are associated lesions appear on CT as single or multiple nodular
with chronic irritation of the airways and occur with irregularities of soft-tissue density, 0.5 to 1.5 mm in
endobronchial foreign bodies, broncholithiasis, or diameter in the tracheal or bronchial lumen
exposure to gases. Squamous cell papilloma is (Fig. 12).49 When extended to the lung paren-
one of the most common benign neoplasms of chyma (< 1% of cases of laryngotracheal papillo-
the central airways. It involves the larynx, bronchi, matosis), papillomatosis produces nodules that
and infrequently the trachea, predominantly in may cavitate and form thin-walled cavities.48
middle-aged men with a history of smoking. The Lesions of papillomatosis are central and periph-
tumor appears as a lobulated, well-limited eral but with a predominance within the posterior
polypoid nodule within the airways without fatty half of the thorax. These lesions may transform
content or calcification.47 into carcinoma and involve careful follow-up.
Laryngotracheal papillomatosis is caused by
infection with human papillomavirus. It can be CHONDROMA
contracted at birth or acquired through sexual
transmission. The lesion arises in the larynx and These rare benign cartilaginous tumors rarely
spreads by seeding from the upper airways to develop in the trachea. CT may show foci of
Fig.11. Endobronchial lipoma. (A) Axial CT shows -90 HU rounded fatty mass (arrow) obstructing right lower lobe
bronchus. VB (B) and real bronchoscopy (C) show excellent correlation.
calcifications within a sharply defined polypoid lesion administration. Definitive diagnosis is demon-
up to 3 cm in diameter.50 Imaging is unable to differ- strated by biopsy under bronchoscopy.
entiate a chondroma from a chondrosarcoma;
however. ADENOMA
Pedonculated tracheobronchial adenoma (adeno-
SCHWANNOMA matous polyp or mucous gland cystadenoma)
arises from bronchial mucous glands and is rare.
This neurogenic tumor is rare and may present at It appears as a solitary, spherical, soft-tissue
any age. It is composed of Schwann’s cells of density polypoid mass.52
nerve sheath. Most cases occur in adults. They
are typically unique encapsulated tumors attached LEIOMYOMA
to a nerve but contain no axon protruding within
the airways.51 On CT, schwannoma appears as This rare tumor accounts for approximately 2%
a well-limited mass of low tissue density before of surgically resected benign lung tumors. Most
contrast administration; the mass is homoge- leiomyomas are seen within the bronchi (70%
neously and strongly enhanced after contrast of cases); 30% are detected in the trachea.53
238 Ferretti et al
Fig. 12. Tracheobronchial papillomatosis in a 23-year-old man. (A) Axial CT shows a small, well-limited nodule
(arrow). (B) Virtual endoscopy demonstrates multiple elevations of the tracheal wall caused by papillomas. (C)
Axial CT at the level of lower lobes shows bilaterally distributed cysts. (Courtesy of D. Tack, MD, PhD, Baudour,
Belgium.)
An iceberg tumor growth pattern with a large ex- to the airway wall. Histologic diagnosis is manda-
traluminal component may be present on CT and tory. See Table 1 for more examples.
should contraindicate bronchoscopic resection
of these tumors. CT description of leiomyomas in
the airways was reported by Kim and colleagues53 DIFFERENTIAL DIAGNOSIS
in a series of 13 tumors: 2 were not depicted using Mucoid Pseudotumor
CT because of their small size; 11 (84%) were
identified using CT (9 as intraluminal nodules and Mucus is the most commonly encountered soft-
2 as iceberg tumors). Tumors are usually oval tissue mass within the airways, creating a mucoid
and are rarely lobulated or round. Obstructive pseudotumor. In most cases, mucoid pseudotu-
pneumonia, atelectasis, or mucus plugging was mor is easily identified because the lesion is of
present in 38% of cases. Calcifications are rare low tissue attenuation, does not enhance after
and are reported in less than 10% of cases. contrast media administration, may contain small
Most of these tumors appear as homogeneous air bubbles, is located in the dependant portions
nodules before contrast administration and of the airway, is not associated with disruption of
become slightly enhanced after IV contrast the cartilaginous rings of the trachea, and is mobile
injection. after coughing. In rare cases, thick mucus does
not contain air, adheres to the wall of the airway,
OTHER BENIGN NEOPLASMS is not mobile after coughing, and may be mistaken
for a real tumor (Fig. 13). Such false-positive
Other neoplasms usually appear as smooth, CT findings are diagnosed using fiberoptic
polypoid, noncalcified nodules or masses limited bronchoscopy.
Fig.13. Mucoid impaction mimicking endobronchial tumor. (A) Axial CT shows small, well-defined nodule within
the right middle lobe bronchus (arrow). (B) Bronchoscopy demonstrates the mucous nature of the lesion (arrow).
Focal Infection CT is more sensitive than bronchoscopy by identi-

fying intraluminal and peribronchial calcifications
Focal infection may be responsible for tumor-like
distal to inflammatory airways stenosis.55
lesions when it produces granulation or endolumi-
nal masses. Diagnosis is impossible with CT and Tracheopathia Osteoplastica
requires fiberoptic bronchoscopy and microbacte-
rial analysis. Specific pathogens include Myco- Tracheopathia osteoplastica occurs almost exclu-
bacterium tuberculosis, mucormycosis, Klebsiella sively in men over 50 years old; most patients are
rhinoscleromatis, and actinomycosis (Fig. 14).54 asymptomatic. This benign condition of unknown
origin is characterized by multiple submucosal
osteocartilaginous growths localized along the
Broncholithiasis
inner anterior and lateral walls of the trachea.56
Broncholithiasis may mimic a centrally located ob- CT shows multiple irregular sessile nodules, 1 to
tructive tumor on bronchoscopy. In these cases, 5 mm in diameter, that can be calcified protruding
Fig. 14. Endobronchial actinomycosis mimicking endobronchial tumor. (A) Contrast-enhanced CT at the level of
the right intermediate bronchus demonstrates complete occlusion of the bronchus (arrow). (B) Coronal reforma-
tion shows right lower lobe atelectasis. (Courtesy of D. Tack, MD, PhD, Baudour, Belgium.)
240 Ferretti et al
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15. Burke AJ, Vining DJ, McGuirt WF Jr, et al. Evaluation
lower airways secondary to the deposits of extra-
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16. Finkelstein SE, Summers RM, Nguyen DM, et al.
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Virtual bronchoscopy for evaluation of malignant
common than diffuse disease.57 CT shows
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23(6):957–8. 1998;8:352–4.
Nonneoplastic Tracheal
and Bronchial Stenoses
Philippe A. Grenier, MDa,*, Catherine Beigelman-Aubry, MDa,
Pierre-Yves Brillet, MD, PhDb
KEYWORDS
Tracheal stenosis Bronchial stenosis
Infectious tracheobronchitis Relapsing polychondritis
Wegener’s granulomatosis Tracheobronchial amyloidosis
Large airway diseases that may result in airway help surgeons and interventional pulmonologists
stenosis are neoplastic or nonneoplastic in origin. to select adequate procedures and determine
Tracheal and bronchial neoplasms are described response to treatment. The use of MDCT with thin
in another article in this issue. Nonneoplastic collimation (0.6–1.5 mm) over the entire chest
diseases of central airways that may lead to airway during a single breath hold at full inspiration allows
lumen narrowing include iatrogenic strictures, infec- acquisition of volumetric high resolution data sets.
tious tracheobronchitis, systemic disease, saber Reconstruction of axial overlapped thin slices
sheath deformity of the trachea, tracheobronchopa- permits multiplanar reformations of high quality
thia osteochrondroplastica, and broncholithiasis. and helps detect and characterize airway wall thick-
Systemic diseases include amyloidosis, inflamma- ening and calcifications. Generally, intravenous
tory bowel disease, relapsing polychondritis, contrast is not required for assessing nonneoplastic
sarcoidosis, and Wegener’s granulomatosis. Tra- airway stenoses; however, it is recommended in
cheobronchomalacia that induces airway narrowing cases in which there is a high likelihood of diseases
only during expiratory maneuver or cough is in the adjacent lymph nodes and for cases in which
described in another article. Clinical recognition of neoplastic airway disease may be suspected.8
tracheobronchial stenosis is notoriously difficult, Additional acquisition during dynamic expiration
especially early in its course. Clinical symptoms of using low dose may be helpful to detect coexisting
airway obstruction are late and nonspecific. Earlier tracheal or bronchomalacia.3,9
diagnosis is often possible with the advent of routine When assessing airway narrowing, it is impor-
CT imaging. tant to carefully assess the location and extent
Multidetector computed tomography (MDCT) is along the airways of the stenosis and characterize
the imaging modality of choice for assessing such the presence, distribution, and type of airway wall
diseases.1–4 It is important to be aware of the limi- thickening. Considering these factors in combina-
tations of the axial plane for assessing airway tion with associated features in the mediastinum,
stenosis. Subtle airway stenosis may be missed, hilum, and lung parenchyma and pertinent clinical
and the craniocaudal extent of disease may be un- and laboratory data should allow radiologists to
derestimated. By providing a continuous anatomic provide a limited number of differential diagnoses.
display of the airways, multiplanar reformations
along and perpendicular to the central axis of the
IATROGENIC STENOSIS
airways, and the three-dimensional reconstruction
images help circumvent these limitations.5–7 The most common iatrogenic airway stenoses are
Such multiplanar and three-dimensional images tracheal strictures secondary to intubation or
a
Hôpital Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris (APHP), Université Pierre et Marie Curie,
Service de Radiologie Polyvalente, Diagnostique et Interventionnelle, 47/83 boulevard de l’Hôpital, 75651 Paris
cedex 13, Paris, France
b
Hôpital Avicenne, Assistance Publique-Hôpitaux de Paris (APHP), Université Paris XIII, UPRES EA 2363, Service
de Radiologie, 125, route de Stalingrad, 93009 Bobigny, France
E-mail address: [email protected] (P. A. Grenier).
Radiol Clin N Am 47 (2009) 243–260

doi:10.1016/j.rcl.2008.11.011
244 Grenier et al
tracheostomy and bronchial anastomosis stenosis cases.14,15 Risk factors include infection, rejection,
after lung transplantation. Strictures of the trachea and immunosuppression. Affected patients typi-
are usually secondary to damage from a cuffed cally present with failure of anticipated improve-
endotracheal or tracheostomy tube. The preva- ments in symptoms in the first months after
lence of stenoses after endotracheal tube place- transplantation and decline in pulmonary function,
ment has decreased substantially to 1% since especially the FEV1. Severe stenosis may result in
the introduction of low pressure cuff endotracheal progressive airflow obstruction that is difficult to
tubes.10 Conversely, the prevalence of tracheal clinically differentiate from other causes of airflow
stenosis after longstanding tracheostomy tube limitation that may occur after lung transplanta-
placement remains high, with a rate of approxi- tion, particularly obliterative bronchiolitis.16 The
mately 30%.11 Infection, mechanical irritation, diagnosis is obtained at bronchoscopy and shows
steroid administration, use of positive pressure a focal cicatricial narrowing at the anastomotic
ventilation, and prolonged intubation may increase site. MDCT with multiplanar reformations and
the risk of stenosis occurrence. volume rendering techniques provides information
The principal site of stenosis after intubation is on the length of the stenosis and the patency of the
the subglottic region at the level of the endotra- distal airways, which is important in planning treat-
cheal balloon. Strictures are believed to occur ment. CT findings consist of focal narrowing at the
when the cuff pressure is high enough to impede bronchial anastomotic site.16 Virtual bronchos-
local blood circulation, with resultant ischemic copy assesses the grade of the stenosis with
necrosis of the mucosa. The most susceptible a good correlation with pulmonary function
portions of the trachea are those in which the tests.15 CT is also used to assess the patency of
mucosa overlies the cartilaginous rings, which the airways distal to high-grade stenosis not
subsequently soften and become fragmented traversable by the endoscope and assess the
with the risk of tracheomalacia. This phase is response to treatment in the follow-up. Treatment
subsequently followed by granulation formation consists of balloon dilatation followed by place-
and fibrosis. Postintubation stenosis is character- ment of a stent.
ized by eccentric or concentric tracheal wall thick-
ening and associated luminal narrowing. The TUBERCULOSIS
craniocaudal length usually ranges from 1.5 to
2.5 cm.12 Posttracheostomy stenosis occurs Tracheobronchial stenosis caused by tuberculosis
most commonly at the stoma site or less may occur in the setting of acute infection or as
commonly at the site where the tip of the tube late as 30 years after infection.14 Endobronchial
has impinged on the tracheal mucosa. It involves tuberculosis has been reported in 10% to 37% of
1.5 to 2.5 cm of tracheal wall.12,13 patients with pulmonary tuberculosis, and a vari-
Patients with mild iatrogenic stenosis may be able degree of stenosis has been reported to
initially asymptomatic. When present, symptoms occur in 90% of cases.14 Isolated tracheal involve-
are often delayed several weeks after extubation ment is likely a rare manifestation. Tuberculosis
and include dyspnea on exertion, stridor, or typically involves the distal trachea and proximal
wheezing. MDCT is the imaging modality of choice bronchi. Spread along peribronchial lymphatic
for detecting and characterizing tracheal stenoses. channels seems to play a more important role
On axial images, CT demonstrates eccentric or than direct airway spread by infected sputum.
concentric soft-tissue thickening with associated Evidence supporting this hypothesis is that in
luminal narrowing. Multiplanar and reformations many patients in whom the central airways biopsy
along the long axis of the trachea and volume is positive for tuberculosis, main tuberculous
rendering reconstruction help assess the location lesions are confined to the submucosa, with the
and extent of the stenosis (Fig. 1). On longitudinal mucosa either remaining intact or having only
images, the focal and circumferential luminal nar- shallow ulceration.17 Another mechanism is local
rowing may produce a characteristic ‘‘hourglass’’ extension from adjacent mediastinal tuberculosis
configuration (Fig. 2).8 Less commonly, tracheal or lymphadenitis. The presence of lymphadenopathy
bronchial stenosis may present as a thin membrane contiguous to the tuberculous lesions in the
or granulation tissue protruding into the airway trachea or main bronchi suggests that local exten-
lumen (Fig. 3). Interventional bronchoscopic (eg, sion is a probable mechanism (Fig. 4).18
balloon dilatation, stenting, or laser therapy) or Tuberculosis indirectly involves the bronchial
surgical procedures (eg, resection and anastomosis) wall, and the disease undergoes several evolutional
may be used to treat symptomatic tracheal stenosis. stages, including early formation of tubercles in the
After lung transplantation, bronchial anasto- submucosal layer, ulceration and necrosis of the
motic stenosis may occur in 10% to 15% of mucosal wall, and healing with a variable degree
Nonneoplastic Tracheal and Bronchial Stenoses 245
Fig.1. Postintubation tracheal stenosis. (A) Transverse scans targeted on the trachea and coronal oblique reformat
along the long axis and mainstem bronchi. (B) Descending virtual bronchoscopy and three-dimensional external
rendering of the tracheobronchial tree. Presence of a short, concentric, and symmetric stenosis of the tracheal
lumen at the level of the upper part of the intrathoracic portion of the trachea. Note on the transverse scan
(A) a regular, concentric wall thickening at the level of the stenosis. Note calcifications of the aortic arch and right
innominate artery.
of fibrosis or residual stenosis. Surprisingly, despite disease may coexist in one patient, the prognosis
active infection, prebronchoscopic sputum is worse at the stage of fibrotic disease than in
samples produce negative results.17 Endoscopic active disease. Not surprisingly, CT findings tend
evaluation may not be diagnostic. Endobronchial to reflect the stage of disease.18–20 Stenosis in
biopsy has proven to be nonspecific in one third active disease occurs by hyperplastic changes
of the cases.19 Although various stages of the and inflammatory edema. On CT scans, loss of
246 Grenier et al
bronchoscopy and biopsy to confirm the diag-

nosis, however. On follow-up after medical treat-
ment, CT findings of irregular airway narrowing,
obstruction, enlarged lymph nodes, and wall thick-
ening, which are observed at the active stage of
disease, are replaced by normal airways or
smooth luminal narrowing with nearly normal wall
thickness. In some patients, the airway disease
progresses and residual fibrostenosis occurs. In
fibrotic disease, usually there is no change in
airway narrowing on follow-up CT studies. This
disease form is resistant to medical treatment.
Radiologic or surgical intervention is usually
needed to restore the luminal patency.14
BRONCHIAL ANTHRACOFIBROSIS
Bronchial anthracofibrosis recently was defined as
Fig. 2. Postintubation tracheal stenosis. Coronal obli-
an inflammatory bronchial stenosis associated
que reformation along the long axis of the trachea.
Circumferential luminal narrowing extended along with anthracotic pigmentation on bronchoscopy
2 cm associated with soft-tissue thickening, which without a relevant history of pneumoconiosis or
produces the characteristic ‘‘hourglass configuration.’’ smoking. Most patients with bronchial anthracosis
have had no exposure to mining or industry and no
definition of the bronchial wall resulting from adja- history of smoking. A potential relationship
cent lymphadenopathy, irregular luminal narrowing between bronchial anthracosis and tuberculosis
with wall thickening, contrast enhancement of the has been suggested, however. It has been hypoth-
tracheal wall, and rim enhancement of enlarged esized that the black pigments in the bronchial
mediastinal node are common findings.18–20 Rarely walls are derived from anthracotic material in the
tuberculous nodes are observed to cavitate, which adjacent lymph nodes. The involved lymph nodes
results in communication with the adjacent airway may perforate into the adjacent bronchi, and
(see Fig. 4). Nodobronchial fistula is depicted by carbon particles in the lymph nodes may penetrate
the presence of gas in cavitated hila or mediastinal the bronchial wall as deep as the mucosa, result-
lymphadenopathy adjacent to the airway. Discrete ing in coloring of the bronchial mucosa. Subse-
visualization of the sinus tract between the bron- quently, healing with fibrotic response may occur
chial lumen and the hypertrophied cavitated lymph and result in bronchial narrowing or obstruction
node can help plan therapy. with anthracotic pigmentation.21,22
Stenosis in fibrotic disease occurs by fibroste- Most patients are elderly women who usually
nosis, and tuberculomas are usually absent in present with cough, sputum, and dyspnea. On
the diseased bronchial wall. On CT scans, smooth bronchoscopy, the right middle lobe bronchi is
narrowing of the tracheobronchial lumen with the most commonly involved site followed by the
minimal wall thickening is typically seen (Fig. 5). right upper, left upper, lingula division, right lower,
The bronchial stenosis is concentric with uniform and left lower lobe bronchi. Multiple site involve-
thickening of the bronchial wall and involvement ment may be seen in up to 50% of patients. On
of the long segment of the bronchus (> 3 cm).19 CT, a segmental collapse distal to the involved
Unlike active infection, which involves the main- bronchi is the most commonly reported finding,
stem bronchi equally, fibrotic tuberculosis has with the right middle lobe being the most
been reported to involve the left mainstem bron- frequently involved lobe. The other findings
chus more often. include smooth bronchial narrowing accompanied
CT findings of central airway tuberculosis are by thickening of the wall and enlarged mediastinal
nonspecific and need to be distinguished from or hilar lymph nodes adjacent to the involved
bronchogenic carcinoma affecting the central bronchi (Fig. 6). Calcified nodes adjacent to the
airways. The differential diagnosis from broncho- bronchi supplying the atelectatic lung are seen in
genic carcinoma can be made by the longer more than 50% of patients. The CT findings are
segment of involvement, circumferential luminal similar to those observed in bronchial tubercu-
narrowing, and absence of an intraluminal mass. losis. Bronchial biopsy is required to eliminate
CT scans must always be supplemented by malignancy.
Fig. 3. Iatrogenic stenosis of the left mainstem bronchus occurred after a selective intubation. Transverse scan (top
left), coronal oblique reformat of the tracheal lumen with minimum intensity projection (bottom left), and three-
dimensional external rendering reformation of the tracheobronchial tree (right). The traumatic lesion occurred
during intubation. Presence of nodular lesions along the inner surface of the left main bronchus (arrow) reflects
granulation tissue and concentric narrowing of the origin of the left main bronchus.
RHINOSCLEROMA strictures of the trachea and bronchi. Mediastinal

or hilar lymphadenopathy and postobstructive
Rhinoscleroma is a slowly progressive infectious consolidation also may be present.14 Antibiother-
granulomatous disease caused by Klebsiella rhi- apy generally results in improvement, but
noscleromatis, a capsulated gram-negative bacte- advanced cases with fibrotic stenosis may benefit
rium that is endemic in tropical and subtropical from interventional procedures.
areas.23,24 Typically involving the upper respiratory
tract, this organism also may involve the trachea
FUNGAL TRACHEOBRONCHITIS
and proximal bronchi. If left untreated, the infec-
tion progresses slowly over many years with alter- Acute tracheobronchitis caused by aspergillosis is
nating periods of remission and relapse. uncommon and is usually restricted to the central
Pathologically in the granulomatous phase, airways. It usually occurs in severely immunocom-
nodules and masses cause partial obstruction of promised individuals, especially persons with
the involved airways (pseudoepitheliomatous underlying malignancies or AIDS, or in individuals
hyperplasia). In the final sclerotic stage, the airway who have undergone bone marrow, lung, or heart
appears deformed, with stenosis developing transplantation.25 Histologically, there is evidence
secondary to fibrosis. The diagnosis is generally of respiratory epithelial ulceration and submucosal
established by biopsy or positive culture results. inflammation. CT reveals nonspecific multifocal or
CT findings include diffuse nodular thickening of diffuse tracheobronchial wall thickening, which
the proximal airway walls with luminal narrowing, results in either smooth or nodular luminal narrow-
nodularity of the tracheal mucosa, and concentric ing (Fig. 7).26,27 Bronchial wall necrosis may lead
248 Grenier et al
characterized by recurrent inflammation of the

cartilaginous structures of the nose, external ear,
peripheral joints, larynx, trachea, and bronchi.29
Relapsing polychondritis is likely immune-medi-
ated and considered to have an autoimmune path-
ogenesis. Although any age group may be
affected, the peak of incidence of the disease is
between the third and the sixth decades with
a slight predominance in women. Airway involve-
ment is present in up to 50% of patients and is
a major cause of morbidity and mortality.29,30
Rarely, it may occur as an isolated manifestation
of the disease.31 Airway involvement may be
asymptomatic in early stages, but most patients
Fig. 4. Transverse scan in a patient with active tubercu- with laryngotracheal involvement present with
losis. Nodobronchial fistula. There is direct communi- nonspecific respiratory tract symptoms, including
cation between the anterior aspect of the right cough, dyspnea, wheezing, aphonia, and
mainstem bronchus and the necrotic mediastinal hoarseness.30
lymphadenopathy (arrow). Multiple small, centrilobu-
The larynx and subglottic trachea are often the
lar, nodular opacities and nodules within the right
initial sites of involvement. As the disease prog-
upper lobe and, to a lesser extent, in the lower lobes
represent endobronchial spread of tuberculosis. resses, the distal trachea and bronchi may be
involved. The airway may be involved focally or
to bronchial rupture, and associated rupture of the diffusely. The distal bronchi may be involved to
adjacent pulmonary artery may lead to death.28 the level of the subsegmental bronchi. Pathologi-
cally, the disease is characterized by an acute
RELAPSING POLYCHONDRITIS inflammatory infiltrate in the cartilage and peri-
chondrial tissue. Airway inflammation may result
Relapsing polychondritis is an unusual multisyste- in luminal narrowing. Dissolution and fragmenta-
mic disease of unknown origin that is tion of the cartilage occur and may be followed
by fibrosis.32 In the late stages of the disease,
this fibrosis-induced contraction of the airway
may lead to severe luminal narrowing. Loss of
structural cartilaginous support also may result in
tracheobronchomalacia. The diagnosis of the
disease is made on clinical criteria according to
the lack of pathognomonic histologic or laboratory
findings. Michet and colleagues33 established
major (auricular, nose, and laryngotracheal
chondritis) and minor (ocular inflammation, hypoa-
cousia, vestibular damage, seronegative inflam-
matory arthritis) criteria. The presence of two
major criteria or one major criterion and two minor
criteria permits the diagnosis.
The most common CT pattern is a combina-
tion of increased airway wall attenuation in
association with smooth tracheal or bronchial
wall thickening that characteristically spares
the posterior membranous portion of the
Fig. 5. Posttuberculosis bronchial fibrotic stenosis. Ob- trachea (Fig. 8).12–14,34 The degree of increased
lique reformat along the long axis of the left upper attenuation may range from subtle to a finding
lobar bronchus. The bronchial stenosis visible at the
of frank calcification (see Fig. 8A,B).34 Narrow-
origin of the lingular bronchus is short and has
ing of airway lumen is more or less present (see
a nodular endoluminal appearance without any ex-
trabronchial soft tissue mass (arrow). There is a distor- Fig. 8C).35 In advanced disease, circumferential
tion of the airways distal to the bronchial stenosis. wall thickening may be seen (Fig. 9). Gross
(From Grenier PA. Imagerie thoracique de l’adulte. destruction of the cartilaginous rings associated
3rd edition. Paris: Flammarion Médecine-Sciences; with fibrotic stenosis may occur. Important
2006; with permission.) flaccidity of the airway wall may lead to
Fig. 6. Anthracofibrosis. Transverse nonenhanced CT scans. There is stenosis of the right upper lobar bronchus by
a soft-tissue mass that extents medially into the mediastinum. Note thickening of the posterior wall of the right
upper lobar and intermediate bronchi and narrowing and deformity of the lumen of the intermediate bronchus.
Enlargement of the precarinal and subcarinal lymph nodes is visible.
considerable collapse on expiratory CT images reconstruction may be used to provide long-term

(see Fig. 8D). Using dynamic expiratory CT, palliation.37
Lee and colleagues9 found malacia and expira-
tory air trapping in 13 and 17 of 18 patients, WEGENER’S GRANULOMATOSIS
respectively, with relapsing polychondritis.
Differential diagnosis is easy when CT images Wegener’s granulomatosis is a disease of
depict the presence of characteristic smooth unknown origin that is characterized by a necro-
thickening of the anterior and lateral walls of the tizing granulomatous vascularitis. Involvement of
trachea, and a diagnosis of relapsing polychondri- the large airways is a common manifestation of
tis can be made with a high degree of confidence. the disease. Its frequency was reported as 16%
Tracheobronchopathia osteoplastica, which also and 23% in two large series.38,39 Although most
spares the posterior membranous wall of the often unassociated with symptoms or a late mani-
trachea, is easily distinguished from relapsing pol- festation of well-established disease, tracheal or
ychondritis by the presence of nodules arising bronchial stenosis is occasionally responsible for
from the submucosa of the tracheal lumen and the initial presentation.40 Subglottic stenosis may
protruding into the airway lumen. occur in Wegener’s granulomatosis without other
Treatment is based on a combination of medica- evidence of pulmonary involvement. In one series,
tions, including corticosteroids, immunosuppres- approximately 50% of tracheal stenoses occurred
sive agents, and nonsteroidal anti-inflammatory independently of other features of active Wege-
drugs.29 Although these drugs may temporarily ner’s granulomatosis.38
decrease the severity of recurrences, disease Histologically, granulomatous inflammation and
usually progresses. CT may play a role in the vascularitis typical of the disorder can be seen in
follow-up to assess the response to therapy.36 the mucosa and submucosa in the early stage.
Tracheostomy, tracheal stenting, and tracheal Fibrosis is seen later. Endoscopic manifestations
250 Grenier et al
Fig. 7. Necrotizing aspergillosis of proximal airways in a young immunocompromised patient suffering from a
B-lymphoma treated by chemotherapy and bone marrow transplantation. Transverse scans through the proximal
airways. Diffuse and circumferential thickening of the tracheobronchial walls and soft tissue infiltration in the
mediastinum fat around the trachea and mainstem bronchi are visible. There is regular narrowing of the left
mainstem bronchus with a nodular appearance of the anterior inner surface of the left main bronchus (arrow).
include inflammatory tracheobronchial stenosis, have demonstrated the high frequency of airway
ulcerating tracheobronchitis, and tracheobron- abnormalities on the more distal airways. For
chial stenosis without an inflammatory compo- instance, Lee and colleagues45 reported that
nent. In a study of 77 patients with documented central airway abnormalities could be identified in
disease, Cordier and colleagues41 found that 30% of patients, whereas segmental and subseg-
55% of patients in whom fiberoptic bronchoscopy mental bronchial wall thickening with or without
was performed proved to have airway involve- luminal narrowing or obliteration was detectable in
ment. In another study by Daum and colleagues42 73% of patients. Bronchial stenosis may result in
that included 51 patients who underwent bron- distal collapse or consolidation of a lobe or lung.41
choscopy, airway abnormalities were found in Virtual bronchoscopy may help detect subtle
59%, including subglottic stenosis in 17%, ulcer- tracheal or bronchial stenosis. In a study of 18
ating tracheobronchitis in 60%, and tracheal or virtual bronchoscopic examinations performed in
bronchial stenosis in 13%. 11 patients with Wegener’s granulomatosis, 32 of
On CT, tracheal stenoses are most commonly 40 bronchoscopically visible stenoses, most in
subglottic (Fig. 10). In a study of ten patients the lobar and intermediate bronchi, were identified
with known tracheal involvement, Screaton and on virtual bronchoscopy by at least one reading
colleagues43 noted that 90% of lesions were sub- radiologist compared with only 22 on axial
glottic and identifiable as short segments of images.46 According to the high prevalence of
circumferential mucosal thickening. Usually subglottic stenoses, this area always should be
tracheal stenosis presents as smooth or irregular included in the imaging volume in patients with
circumferential narrowing approximately 2 to Wegener’s granulomatosis.8
4 cm long.43,44 CT shows abnormal intratracheal CT has proved valuable in follow-up of airway
soft tissue, which is often associated with thick- abnormalities. In the study by Lee and
ening and calcification of the tracheal ring. Carti- colleagues,45 20 patients had follow-up CT exam-
laginous erosion also may be seen. CT studies inations. There was total resolution of previously
Fig. 8. Relapsing polychondritis. (A) Transverse scan through the trachea and mainstem bronchi. (B) Coronal
oblique reformat along the proximal airways and three-dimensional external rendering of the tracheobronchial
air content. (C) Transverse scan through the trachea and main and lobar bronchi. (D) Transverse scan of the carina
at full inspiration (top) and during forced dynamic expiratory maneuver (bottom). There is diffuse and regular
thickening of the anterior and lateral walls of the trachea and thickening of the anterior wall of the main
and lobar bronchi. This wall thickening contains calcific deposits. Diffuse narrowing of the lumen of the main-
stem and lobar bronchi is present. Tracheobronchomalacia with complete collapse of the mainstem bronchi
during expiration was noted. (Fig. 8 continued on next page.)
252 Grenier et al
Fig. 8 (continued).
Fig. 9. Relapsing polychondritis in a patient with advanced disease. Transverse scan (left) and descending virtual
endoscopic views (right). A circumferential wall thickening is present with narrowing and deformity of the
tracheal and bronchial lumens.
identified airway abnormalities in 5 patients, lung Diffuse involvement is most common. It may
parenchyma and airway lesions improved with involve the larynx, trachea, main bronchi, and
partial disappearance in 12 patients, and 3 lobar or proximal segmental bronchi and often
patients demonstrated evidence of recurrent involves contiguous segments of the airway.
disease. Tracheal stenosis is often unresponsive Histologically the amyloid tends to be deposited
to systemic therapy, however, and local interven- initially in relation to tracheal gland acini and the
tion is favored. Airway lesions may occur in the walls of small blood vessels in the mucosa. As
course of therapy, with symptomatic airway the amount of disease increases, the glands
lesions occurring in the course of relapses.47 CT atrophy and the amyloid generates irregular pla-
may prove invaluable by demonstrating optimal ques and nodules in the mucosa that are usually
sites for tracheostomy and by defining the true multifocal,or less commonly forms a single mass-
extent of disease when bronchial narrowing like syndrome. The overlying mucosa is intact.
precludes complete bronchoscopic evaluation. Dystrophic calcification and ossification are
Virtual bronchoscopy is particularly appreciated frequently present at histologic examination.
in this setting.46 Park and colleagues48 described Affected patients are often asymptomatic for
MR findings of Wegener’s granulomatosis a long time before diagnosis, which suggests
stenosis. Abnormal soft tissue at the level of the that the disease progresses relatively slowly. In
stenosis is of intermediate signal on T1-weighted patients with proximal subglottic or laryngeal
images, high signal on T2-weighted images, and involvement, the disease manifests by hoarseness
enhances with contrast agent. or stridor, whereas patients who have distal
tracheal or bronchial involvement suffer from
TRACHEOBRONCHIAL AMYLOIDOSIS cough, wheezing, dyspnea, or hemoptysis.49,50 In
case of bronchial obstruction, patients may
Deposition of amyloid in the tracheal bronchi may present with fever caused by obstructive pneumo-
be seen in association with systemic amyloidosis nitis. Endoscopic examination of the disease
or as an isolated manifestation.49,50 Airway shows either submucosal plaques and nodules
involvement may be focal, multifocal, or diffuse. with a cobblestone appearance or a tumor-like
254 Grenier et al
Fig. 10. Wegener’s granulomatosis. Transverse scan over the upper part of the trachea (left) and coronal oblique
reformat along the long axis of the trachea targeted on the larynx and cervical part of the trachea (right). Note
short concentric stenosis of the subglottic area of the trachea (large arrow). Circumferential thickening of the
tracheal wall at the level of the stenosis is visible. Note the presence of two small ulcerations within the posterior
wall of the trachea (small arrows).
appearance or circumferential wall thickening. membrane of the trachea.50 Local lesions give
Endoscopic biopsies are diagnostic.51 rise to endoluminal masses (amyloidomas) that
On CT scans, amyloid results in circumferential may be radiologically indistinguishable from
tracheal or bronchial wall thickening caused by neoplasms.8
the submucosal deposition of nodules and pla- There have been rare reports of concurrent
ques that induces a luminal narrowing tracheobronchial amyloidosis and tracheobron-
(Fig. 11).12,49,50,52–55 There may be multiple chopathia osteoplastica.49,51 In most cases,
concentric or eccentric strictures. Mural calcifica- however, these entities prove pathologically
tions are prominent features that have to be distin- distinct. In patients with severe narrowing, the
guished from calcified lymph nodes. Some amyloid deposits may be removed by intermittent
patients with tracheobronchial amyloidosis have bronchoscopic resections using either forceps or
hilar or mediastinal calcified or noncalcified laser.57 Resection is not curative, however, and
enlarged lymph nodes.56 In patients with recurrent lesions often recur 6 to 12 months after treatment.
obstructive pneumonias, bronchiectasis also may Other options include stenting and radiation
be identified. Other patterns may be seen, such therapy.58,59 Tracheostomies may be required in
as eccentric strictures sparing the posterior case of subglottic involvement.
Fig.11. Tracheobronchial amyloidosis as seen on transverse scans on the trachea and proximal bronchi. Thickening
of the walls of the trachea is associated with tracheal lumen deformity. There is diffuse thickening of the bron-
chial walls containing calcific deposits. Luminal narrowing of the upper lobar and right intermediate bronchi and
occlusion of the lumen of the right middle and lower lobar bronchi is visible.
SARCOIDOSIS important, because endobronchial biopsy of an

abnormal site is likely to yield granulomas.
Involvement of the trachea is rare, and when it Obstruction of lobar or segmental bronchi may
occurs, it is usually associated with laryngeal occur as a result of airway wall fibrosis and granu-
involvement.60 The proximal and distal parts of lomas or peribronchial lymph node compression
the trachea may be affected, and the appearance
of the stenosis may be smooth, irregular and
nodular, or even mass-like. Bronchial involvement
is much more common as a manifestation of
sarcoidosis.61 It was reported in 65% of 60
patients with sarcoidosis in a study by Lenique
and colleagues62 using high resolution computed
tomography. The most common findings are
regular or nodular bronchial wall thickening of the
lobar, segmental, or subsegmental airways. The
thickening likely reflects the presence of granu-
lomas and fibrous tissue in the peribronchial inter-
stitium. This bronchial wall thickening may result in
smooth or irregular luminal narrowing, as was
observed in 23% of patients by Lenique and
colleagues (Fig. 12).62 The luminal narrowing
correlates with the presence of mucosal thick- Fig. 12. Sarcoidosis. Transverse scan targets the right
ening at bronchoscopy and presumably reflects upper lobe. Stenosis of the distal part of the right
prominent inflammation in this location. Recogni- upper lobar bronchus extending to the anterior
tion of these abnormalities may be diagnostically segmental bronchus is visible (arrow).
256 Grenier et al
and conglomerate fibrosis or some combination of interventional bronchoscopic techniques may be

these phenomena.63 Lobar or segmental atelec- considered.8
tasis remains an uncommon manifestation, occur-
ring in approximately 1% of cases of SABER SHEATH TRACHEA
sarcoidosis.64 Bronchial stenosis caused by
Sabear sheath trachea is a relatively uncommon
sarcoidosis may clear spontaneously or with steroid
deformity of the trachea characterized by reduc-
treatment.65 Mechanical dilatation or stenting may
tion in coronal diameter and elongation of the
be proposed in case of refractory stenosis.66,67
sagittal diameter. It is defined by a coronal diam-
eter equal to or less than one-half its sagittal diam-
INFLAMMATORY BOWEL DISEASE eter, measured at 1 cm above the top of the aortic
arch.71,72 This deformity affects only the intratho-
Chronic inflammatory bowel diseases, including
racic portion of the trachea, with abrupt widening
ulcerative colitis and Crohn’s disease, occasion-
of the tracheal lumen above the thoracic inlet. It
ally may demonstrate extraintestinal manifesta-
may extend downward on the mainstem bronchi.
tions. Among them, airway disease is relatively
This deformity is almost always associated with
uncommon and may take several forms, including
chronic obstructive pulmonary disease and has
ulcerative tracheitis and tracheobronchitis, bron-
been described exclusively in men.71 It has been
chiectasis, and small airway disease, most
postulated to be the consequence of abnormal
commonly obliterative bronchiolitis.68–70 Tracheo-
pattern and magnitude pressure changes related
bronchial complications are rare and occur more
to hyperinflated lungs.13 The deformity is often de-
often in association with ulcerative colitis than
tected incidentally on chest radiograph or CT
Crohn’s disease. In most—but not all—cases,
(Fig. 13). The inner contour of the trachea is often
the diagnosis of inflammatory bowel disease
smooth but occasionally has a nodular contour.
precedes the presence of airway disease.
Calcification of tracheal cartilage is frequently
Tracheobronchitis is characterized histologically
evident.73 Although saber sheath trachea is classi-
by more or less concentric mucosal and submu-
cally described as a static deformity, further nar-
cosal fibrosis and chronic inflammation. Ulceration
rowing of the tracheal lumen can be documented
and luminal narrowing may be evident. The carti-
when patients are examined during forced expira-
laginous plates may be calcified but are not
tion, reflecting excessive collapsibility of lateral
destroyed. Affected patients present with nonspe-
walls (tracheomalacia).74
cific symptoms of airway obstruction, including
stridor, dyspnea, and cough. CT findings are TRACHEOBRONCHOPATHIA
nonspecific.14 The tracheobronchial walls are OSTEOCHONDROPLASTICA
thickened and produce irregular luminal narrow-
ing. Bronchial wall thickening and bronchiectasis This rare disorder is characterized by the pres-
also may be present with or without mucoid ence of multiple submucosal cartilaginous or
impaction. If medical therapy (intravenous steroids bony nodules projecting into the tracheobron-
and cyclosporine) fails to resolve symptoms, chial lumen.12–14,75 It is a benign disease of
Fig.13. Saber sheath trachea in a patient with severe chronic obstructive pulmonary disease. Transverse scan (left)
and descending virtual endoscopy view (right). Characteristic deformity of the tracheal lumen is present.
unknown origin. Men are more frequently posterior wall, but the thickening of the wall is
involved than women, and most patients are not nodular in appearance. In most patients, the
older than age 50. Several potential causes or disease progresses slowly. Therapy is requisite
associations have been postulated, including only when the tracheal or bronchial lumens
amyloidosis, hereditary factors, chemical irrita- become compromised. Therapeutic options
tion, and infection. Most cases are asymptom- include surgical or laser resection, radiation
atic, but patients may present with chronic therapy, and stent placement.
cough, hoarseness, stridor, or wheezing that is
sometimes confused with asthma. It has been re-
BRONCHOLITHIASIS
ported to cause hemoptysis.76 Histologically, the
nodules are recognized as submucosal osteocar- Broncholithiasis is a condition in which calcified
tilaginous growths. The mucosal surface is intact, lymph nodes distort and erode into the tracheo-
and a connection between the nodule and the bronchial tree, and patients may expectorate or
perichondrium of the tracheal cartilaginous ring aspirate the calcified material.80 Most broncholiths
is frequently identified. Because it contains no are composed of fragments of calcified material
cartilage, the posterior wall of the trachea is that were originally located in a peribronchial
spared. lymph node. Broncholithiasis is considered as
CT is the imaging modality of choice for this a late complication of granulomatous lymphade-
condition.75,77–79 It demonstrates a pattern of nitis caused by Mycobacterium tuberculosis or
multiple calcified nodules arising from the ante- fungi such as Histoplasma capsulatum. Pathologi-
rior and lateral walls of the trachea and cally the airway is fibrotic and distorted, and
protruding into the lumen. The nodules typically erosion by calcified lymph nodes may be
range in size from 3 to 8 mm. They result in apparent. Bronchial wall fibrosis and obstruction
diffuse luminal narrowing and are associated pneumonitis may be present. Identification of
with thickening and deformity of the tracheal calcified material within an acute inflammatory
rings. The posterior membranous portion of the exudate or granulation tissue is key for diagnosis
trachea is spared. The differential diagnoses on bronchoscopic biopsy specimen. The diag-
include amyloidosis and tuberculosis, but these nosis may be made at CT by identifying a focus
diseases do not respect the posterior wall. of calcified material within the bronchial lumen
Relapsing polychondritis also affects the without any mass (Fig. 14).80–82 Peribronchial
Fig.14. Broncholithiasis. Coronal oblique reformat targets the upper part of the left upper lobe. There is complete
obliteration of the lumen of the subsegmental bronchus by endoluminal calcified material, complicated by post-
obstructive bronchiectasis.
258 Grenier et al
lymph node calcification is commonly seen. Post- 9. Lee KS, Ernst A, Trentham DE, et al. Relapsing poly-
obstructive abnormalities are often present, chondritis: prevalence of expiratory CT airway abnor-
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Multidetector C T
Evaluation of
Tracheobronchomalacia
EdwardY. Lee, MD, MPHa, Diana Litmanovich, MDb,
Phillip M. Boiselle, MDb,*
KEYWORDS
Tracheomalacia
Tracheobronchomalacia
Bronchomalacia
Multidetector computed tomography (MDCT)
Clinical indications Diagnostic criterion
MDCT protocols Image interpretation
Tracheobronchomalacia (TBM) is a disorder that 3-dimensional images, image interpretation, and

results from a weakness of the tracheobronchial pre- and postoperative assessment. An emphasis
walls and/or supporting cartilage, resulting in is placed on providing the reader with practical
excessive expiratory collapse (Fig. 1).1–5 This information that will enhance the ability to opti-
condition may arise congenitally from disorders mally perform and interpret CT studies for diag-
related to underlying impaired cartilage maturation nosing TBM in daily clinical practice.
or may be acquired from conditions related to prior
intubation, infection, trauma, long-standing
extrinsic central airway compression, or chronic CLINICAL INDICATIONS
inflammation.1–5
Although TBM has been increasingly recognized Although clinical indications have yet to be estab-
as an important cause of chronic respiratory lished for screening for TBM, the combination of
symptoms, it is still considered a relatively under- chronic respiratory symptoms and one or more
diagnosed condition.3 Because it escapes detec- risk factors should raise the suspicion for this
tion on routine end-inspiratory chest radiographs disorder. Risk factors for the acquired form of
and CT scans, the diagnosis of TBM usually TBM are reviewed in Box 1.
requires evaluating the airway during an active It is important to be aware that extrinsic airway
respiratory maneuver such as dynamic exhalation compression caused by paratracheal masses
or coughing.3,4,6 Recent advances in CT tech- (such as mediastinal vascular anomalies and
nology now allow the radiologist to accurately thyroid goiters) is frequently associated with tra-
diagnose this condition noninvasively with similar cheomalacia (TM) (Fig. 2).3,8,9 For example, in chil-
accuracy to bronchoscopy, the historical refer- dren with paratracheal vascular anomalies such as
ence standard.6,7 innominate artery compression or vascular rings,
This article provides an up-to-date review of surgical correction of the vascular anomalies alone
imaging for TBM, including clinical indications, may not adequately treat the respiratory symp-
physiologic principles, diagnostic criterion, multi- toms if extrinsic compression is accompanied by
detector CT (MDCT) protocols, multiplanar and intrinsic TM.8,9 Thus, preoperative evaluation of
a
Department of Radiology and Medicine, Children’s Hospital Boston, Harvard Medical School, 300 Longwood
Avenue, Boston, MA 02115, USA
b
Department of Radiology, Center for Airway Imaging, Beth Israel Deaconess Medical Center, Harvard
Medical School, Boston, MA 02115, USA
* Correspondence author.
E-mail address: [email protected] (P.M. Boiselle).
Radiol Clin N Am 47 (2009) 261–269

doi:10.1016/j.rcl.2008.11.007
262 Lee et al
Fig.1. CT diagnosis of TM in a 72-year-old man with shortness of breath. Paired inspiratory CT image (A) demon-
strates a normal oval shape of the tracheal lumen. Dynamic expiratory CT image (B) shows excessive expiratory
collapse of the trachea (arrow) consistent with TM.
such patients should routinely include a dedicated zero, intraluminal pressure equals alveolar pres-
expiratory sequence to assess for TM. sure and differs from pleural pressure only by the
Although pulmonary function studies (PFTs) are elastic recoil pressure of the lung, which depends
potentially useful in evaluating a patient with sus- on lung volume. At maximal lung volume with no
pected TBM, they are not diagnostic of this entity.3 flow (end-inspiration), the intraluminal pressure is
On forced expiratory spirograms, patients with 20 to 30 cm H2O greater than pleural pressure,
TBM frequently show a characteristic ‘‘break’’ or and the pressure difference expands the trachea.
notch in the expiratory phase of the flow-volume
loop;3 however, this pattern may also be seen in
patients with emphysema without coexisting Box 1
TBM. Thus, MDCT is still necessary for confirming Risk factors for acquired tracheomalacia
and characterizing TBM in such patients. Chronic obstructive pulmonary disease
Iatrogenic
PHYSIOLOGIC PRINCIPLES Prior intubation
Functional CT imaging for TBM requires acquiring Prior tracheostomy
imaging data either during or after a provocative Radiation therapy
maneuver such as expiration and coughing. In
Lung transplantation
order to understand why certain respiratory
maneuvers are more effective than others at elicit- Chronic bronchitis
ing tracheal collapse, it is important to review the
Relapsing polychondritis
relationship of tracheal collapse to intrathoracic
pressures. Changes in size of malacic trachea Chest trauma
and bronchi depend on the difference between Chronic external compression of the trachea
the intraluminal pressure inside the airways and Paratracheal neoplasms (benign and malignant)
the pleural (intrathoracic) pressure outside.4,10
Pleural pressure depends mostly on respiratory Paratracheal masses (eg, goiter, congenital cyst)
muscles, and is high during expiratory efforts. In Aortic aneurysms and vascular rings
contrast, intraluminal pressures are highly vari- Skeletal abnormalities (eg, pectus, scoliosis)
able, and depend on airflow. When airflow is
MDCT Evaluation of Tracheobronchomalacia 263
Fig. 2. TM in a young child associated with extrinsic compression from mediastinal vascular anomaly. Inspiratory
contrast-enhanced CT image (A) demonstrates mild tracheal compression from a right-sided aortic arch (R) with
aberrant left subclavian artery. Dynamic expiratory CT image (B) at similar level demonstrates near collapse of the
trachea (arrow), consistent with severe TM. Prominent thymic tissue is noted in the mediastinum, consistent with
the patient’s young age.
At low lung volumes with no flow (end-expiration), robust provocative maneuver to elicit tracheal
the intraluminal pressure is nearly equal to pleural collapse.13
pressure, and the trachea is unstressed. The Based on the results of these studies, there is
trachea is most compressed during cough and a need to obtain normative data regarding the
dynamic expiration at low lung volume, when range of tracheal collapse using forced exhalation
pleural pressure is high (w100 cm H2O), and expi- among patients of varying ages, ethnicities, and
ratory flow limitation in the small airways prevents both genders, both with and without coexistent
transmission of the high alveolar pressures to the pulmonary disease. Until such data are published,
central airways. Under these conditions, intralumi- one should keep in mind that there is substantial
nal pressure is nearly atmospheric, and the large overlap with normal physiologic changes at the
transmural pressure causes tracheal collapse.4,10 lower range of positive results. Thus, MDCT results
Thus, based on physiologic principles, imaging should be carefully correlated with respiratory
during a forced exhalation or coughing maneuver symptoms and functional impairment.
is more sensitive for eliciting TBM than imaging
at end-expiration. MULTIDETECTOR CT TECHNIQUES
Three main types of MDCT techniques are
DIAGNOSTIC CRITERION currently used for evaluating TBM: (1) paired
end-inspiratory and end-expiratory MDCT; (2)
Although greater than 50% expiratory reduction in paired end-inspiratory and dynamic expiratory
cross-sectional area of the airway lumen is widely MDCT; and (3) cine MDCT combined with a cough-
considered diagnostic of TM, it is important to be ing maneuver.
aware that asymptomatic, healthy individuals
Paired End-Inspiratory/End-Expiratory
may demonstrate levels of expiratory collapse
Multidetector CT
that exceed this diagnostic threshold (Fig. 3). For
example, Stern and colleagues11 obtained With this method, inspiratory and expiratory
a degree of tracheal collapse greater than 50% phases of volumetric CT data acquisition are ob-
at end-expiration in 4 of 10 healthy young adult tained at the end of inspiration and expiration,
male volunteers scanned with an electron-beam respectively. Because imaging at the end of expi-
CT. Based on their findings, these authors recom- ration is the least sensitive method for eliciting
mended a more conservative threshold of 70% of expiratory collapse, this technique should be
collapse as indicative of TM. Similarly, Heussel limited to the assessment of infants and children
and colleagues12 reported that healthy volunteers younger than 5 years of age who are unable to
can sometimes exceed the standard diagnostic cooperate with dynamic expiratory breathing
criterion. Moreover, when using 64-MDCT ‘‘cine’’ instructions. For such patients, end-inspiratory
imaging to assess the trachea during coughing, it and end-expiratory phases of the CT scanning
has been suggested that a higher threshold value are obtained following intubation by alternatively
of 70% should be considered when using this applying and withholding positive pressure
264 Lee et al
Fig. 3. Dynamic tracheal changes in a 45-year-old asymptomatic male volunteer with normal pulmonary function.
Axial end-inspiratory CT image above level of aortic arch (A) demonstrates normal oval-shaped tracheal lumen.
Axial dynamic-expiratory CT image at same level (B) demonstrates moderate anterior bowing of posterior
membranous wall of trachea (arrow). Cross-sectional area of the tracheal lumen decreased by 51% during expi-
ration, slightly exceeding the diagnostic criterion for TM.
ventilation during inspiration and expiration, include the entire lungs to assess for potential
respectively.8,9 For detailed information about complications of malacia such as bronchiectasis.
this method, the reader is referred to Lee and Helical scanning is performed in the craniocaudal
colleagues.8,9 direction for both end-inspiratory and dynamic-
expiratory scans.
Paired End-Inspiratory/Dynamic Expiratory The end-inspiratory scan is performed first (170
Multidetector CT mAs, 120 kVp, 2.5 mm collimation, pitch equiva-
lent of 1.5). Following the end-inspiratory scan,
This technique includes imaging during two patients are subsequently coached with instruc-
different phases of respiration: end inspiration tions for the dynamic expiratory component of
(imaging during suspended end inspiration) and the scan (40 mAs, 120 kVp, 2.5 mm collimation,
continuous dynamic expiration (imaging during high-speed mode, with pitch equivalent of 1.5).
forceful exhalation). This protocol can be success- For this sequence, patients are instructed to take
fully performed with any type of MDCT scanner. a deep breath in and to blow it out during the CT
However, the best results are produced with acquisition, which is coordinated to begin with
scanner configurations of eight or more detector the onset of the patient’s forced expiratory effort.
rows. Detailed ‘‘scripts’’ of breathing instructions for
At the authors’ institutions, this technique is the this protocol can be found in an article by Bankier
method of choice for imaging adults and children and colleagues.14
older than 5 years for suspected malacia. In the
following paragraphs, scanning parameters for
Cine CT During Coughing
imaging adults are reviewed. For detailed informa-
tion about imaging children with this protocol, the This technique requires use of a 64-row or greater
reader is referred to Lee and colleagues.8,9 MDCT scanner. At the authors’ institution this
Before helical scanning, initial scout topo- protocol is performed with detector collimation 0.5
graphic images are obtained to determine the mm 64; mA 5 80; kVp 5 120; gantry rotation 5
area of coverage, which extends from the proximal 0.4 seconds.
trachea through the main bronchi, corresponding An initial scout topographic image is obtained to
to a length of approximately 10 to 12 cm in adults. determine the area of coverage, which extends 3.2
If the patient has not had a recent CT scan, the to 4.0 cm in craniocaudad length depending on the
end-inspiratory acquisition can be extended to scan manufacturer. In order to ‘‘sample’’ the
trachea and proximal main bronchi within a single configuration). If repeated at multiple levels to
acquisition, the inferior aspect of the acquisition is provide similar coverage to the dual phase CT,
set at the level of the carina, and the superior the total dose for serial cine acquisitions would
aspect of the acquisition is set 3.2 cm above this be greater than the dual-phase technique. Esti-
level, which corresponds to approximately the mated doses for cine imaging of the airways using
level of the aortic arch in most adults. A 3- to the new 320 MDCT scanner are not currently avail-
5-second acquisition is acquired in cine mode able, but careful attention to dose reduction
beginning at end-inspiration and followed by methods will be important with this scanner to
repeated coughing maneuvers. Images are recon- avoid excessive radiation exposure.
structed at 8-mm collimation in a standard algo-
rithm, creating four contiguous cine datasets Image Quality
from a single acquisition.
In order to ensure a high-quality study, technolo-
The recently introduced dynamic-volume 320
gists should be trained to coach and monitor
MDCT scanner (Aquilion ONE, Toshiba Medical
patients as they perform the respiratory tech-
Systems) provides 16 cm of anatomic coverage
niques. Technologists should also be trained to
in a single rotation.15 This technique holds great
recognize the characteristic appearance of inspi-
promise for evaluating TBM because it provides
ratory and expiratory CT scans to ensure that the
coverage of the entire intrathoracic trachea in
imaging sequences have been successfully per-
most older children and adults.
formed during the appropriate respiratory maneu-
vers (see Fig. 1). For sites using these protocols for
Radiation Exposure the first time, the radiologist should observe and
monitor cases until the technologists have
Because paired inspiratory-expiratory CT requires
become comfortable coaching patients with these
imaging during two phases of respiration, it has the
maneuvers.
potential to result in a ‘‘double dose’’ compared
with a traditional single-phase CT scan unless
methods for dose reduction are used. Cine ROLE OF MULTIPLANAR AND 3-DIMENSIONAL
imaging also has the potential for high radiation RECONSTRUCTIONS
exposure. Volumetric MDCT imaging allows for the creation
Because of the high inherent contrast between of high-quality, three-dimensional (3D) reconstruc-
the air-filled trachea and soft tissue structures, it tions and multiplanar reformations (MPR), which
is possible to substantially reduce dose without have the potential to aid diagnosis and preopera-
negatively influencing image quality for assessing tive planning.4,18–21 Virtual bronchoscopic images,
luminal dimensions of the airway.4,6 For example, which provide an intraluminal perspective similar
a clinical study by Zhang and colleagues16 showed to conventional bronchoscopy, are particularly
no difference between standard (240 to 260 mA) helpful for assessing dynamic changes in the
and low-dose (40 to 80 mA) images for assessing lumen of the main bronchi, which course obliquely
the tracheal lumen during dynamic expiration. to the axial plane and are not optimally evaluated
Thus, a low-dose (30 to 40 mAs) technique by traditional axial CT images. External 3D recon-
should be used when imaging during coughing or structions are valuable for displaying complex 3D
expiration. Although a standard mAs level is typi- relationships in patients with extrinsic paratracheal
cally used for the end-inspiratory scan, dose masses such as aortic vascular anomalies.
modulation can be used to modify the mAs level Paired end-inspiratory and dynamic-expiratory
during the acquisition to further reduce radiation sagittal reformation images along the axis of the
exposure. trachea are helpful for displaying the craniocaudad
The estimated radiation dose (expressed as extent of excessive tracheal collapse during expi-
dose-length product) for a dual-phase study (stan- ration (Fig. 4).4
dard-dose end-inspiratory sequence 1 low-dose
dynamic expiratory sequence) for a 70-kg patient IMAGE INTERPRETATION
is approximately 500 mGy.cm, which is compa-
rable to a routine chest CT (reference value 600 Interpretation of CT images requires careful rev
mGy.cm).17 By comparison, the estimated dose iew and comparison of both end-inspiratory and
for a low-dose cine CT is approximately 200 to dynamic-expiratory images.
220 mGy.cm. However, unlike the dual-phase End-inspiratory images provide important
scan, which covers the entirety of the central anatomic information about tracheal size, shape,
airways, a single cine acquisition covers only 3.2 and wall thickness, as well as the presence or
to 4.0 cm in the z-axis (depending on the scanner absence of extrinsic masses compressing the
266 Lee et al
Fig. 4. Assessment of craniocaudad extent of TM in a 50-year-old woman with chronic cough. Paired end-inspira-
tory (A) and dynamic-expiratory (B) sagittal reformation images enhance display of craniocaudad length of TM.
Note diffuse expiratory narrowing of trachea during expiration (B), consistent with diffuse TM.
trachea. In patients with TM, the tracheal lumen is ensure that the same anatomic level is compared
almost always normal in appearance on end-inspi- between the two sequences by comparing vascular
ration CT.2 There are four notable exceptions. structures and other anatomic landmarks.
First, patients with relapsing polychondritis Although quantitative methods are preferable to
(Fig. 5) frequently demonstrate characteristic wall visual assessment, it is important to be aware that
thickening and calcification that spares the poste- about half of patients with acquired TM will
rior membranous wall of the trachea. Second, demonstrate an expiratory ‘‘frown-like’’ configura-
patients with lunate (coronal > sagittal dimension) tion, in which the posterior membranous wall is
tracheal configurations (Fig. 6). Third, patients excessively bowed forward and parallels the
with tracheomegaly (coronal diameter > 25 mm). convex contour of the anterior wall with less than
Fourth, patients with extrinsic tracheal compres- 6-mm distance between the anterior and posterior
sion from adjacent vascular anomalies or thyroid walls (Fig. 8).22 This appearance, which has been
masses (see Fig. 2). coined the ‘‘frown sign,’’ has the potential to aid
When there is near or complete collapse of the the detection of TM when patients inadvertently
airway lumen during expiration, the diagnosis of breathe during routine CT scans.22
malacia can be confidently made based on visual With regard to interpreting cine coughing CT
analysis of the images (see Fig. 1). However, the studies, these exams are ideally viewed in ‘‘cine’’
most accurate means for diagnosing malacia on fashion at either a picture archiving and comm-
CT in patients with subtotal expiratory collapse is unication systems workstation or 3D workstation.
to use an electronic tracing tool to calculate the Quantitative measurements are obtained on
cross-sectional area of the airway lumen on individual static images in a similar fashion to
images at the same anatomic level obtained at the technique described for paired-inspiratory–
inspiration and dynamic expiration (Fig. 7).4 Such dynamic-expiratory CT. As described earlier, greater
tools can be found on commercially available than 70% reduction in cross-sectional area during
picture archiving and communication systems coughing is considered diagnostic. A commercial
stations as well as with 3D workstations. software program (Analyze 6.0, AnalyzeDirect, Inc.,
As described in the previous section, greater than Lenexa KS) can also be used to provide automated
50% expiratory reduction in cross-sectional area is measurement of changes in tracheal lumen cross-
considered diagnostic. Care should be taken to sectional area values during the cine sequence.13
Fig. 5. TM in a 50-year-old woman with relapsing polychondritis presenting with intractable cough and dyspnea.
End-inspiratory (A) and dynamic-expiratory (B) axial images of the upper trachea show calcified wall thickening
with characteristic sparing of posterior membranous trachea. Excessive expiratory collapse of the trachea (arrow)
is consistent with TM.
When interpreting functional CT scans of symptoms, severity and distribution of disease,

patients with TM, it is important to report the and underlying cause of TM.23
severity, distribution, and morphology. These Because there is not a single widely accepted
factors have an important impact on treatment scale for reporting the severity of TM, it is impor-
decisions, which are based on a combination of tant to report the quantitative degree of
Fig. 6. Lunate configuration of the trachea in a 71-year-old woman with chronic cough and dyspnea. End-inspi-
ratory CT image (A) demonstrates widening of coronal diameter of trachea with respect to the sagittal diameter,
consistent with a lunate configuration. Dynamic-expiratory CT image (B) shows excessive expiratory collapse of
airway lumen, consistent with TM.
268 Lee et al
Fig. 8. ‘‘Frown sign’’ of TM in 64-year-old man with

chronic cough. Dynamic expiratory CT image demon-
strates excessive collapse of trachea with crescenteric,
Fig. 7. Example of electronic tracing method for ‘‘frown-like’’ configuration of airway lumen (arrow),
measuring cross-sectional area of tracheal lumen at consistent with TM.
the level of the aortic arch. The tracing line has
been electronically thickened to enhance visibility
for photographic reproduction. (Reprinted from
if it occurs primarily because of either excessive
Baroni RH, Feller-Kopman D, Nishino M, et al. Trache- bulging of the posterior membranous wall or
obronchomalacia: comparison between end-expira- collapse of the anterolateral cartilaginous struc-
tory and dynamic expiratory CT for evaluation of tures. For example, patients with collapse primarily
central airway collapse. Radiology 2005;235(2): because of bulging and flaccidity of the posterior
635–41; with permission.) membranous wall are potential candidates for tra-
cheoplasty surgery, a novel surgical technique in
collapsibility rather than simply using a qualitative which in the posterior wall of the trachea is rein-
descriptor. A severity scale that has been used forced by a Marlex graft.24 Surgical reinforcement
by some investigators includes three grades of of the posterior membranous wall enhances the
severity based on the degree of airway collapse: rigidity of this structure and makes it less suscep-
(1) mild: 50% to 74%; (2) moderate: 74% to tible to bowing during expiration.
99%; and (3) severe: 100% collapse.7,23 In
contrast, we consider more than 90% expiratory PREOPERATIVE AND POSTOPERATIVE
collapse as indicative of severe TM. ASSESSMENT
Murgu and Colt23 recently proposed a functional
class/extent/morphology/origin/severity (FEMOS) CT plays several potentially important roles in eval-
classification for TM. In this classification, a focal uating severely symptomatic TM patients who are
distribution of malacia is defined as involvement undergoing evaluation for curative tracheoplasty
of one tracheal region (upper, middle, or lower) surgery.24 Preoperative roles include (1) precise
or involvement of one main or lobar bronchus. characterization of airway shape and determina-
Multifocal distribution is defined as involvement tion of which parts of the airway wall contribute
of two contiguous or at least two noncontiguous to excessive airway collapsibility; (2) evaluation
regions, and diffuse involvement is defined as for systemic diseases such as relapsing polychon-
involvement of more than two contiguous regions. dritis that are not amenable to surgical therapy; (3)
From a practical perspective, accurate determina- identification of extrinsic, paratracheal masses
tion of distribution has implications for treatment. that may alter surgical planning; and (4) baseline
For example, focal areas of malacia may benefit measure of airway collapsibility by which to
from stenting, whereas diffuse disease is more compare postoperative scans for evaluating
amenable to tracheoplasty surgery. response to surgery. In the postoperative setting,
Regarding morphology, one should describe CT provides a noninvasive method for assessing
whether the collapse occurs circumferentially, or for postoperative complications and noninvasively
quantifying the degree of improvement in airway 9. Lee EY, Zurakowski D, Waltz DA, et al. MDCT evalu-
collapsibility. ation of the prevalence of tracheomalacia in children
Our surgeons and pulmonologists have found with mediastinal aortic vascular anomalies. J Thorac
a combination of subjective symptomatic improve- Imaging 2008;23(4):258–65.
ment and quantitative reduction in airway collaps- 10. Baroni RH, Feller-Kopman D, Nishino M, et al. Tra-
ibility at CT to be the most helpful measurements cheobronchomalacia: comparison between end-
of determining response to surgery.24 Our prelimi- expiratory and dynamic expiratory CT for evaluation
nary findings comparing preoperative and postop- of central airway collapse. Radiology 2005;235(2):
erative scans showed that tracheoplasty resulted 635–41.
in a decrease in the degree of airway collapse 11. Stern EJ, Graham CM, Webb WR, et al. Normal
that was accompanied by a qualitative improve- trachea during forced expiration: dynamic CT
ment of respiratory symptoms.24 measurements. Radiology 1993;187(1):27–31.
12. Heussel CP, Hafner B, Lill J, et al. Paired inspiratory/
SUMMARY expiratory spiral CT and continuous respiration cine
TBM refers to excessive expiratory collapse of the CT in the diagnosis of tracheal instability. Eur Radiol
trachea and bronchi as a result of weakening of the 2001;11:982–9.
airway walls and/or supporting cartilage. This 13. Boiselle PM, Lee KS, Lin S, et al. Cine CT during
disorder has recently been increasingly recog- coughing for assessment of tracheomalacia: prelim-
nized as an important cause of chronic respiratory inary experience with 64-MDCT. Am J Roentgenol
symptoms. MDCT technology allows for noninva- 2006;187(2):W175–7.
sive imaging of TBM with similar accuracy to the 14. Bankier AA, O’Donnell C, Boiselle PM. Respiratory
historical reference standard of bronchoscopy. instructions for CT examinations of the lungs: a hands-
Paired end-inspiratory, dynamic expiratory on guideline. Radiographics 2008;28:919–31.
MDCT is the examination of choice for assessing 15. Johns Hopkins Installs First 320-slice CT scanner in
patients with suspected TBM. Radiologists should North America. Johns Hopkins Medicine Web site.
become familiar with imaging protocols and inter- Available at: http://www.hopkinsmedicine.org/Press_
pretation techniques to accurately diagnose this releases/2007/11_26_07.html. November 26, 2007.
condition using MDCT. Accessed July 28, 2008.
16. Zhang J, Hasegawa I, Feller-Kopman D, et al.
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Imaging^Bronchoscopic
Correlations for
Interventional
Pulmonology
Tshering Amdo, MDa, Myrna C.B. Godoy, MDb,
David Ost, MD, MPHc, David P. Naidich, MD, FACCPb,*
KEYWORDS
Interventional pulmonology CT Virtual bronchoscopy
Endobronchial ultrasound (EBUS)
Transbronchial needle aspiration (TBNA)
The development and rapid advancement of both correlations in the evaluation and treatment of
bronchoscopic, CT and ultrasound imaging tech- central airway disease, followed by CT- broncho-
nology has had considerable impact on the scopic correlations in the evaluation of peripheral
management of a wide variety of pulmonary lung disease, in particular, pulmonary nodules.
diseases. The synergy between these newer Following this, the rapidly evolving topic of inter-
imaging modalities and advanced interventional ventional bronchoscopic approaches to the treat-
endoscopic procedures has led to a revolution in ment of emphysema will be reviewed.
diagnostic and therapeutic options in patients Although attention will be primarily be placed on
with both central and peripheral airway disease. CT bronchoscopic correlations (including CT-fluo-
Given the broad clinical implications of these tech- roscopy and virtual bronchoscopy), emphasis will
nological advances, only the most important areas also be placed on newer imaging technologies
of interventional pulmonology in which imaging including endobronchial ultrasound (EBUS), elec-
has had a major impact will be selectively reviewed tromagnetic navigation and guidance, and
to highlight fundamental principles. Doppler ultrasound site selection for broncho-
Whereas interventional pulmonology is often scopic treatment of emphysema.
conceptually organized around different technolo-
gies and instruments such as stents, lasers, and
electrocautery, among others, it is important to BRONCHCOSCOPIC IMAGING CORRELATIONS IN
emphasize applications of the same technology THE EVALUATION OF CENTRAL AIRWAY DISEASE
may vary widely in terms of their methods, risks, CT Imaging Technique
and benefits depending on the nature of the indi- Key to the recent ability of imaging to serve as
cation. For example, while the method in which a guide for interventional bronchoscopic proce-
a stent is placed may not alter, indications and dures has been the introduction and now wide-
complications are significantly different between spread availability of multidetector CT scanners
patients with benign and malignant disease.1 As capable of acquiring contiguous and/or overlap-
a consequence, particular emphasis will be placed ping high-resolution images throughout the entire
first on evaluation of CT-bronchoscopic thorax in a single breathhold. This has led to
a
Division of Pulmonary and Critical Care Medicine, New York University–Langone Medical Center, Tisch
Hospital, 560 First Avenue, New York, NY 10016, USA
b
Department of Radiology, New York University–Langone Medical Center, Tisch Hospital 560 First Avenue,
New York, NY 10016, USA
c
Division of Pulmonary Medicine, MD Anderson Cancer Center, Houston, TX 77030, USA
E-mail address: [email protected] (D.P. Naidich).
Radiol Clin N Am 47 (2009) 271–287

doi:10.1016/j.rcl.2008.11.005
272 Amdo et al
a near revolution in the variety of methods by to look backward from distal to proximal, ‘‘retro-
which the airways and lung can be visualized flexing’’ the virtual bronchoscope, which is not
and evaluated, including the use of quantitative possible with the conventional bronchoscope.6
CT techniques.2,3 Although a truly detailed discus- Virtual bronchoscopy is especially complementary
sion of this topic is beyond the scope of the to bronchoscopy in the assessment of patients
present review, the following general points with high-grade airway stenoses, particularly for
regarding CT technique for evaluating the airways assessing the patency of the airways beyond the
are emphasized. site of a stenosis. In one study7 comparing virtual
Optimal evaluation of both the central and and conventional bronchoscopy in 20 patients
peripheral airways requires at a minimum contig- with malignant airway stenoses, while high-grade
uous high-resolution images throughout the entire stenoses were viewed equally well with both tech-
chest (Fig. 1). While contiguous 1- to 1.5-mm niques, virtual bronchoscopy offered the advan-
sections are sufficient for evaluating both the tage of viewing the airway beyond the site of
central and peripheral airways, in our experience, stenosis in 5 (25%) of 20 patients in whom the
optimal visualization of the peripheral airways is bronchoscope could not pass the lesion.
best obtained with use of submillimeter overlap- However, lack of sensitivity for mucosal detail is
ping sections whenever possible (typically 0.75 an important limitation especially when attempting
mm every 0.5 mm) especially in those cases for to assess the extent of airway wall involvement in
which three-dimensional (3D) segmentation or patients in whom a distinction between intrinsic
virtual bronchoscopic evaluation of the peripheral and extrinsic tumor is of clinical consequence.6,8
(sixth to ninth order) airways is deemed clinically Finally, it is worth emphasizing that in those case
important.4 Although the use of low-dose tech- in which there are central lesions accompanied by
nique (50 to 80 mAs) is more than sufficient to eval- peripheral volume loss, administration of a bolus of
uate the central airways and in most cases the intravenous contrast medium may prove indis-
peripheral airways as well, in those cases for which pensable by allowing precise delineation of the
3D and/or virtual endoscopic views are intended, true extent of tumor versus atelectatic lung, find-
best results necessitate the use of routine stan- ings often indispensable for selecting optimal ther-
dard CT exposure factors. Additional consider- apeutic interventions.
ations include acquisition of select expiratory
high-resolution images in cases in which tracheal
Transbronchial Needle Aspiration and Biopsy
and bronchial dynamics are of concern, or to
confirm the presence of obstructive small airway CT-bronchoscopic correlations
disease. Transbronchial needle aspiration and biopsy
Axial CT images are sufficient for evaluating (TBNA) is a minimally invasive procedure done
most airway abnormalities;3,5 however, there are via flexible fiber-optic bronchoscopy (FB) that
inherent limitations of these for assessing the allows sampling of tissue through the tracheal or
central airways, including (1) limited ability to bronchial wall not directly visualized broncho-
detect subtle airway stenosis; (2) underestimation scopically. TBNA was first introduced by Schiep-
of the craniocaudad extent of disease; (3) difficulty pati9 in 1958 for use with the rigid bronchoscope
displaying the complex 3D relationships of the and was later adapted for flexible bronchoscopy
airway to adjacent mediastinal structures; (4) inad- (FB) by Wang and colleagues in 1983.10 This tech-
equate representation of airways oriented nique has been successfully used since then as
obliquely to the axial plane; and (5) difficulty as- a nonsurgical means most often to sample medi-
sessing the interfaces and surfaces of airways astinal nodes or less commonly hilar nodes, endo-
that lie parallel to the axial plane. Another relative bronchial lesions, and peripheral lesions for the
limitation of axial CT scanning is the generation diagnosis of both benign and neoplastic
of a large number of images for review, especially conditions.
with multidetector scanners, which may generate The ability of TBNA to sample mediastinal and
data sets containing hundreds of images. As hilar nodes can reduce the need for invasive me-
a consequence, use of retrospectively recon- diastinoscopy for assessing paratracheal lymph
structed 2D and 3D images should be considered nodes and the need for open thoracotomy for
routine for bronchoscopic correlation to overcome posterior, subcarinal, and hilar lymph nodes,
these limitations. respectively.11,12 Importantly, TBNA may provide
Virtual bronchoscopy (VB) in particular may the only bronchoscopic specimen diagnostic for
facilitate central airway evaluation by allowing the lung cancer in up to 18% of patients in one re-
user to ‘‘bypass’’ an obstructing lesion, accurately ported series.13 However, TBNA is often a ‘‘blind’’
measure its length and cross-sectional area, and procedure, especially when limited to sampling
Imaging–Bronchoscopic Correlations 273
Fig. 1. Central airway lesions. (A) Section through the lower trachea imaged with lung windows shows a well-
defined obstructing lesion. (B) Identical image as in A imaged with mediastinal window clearly demonstrates
the true extent of tumor which appears denser then adjacent peripheral atelectasis. The ability to demonstrate
the extent of tumor is critical to deciding the optimal method for interventional bronchoscopic therapy.
peribronchial lesions in the absence of endobron- CT fluoroscopic guidance

chial abnormalities. As a consequence, use of In 1998, Rong and Cui19 demonstrated an increase
TBNA has been limited with an overall sensitivity in the yield by performing TBNA under CT guid-
for diagnosing malignant mediastinal adenopathy ance. Using conventional CT scanners to provide
of 78% with values ranging from 14% to 100%.14 cross-sectional views of the relevant anatomy
Although the sensitivity and specificity of TBNA during the procedure, the tip of the needle was
largely depends on operator technique, the yield located and then adjusted until it was documented
of TBNA has been shown to be markedly enhanced to be inside targeted mediastinal lymph nodes.
by using CT scans to plan optimal biopsy sites Slow CT reconstruction times and significant radi-
before bronchoscopy.12,15 It should be empha- ation exposure, however, limit the practical value
sized, however, that merely identifying an enlarged of this approach.
node adjacent to a central airway on CT may not With the introduction of CT fluoroscopy, real-
lead to successful TBNA, as not all endoscopic time imaging during the procedure became
sites are equally amenable to transbronchial feasible enabling continuous image acquisition,
biopsy. As a consequence, optimal endoscopic permitting precise, real-time localization of the
anatomic landmarks for 14 hilar, intrapulmonary, bronchoscopic tip and needle (Fig. 2). CT fluoros-
and mediastinal lymph node stations have been copy is less cumbersome than conventional CT
proposed.11 This classification provides for consis- guidance, as it is controlled via a foot pedal with
tent, reproducible, lymph node mapping that is the imaging screen mounted next to the bronchos-
compatible with the international staging system copist obviating reliance on the technologist’s
for lung cancer16 and is applicable for clinical and workstation to check the position of the catheter
surgical-pathologic staging. tip. To date, several case series20,21,22,23 have
Whereas TBNA is most often performed to diag- demonstrated the safety and ease of performing
nose and stage NSCLC, it has also been used TBNA under CT fluoroscopic guidance with an
successfully to diagnose benign diseases as well, increase in yield of diagnosis in patients previously
including, in particular, sarcoidosis17 and more undergoing nondiagnostic conventional
recently tuberculous lymphadenopathy in patients TBNA.20,21,23 White and colleagues23 performed
with HIV1/AIDS.18 Especially in the setting of TBNA with CT fluoroscopic assistance on 27
HIV1/AIDS, active tuberculous nodes may appear patients, of whom 15 had mediastinal nodes and
as rim-enhancing low-density lesions following 12 had lung nodules or focal infiltrates. Mean lesion
intravenous contrast administration. In one series, size was 1.7 cm in the mediastinum and 2.2 cm in
using CT as a guide, mycobacterial infection was the lung. A correct diagnosis was established in
diagnosed by TBNA in 21 of 23 cases of docu- 10 of 12 mediastinal lesions (83%) for which
mented mycobacterial infection, with TBNA follow-up was available (three patients were lost
providing the only diagnostic specimen in 13 to follow-up) and in eight lung lesions (67%).23
(57%).18 These investigators concluded that CT fluoroscopy
274 Amdo et al
Transbronchial Needle
Aspiration–Endobronchial Ultrasound
As discussed above, the yield of TBNA is
clearly enhanced by pre-procedural review of CT
findings: further advantages accrue from perform-
ing TBNA with fluoroscopic guidance. Despite
these advantages, the overall yield of TBNA
remains suboptimal.14 As a consequence, and
not surprisingly, attention has increasingly focused
on developing newer methods for performing
image-guided TBNA, most importantly endobron-
chial ultrasound.
Endobronchial ultrasound (EBUS)-TBNA is
a relatively new technique in which a convex ultra-
sound probe is inserted through the working
Fig. 2. CT fluoroscopy-guided bronchoscopy. Axial CT channel of the bronchoscope allowing real-time
section through the distal trachea shows the tip of
ultrasound-guided TBNA. After imaging the target
a TBNA needle in an enlarged right paratracheal
lymph node (arrow), which on cytology proved to
lymph node, a specially designed TBNA needle is
be due to metastatic non–small cell lung cancer. As passed through the ultrasound catheter into the
demonstrated in this case, TBNA not only allows diag- desired location under real-time ultrasound guid-
nosis, often the sole means for acquiring histologic ance (Fig. 4). EBUS-TBNA can be used to sample
diagnosis, but may also provide precise tumor all except anterior, prevascular, and aorticopulmo-
staging—in this case Stage IIIA disease, obviating nary lymph nodes. This includes the highest medi-
more invasive procedures including mediastinoscopy astinal, upper and lower paratracheal, and
and/or surgery. subcarinal as well as hilar lymph nodes.26,27,28
Compared with conventional TBNA, EBUS guid-
can provide effective, real-time guidance for TBNA ance significantly increases the yield of TBNA in
and might be particularly valuable in patients with all mediastinal lymph node locations except for
small or less accessible mediastinal nodes.23 subcarinal nodes.29
Despite these results, a recent randomized trial Of particular interest, EBUS has been shown to
failed to demonstrate a significant difference diagnose malignant mediastinal and hilar nodes in
between CT fluoroscopic-guided bronchoscopy patients in whom both CT and positron emission
and conventional approaches, although this may tomography (PET) scans have proved negative.30
reflect the small sample size of this study as there Similar findings have been reported by others.
was a trend toward higher diagnostic yield with CT Yasufuku and colleagues,31 for example, in
guidance on a per lymph node versus a per patient a prospective comparison of EBUS-TBNA with
basis.24 Finally, it should be noted that some have PET and thoracic CT for detection of mediastinal
advocated the use of virtual bronchoscopic guid- and hilar lymph node metastasis in patients with
ance as a means for enhancing the yield of lung cancer using surgical nodal sampling as
TBNA (Fig. 3). Vining and colleagues6 compared a gold standard, showed that the sensitivities of
VB images with videotaped bronchoscopy results CT, PET, and EBUS-TBNA for mediastinal and
in 20 patients who had undergone both helical hilar lymph node staging were 76.9%, 80.0%,
chest CT and FB during clinical evaluation of and 92.3%, respectively, with corresponding
central airway abnormalities and observed that specificities of 55.3%, 70.1%, and 100%, and
virtual bronchoscopy simulations accurately rep- diagnostic accuracies of 60.8%, 72.5%, and
resented major endobronchial anatomic findings. 98.0%. Other investigators, however, have
As an extension of these findings, McAdams and demonstrated that although EBUS may be useful
colleagues25 assessed the role of virtual bron- in this situation, the frequency of occult medias-
choscopy in guiding TBNA in 17 patients and tinal disease may be too low to make routine
found that it improved the diagnostic yield of this EBUS worthwhile. Cerfolio and colleagues32 con-
procedure, with an overall sensitivity of 88% on ducted a prospective trial on patients with NSCLC
a per-node basis, and reduced both the amount who were clinically staged N2 negative by both
of pre-procedure preparation and the actual dedicated CT scanning and/or integrated PET/
procedural time. To date, while suggestive, an CT. All underwent mediastinoscopy and endo-
actual role for routine VB-guided TBNA remains scopic ultrasonography (EUS) esophageal endo-
to be established. scopic ultrasound-guided fine-needle aspiration
Fig. 3. TBNA: virtual bronchoscopic guidance. Axial (A) and coronal (B) CT sections imaged with narrow windows
at the level of the bronchus intermedius show enlarged bilateral hilar and subcarinal nodes. In this case, right
hilar nodes have been semiautomatically outlined and appear in color. Note that the optimal angle for accessing
these nodes is also presented (yellow lines). (C) Virtual bronchoscopic image looking inferiorly from the distal
bronchus intermedius showing the bifurcation of the right upper lobe bronchus and bronchus intermedius.
The exact location of the right hilar nodes outlined in A and B are now superimposed on the virtual broncho-
scopic image. In this case, virtual bronchoscopy serves as a road map assisting TBNA in accessing nodes in loca-
tions less frequently biopsied. Cytologically proven metastatic non–small cell carcinoma. (From Naidich DP,
Webb WR, Grenier PA, Harkin TJ, Gefter WB. Imaging the airways. Philadelphia: Lippincott Williams and Wilkins;
2005. p. 49; with permission.)
(FNA). Those with negative N2 nodes underwent only 3.7% had positive EUS-guided FNA results.
thoracotomy with thoracic lymphadenectomy to Based on these findings, these authors concluded
confirm the findings. Only 2.9% of patients clini- that there was no indication for the use of routine
cally staged as N0 after integrated PET/CT and/ EUS-guided FNA in patients with radiographic
or CT had positive mediastinoscopy results and N0 disease. However, as patients with clinical
276 Amdo et al
Fig. 4. EBUS-TBNA. (A) Contrast-enhanced axial CT image at the level of the carina shows slightly enlarged pre-
carinal nodes. (B) Endobronchial ultrasound image shows these same nodes adjacent to the airway wall noted
at the top of the image, measured at a depth of 1.1 cm. Nodes are especially easy to identify with EBUS separable
from adjacent airways and vessels. (C) Endoscopic image confirming in real time that the TBNA needle tip is
within the center of these nodes (arrow). Cytologically proven non–small cell lung cancer. (Case courtesy of
Michael Zervos, MD, New York University–Langone Medical Center, New York NY.)
N1–hilar adenopathy by PET/CT had a relatively central airway disease. To date, these procedures
higher prevalence of unsuspected N2 disease are most often performed either as complemen-
(a total of 17.6% after mediastinoscopy and tary to and/or alternatives to surgery, radiation,
23.5% after EUS-guided FNA) these investigators and/or chemotherapy in the palliative treatment
did conclude that patients with N1 disease be of patients with malignant airway disease. Approx-
considered for routine EUS-FNA. Despite these imately 30% of lung cancer patients present with
findings, the need for preoperative EBUS-TBNA lesions obstructing either the trachea or main
of otherwise normal-appearing mediastinal nodes bronchi resulting in dyspnea, cough, hemoptysis,
remains to be determined. or symptoms secondary to obstructive pneu-
monia, especially when the degree of obstruction
exceeds 50%.33,34 Importantly, the ability to
Therapeutic Interventional Bronchoscopy:
recanalize obstructed airways has proved of clin-
Imaging Correlations
ical benefit by alleviating symptoms and, in select
The past decade has seen the development of cases, prolonging survival.
a number of interventional bronchoscopic alterna- Currently available ablational procedures include
tives to routine therapeutic modalities for treating Nd:YAG laser bronchoscopy, photodynamic
therapy, argon plasma coagulation, endobronchial by delineating the true extent of disease, both
stents, brachytherapy, and cryosurgery.35 intrinsic and extrinsic, as well as allowing visualiza-
Although a detailed description of these various tion of airways peripheral to points of obstruction
procedures, including their mechanisms of action, otherwise invisible to the bronchoscopist.3,38
is outside the intended scope of the present review, To date, CT has proved especially useful in the pre-
a few basic principles regarding their use warrant and post-procedural assessment of airway stents.
emphasis. Airway obstruction may result either For airway stenting, routine 2D multiplanar recon-
from intrinsic tumor, extrinsic disease (typically structions (MPRs) have proved of greatest utility clin-
from adjacent adenopathy, but also including other ically.39 Easily obtained, MPRs can be displayed in
mediastinal malignancies) or a combination of routine coronal and sagittal planes, orthogonal to
these causes. In one study of 143 patients in a point of reference, or in a curved fashion along
whom stents were placed to alleviate obstruction, the axis of the airway. The 2D reformatted images
of 67% with malignant disease, 42% were because performed along the axis of the airway offer the
of extrinsic compression whereas 27% had intralu- advantage of quickly displaying the regional extent
minal tumors.36 Differentiation between these of a stenosis on a single image, although more diffi-
causes is of significance: stents and brachyther- cult and time consuming to reconstruct.
apy, for example, are best suited for extrinsic As an aid, pre-procedural CT planning may
compression or in cases in which there is extensive prove invaluable by precisely delineating the
submucosal disease (Fig. 5).33 In distinction, in anatomy, pathology, and severity of airway
patients with intrinsic airway lesions, options pref- obstruction.39 Information provided by CT can
erentially include tumor debulking with the rigid help to determine whether the airway obstruction
bronchoscope, laser, cryotherapy, argon plasma is caused by extrinsic compression, intraluminal
coagulation, and photodynamic therapy (Fig. 6).33 disease, or intrinsic airway disease, as occurs
Among these latter, cryotherapy, photodynamic in patients with tracheobronchomalacia, for
therapy, and brachytherapy are generally reserved example.40 CT also provides important comple-
for smaller endobronchial tumors where distal mentary information regarding the relationship of
bronchial segments can be visualized37 Although the central airways to adjacent structures that
choice among these various approaches is indi- are not visible at bronchoscopy.39 By determining
vidual depending on availability and technical the cause, location, length, and extent of the
expertise, in all cases CT may prove indispensable airway obstruction, CT can help stratify patients
Fig. 5. Airway compression caused by extrinsic disease. (A) Non–contrast-enhanced multiplanar coronal recon-
struction through the distal trachea and carina shows marked narrowing of these airways with apparent
complete obstruction of the left mainstem bronchus resulting in marked volume loss in the left lung. (B) Coronal
image at the same level as A showing the placement of a Y-shaped dynamic silicone stent in the distal trachea and
proximal mainstem bronchi resulting in improved aeration of the left lung. Multiplanar reconstructions, in partic-
ular, facilitate identification of appropriate candidates for stenting as well as facilitating more precise measure-
ment of airway diameter, lesion length, and airway visualization beyond the reach of the bronchoscope because
of obstruction.
278 Amdo et al
Fig. 6. Airway obstruction caused by intrinsic tumor. (A) Contrast-enhanced axial image just below the carina
shows near complete obstruction of the right mainstem bronchus by a well-defined soft tissue mass. (B) Endo-
scopic view showing bulky tumor mass appearing to completely occlude the airway lumen. (C) Endoscopic
view following photodynamic therapy documenting marked decrease in the size of this lesion, which proved
to be a mucoepidermoid carcinoma. CT may play an invaluable role by demonstrating the true extent of disease
allowing optimal choice of interventional technique.
into those who are amenable to surgical resection bronchoscopy, considered as the gold standard.
and those who are candidates for palliative treat- Two-dimensional images and 3D VB of tracheal
ment (see Fig. 5). In patients deemed appropriate stenoses proved to be efficient and complementary
for airway stenting, CT findings can help to deter- to rigid bronchoscopy, permitting a reliable endolu-
mine the type, size, and length of stent needed. minal 3D view and evaluation of the surrounding
For cases in which the initial bronchoscopic evalu- anatomic structures.
ation fails to visualize the airways beyond the site In addition to assisting in pre-procedural plan-
of obstruction, CT serves as an important adjunct ning, CT is also being increasingly used as a first-
study by providing detailed anatomic information line study for stent surveillance.39,42 Because
of the distal airways.39 nearly all stents, including metallic stents, cause
Less common, although as important, indications minimal artifact on multislice CT, the location,
for interventional bronchoscopic procedures include shape, and patency of the stents and adjacent
the treatment of benign tumors or a number of benign airways can be clearly visualized on CT. CT is highly
conditions, most often postintubation tracheal stric- accurate at detecting stent complications,
tures. Gluecker and colleagues,41 for example, including malpositioning, migration, fracture,
compared 2D and 3D CT imaging in the pre- and incongruence between the stent and airway diam-
postoperative evaluation of complex benign laryng- eters, external compression with continued
eo-tracheal airway stenoses with rigid stenosis, and local recurrence of malignancy
(Fig. 7). When compared with fiber-optic bronchos- The CT Bronchus Sign/CT Fluoroscopy
copy, both MPRs and 3D renderings have been
It has been shown that the likely yield of TBNA for
shown to be 88% to 100% sensitive and 100%
peripheral lesions is significantly improved in those
specific in detecting stent complications.42,43 In
cases in which a bronchus can be identified
a recent study by Dialani and colleagues,42 for
leading to or traversing a nodule, a so-called
example, CT accurately detected 15 (100%) of 15
‘‘positive bronchus sign.’’45,46 It has also been
stent complications in a group of patients under-
suggested that improved results from attempted
going surveillance with both CT and bronchoscopy
transbronchial biopsy may result when CT fluoros-
leading these investigators to suggest that CT may
copy has been used to aid in the diagnosis of
be able to replace bronchoscopy for routine
peripheral lesions (Fig. 8). To date, while there
surveillance of stents.
have been several small promising case series re-
ported,19,20,22,23,47 there has been only one small
Bronchoscopic Imaging Correlations
randomized controlled trial24 comparing CT fluo-
for Peripheral Lesions
roscopy–guided bronchoscopy with conventional
It has long been noted that the likely yield of trans- bronchoscopy for the diagnosis of peripheral
bronchial needle aspiration or biopsy (TBBx) of lesions. In this study, there was no significant
peripheral lung nodules or masses is affected by difference between CT fluoroscopy–guided bron-
the size and location of lesions. Peripheral lesions choscopy and conventional bronchoscopy;24
(defined as those not visible bronchoscopically however, when CT confirmed entry of the biopsy
beyond the segmental bronchial level) in particular forceps or needle into peripheral lesions, the diag-
are problematic with sensitivities of 34% for nostic yield did prove considerably higher. This
peripheral lesions smaller than 2 cm in diameter result suggests that the overall yield of CT-guided
versus 63% for lesions with a diameter larger bronchoscopy could be considerably enhanced if
than 2 reported in one recent study including 30 combined with improved methods of steering,
patients with peripheral lung lesions.44 Additional not only using conventional but ultrathin broncho-
factors include the type of lesion (solid versus scopes as well.
groundglass), the type of procedure performed In this regard, VB has recently been applied to aid
(bronchoalveolar lavage versus bronchial brushing in the diagnosis of peripheral lesions. Asano and
versus TBBx), or whether the procedure is per- colleagues48 used VB and ultrathin bronchoscopy
formed under fluoroscopic control. for peripheral pulmonary lesions. The diagnosis
Fig. 7. Stent surveillance. (A) Enlargement of a contrast-enhanced axial CT image below the carina shows exten-
sive mediastinal tumor associated with bilateral pleural effusions. Note that there is a stent seen in the left main
stem bronchus within which soft tissue density is clearly identifiable, in this case because of tumor invasion of the
stent. CT is an effective means for monitoring stents following placement. (B) Endoscopic view confirming tumor
infiltrating the lumen through the stent.
280 Amdo et al
through the working channel of a flexible bron-

choscope (FB). Under fluoroscopic guidance,
the whole unit is maneuvered to the region of
interest. Although the radial probe provides
cross-sectional images of the tracheobronchial
wall and adjacent mediastinal structures and
produces a 360-degree image relative to the
long axis of the FB, a major limitation has been
the necessity to withdraw the US probe before in-
serting a biopsy instrument, eliminating the possi-
bility of real-time positioning.
Paone and colleagues53 conducted a study to
compare the diagnostic yield of two bronchoscopic
procedures: EBUS transbronchial biopsy (EBUS-
TBB) and conventional transbronchial biopsy
(TBB) for diagnosing peripheral pulmonary lesions
and reported a sensitivity of 79% in the EBUS
group versus 55% in the TBB group for estab-
Fig. 8. CT fluoroscopy: peripheral lung disease. Axial
lishing malignant diagnoses. In patients with
non–contrast-enhanced image obtained during fluo-
roscopic-guided transbronchial biopsy. Note that lesions smaller than 3 cm, they found a consider-
images obtained during CT fluoroscopy suffer from able decline in TBB sensitivity and accuracy (31%
some degree of motion artifact and increased image and 50%), whereas EBUS-TBB maintained its
noise. In this case it is possible to identify the tip of diagnostic yield (75% and 83%). A similar differ-
the biopsy needle within the center of a lesion in ence in sensitivity was observed when lesions
the superior segment of the left lower lobe. Cytolog- smaller than 2 cm (23% versus 71%) were
ically proven non–small cell lung cancer. compared. Based on these data it may be
concluded that in select cases EBUS using
a radial probe represents an important option
rate of this procedure was 81.6% for the 38 lesions
in the diagnosis of peripheral lung cancer, espe-
examined by ultrathin bronchoscopy: 80.8% for 26
cially in small lesions and/or patients considered
lesions 2 cm in size, and 83.3% for 12 lesions larger
ineligible for surgery.
than 2 cm. Merritt and colleagues49 and Shinagawa
and colleagues50 have also demonstrated that
Electromagnetic navigation
combining VB and ultrathin bronchoscopy was
The electromagnetic navigation system is an
superior to conventional bronchoscopy for diag-
image-guided localization device that assists in
nosing peripheral lung lesions. Asahina and
placing endobronchial accessories (forceps,
colleagues51 successfully performed transbronchial
brush, or needle) to desired regions of the lung
biopsy (TBB) using EBUS and VB navigation for
(Fig. 9). The use of this novel technology in animal
small peripheral pulmonary lesions smaller than
models followed by human subjects was first
3 cm in diameter.
published by Schwarz and colleagues.54,55 In
Despite these findings, it should be noted that
brief, following an initial CT examination requiring
currently available biopsy forceps for use in ultra-
contiguous 1-mm sections, images are then up-
thin bronchoscopes present an important barrier
loaded into a navigating computer. The electro-
to the use of this approach, as only minimal tissue
magnetic navigation system creates an
can generally be obtained, severely limiting diag-
electromagnetic field around the chest by placing
nostic efficacy. More promising is the potential
the subject on an electromagnetic localization
use of VB-guided bronchoscopy to precisely
board. An electromagnetic position sensor is
direct a pediatric bronchoscope into the lung
then attached to the same navigating computer
periphery to sample specific regions in patients
already loaded with the initial CT scan data,
with otherwise ‘‘diffuse’’ lung diseases identified
now displayed in virtual bronchoscopic format.
on high-resolution CT (HRCT).4
As the sensor is positioned within the electromag-
netic field, precise spatial coordinates are fed to
Endobronchial ultrasound with radial probe the computer in real time and correlated with
EBUS using a radial ultrasonic probe has also the virtual bronchoscopic CT data by a process
been used to locate peripheral parenchymal of registration. This involves selecting predeter-
lesions.52 For this purpose a thin ultrasonic probe mined targets on the virtual bronchoscopic image
is inserted through a guide sheath that is passed and then touching the corresponding identical
Fig. 9. Electromagnetic registration and guidance: peripheral lung lesions. Screen shot from a commercially avail-
able electromagnetic navigation system (SuperDimension, Inc) shows typical interface with axial, sagittal, and
coronal CT images, respectively, coupled with virtual bronchoscopic images. Following an initial CT study, data
are loaded into the navigating computer. Using virtual bronchoscopy (lower right image), anatomic landmarks
are chosen as registration points. Typically these are the main carina, right upper lobe carina, middle lobe carina,
right lower lobe carina, left upper lobe carina, and left lower lobe carina displayed in this image as purple dots.
During actual bronchoscopy, the electromagnetic sensor is touched to each of these points enabling electronic
registration of these locations by the computer. A separate path to the target lesion of interest (green dots) is
also reconstructed. Once the registration process is complete, the bronchoscopist pilots from green dot to green
dot until the target lesion is reached.
endoscopic targets in real time using the bron- data to real-life coordinates (see Fig. 9). Diver-
choscopic probe. Targets chosen are points of gence, a measure of the error between the pro-
airway bifurcation and include the main carina jected coordinates using these registration
and most proximal lobar bifurcations. Once five points and the actual points, is usually in the
to six of these anatomic locations have been so range of 3 to 7 mm.
co-registered with the corresponding VB images, Importantly, the electromagnetic navigation
the computer can then map the rest of the CT system includes two critical features. The
282 Amdo et al
electromagnetic position sensor, which is the nondiagnostic. The diagnostic yield of the
size of a bronchoscopy forceps, first is steerable combined procedure (88%) was greater than
and second may be passed through an extended EBUS (69%) or electromagnetic navigation (59%)
working channel, which is simply a hollow cath- alone, and proved independent of lesion size or
eter. Consequently, once the probe reaches its lobar distribution. Based on this initial study, it
intended target, it can be removed while keeping was concluded that combining EBUS and electro-
the extended working channel in place. Biopsy magnetic navigation can improve the sensitivity of
instruments such as forceps and brushes can flexible bronchoscopy when compared with either
then be passed back down the extended working EBUS or electromagnetic navigation individually
channel to the same location. for diagnosing peripheral lung lesions without
Schwarz and colleagues54,55 successfully compromising safety.60 Similarly improved results
demonstrated, initially in animal models and using a combined approach have also been
subsequently in a human study, safe use of reported.61
electromagnetic navigation technology coupled
with preoperative CT scanning in precisely local-
Emphysema: CT-Interventional Bronchoscopic
izing peripheral lung lesions with a variety of
Correlations
endobronchial accessories. They also demon-
strated safety and efficacy in navigating to Bronchoscopic lung volume reduction in severe
peripheral lung lesions located beyond the range emphysema
of a standard FB.54,55 Subsequent larger studies Current treatment of severe emphysema is limited
in human subjects56,57,58,59 have been con- to palliative measures that include supplemental
ducted with reproducible results, proving that oxygen, bronchodilators, anti-inflammatory drugs,
electromagnetic navigation bronchoscopy is and pulmonary rehabilitation or lung transplanta-
a safe method for sampling peripheral and tion. Various surgical procedures have been used
mediastinal lesions with high diagnostic yield to treat severe emphysema, including thoraco-
independent of lesion size and location. None- plasty, excision of bullae, costochondrectomy,
theless, despite the potential of widespread clin- phrenic nerve division, autonomic denervation,
ical applicability, it should be emphasized that, and lung transplantation. Until the completion of
to date, no randomized trials demonstrating its the National Emphysema Treatment Trial
superiority to conventional methods for diag- (NETT),62 none of these techniques were accepted
nosing peripheral lung lesions has been as beneficial except for resection of giant bullae
reported. and lung transplantation, each indicated in only
a small percentage of emphysema patients. The
Electromagnetic navigation plus NETT trial established that Lung Volume Reduc-
endobronchial ultrasound tion Surgery (LVRS) is associated with improve-
Although both EBUS using radial probes and elec- ment in health status, dyspnea, and exercise
tromagnetic navigation bronchoscopy have been capacity and lung function, when compared with
used individually to try to improve bronchoscopic medical treatment in select populations.62,63 In
diagnosis of peripheral lung lesions, a recent study this regard, the extent and severity of disease as
by Eberhardt and colleagues60 evaluated whether identified by CT has proved one of the most impor-
or not the combination of radial EBUS plus electro- tant predictors of a successful outcome, with
magnetic navigation might be superior to either patients with predominant upper lobe centrilobular
system alone. Focusing on the diagnosis of periph- disease most likely to respond successfully
eral lung lesions, this study included three arms: (Fig. 10), while those with more diffuse disease
EBUS only, an electromagnetic navigation system are noncandidates. However, because LVRS is
only, and a combined procedure using both tech- associated with significant morbidity, mortality,
nologies together. In the combined group, and cost, nonsurgical alternatives for achieving
following initial electromagnetic navigation, an volume reduction are being developed.
ultrasound probe was passed through the To date, three bronchoscopic lung volume
extended working channel to enable direct visuali- reduction strategies have shown promise and are
zation of lesions. Biopsies were taken if ultrasound currently entering into clinical trials. All are de-
visualization confirmed that the extended working signed to reduce hyperinflation within emphsyem-
channel was within the target with retargeting of atous portions of the lung and thus achieve lung
lesions performed as necessary. For the purpose volume reduction without the significant mortality
of this study, primary outcome was defined as diag- and morbidity associated with surgical
nostic yield with the reference standard surgical LVRS.64,65,66,67 These include (1) placement of en-
biopsy if bronchoscopic biopsy proved dobronchial one-way valves designed to promote
Fig.10. Emphysema: quantitative CT evaluation. Axial (A, upper left image), coronal (B, middle image), and volu-
metric rendered coronal sections (C, upper right image) show typical appearance of severe centrilobular emphy-
sema almost exclusively restricted to the upper lobes. In this case, contiguous 1-mm images were acquired with
quantitative analysis using 950 HU as a cut-off to demonstrate emphysematous foci, visually color-coded. The
table below the images shows quantitative assessment of the extent of emphysema as measured by the so-called
‘‘cluster’’ analysis. This allows evaluation of the relative size of low-density foci providing a ‘‘bulla index’’ (3d BI) in
which voxels are subdivided as in this example into four arbitrary classes, each separately color coded, with class 1
(blue) 5 all low-density foci % 2 mm; class 2 (green) 5 2 to 8 mm; class 3 (yellow) 5 8 to 12 mm; and class 4 (pink)
5 12 mm, respectively. Note that in this case the majority of low-density foci fall into the class 4 category and are
clearly upper lobe in distribution without evidence of discrete subpleural bullae noted—findings ideal for both
LVRS and/or bronchoscopic lung volume reduction.
Fig. 11. Bronchoscopic lung volume reduction—one-way valve insertion. With this approach, a one-way valve is
inserted under endoscopic guidance in an upper lobe bronchus to block air from entering the emphysematous
portion of on inspiration while allowing gas to exit during expiration. (A) The appearance of the valve before
insertion against the background of a person’s finger. (B) The appearance of the valve 1 month following deploy-
ment within the right upper lobe bronchus, follow-up 9 months later showed marked narrowing of the right
upper lobe bronchus consistent with marked volume loss in the right upper lobe.
284 Amdo et al
Fig.12. Airway bypass procedure—drug-eluting airway stents. (A–D, label from left to right, top to bottom) Select
coned down views of the proximal left lower lobe basilar bronchi in a patient with diffuse emphysema. CT scans
are used not only to assess the severity and distribution of emphysema but also to identify blood vessels that need
to be avoided during stent placement. With this approach, all lobes except the middle lobe are potential targets
for intervention. Drug-eluting airway stents are placed in segmental airways leading to regions with the highest
residual volume with the expectation that these will facilitate the escape of trapped air. Virtual bronchoscopic
images are also used to further assist in determining optimal stent placement (lower right image). (E) Endoscopic
view showing the appearance of the airway following stent placement. Note that the stent leads directly from
a segmental airway to more distal respiratory bronchioles and alveoli.
atelectasis by blocking inspiratory flow; (2) forma- this regard, CT imaging often plays an indispens-
tion of airway bypass tracts using drug eluting able role in both the selection of appropriate
stents designed to facilitate emptying of candidates for therapy as well as the choice of
‘‘damaged’’ regions of the lung defined by long optimal interventional techniques. However, it is
expiratory times; and (3) instillation of biological apparent that alternate methods for evaluating
adhesives designed to collapse and remodel the airways and lung including ultrasound and
hyperinflated lung.64,65,66,67 electromagnetic navigation will likely play an
For any of these techniques to work, careful increasingly important diagnostic role, necessi-
delineation of the extent and type and severity of tating a thorough understanding of their
emphysema is essential.68 Imaging therefore plays advantages and limitations. Disease-specific
a pivotal role in bronchoscopic treatment of emphy- applications for which imaging technologies,
sema, because it facilitates measurement of the including CT and VB, are either currently routinely
extent and distribution of emphysema as well as used or show the greatest promise are for sus-
identifying concomitant conditions that either pected or diagnosed lung cancers, central and
require additional evaluation (such as presence of peripheral, and emphysema. It may be anticipated
associated severe bronchial disease, concomitant that with growing experience, the potential for
interstitial fibrosis, or potentially malignant lung additional indications of these remarkable technol-
nodules) that may represent contraindications to ogies are likely to increase in the near future.
routine LVRS. Finally, delineation of airway
anatomy relative to adjacent foci of emphysema
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Bronchiectasis
Cylen Javidan-Nejad, MD*, Sanjeev Bhalla, MD
KEYWORDS
Airway Bronchiectasis HRCT
High-resolution CT Cystic fibrosis Ciliary dyskinesia
Bronchiectasis is defined as the irreversible dilata- a vicious cycle of progressive wall damage, mucus
tion of the cartilage-containing airways or bronchi. plugging, and increased bacterial proliferation.3,4
Enlargement of the bronchi in acute illness, as can Once bronchiectasis begins, therefore, it is sure to
be seen in the setting of infectious pneumonia, is progress.
usually reversible and would, therefore, not qualify Airway obstruction is most commonly caused
as bronchiectasis (Fig. 1). Care must be taken to by an intraluminal lesion such as carcinoid tumor,
distinguish this large airway dilatation from dilata- inflammatory myofibroblastic tumor, or a fibrous
tion of the small airways (bronchioles) that do not stricture usually from prior granulomatous infec-
contain cartilage.1 tion such as histoplasmosis or tuberculosis. The
presence of bronchiectasis can be useful in the
MECHANISMS OF DEVELOPMENT differential diagnosis of an endoluminal mass, as
its presence usually implies a chronic component.
Bronchiectasis may result from one of three main Although it may be seen with squamous cell carci-
mechanisms: bronchial wall injury, bronchial nomas arising from papillomas, the presence of
lumen obstruction, and traction from adjacent bronchiectasis is more suggestive of a slowly
fibrosis.2 The latter two mechanisms are usually growing less malignant lesion, such as a carcinoid
apparent on imaging, and are suggested by an en- tumor (Fig. 2). Distal atelectasis and/or postob-
dobronchial filling defect or adjacent interstitial structive pneumonitis are common in these condi-
lung disease. It is when the first group is encoun- tions.2 In the post lobectomy or post–lung
tered that the radiologist is faced with a differential transplant patient, granulation tissue at the suture
diagnosis and a potential diagnostic conondrum. line can occasionally result in an intraluminal
In this article, we aim to cover the CT appearance occlusion and distal bronchiectasis. In these
of bronchiectasis, potential pitfalls, and a diag- patients, immediate postoperative detection may
nostic approach to help narrow the diverse spec- allow for bronchoscopic removal of granulation
trum of conditions that may cause bronchiectasis. tissue and avoid irreversible damage.
Many conditions may lead to bronchial wall injury When bronchiectasis is from bronchial wall
and subsequent bronchiectasis. These include infec- damage or bronchial obstruction, the bronchial
tion and recurrent infections, impaired host defense wall becomes thickened because of infiltration by
leading to infection, exaggerated immune response, mononuclear cells and fibrosis. In cystic fibrosis
congenital structural defects of the bronchial wall, (CF), an additional neutrophilic infiltration of the walls
and extrinsic insults damaging the airway wall and airway lumen can be seen. This mural inflamma-
(Table 1). These conditions share the common tory process progressively destroys the elastin,
denominator of mucus plugging and superimposed muscle, and cartilage. This leads to airway dilatation.
bacterial colonization. The mucus plugging is either The dilatation can be classified by its gross
a result of abnormal mucus constituency or appearance as tubular (cylindric), varicose, or
abnormal mucus clearance. The toxins released by cystic (saccular). In the former, the bronchiectasis
the bacteria and the cytokines and enzymes released is manifest as parallel bronchial walls with failure of
by the surrounding inflammatory cells create normal tapering and squared-off ends of the
Section of Cardiothoracic Imaging, Mallinckrodt Institute of Radiology, Washington University School of

Medicine, 510 South Kingshighway, St. Louis, MO, USA
E-mail address: [email protected] (C. Javidan-Nejad).
Radiol Clin N Am 47 (2009) 289–306

doi:10.1016/j.rcl.2008.11.006
0033-8389/08/$ – see front matter. Published by Elsevier Inc.
290 Javidan-Nejad & Bhalla
Fig.1. Reversible lower lobe bronchial dilatation due to pneumonia. Initial CT (A) in this 12-year-old girl with hy-
pogammaglobulinemia and pneumonia showed bilateral dilated lower lobe bronchi. Subsequent CT (B) per-
formed 6 months later showed resolution of the bronchial dilatation, although it revealed a right middle lobe
pneumonia. Because this dilatation is reversible, it would not qualify as bronchiectasis.
bronchus. As the process worsens, the bronchi lobe distribution in cases of radiation fibrosis,
become serpentine with a beaded appearance. sarcoidosis, and sequela of tuberculosis (Table 2)
This varicose bronchiectasis serves as an interme- (Fig. 3). In cases of usual interstitial pneumonitis
diate step before the development of grossly (UIP) (idiopathic pulmonary fibrosis) and fibrosing
dilated, cystic airways. As the airway dilatation nonspecific interstitial pneumonitis (NSIP), the trac-
increases, there may be progressive collapse tion bronchiectasis tends to be mostly in the
and fibrosis of the distal lung parenchyma.2 periphery and the lung bases (see Fig. 4).5
Traction bronchiectasis, as its name implies, is
caused by retraction of mature fibrosis of the
CLINICAL PRESENTATION AND DIAGNOSIS
parenchyma around the bronchi. Such bronchiec-
tasis follows the distribution of the underlying Symptoms can be very nonspecific. Mild disease
fibrosis. The traction bronchiectasis has an upper may manifest with a mild cough or minimal
Table 1
Bronchiectasis caused by airway wall damage
Mechanism Disease
Congenital structural defect Mounier-Kuhn syndrome
William-Campbell syndrome
Infection Pertussis (whooping cough)
Tuberculosis
Atypical mycobacterium
Impaired immune response
Abnormal mucociliary clearance Cystic fibrosis
Primary ciliary dyskinesia
Decreased systemic immunity Hypogammaglobulinemia
Lung and bone transplantation
Exaggerated immune response Allergic bronchopulmonary aspergillosis
Inflammatory bowel disease
c-ANCA-positive vasculitis
Rheumatoid arthritis
Inhalational injury Smoke and gaseous toxins
Chronic gastroesophageal reflux and aspiration
Bronchiectasis 291
chronic hypoxemia and hypercarbia in more severe

disease. Severe sinusitis can be seen if the bronchi-
ectasis is due to primary cilia dyskinesia, Kartage-
ner syndrome, cystic fibrosis, and diffuse
panbronchiolitis.6
Hemoptysis, sometimes life-threatening, is
caused by chronic airway inflammation and
hypoxemia, which leads to bronchial arterial neo-
vascularization.7,8 These enlarged bronchial arteries
are quite fragile and may rupture with even minimal
trauma. Pulmonary hypertension can ensue
because of underlying hypoxemic vasoconstriction
and obstructive endarteritis.9 The bronchial arteries
anastamose with pulmonary arterioles, leading to
left-to-right shunting, and if severe enough, can
contribute to the pulmonary hypertension.9
Fig. 2. Bronchiectasis due to obstructing carcinoid
The severity of airflow limitation is related to the
tumor. CT through the right middle lobe shows
extent and severity of the bronchiectasis. This can
partially mucus-filled bronchiectasis and an endo-
bronchial mass (arrow). be seen in ventilation-perfusion mismatches and
retained washout on the ventilation images of venti-
lation-perfusion scintigraphy. Decreased FEV1
dyspnea. As it becomes more severe, patients may (forced expiratory volume in 1 second) on spirom-
present with chronic cough, regular and copious etry can also be seen in the setting of bronchiec-
sputum production, progressive dyspnea, and tasis. Although useful in quantifying the ventilation
repeated pulmonary infections.3 Digital clubbing, impairment, spirometry has proven insensitive in
anemia, and weight loss can develop because of detecting early structural damage.
Table 2
Bronchiectasis based on distribution
Location Disease
Focal Congenital bronchial atresia
Foreign body
Broncholithiasis
Endobronchial neoplasm
Diffuse
Upper lung Cystic fibrosis
Sarcoidosis
Progressive massive fibrosis of pneumoconiosis
Radiation fibrosis
Central lung Allergic bronchopulmonary aspergillosis
End-stage hypersensitivity pneumonitis (also upper
lobes)
Mounier-Kuhn (also lower lobes if repeated infections)
Lower lung Usual interstitial pneumonia (IPF)
Nonspecific interstitial pneumonitis
Hypogammaglobulinemia
Lung and bone transplantation
Chronic aspiration
Idiopathic
Right middle lobe and lingula Atypical mycobacterial infection
Immotile cilia syndrome (PCD) (also lower lobes)
Fig. 3. Upper lobe traction bronchiectasis from end-stage sarcoid. Transaxial image at the level of the carina (A)
demonstrates tortuous, shortened bronchi amidst upper lobe fibrosis and volume loss. Upper lobe predominance
of the bronchiectasis is better appreciated on coronal reconstruction (B).
Because of the insensitivity of the other tech- electrolyte test, serum immunoglobulin levels, or
niques and the high-spatial resolution of CT, serum Aspergillus antibody and precipitin, or
high-resolution computed tomography (HRCT) even genetic testing. Occasionally electron
has become the favored diagnostic tool for detec- microscopy of the ciliated cells may be used.6
tion of bronchiectasis.10,11 Rapid disease progres- Bronchoscopy is diagnostic and sometimes
sion has a poorer prognosis and has been shown therapeutic. In localized bronchiectasis caused
to be associated with increased wall thickening, by a foreign body, an endobronchial neoplasm,
colonization by Pseudomonas aeruginosa, and bronchiolithiasis, and fibrotic stenosis, bronchos-
high concentrations of proinflammatory markers copy may be used to remove the obstructing
in sputum or serum, such as neutrophilic lesion. Although the bronchiectasis itself will not
elastase.4,12 revert, the clearance of the airway will improve
Diagnosis is usually suspected by history and any postobstructive pneumonia and may prevent
confirmed by spirometry and HRCT. Depending progression of the airway damage.13 Bronchos-
on the associated symptoms and age of presenta- copy and lavage is helpful in patients with more
tion, the diagnostic workup may include a sweat diffuse bronchiectasis who present with acute
Fig. 4. Lower lobe traction bronchiectasis from usual interstitial pneumonitis. Transaxial image at the level of the
superior segmental bronchi (A) demonstrates traction bronchiectasis, within peripheral-dominant honeycombing
in this 52-year-old man with idiopathic pulmonary fibrosis. Coronal reformatted image (B) shows that the basilar
and peripheral bronchiectasis follows the distribution of the fibrosis.
Bronchiectasis 293
exacerbation or sudden worsening of underlying use of low-dose techniques for HRCT, but we
symptoms. This is usually a sign of acute airway have not routinely relied on these.17
infection, commonly by Pseudomonas and Staph-
ylococcus, and in such patients bronchoscopy is
helpful in obtaining samples for culture and deter- IMAGING FINDINGS
mining antibiotic sensitivity, when usual measures
of sputum sampling are unsuccessful.6 The imaging findings on conventional radiography
are based on visualizing the bronchial wall thick-
HIGH-RESOLUTION CT IMAGING TECHNIQUE ening. This results in ill-defined perihilar linear
densities associated with indistinctness of the
Because of the nonspecific nature of symptoms margins of the central pulmonary arteries. This
associated with bronchiectasis, an accurate way appearance simulates interstitial pulmonary
of diagnosis is needed. HRCT provides the most edema, but lacks the peripheral Kerley B lines.
accurate, least invasive technique. It enables When the bronchus is seen on end, ill-defined
the assessment of bronchial abnormalities to the ring shadows can be identified because of bron-
level of the secondary pulmonary lobule level. chial wall thickening. The presence of tram lines
Conventional HRCT is performed by acquiring or parallel lines along the expected courses of
1.0- to 1.5-mm thick images, every 10 mm, recon- the bronchi indicates more severe bronchiectasis,
structed using a high spatial frequency algorithm. where the dilated bronchial lumen becomes visible
Images can be obtained in a spiral or sequential on conventional radiography. Tram lines are best
mode. Using conventional technique, visualization identified in the lower lobes, right middle lobe,
of bronchi 1 to 2 mm in diameter and vessels 0.1 to and lingula (Fig. 5). Oftentimes, an abnormality is
0.2 mm in diameter may be achieved.1 The lungs detected but the specific diagnosis of bronchiec-
are scanned twice, during suspended end- inhala- tasis is not.
tion and suspended end-exhalation. The latter Mucus plugging may appear as elongated
phase is performed to reveal subtle air trapping. opacities, which may be sometimes calcified.
Multidetector computed tomography (MDCT) These tubular opacities can be confused for
scanners allow fast, volumetric data acquisition, pulmonary vascular enlargement. In cystic bron-
creating contiguous thin sections through the chiectasis, air-fluid levels in thick-walled cysts
lungs with excellent z-axis spatial resolution. are seen, associated with variable degrees of
Similar scanning technique is used for all multide- surrounding consolidations and atelectasis. Based
tector scanners that have 16 detector rows or on the cause and extent of bronchiectasis, the
higher, where a collimation of 0.50 to 1.25 mm is overall lung volume may be increased (Fig. 6).1,2,6
selected. The scans are performed in inspiration CT, especially HRCT, is quite reliable in diag-
and reconstructed as contiguous 1-mm images nosing bronchiectasis. On CT a diagnosis of bron-
or 1-mm-thick images with 10-mm reconstruction chiectasis is made when the internal luminal
interval. Additional advantages are improved diameter of one or more bronchi exceeds the
anatomic matching of airways with regions of air diameter of the adjacent artery. Other diagnostic
trapping during inspiration and expiration and criteria of bronchiectasis are the lack of normal
providing the ability for postprocessing the axial tapering of a bronchus, a visible bronchus abutting
images to create 3-dimensional (3D) assessment the mediastinal pleura, or a visible bronchus within
of the airways.14,15 These postprocessed multipla- 1 cm of the pleura. Signs of bronchiectasis on CT
nar images have become known as volumetric include the signet ring sign, denoted by the artery
HRCT.16 simulating a jewel abutting the ring, the thick-
In our center, we use a 16- or 64-row multidetec- walled dilated bronchus on a transaxial view, and
tor scanner and image the thorax without use of the tram-track sign, from parallel, thickened walls
intravenous contrast during end-inspiration and of a dilated bronchus (Fig. 7).
end-exhalation. Inspiration images are acquired In cylindric bronchiectasis the luminal dilatation
helically and reconstructed as 5 5-mm images is uniform and the wall thickening is smooth. Vari-
and 1 10-mm images; 1 1-mm images are cose bronchiectasis denotes a more severe form
also created in case any mutiplanar or 3D imaging of disease, where the luminal dilatation is charac-
is needed. Exhalation images are also obtained terized by alternating areas of luminal dilatation
helically and reconstructed as 1 10 mm. and constriction, creating a beaded appearance,
When radiation dose is a consideration (espe- and the wall thickening is irregular. In cystic bron-
cially in younger patients), a low-dose technique chiectasis, the most severe form of bronchiec-
of 30 mAs is used in the expiratory phase. A tasis, a dilated, thick-walled bronchus terminates
growing number of authors have advocated the in a thick-walled cyst.18 Oftentimes, more than
Fig. 5. Diffuse bronchiectasis on conventional radiography. Diffuse bronchiectasis can obscure the perihilar vascular
markings, similar to early interstitial pulmonary edema on a pulmonary artery (PA) radiograph (A). Identifying
dilated bronchi on end (short arrow) and tram lines (long arrow) leads to the correct diagnosis of bronchiectasis.
In this case of cystic fibrosis in a 42-year-old man, the findings are best seen on the lateral radiograph (B).
one type of bronchiectasis can be seen in the A potential pitfall in the diagnosis of bronchiec-
same patient (Fig. 8). tasis is the double image of a vessel created by
Smooth bronchial wall thickening is seen in all cardiac or respiratory motion artifact simulating
cases of bronchiectasis, except those caused by a dilated bronchus. This is most common in the lin-
congenital cartilage deficiency (William-Campbell gula and left lower lobe where the effect of cardiac
syndrome, Mounier-Kuhn syndrome) or in allergic motion is most prominent (Fig. 9). Caution should
bronchopulmonary aspergillosis (ABPA). Bronchial be made when diagnosis of bronchial wall thick-
wall thickening alone is a nonspecific finding, as it ening is made on HRCT images that have been re-
is also seen in asthma and chronic bronchitis.6 constructed with a very high frequency algorithm.
Fig. 6. Cystic bronchiectasis on conventional radiography. Cystic changes are seen in both mid and lower lungs
(arrows) on the PA chest radiograph (A) in this 61-year-old woman with chronic Mycobacterium avium intracel-
lulare/complex (MAC) infection. The lateral (B) radiograph demonstrates an air-fluid level in cystic bronchiectasis
of the right middle lobe (arrow).
Bronchiectasis 295
Fig.7. CT findings of diffuse bronchiectasis in a 27-year-old woman with cystic fibrosis. Transaxial images show the
dilated bronchus and adjacent arteriole, seen along their short axis, creating the signet ring sign (arrow) (A).
When the bronchi are visualized along their course, the lack of normal tapering and smooth bronchial wall thick-
ening can be appreciated (B). In cystic fibrosis the fatty attenuation of the pancreas is indicative of the pancreatic
insufficiency (C).
Such algorithm causes the interstitium to appear Mucus plugging of the dilated bronchi and bron-
very thickened and the prominence of the bronchioles appear as branching dense tubular struc-
chial walls results in a ‘‘pseudo-bronchiectasis’’ tures, coursing parallel to, but thicker in diameter,
appearance (Fig. 10). than the adjacent artery and arteriole. These
Fig. 8. CT findings of varicose and cystic bronchiectasis. CT images from the same patient as in Fig. 6 show a more
severe form of bronchiectasis, where both varicose (long arrow) and cystic (short arrow) bronchiectasis is present
(A). In varicose bronchiectasis, the bronchial lumen has a beaded appearance and nodular wall thickening is seen
(arrowhead) (B).
fact, in two retrospective studies evaluating the

etiology of bronchiectasis in large cohorts of
patients with bronchiectasis, almost one third
of the patients were found to have bronchiec-
tasis from prior infection. Idiopathic bronchiec-
tasis, where despite extensive workup the
cause was not found, and bronchiectasis related
to abnormal mucociliary clearance (cystic
fibrosis and primary ciliary dyskinesia) each ac-
counted for almost a quarter of the cases.
ABPA and systemic immunodeficiency, due to
congenital and acquired causes, were the next
most common causes.3,19 Some of the more
commonly encountered etiologies are presented
in the following sections.
Fig. 9. Pseudo-bronchiectasis due to cardiac motion.
The cardiac motion creates a double image, simulating Traction Bronchiectasis
bronchiectasis, usually seen in the left lower lobe and
lingula. The artifactual nature of this observation is Traction bronchiectasis is common in UIP, NSIP,
confirmed by the motion artifact along the posterior and sarcoid and end-stage hypersensitivity
wall of the heart. pneumonitis.20,21
The mechanism of bronchiectasis in fibrotic lung
is based on both physiology and mechanical
plugged bronchi are accompanied by adjacent forces involved in inspiration. Patients with wide-
aggregates of nodules, in a ‘‘tree-in-bud’’ pattern, spread fibrosis require increased inspiratory
consistent with mucus-filled bronchiolectasis in work, leading to a more negative pleural pressure
the center of the secondary pulmonary lobule and therefore a greater transpulmonary pressure
(Fig. 11). during inspiration. On the other hand, pulmonary
Mosaic attenuation and air trapping are fibrosis increases the elastic recoil of the lung,
common associated findings. This is felt to be creating even further expansion of the bronchi
because of coexisting constrictive bronchiolitis in during inspiration.
patients with bronchiectasis. The air trapping In our practice, we find it helpful to compare
may be more pronounced when the bronchiectatic bronchiectasis to the adjacent fibrosis. When the
airway has a component of bronchomalacia bronchiectasis is out of proportion with the adja-
(Fig. 12). cent fibrosis, then NSIP secondary to collagen
vascular disease may be considered. The expla-
BRONCHIECTASIS BASED ON ETIOLOGY nation may stem from bronchial wall injury caused
by collagen vascular disease–related inflammation
A majority of bronchiectasis encountered in clin- or secondary to the high incidence of chronic aspi-
ical practice will be postinfectious in origin. In ration in this subgroup of patients (Fig. 13).
Fig.10. Imaging pitfall. A very sharp reconstruction algorithm (B80f) of the CT image may result in increased noise,
creating the illusion of bronchial wall thickening (A). When the same image is reconstructed with a less sharp
algorithm (B60f), it becomes clear that the bronchiectasis is not real (B).
Bronchiectasis 297
Fig.11. Mucus plugging on CT. On transaxial images, the filling of bronchiectasis by mucus appears as tubular and
branching opacities, with club-like, rounded ends (arrow) (A). The mucus-filled smaller branching bronchi and
bronchioles appear as tree-in-bud opacities (arrowheads) (B). The mosaic attenuation of the surrounding lung
is due to air trapping. In this case, the bronchiectasis was from cystic fibrosis.
Fig.12. Bronchomalacia and bronchiectasis: a 68-year-old man with a reported history of asthma nonresponsive to
steroid therapy. CT images show a normal caliber of the central bronchi (A), and marked collapse of their lumina
when imaged during forced exhalation (B), consistent with bronchomalacia. The distal bronchi are diffusely
dilated and have smooth wall thickening, consistent with mild bronchiectasis (C).
Fig.13. Bronchiectasis out of proportion of surrounding fibrosis. Transaxial CT of the chest of a 34-year-old woman
with scleroderma shows a dilated esophagus, basilar pulmonary fibrosis, and significant bronchiectasis without
evidence of honeycombing (A). The volume-rendered image demonstrates the extent of the bronchiectasis (B)
and the markedly dilated esophagus.
Congenital Airway Wall Abnormality a progression of the bronchiectasis and lung

disease, which, in turn, may result in increased tra-
Congenital defects of the cartilage, collagen, or
cheobronchomegaly (Fig. 14).
other components of the bronchial wall lead to
abnormal physiologic clearing of mucoid
excretions, predisposing the bronchial epithelium CONGENITAL BRONCHIAL ATRESIA
to repeated infections and a vicious cycle of OR MUCOCELE
progressive bronchial dilatation. Structural wall
A focal area of bronchiectasis surrounded by
defect is the common feature of Mounier-Kuhn
lucent lung is typical of congenital bronchial
disease or tracheobronchomegaly, William-
atresia or congenital mucocele. This condition is
Campbell syndrome, and congenital bronchial
characterized by congenital focal obliteration of
atresia.
the lumen of a segmental bronchus, resulting in
focal bronchiectasis and air trapping more distally.
TRACHEOBRONCHOMEGALY (MOUNIER-KUHN The dilated airway is commonly filled by inspis-
DISEASE) sated mucus, which may occasionally calcify.
Congenital bronchial atresia is usually focal, and
Tracheobronchomegaly is an uncommon disease is commonly discovered incidentally, as an ovoid,
that presents mostly in men, in the fourth and fifth tubular, or branching density on a chest radio-
decades. Although believed to be congenital, it graph. It may be confused with a pulmonary
may be associated with Ehlers-Danlos syndrome, nodule. CT reveals its bronchial, branching nature,
Marfan syndrome, and generalized elastosis (cutis and the presence of surrounding and more distal
laxa). Pathological thinning of the muscle, carti- hyperexpanded and hyperlucent lung paren-
lage, and elastic tissue of the airway walls is chyma, due to the associated air trapping (Fig. 15).
seen. This results in uniform dilatation of the Conversely, acquired mucocele is caused by
tracheal and bronchial lumina and increased focal scarring of a segmental bronchus because
distensibility of the tracheal and bronchial walls. of prior granulomatous infection or from an endo-
This tracheobronchomalacia leads to recurrent bronchial lesion. It should be differentiated from
infections in the dependent lungs.6 congenital bronchial atresia by the absence of air
The disease involves the entire trachea and trapping of the distal lung parenchyma.24,25 The
bronchi of first to fourth order. On imaging, presence of an acquired mucocele should prompt
a tracheal diameter exceeding 3 cm in both coronal further interrogation to exclude the possibility of an
and sagittal planes and central bronchiectasis is endobronchial neoplasm (Fig. 16).
seen without associated airway wall thickening.
More distal bronchiectasis, bronchial wall thick- WILLIAMS-CAMPBELL SYNDROME
ening, occasionally fibrosis, and cystic changes in
the lower lobes are common because of sequela Williams-Campbell syndrome is a rare disease in
of repeated pneumonia.22,23 The net effect is which the cartilage of the fourth-, fifth-, and
Bronchiectasis 299
Fig. 14. Mounier-Kuhn Syndrome. Transaxial CT images show tracheomegaly (A), and basilar-predominant vari-
cose and cystic bronchiectasis (B) seen in Mounier-Kuhn syndrome. The coronal reconstructed image demon-
strates the typical corrugated appearance of the tracheal wall (C).
Fig. 15. Congenital bronchial atresia: a 60-year-old woman with pleuritic chest pain. CT with contrast (A) shows
a nodular density in the left lower lobe with surrounding hyperlucency of the lung parenchyma, due to focal
air trapping. Coronal reconstruction (B) better demonstrates the tubular nature of that structure, which connects
to a dilated bronchus. These features (mucocele with surrounding air trapping) are diagnostic for this condition.
especially mycobacterium avium intracellulare-

complex (MAC), are increasingly being recognized
as the cause of chronic lung infection in adults with
normal immunity and no underlying lung disease,
especially older women. In immunocompetent
people, MAC has three different forms: a fibrocavi-
tary form, a nodular bronchiectatic form, and
hypersensitivity pneumonitis.
The fibrocavitary form, similar to postprimary
tuberculosis, involves the apices and upper lobes
and causes traction bronchiectasis in the affected
lung. It occurs mostly in older men with emphy-
sema. The nodular bronchiectasis form represents
a slowly progressive disease, often resistant to
treatment and more common in older women.
Fig. 16. Acquired mucocele. CT image through the On CT and HRCT imaging it appears as multiple
right upper lobe in this 68-year-old man reveals clusters of centrilobular micronodules, in a branch-
a branching tubular structure representing a mucus- ing or tree-in-bud pattern, aggregating around air-
filed dilated subsegmental bronchus or mucocele. or mucus-filled cylindric bronchiectasis and bron-
The lack of surrounding air trapping suggests that it chiolectasis. Multifocal consolidations and cavities
is not congenital and likely because of an acquired can occur. There is associated mosaic attenuation
obstruction of the bronchus. In this case, it was from and air trapping. The disease has a predilection for
a small squamous cell cancer that was not seen before
the right middle lobe, upper lobes, and lingula, but
surgery.
involvement of other lobes may also be seen
(Fig. 17).28–30 Scarring and traction bronchiectasis
sixth-generation bronchi is defective. The disease of the right middle lobe and lingula are indicative of
may involve the lung focally or diffusely.26 The long-standing disease (Fig. 18).
congenital form presents in childhood and is
commonly associated with congenital heart Mucociliary Clearance Abnormalities
disease, polysplenia, bronchial isomerism, and The ciliary ladder is responsible for effective
situs inversus. The acquired form is likely a sequela clearing of mucoid excretions of the airway epithe-
of prior adenovirus (measles and pertussis) infec- lium. Abnormalities in the consistency of mucus in
tions.27 CT imaging shows cystic bronchiectasis
distal to the third-generation bronchi, and inspira-
tory-expiratory CT imaging reveals ballooning on
inspiration and collapse on exhalation.1
Acquired Wall Abnormalities

Chronic or past infections, inhalational injury, and
cellular infiltration in the setting of graft versus
host disease lead to inflammation of the bronchial
wall, resulting in structural damage of the bron-
chial wall. This leads to irreversible bronchial dila-
tation and increased mucus production, leading to
a vicious cycle of inflammation and wall damage.
ATYPICAL OR NONTUBERCULOSIS
MYCOBACTERIA Fig. 17. Mycobacterium avium intracellulare/complex
(MAC) infection: a 68-year-old Chinese woman who
Atypical or nontuberculosis mycobacterial pulmo-
developed mild cough and intermittent hemoptysis
nary infection was previously considered to occur
after a trip to China, which did not respond to routine
only in adults with chronic lung disease, such as antibiotic therapy. CT shows extensive micronodules,
CF, lung cancer, or emphysema; adults with mostly in the right middle lobe, right lower lobe,
impaired immunity, especially acquired immuno- and lingula, which aggregate in a tree-in-bud pattern
deficiency syndrome (AIDS), or those with thoracic around the mildly dilated subsegmental bronchi
skeletal abnormalities. These organisms, however, (arrow). Sputum specimen was positive for MAC.
Bronchiectasis 301
Fig. 18. Bronchial artery collateral formation in bronchiectasis: a 76-year-old woman with chronic productive
cough has new-onset of severe, recurrent hemoptysis. CT of chest with intravenous contrast shows severe bron-
chiectasis and scarring of the right middle lobe (A). The dilated bronchial arteries (white arrow) providing collat-
eral flow to the lungs are better demonstrated on the coronal thin-MIP (6 mm) reformatted image (B).
cystic fibrosis, and abnormalities of the structure CYSTIC FIBROSIS

and function of the cilia of the airway epithelium,
as seen in primary ciliary dyskinesia or immotile CF is an autosomal recessive trait and occurs in
ciliary syndrome, leads to ineffective mucus clear- approximately 1 in 3000 live births in the United
ance and secondary colonization of the airway States and Europe. It is caused by a mutation in
lumina by bacteria. This chronic infection and the CF transmembrane conductance regulator
repeated bouts of pneumonia lead to bronchiec- (CFTR). This results in failed secretion of chloride
tasis. The bronchiectasis of cystic fibrosis is typi- through the CFTR and associated ion channels,
cally worse in the upper lobes, as opposed to leading to dehydration of the endobronchial
primary and acquired ciliary dyskinesia, such as secretions. This thickened mucus cannot be effi-
Young syndrome where bronchiectasis is associ- ciently cleared by the mucociliary system, leading
ated with azospermia, where the bronchiectasis to obstructed airways and bacterial infection.4
is worse in the dependant or lower lungs. Colonization and recurrent infection with
Fig. 19. Initial diagnosis of cystic fibrosis late in life. A 68-year-old woman had undergone left lower lobectomy
more than 35 years ago because of recurrent pneumonia of the left lower lobe. Recent dyspnea on exertion
and wheezing prompted further workup, which revealed a sweat chloride 5 81 mmol/L (normal <40 mmol/L).
Coronal reformatted image of HRCT of chest (A) shows severe bronchiectasis of the left upper lobe, and relatively
mild bronchiectasis throughout the right lung. Transaxial nonenhanced CT of abdomen does not show fatty
pancreatic parenchyma (B).
Staphylococcus aureus, Haemophilus influenza, thickening. Extensive mucus plugging of the

and Pseudomonas aeruginosa is common, leading dilated bronchi and bronchioles manifests as cen-
to progression of airway destruction. A poor trilobular nodules and branching densities (see
prognosis is made when atypical mycobacteria Fig. 7).1 In early or mild forms of CF, these findings
or Burkholderia cepacia colonize the dilated may be confined to the right upper lobe.6 In adults
airways.6 with long-standing diffuse disease, the findings are
Although CF is usually diagnosed in childhood, widespread throughout the lungs and the upper
the heterogeneity of severity of disease leads to lobes may be completely scarred and collapsed
patients with milder disease, first diagnosed in with associated traction bronchiectasis. This lobar
adulthood. As genetic testing improves and scarring is due to chronicity of disease where the
awareness of CF increases, milder forms are bronchiectasis has been most severe and present
increasingly being detected later in adulthood. In longest. Despite the upper lobe volume loss, the
fact, at our institution, the oldest first-time diag- lungs remain hyperinflated.
nosis of CF was in a 72-year-old woman with Typically patients with CF have pancreatic insuf-
mild bronchiectasis. Sweat chloride test greater ficiency and on CT the pancreas has a homoge-
than 40 mmol/L indicates the presence of nous fat attenuation. However, in cases of
disease. a milder mutation, which leads to an initial diag-
CT findings include diffuse cylindric, varicose, or nosis later in life, the pancreatic insufficiency is
even cystic bronchiectasis, bronchial wall commonly absent (Fig. 19).31
Fig. 20. Kartagener syndrome. Transaxial CT of chest (A) in a 12-year-old boy with Kartagener Syndrome shows
dextrocardia and bronchiectasis of the lower lobes and scarring of the left middle lobe caused by more severe
bronchiectasis and repeated pneumonia. Nonenhanced CT of the paranasal sinuses (B) show marked mucosal
thickening and opacification of the maxillary sinuses. The transaxial nonenhanced CT image of the upper
abdomen (C) demonstrates the situs inversus associated with this syndrome.
Bronchiectasis 303
Fig. 21. Primary ciliary dyskinesia: a 37-year-old woman with primary ciliary dyskinesia and chronic pseudomonas
aeruginosa infection being evaluated for bilateral lung transplantation. HRCT of chest (A) shows mixed tubular,
varicose, and cystic bronchiectasis. Minimal intensity reformatted images in axial (B) and sagittal (C) planes and
volume-rendered image (D) demonstrate the extent of bronchiectasis and the associated mosaic attenuation.
PRIMARY CILIARY DYSKINESIA PCD tends to be diagnosed relatively late,

because of its nonspecific presenting symptoms
Primary ciliary dyskinesia (PCD) or immotile ciliary in children. Clinical suspicion based on a focused
syndrome is caused by a defect in structure and history leads to diagnosis. The current diagnostic
function of the airway cilia. This leads to impaired test of choice is electron microscopic analysis of
mucociliary clearance.32 It is a genetically hetero- respiratory cilia in samples of nasal or airway
geneous, autosomal recessive trait with a preva- mucosa. This reveals defects in the outer or inner
lence of approximately 1 in 15,000 to 1 in 30,000 dynein arms of the cilia.33
of live births. Patients present with recurrent infec- The CT findings of PCD are bronchiectasis of
tions of the lungs, sinuses, and middle ear. Similar variable severity, associated with tree-in-bud
to CF, PCD causes progressive bronchiectasis. nodules and branching densities because of
Thoracoabdominal asymmetry occurs in approxi- mucus plugging, and lobar scarring and air trap-
mately 50% of patients. Kartagener syndrome or ping (Fig. 21). Bronchiectasis in PCD is predomi-
triad is present in half of the PCD patients. This nantly in the lingual and middle and lower lobes
triad consists of situs inversus, bronchiectasis, of the lung. Isolated upper lobe involvement and
and sinusitis (Fig. 20).33,34 isolated peripheral bronchiectasis is very rare.
Fig. 22. Allergic bronchopulmonary aspergillosis (ABPA). HRCT of the chest in a 55-year-old woman with history of
reactive airway disease at the level of the apices (A) and lung bases (B) show dilated, thick-walled bronchi. The
bronchiectasis is worse in the apices where mucus plugging is seen. Coronal (C) and sagittal (D) reformatted
images better demonstrate the distribution of the bronchiectasis.
Hyper-Immune Response may cough up brown mucus plugs. Clinical diag-

nosis could be made by detecting an elevated
Inflammatory bowel disease, rheumatoid arthritis,
serum IgE level, eosinophilia on peripheral blood
Sjogren disease, antineutrophilic cytoplasmic anti-
smears, or positive skin reaction to Aspergillus
body (c-ANCA)–positive vasculitis (Wegener
antigen. Mycelia can occasionally be identified in
disease), and allergic bronchopulmonary aspergil-
the exporated mucus plugs.
losis all can lead to bronchiectasis, possibly
Chest radiographs and CT show migratory pneu-
because of inflammation of the airway wall in the
monitis early in the disease. This usually involves
setting of a hyperimmune response to internal or
the upper lobes. Central and upper lobe bronchiec-
external antigens. The chronic inflammation
tasis, varicose or cystic subtypes, is best detected
damages the bronchial walls, leading to
by CT, which also helps monitor progression and
bronchiectasis.
response to treatment. In more chronic disease,
inspissated mucus in the dilated central bronchi
ALLERGIC BRONCHOPULMONARY ASPERGILLOSIS created the appearance of the classic finger-
Allergic brochopulmonary aspergillosis (ABPA) is in-glove appearance on the chest radiographs
due to a hypersensitivity reaction to Aspergillus (Fig. 22). Atelectasis or hyperinflation of the lung
fumigatis antigens, leading to development of distal to the impacted bronchi can occur.18,35
bronchocentric granulomata in the bronchi and
bronchioles, associated with mucus impaction.35 SUMMARY
It is most commonly seen in patients with atopic
rhinitis, asthma, or CF.36 Patients present with Bronchiectasis, or the irreversible dilatation of
wheezing, fever, and pleuritic chest pain and bronchi, can present with a host of nonspecific
Bronchiectasis 305
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and hypoxia. The radiologist, then, can play an chiectasis: clinical and HRCT evaluation. Pediatr Pul-
important role in its detection and characterization. monol 2003;35:477–83.
Bronchiectasis must be differentiated from motion 10. Eshed I, Minski I, Katz R, et al. Bronchiectasis:
artifact and transient bronchial dilatation in acute correlation of high-resolution CT findings with
lung disease. When diagnosed, a logical approach health-related quality of life. Clin Radiol 2007;62:
may allow for proper triage of the patient to prevent 152–9.
progression of disease. 11. Martınez-Garcıa MA, Soler-Cataluna JJ, Perpina-
The radiologic approach usually begins with CT, Tordera M, et al. Factors associated with lung
which is fast and accurate. The diagnostic function decline in adult patients with stable non-
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Imaging of Small
Airway Disease ( SAD)
Sudhakar N.J. Pipavath, MBBS, Eric J. Stern, MD*
KEYWORDS
Small airways disease Bronchiolitis
High resolution computed tomography
Follicular bronchiolitis Panbronchiolitis
DEFINITION branches; the alveolar ducts, sacs, and alveoli

succeed them. The respiratory bronchiole is both
Small airways are generally considered synony- a conducting and gas exchange unit. The acinus
mous with those airways at the bronchiolar level is defined as a unit of the lung that is distal to the
and beyond, have a luminal diameter of less than terminal bronchiole, and it typically measures
1 to 2 mm, and contain no cartilage in their walls. 7 mm in diameter.
Although distinct, small airways disease (SAD)
and bronchiolitis are often used interchangeably.
The two types of bronchioles—membranous and APPROACH TO EVALUATING AND DIAGNOSING
respiratory—have distinct histology and function.1 SMALL AIRWAY DISEASE
Membranous bronchioles are conducting airways,
and respiratory bronchioles are noted to have Imaging signs of SAD can be direct or indirect. Two
alveoli and alveolar ducts. common direct signs are (1) centrilobular branching
nodules that often appear as V- or Y-shaped and
sometimes are referred to as ‘‘tree in bud’’ opaci-
ANATOMY OF THE SECONDARY ties (Fig. 2)3 and (2) centrilobular or peribronchiolar
PULMONARY LOBULE ground glass opacities. A less common direct sign
includes bronchiolectasis, a feature seen only rela-
Understanding the anatomy of the secondary tively late and with chronic forms of SAD.
pulmonary lobule is essential for understanding Air trapping is an indirect sign of bronchiolar
SAD successfully. The secondary pulmonary disease. In the clinical setting of actively inflamed
lobule (Fig. 1) is the smallest, irregular/polyhedral- bronchioles, inflammation or mucous plugging
shaped unit of the lung marginated by connective leads to premature closure or complete obstruc-
tissue.2 The center of the lobule is supplied by tion, with air trapping as a consequence.4 In the
a paired bronchiole and pulmonary arteriole setting of mature obliterative (constrictive) bron-
branch, and the secondary pulmonary lobule is chiolitis, the airway lumen is narrowed or obliter-
marginated by connective tissue—the interlobular ated, and the lobule is ventilated by collateral
septa, which contains pulmonary venules and drift. It is not an issue of premature closure as in
lymphatic channels. The preterminal bronchiole the setting of acute inflammation; the closure is
supplies the secondary pulmonary lobule, also typically fixed or permanent. To diagnose air trap-
called the lobular bronchiole, which gives rise ping on CT, expiratory images are essential.5
to approximately three terminal bronchioles. Although lobular and segmental air trapping is
The terminal bronchioles end in respiratory bron- appreciable on inspiratory scans, lobar air trapping
chioles. Respiratory bronchioles have three order often requires expiratory scans performed with
Department of Radiology, University of Washington Medical Center, 1959 NE Pacific Street, # 357115, Seattle,
WA, USA
E-mail address: [email protected] (E. J. Stern).
Radiol Clin N Am 47 (2009) 307–316

doi:10.1016/j.rcl.2009.01.002
308 Pipavath & Stern
Fig. 1. Secondary pulmonary lobule. Line drawing with schematic depiction of secondary pulmonary lobule
anatomy. (Courtesy of the University of Washington, Seattle, WA; with permission. Copyright ª 2009 University
of Washington.)
good patient effort. The most important confounder INFLAMMATORY BRONCHIOLITIS

in diagnosing air trapping that results from SAD is Infectious Bronchiolitis
the similar pulmonary mosaic attenuation pattern
Acute infections can cause inflammation of the
that results from pulmonary vascular disease. In
small airways and a clinically severe form of lung
other words, the mosaic attenuation pattern that
dysfunction. Pathologically, epithelial necrosis,
results from SAD occurs in combination with the
inflammation of the bronchiolar walls, and
primary airway constriction and secondary hypoxic
vasoconstriction. This is in contrast to the similar
pattern of primary pulmonary vascular disease
and associated secondary hypoxic bronchiolar
constriction. Air trapping can be seen as a result
of primary pulmonary vascular disease,6 and
possibly the original concept of being able to differ-
entiate these two entities depends more on the
associated abnormalities.
In the evaluation of patients who are suspected
of having SAD, one should take into consideration
the fact that some mild air trapping, mostly lobular,
can be seen in otherwise normal healthy,
nonsmoking individuals7 and even in asymptom- Fig. 2. Kartgener’s syndrome. Axial HRCT images
atic, otherwise healthy cigarette smokers. The demonstrate fine centrilobular nodules and tree in
extent of air trapping and associated clinical bud pattern in the lower lobes with cystic and varicose
symptomatology helps in decision making. bronchiectasis.
Table 1
Classification, causes, imaging features and differential diagnosis of SAD
Type Based on Imaging Prototype Cause

Category or Pathologic Pattern and Other Causes Imaging Features (HRCT) Differential Diagnosis
Inflammatory
— Infectious Viruses, mycoplasma, Centrilobular nodules, tree in Hypersensitivity pneumonitis,
mycobacteria bud pattern, bronchial wall aspiration (in lower lung
thickening, ground glass distribution)
abnormality in a patchy
distribution
Respiratory bronchiolitis Cigarette smoking Centrilobular nodules and patchy Hypersensitivity pneumonitis,
ground glass opacities in an pulmonary neovascularity in
upper lung distribution Eisenmenger’s syndrome27
Follicular bronchiolitis Rheumatoid arthritis and Lower lung dominant Asian panbronchiolitis,
Sjögren’s syndrome centrilobular and immunodeficiency syndromes,
peribronchiolar nodules, infectious bronchiolitis
bronchiolectasis and
bronchiectasis
Asian panbronchiolitis Idiopathic, relatively exclusive Lower lung bronchiectasis, Follicular bronchiolitis,
Imaging of Small Airways Disease

in patients of Asian origin bronchiolectasis, centrilobular immunodeficiency syndromes,
nodules and infectious bronchiolitis
Fibrotic bronchiolitis
— Constrictive or obliterative Postinfectious, Swyer-James Patchy air trapping, Differential perfusion from
bronchiolitis Macleod syndrome, toxic fume bronchiectasis, and pulmonary hypertension
inhalation bronchiolectasis
Obliterative bronchiolitis in Postlung transplantation, chronic Lower lung dominant mosaic Postinfectious obliterative
postlung or hematopoietic graft-versus-host disease attenuation and air trapping bronchiolitis
stem cell transplant population with diminished vascularity
309
intraluminal exudation are seen in this setting. so-called ‘‘Lady Windermere syndrome.’’9 Coloni-
Acute viral infections are by far the most common zation or superinfection of bronchiectatic airways
infections to cause bronchitis and bronchiolitis. with bacterial or nontuberculous mycobacteria
Although in children infectious bronchiolitis most cannot be differentiated from isolated infectious
frequently is the result of acute viral infections, in bronchiolitis alone (Table 1).
adults, viruses, mycoplasma, and bacteria are all
Smoking-related Small Airways Disease
equally responsible for acute infectious bronchioli-
tis. More chronic infections and nontuberculous Centrilobular emphysema, which falls under the
and tuberculous mycobacterial infections can broad category of chronic obstructive pulmonary
cause bronchiolitis. In immunosuppressed disease, is not discussed in this article. This section
patients, causes for infectious bronchiolitis can focuses on respiratory bronchiolitis and respiratory
include Aspergillus sp. cytomegalovirus, respira- bronchiolitis–associated interstitial lung disease
tory syncitial virus, and Pseudomonas aeruginosa. (RB/RB-ILD). RB is mostly an asymptomatic condi-
Normal bronchioles at routine thin section tion seen in smokers. RB-ILD, however, is a form of
imaging are not typically visible. When they interstitial pneumonia associated with RB. As the
become acutely or subacutely inflamed or filled name suggests, cigarette smoking causes inflam-
with inflammatory exudate, they are much more mation in and around the respiratory bronchioles.
likely to be visible4 and demonstrate distinct Hemosiderin-like pigment-laden macrophage
sharply defined centrilobular nodules, centrilobu- accumulation within the respiratory bronchioles at
lar nodules with a ‘‘tree in bud’’ pattern, bronchi- pathology and ill-defined centrilobular nodules
olar wall thickening, or indistinct, more ground and, occasionally, centrilobular ground glass opac-
glass–appearing centrilobular nodules, all with or ities at high resolution CT (HRCT) are common.
without associated air trapping.8 The secondary RB-ILD tends to have more dominant interstitial
or indirect finding of air trapping occurs less inflammation and accumulation of hemosiderin-
frequently in acute infectious bronchiolitis. like pigment-laden macrophages in the alveolar
A feature that the authors have found useful in spaces in addition to the respiratory bronchioles.
suggesting infectious etiology is the patchy distri- At HRCT, RB-ILD manifests as dominant ground
bution (Fig. 3). Noninfectious inflammatory glass abnormality with centrilobular nodules
bronchiolitis often has a more uniform and mostly (Fig. 6). Air trapping is not a common feature. RB
bilateral or symmetric involvement (eg, in patients and RB-ILD tend to have upper lung predomi-
with Asian panbronchiolitis or follicular bronchioli- nance.10 Imaging differential diagnosis for RB
tis). Associated areas of nonspecific ground glass includes infectious bronchiolitis and hypersensi-
opacities or even areas of focal or more dense tivity pneumonitis. Hypersensitivity pneumonitis,
consolidation may be seen in patients with infec- desquamative interstitial pneumonia, and nonspe-
tious bronchiolitis. More extensive or chronic cific interstitial pneumonitis are the imaging differ-
airway infections are much more likely to have ential considerations for RB-ILD. Desquamative
associated bronchiectasis or bronchiolectasis interstitial pneumonia has lower and peripheral
(Fig. 4). If the pattern of bronchiolitis involves lung dominant ground glass opacity. Well-defined
predominantly the right middle lobe and lingula, cysts may be present within areas of ground glass
one should strongly consider mycobacterium opacity, a less sensitive but relatively more specific
avium intracellulare infection (Fig. 5), clinically the feature. Desquamative interstitial pneumonia
Fig. 3. Infectious bronchiolitis. A 37-year-old woman before matched unrelated peripheral blood stem cell trans-
plant for AML, for a second transplant after relapsing after the first unrelated transplant. Axial HRCT (A) and MIP
(B) images demonstrate patchy centrilobular nodules and tree in bud pattern, presumed to be bacterial infection.
Imaging of Small Airways Disease 311
Fig. 4. Reactivation tuberculosis (Tb) with endobronchial spread of disease. (A) Thin collimation axial CT image and
(B) MIP reformat demonstrate patchy centrilobular nodules and tree in bud appearance (arrows) in lower lobes and
lingula. Bronchiectasis/bronchiolectasis is present in left lower lobe. (Courtesy of C. Beigelman-Aubry, MD, Paris,
France.)
characteristically lacks centrilobular nodules that are dominant features,12 whereas ground glass
are otherwise common in RB-ILD. opacity is uncommon. A more common form of
Hypersensitivity pneumonitis has imaging bronchiolitis seen in patients with rheumatoid
features similar to RB and RB-ILD. Both demon- arthritis is constrictive bronchiolitis (discussed in
strate ill-defined centrilobular opacities and more detail later). There is also some association
ground glass opacity (Fig. 7). Patients who between treatment of rheumatoid arthritis with
develop hypersensitivity pneumonitis are usually D-penicillamine therapy and occurrence of
nonsmokers, however, and smokers are thought constrictive bronchiolitis.
to have some protective effect from developing
hypersensitivity pneumonitis.11 FIBROTIC /CONSTRICTIVE BRONCHIOLITIS
Constrictive bronchiolitis, which is also known as
Follicular Bronchiolitis
obliterative bronchiolitis, is a category of disorders
This is a form of bronchiolitis that results from recognized by a pattern of peribronchiolar fibrosis
hyperplasia of the lymphoid tissue around and resulting in complete cicatrization of bronchiolar
along the small airways/bronchioles. It is classically lumen.1 Although most commonly idiopathic,
associated with collagen vascular disease, known causes include infections, toxic fume inha-
specifically rheumatoid arthritis and Sjögren’s lation (oxides of nitrogen, chlorine), autoimmune
syndrome. The lymphocytes are polyclonal on disorders, including rheumatoid arthritis,
immunohistochemistry. On HRCT, centrilobular graft-versus-host disease, lung transplantation,
nodules, tree in bud, and peribronchial nodules inflammatory bowel disease,13 and drug reactions,
Fig. 5. Postinfectious bronchiectasis with bronchiolitis from super-added infection or colonization by mycobacte-
rium avium intracellulare. Axial thin section CT demonstrates multiple centrilobular nodules in the right upper
lobe and bronchiectasis in right middle lobe, lingual, and the left lower lobe.
Fig. 6. Respiratory bronchiolitis from smoking. (A) Thin axial image and (B) coronal MIP reconstruction demon-
strate multiple centrilobular ground glass opacities (arrow, curved arrow). (Courtesy of C. Beigelman-Aubry,
MD, Paris, France.)
such as can be seen with D-penicillamine therapy. Postinfectious Constrictive Bronchiolitis

Direct CT signs of disease in and around airways
Pulmonary infections with agents such as adeno-
are usually absent in this form of bronchiolitis.
virus, measles, pertussis, mycoplasma, and tuber-
Bronchiectasis of larger airways can be associ-
culosis can cause postinfectious bronchiolitis.14
ated. Air trapping as an indirect finding of airway
Postinfectious constrictive bronchiolitis also
narrowing/obliteration is the most common and
demonstrates mosaic attenuation/air trapping
identifying imaging feature of this broad category
and rarely bronchiectasis or centrilobular nodules.
of bronchiolitis.
As discussed earlier, infections and postinfe
ctious constrictive bronchiolitis have a patchy
distribution.15
Swyer-James or Macleod Syndrome

Swyer-James (or MacLeod) syndrome, a long-
term complication of postinfectious constrictive
bronchiolitis that occurs in childhood, is the devel-
opment of unilateral hyperlucent lung with
evidence of air trapping and decreased vascu-
larity. Alveolar maturation occurs in children by
the age of 8. Children who have infectious bron-
chiolitis before this age tend to either heal
completely or heal with fibrosis, which affects
alveolar maturation and results in a decrease in
the number of alveoli and pulmonary vessels.
Imaging of Swyer-James syndrome manifests as
asymmetric patchy lobar or lobular air trapping
that affects large areas. Originally this was thought
to be unilateral and unilobar,16 the advent of CT
has made it increasingly clear that bilateral
involvement is a rule rather than exception. Air
trapping with diminished vascularity (Fig. 8), bron-
chiectasis, or hypoplastic lobe or lung is another
typical feature.17
Fig. 7. Hypersensitivity pneumonitis. Axial thin section Noxious Fume Exposure

chest CT demonstrates multiple ill-defined centrilobu-
lar opacities (arrow). The image on the right from Exposure to various toxic fumes has been related
another patient demonstrates somewhat more to subsequent development of constrictive bron-
discrete centrilobular nodules. chiolitis. Initially some of these fumes may cause
Fig. 8. (A) HRCT of the chest demonstrates asymmetric lobular air trapping with diminished vascularity seen in the
lungs bilaterally from Swyer-James-Macleod syndrome. (B) Distribution of abnormality is better appreciated on
the coronal reformatted image. (Courtesy of J.D. Godwin, MD, Seattle, WA.)
acute inflammatory disease. Imaging features are fibrosis occurs as the healing progresses in 2 to 6
similar to other causes of constrictive bronchiolitis. weeks.
Silo filler’s lung from nitrogen dioxide exposure18
and popcorn flavor manufacturing lung from di-
Transplant-Related Bronchiolitis
acetyl exposure19 are known to cause constrictive
Obliterative Syndrome
bronchiolitis. Post–nitrogen dioxide exposure
bronchiolitis results from exuberant healing phase Bronchiolitis obliterative syndrome can be defined
with formation of granulation tissue that accumu- as graft deterioration secondary to persistent
lates at the site of the previous bronchiolar injury. airflow obstruction. Diagnosis does not neces-
Widespread obstruction and obliteration of small sarily require histologic confirmation; in contrast,
airways with or without associated peribronchiolar the term ‘‘bronchiolitis obliterans’’ is used for
a histologically proven diagnosis. This histologic Hyperpolarized helium (3He) MRI is an upcoming
diagnosis is restricted anatomically to the tool that offers morphologic and functional assess-
membranous and respiratory bronchioles and ment of lung ventilation and air trapping. It has been
requires the presence of eosinophilic fibrous scar- studied in post lung transplant patients. 3He MRI
ring of the wall of these small conducting airways has high spatial and temporal resolution compared
with partial or complete obliteration of the lumen.20 with thin section CT, proton MRI, and radionuclide
In advanced cases there is bronchial wall thick- ventilation scan. The dynamic distribution of venti-
ening and bronchiectasis. lation during continuous breathing after inhalation
At imaging, the presence of more than 32% air of a single breath of 3He gas demonstrates homo-
trapping has 87.5% sensitivity and specificity for geneous and fast distribution in normal individuals.
the diagnosis of bronchiolitis obliterative In patients with air trapping, irregular and delayed
syndrome, and in some patients this precedes the patterns with redistribution are seen.25
spirometric criteria for bronchiolitis obliterative
syndrome.21 Conversely, having less than 32% of
air trapping has a high negative predictive value
ASIAN DIFFUSE PANBRONCHIOLITIS
until the fifth postoperative year. In another, smaller
study, an air-trapping score provided a sensitivity Diffuse panbronchiolitis is an idiopathic chronic
of 74% and a specificity of 67% for histopatholog- bronchiolitis seen most often in patients of Asian
ically proven obliterative bronchiolitis.22 A more origin but is not limited to them. Imaging findings
recent study, however, indicated that the value of include centrilobular nodules, tree-in-bud, bron-
the finding of air trapping in early stages of bronchiolectasis, bronchiectasis in a lower lobe with
chiolitis obliterative syndrome is lower than was re- dominant distribution, which affects both the lower
ported previously. The role of thin section CT as lobes (Fig. 10). Not surprisingly, mosaic attenua-
a screening test to evaluate patients with lung tion may be seen on expiratory images.26 Nonspe-
transplants was questioned.23 Air trapping and, cific patchy consolidation, mucoid impaction, and
occasionally in advanced cases, bronchial wall segmental atelectasis are some other imaging
thickening and bronchiectasis are seen (Fig. 9).24 features.15
Fig. 9. HRCT of the chest demonstrates mosaic attenuation on inspiratory scans (A, B), with accentuation of the
dark areas on expiratory scans (C, D) indicating severe air trapping in a patient with obliterative bronchiolitis
from chronic graft-versus-host disease.
Fig. 10. Asian panbronchiolitis. HRCT images demonstrate bilateral relatively symmetric, lower lung dominant
cylindrical bronchiectasis, bronchiolectasis, mucus plugging, and centrilobular nodules. (Courtesy of S. Young
Kim, MD, Seoul, South Korea).
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ACKNOWLEDGMENTS
computed tomography and correlation with pulmo-
The authors thank Professor J.D. Godwin for his nary function tests. J Comput Assist Tomogr 2003;
guidance and support and Drs Catherine Beigel- 27(5):735–42.
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Asthma : An Imaging
Update
Alyn Q.Woods, MDa,b,*, David A. Lynch, MBa
KEYWORDS
Asthma Radiology CT MRI Imaging
Synchrotron radiation Hyperpolarized 3He
The incidence of asthma continues to increase and subacute exacerbations being driven by envi-
worldwide, and in 2005 approximately 7.7% of ronmental triggers, including allergens, microor-
the population of the United States (or 22.2 million ganisms, and pollutants.
individuals) carried the diagnosis.1 Significant Rather than being a single disease entity,
recent advances in imaging of asthma have asthma seems to consist of related, overl-
yielded both a better understanding of the under- apping syndromes.4 Clinically important pheno-
lying pathophysiology as well as more effective types of asthma include allergic asthma,
assistance in guiding appropriate clinical therapy. aspirin-sensitive asthma, glucocorticoid-resistant
asthma, and asthma with fixed airflow limitation.4
ASTHMA DEFINED Additionally, obese individuals may have a specific
asthma phenotype, characterized by greater
Asthma is characterized by all of the following:2 severity and poorer control with medications.5
More recently, there is a suggestion that there is
(1) airways obstruction that is usually reversible
a phenotype of severe asthma characterized by
(2) chronic airway inflammation, and
air trapping visible on CT.6
(3) nonspecific airways hyperreactivity.
Asthma may occur at any age, but most patients
The clinical diagnosis of asthma is most with asthma experience their first symptoms before
commonly made by documenting physiologic age 5.4 Most early-onset asthma has an allergic
airway obstruction that improves following admin- component.
istration of bronchodilator. In more occult cases, Morphologically, asthma is characterized by
asthma may be diagnosed by the presence of airway remodeling, with infiltration of the airway
airway hyperreactivity to an inhaled substance, wall by inflammatory cells, deposition of connec-
most commonly methacholine. tive tissue, increase in smooth muscle mass,
Beyond this basic definition, it is increasingly vascular changes, and hypertrophied mucous
clear that asthma is heterogeneous with regard to glands.7–9 The airway remodeling of asthma is
its immunopathology, clinical phenotypes, natural present by the age of 3.10
history, and response to treatment. It was once Asthma manifests clinically as periodic wheezing
considered to be an allergic disorder mediated by and shortness of breath. On physiologic evaluation,
Th2-type lymphocytes, IgE, mast cells, eosino- the forced expiratory lung volume in 1 second
phils, macrophages, and cytokines. However, it is (FEV1) is reduced below 80% of normal, and the
now evident that the pathophysiology of asthma ratio of FEV1 to forced vital capacity (FVC) is less
also involves local factors such as local epithelial, than 70%. The ratio of lung residual volume to the
mesenchymal, vascular, and neural structures.3 total lung capacity (RV/TLC) is increased. The clin-
These structural cells contribute to the develop- ical diagnosis of asthma is most commonly made
ment of a chronic asthma phenotype, with acute by documenting physiologic airway obstruction
a
Division of Radiology, National Jewish Health, 1400 Jackson Street, Denver, CO 80206, USA
b
University of Colorado Denver, Radiology Academic Office, Mail Stop 8200, Building L15, Room 2414, 12631
East 17th Avenue, PO Box 6511, Aurora, CO 80045, USA
* Corresponding author. University of Colorado Denver, Radiology Academic Office, Mail Stop 8200, Building
L15, Room 2414, 12631 East 17th Avenue, PO Box 6511, Aurora, CO 80045, USA.
E-mail address: [email protected] (A.Q. Woods).
Radiol Clin N Am 47 (2009) 317–329

doi:10.1016/j.rcl.2008.11.008
318 Woods & Lynch
that reverses with bronchodilators. In more occult radiologic identification of signs of esophageal re-
cases, asthma may be diagnosed by the metha- flux such as esophageal wall thickening18 may be
choline challenge test: FEV1 is measured after helpful in asthmatic patients (Fig. 2).
inhalation of progressively increasing concentra-
tions of methacholine to identify bronchial
hyperreactivity.11
IMAGING FEATURES
CONDITIONS ASSOCIATED WITH ASTHMA Chest Radiograph
Chronic rhinosinusitis has a well-established asso- The radiographic features of asthma are not
ciation with asthma12 and the greater the degree of particularly specific, but the most common abnor-
mucosal thickening the higher the likelihood of mality is bronchial wall thickening, with hyperinfla-
airway obstruction.13 Extensive sinus mucosal tion the second most common (though less
thickening is more common in those with acute reliable) finding (Fig. 3).19 Whereas some series
exacerbations of asthma than in those without identify hyperinflation radiographically in asth-
exacerbations.14 The degree of mucosal thick- matic patients up to 24% of the time,20 it is
ening appears to correlate with blood and sputum uncommon to see marked hyperinflation in asth-
eosinophilia.12 The relationship between sinus matic patients who do not also have emphysema.
disease and asthma appears most marked in Indeed, many patients with asthma have normal or
those with severe mucosal changes on CT scan, reduced lung volumes even during acute exacer-
suggesting that the thickened sinus mucosa is bations of their condition.
a site of immunologic activity, which intensifies It is important to reduce the number of chest
the atopic response in the airways.15 Sinus CT is radiographs obtained in the clinical evaluation of
commonly performed to document the extent of known asthmatic patients, particularly children,21
sinus disease in asthma (Fig. 1). In these patients, in whom radiation exposure has greater potential
appropriate pretreatment with antibiotics, muco- harm. Routine chest radiographs are not usually
lytic agents, nasal steroids, or nasal saline, is help- obtained in patients with asthma, except when
ful to reduce or eliminate sinus changes caused by there are atypical features, or there is concern for
acute inflammation or infection.15 a complication. The indication for chest radio-
Similarly, there is an established relationship graphs in asthma is open to debate. Tsai and
between gastroesophageal reflux disease colleagues22 suggested guidelines for selective
(GERD) and asthma. Treatment of gastroesopha- performance of chest radiographs in adult patients
geal reflux may reduce symptoms and physiologic admitted with acute exacerbations of obstructive
impairment due to asthma.16,17 Therefore, airway disease, proposing that chest radiographs
should be performed only in patients who fulfill
one or more of the following criteria: a clinical diag-
nosis of chronic obstructive pulmonary disease (as
defined by the American Thoracic Society);
a history of fever, or temperature more than
37.8 C; clinical or ECG evidence of heart disease;
history of intravenous drug abuse; seizures; immu-
nosuppression; evidence of other lung disease; or
prior thoracic surgery. In a prospective study using
these criteria, management was changed on the
basis of the chest radiograph in 31% of the
patients evaluated.22 It is clear that use of guide-
lines and education can substantially reduce the
use of chest radiographs in asthmatic patients.23
Buckmaster and Boon21 suggested that radio-
graphs are unnecessary in those with known
asthma and those who are improving with treat-
ment, unless pneumothorax is suspected, or the
patient is in the intensive care unit. In their study,
the performance of unnecessary chest radio-
Fig.1. Diffuse sinusitis in a patient with severe asthma. graphs, identified by these criteria, was reduced
Coronal CT shows mucosal thickening involving substantially following implementation of an
frontal, ethmoid and maxillary sinuses. educational program.
Asthma: An Imaging Update 319
Fig. 2. Asthma with reflux esophagitis. (A) Axial CT shows moderate airway wall thickening. (B) Axial CT shows
circumferential esophageal wall thickening (arrowhead).
CT A recent prospective study of CT in patients

with asthma used automated quantitative
In patients with asthma, CT is indicated to identify
3-dimensional (3-D) CT analysis (Fig. 5) to show
suspected complications, particularly allergic
that airway wall thickness and area seen on
bronchopulmonary aspergillosis, and mimics of
MDCT correlated with airway epithelial thickness
asthma such as hypersensitivity pneumonitis.
on endobronchial biopsy patients with severe
When it is performed, CT is helpful for evaluating
asthma.26 These authors also showed that
the extent of airway thickening and expiratory air
patients with severe asthma have thicker airway
trapping (Fig. 4). Several studies using multidetec-
walls on MDCT than mild asthmatic patients or
tor CT (MDCT) have clearly demonstrated the
healthy subjects, but overlap between these
extent to which the airway walls of asthmatic
groups limits the diagnostic value of this
patients are thicker than healthy individuals.24
measurement. As the authors acknowledge,
Furthermore, the degree of airway wall thickness
a pivotal goal in those caring for individuals with
directly correlates with the severity of airflow
severe asthma is to develop reliable and
obstruction and clinical disease.25,26
Fig. 3. (A, B) Chest radiograph with detail view in a patient with asthma. The lung volumes are increased, and
there are numerous thick-walled airways around the hila (arrows).
320 Woods & Lynch
Fig. 4. CT findings in uncomplicated asthma (same patient as Fig. 3). (A) Inspiratory CT image shows diffuse airway
wall thickening. (B) Expiratory CT shows diffuse air trapping.
reproducible yet noninvasive means to measure that quantitative CT cannot only determine air
airway remodeling, which would allow providers trapping in asthmatic subjects, but also identify
both to identify those individuals more prone to a group of individuals with a high risk of severe
develop severe disease as well as a means to disease. In this study, expiratory MDCT was per-
effectively gauge the response to therapy. CT formed in a subset of the subjects in the Severe
currently stands as the modality most likely to Asthma Research Program. In this study, air trap-
deliver this, and automated 3-D quantitative soft- ping was defined as the percentage of lung less
ware will probably be the vehicle by which it is than 850 HU on expiratory CT, and those individ-
achieved.27 uals with an air trapping percentage above the
Air trapping can also be effectively quantified median value of 9.66% were defined as having
with CT, generally during expiration (Fig. 6).28 A an air-trapping phenotype. The threshold value of
recent study by Busacker and colleagues6 showed 850 was selected because the specific volume
Fig. 5. Quantitative analysis of airway wall thickening quantification in a young patient with severe asthma (FEV1/
FVC ratio 0.59, residual volume 306% predicted). (A) Multidetector CT permits reconstruction of airway tree using
proprietary software (VIDA Diagnostics Inc, Iowa City, IA). (B) Orthogonal image through the airway provides
measurement of airway dimensions. The airways are moderately thick-walled. (Courtesy of Paul Szefler, Stanley
Szefler, MD, and Ronina Covar, MD, National Jewish Health, Denver, CO.)
Fig. 7. Bronchial dilation in asthma. Inspiratory CT

shows moderate dilation of central bronchi relative
to adjacent vessels.
colleagues30 confirmed that bronchial dilation is

more prevalent in asthmatic patients than in
healthy subjects, and like the study performed by
Fig. 6. Expiratory CT in asthma (same patient as Fig. 5). Harmenci and colleagues,31 they also suggest an
Coronal expiratory CT image shows moderate air trap- association between an increased severity of
ping. Voxels with CT attenuation less than 856 asthma with a higher prevalence of bronchiec-
Hounsfield Units are highlighted in color, with tasis, as assessed by MDCT.
different colors for different lobes; 23% of the lung A difficult but important distinction for the radiol-
showed air trapping by this definition. ogist and clinician is whether or not bronchiectasis
observed in an asthmatic patient is associated
with allergic bronchopulmonary aspirgillosis
of the normal lung at total lung capacity is 6.0 mL/
(ABPA). Although central bronchiectasis is impor-
gm, corresponding to a CT attenuation of 856
tant in the diagnosis of ABPA, the presence of
HU. It seems reasonable therefore to assume
dilated airways combined with an existing diag-
that pixels with attenuation values less than this
nosis of asthma should not automatically decree
value represent persistently inflated lung on expi-
a diagnosis of ABPA. In fact, Neeld and
ration. This threshold value has also been used
colleagues,34 and others,35 have established that
to identify air trapping on single-slice CT in asth-
cylindric bronchiectasis can certainly be present
matic children.29 Using both univariate and multi-
without concomitant ABPA. Mitchell and
variate statistical analysis, the clinical and
colleagues35 described the CT and radiographic
demographic features of subjects with the air-
findings in 19 patients with documented ABPA,
trapping phenotype were compared with those
10 patients with probable ABPA, and 18 asthmatic
without the phenotype. Individuals in the group
controls without ABPA. All but one (89%) of the
with air trapping were significantly more likely to
ABPA patients demonstrated central cystic or vari-
have a history of asthma-related hospitalizations,
coid bronchiectasis in at least one lobe, and all 10
ICU visits, and/or mechanical ventilation. Duration
(100%) of the probable ABPA patients had
of asthma, history of pneumonia, high levels of
evidence of bronchiectasis on HRCT, while 3
airway neutrophils, airflow obstruction (FEV(1)/
(17%) of the asthmatic controls had findings of
FVC), and atopy were identified as independent
cylindric bronchiectasis. In patients with asthma,
risk factors associated with the air-trapping
CT imaging features that support the diagnosis
phenotype.6
of ABPA rather than asthma are varicose or cylin-
Bronchial dilation, or bronchiectasis (defined as
dric bronchiectasis (Fig. 8),35 mucoid impaction
a bronchus with a larger diameter than the internal
(see Fig. 8), and centrilobular nodules.36 ABPA is
diameter of the adjacent pulmonary artery), has
further discussed elsewhere in this issue.
been well documented on CT in asthmatic patients
(Fig. 7).30–32 Lynch and colleagues32 reported
Synchrotron Radiation CT and Dual-Energy CT
77% of asthmatic patients had one or more dilated
bronchi compared with 59% of healthy control While MDCT has good spatial resolution, and hy-
subjects, whereas Park and colleagues33 reported perpolarized 3He MR offers temporal resolution
a prevalence of 31% in asthmatics and 7% in and quantitative measurements of functional lung
healthy controls. A study by Takemura and parameters, some are investigating an innovative
322 Woods & Lynch
air space size, and determine regional oxygen

partial pressure.41 Although inhalation of hyperpo-
larized 3He is generally safe, it is still classified as
an investigational contrast agent by the US Food
and Drug Administration and thus is approved for
investigational, but not for clinical use. A recent
retrospective study by Lutey and colleagues41
demonstrated that in 100 consecutive patients,
there were no serious adverse events and no clin-
ically important effects of 3He MR imaging on vital
signs. Although some unpredictable transient de-
saturations were noted, which suggest that poten-
tial subjects be screened for comorbidities, the
agent was used and tolerated not only in healthy
subjects but also in heavy smokers and those
Fig. 8. Varicose bronchiectasis and mucoid impaction with severe obstructive pulmonary disease.
in allergic bronchopulmonary aspergillosis. CT shows Altes and colleagues42 first presented hyperpo-
marked dilation of multiple subsegmental airways larized 3He MR lung ventilation imaging findings in
(arrows) and mucoid impaction (arrowhead). asthmatic patients, and showed that ventilation
defects were present in 7 of 10 asthmatic patients
approach to effectively image both function and but in none of 10 healthy subjects (Fig. 9). de
morphology simultaneously.37 Synchrotron radia- Lange and colleagues43 subsequently demon-
tion CT using inhaled xenon as a contrast agent strated that the amount of regional airflow obstruc-
is an investigative technique pioneered by Bayat tion depicted by hyperpolarized 3He MR
and colleagues38 that appears preliminarily to fulfill correlated with spirometric severity of asthma.
these criteria. Their technique involves imaging The technique is very sensitive for identifying
with two monochromatic x-ray beams tuned to methacholine-induced air trapping.44 In patients
slightly different energies above and below the scanned on two occasions, ventilation defects
K-edge of xenon (Xe), with subsequent subtraction identified with hyperpolarized 3He were un-
of the high-energy image from the low-energy changed in location in about 40% of cases, and
image on a logarithmic scale, which subtracts postmethacholine defects are unchanged in
the contributions of soft tissue and bone, yielding location in about 70% of cases, suggesting that
only the distribution of the contrast agent the regional changes of asthma are relatively fixed
dispersed throughout the lungs.38 Using this K- within the lung.44 Recent emphasis has focused on
edge subtraction method (KES), Bayat and comparing the utility of hyperpolarized 3He MR
colleagues are able to quantitatively measure Xe imaging against that of the established gold stan-
contrast within the airways permitting the dard for airway evaluation, MDCT. Fain and
measurement of regional ventilation while also colleagues45 sought to compare regional ventila-
giving the high-resolution structural data. Future tion defects in asthmatic patients on hyperpolar-
directions may include the simultaneous measure- ized 3He MR with air trapping on MDCT and
ment of ventilation/perfusion maps using KES inflammatory makers on bronchoscopy. They
synchrotron CT imaging.39 Current practical limi- found significant overlap of ventilatory defects on
tations include high radiation exposure, limited hyperpolarized 3He MR with hyperlucency on
availability of synchrotron x-ray sources, and the MDCT, both of which correlated with elevated
requirement for the x-ray beam plane to remain inflammatory markers (neutrophils) yielded by tar-
stationary, requiring coordinated automated geted bronchoalveolar lavage. Their findings again
movement of the patient for image acquisition.37 suggest that the areas of air trapping in the lungs
Similar techniques have been developed using of asthmatic individuals are relatively fixed. Tzeng
dual-energy CT,40 which is more widely available. and colleagues46 used both hyperpolarized 3He
MR and MDCT to compare the airways of seven
volunteers (five asthmatic, two healthy) before
MR Imaging
and after a methacholine challenge. Their tech-
Magnetic resonance (MR) imaging using hyperpo- nique yielded no meaningful correlation between
larized helium 3 (3He) gas shows burgeoning the airway caliber changes measured on MR and
promise in the evaluation of the lungs because of MDCT. Airway measurement by hyperpolarized
3
the ability to evaluate the spatial and temporal He MR failed to match MDCT in 37% to 43% of
distribution of ventilation, quantitatively assess the airway diameters from the first six generations
Fig. 9. Demonstration of ventilation defects by hyperpolarized 3He MR. (A) Coronal MR image in a mild-moderate
asthmatic patient (FEV1 109% predicted) shows several small ventilation defects. (B) Follow-up image obtained
after the patient was exposed to second-hand smoke, shows increase in number and size of ventilation defects.
The FEV1 fell to 94% predicted, but remained within the normal range. (Courtesy of Dr Sean Fain, University of
Wisconsin, Madison, WI.)
(at the two lung volumes tested, FRC and FRC 1 COMPLICATIONS OF ASTHMA
1L). Although it may not yet rival the gold standard
of MDCT because of poorer resolution and thus Radiologic imaging is important in identifying compli-
less anatomic detail, hyperpolarized 3He MR cations of asthma. Acute complications of asthma
remains promising. In addition to providing greater may include pneumothorax, pneumomediastinum
patient safety because of the lack of ionizing radi- (Fig. 10) (and rarely pneumopericardium, pneumoper-
ation, the modality promises better functional itoneum, pneumoretroperitoneum, pneumorrhachis,
physiologic data. Recent advances include those and even subdural emphysema),50–52 mucus impac-
described by Wang and colleagues,47 who have tion with or without atelectasis, and pneumonia.
developed a novel hybrid MR pulse sequence Chronic complications of asthma include
that obtains coregistered maps of the apparent allergic bronchopulmonary aspergillosis (dis-
diffusion coefficient (ADC) of helium at both short cussed elsewhere in this issue), eosinophilic pneu-
and long time-scales (during a single breath monia, and Churg-Strauss vasculitis. About 50%
hold). The ADC appears to be a sensitive method of patients with chronic eosinophilic pneumonia
for detecting early alveolar destruction in emphy- have a history of atopy, with or without asthma.
sema,48 and the study by Wang and colleagues47 The typical radiographic findings in chronic eosin-
demonstrated significant elevations in ADC values ophilic pneumonia are patchy airspace opacities,
in asthmatic patients compared with healthy usually with upper lobe predominance. The
controls suggesting that the alveolar spaces may peripheral distribution of the infiltrates may be
also be expanded in asthma. Tsai and evident on the chest radiograph as the ‘‘negative
colleagues49 have designed an open-access, pulmonary edema pattern,’’ but may be more
low-field MR system for both horizontal and obvious on CT scan.53,54
upright hyperpolarized 3He imaging of the human Churg-Strauss vasculitis (allergic granulomato-
lungs, which has important physiologic implica- sis and angiitis) is a granulomatous vasculitis that
tions given the limitations of some patients who occurs in patients with asthma, and is commonly
are unable to remain horizontal for the required associated with eosinophilia. This vasculitis may
amount of time to be adequately imaged, as well also affect the skin, kidneys, and peripheral
as the inherent physiologic changes between the nerves. Pericardial or myocardial involvement
supine or prone lung with that of the upright lung. may occur (Fig. 11).55,56 CT shows lung paren-
With continued advances in hyperpolarized 3He chymal abnormalities in about 75% of cases.57
MR, widespread clinical use is possible, but may Radiologically, it presents with patchy, often
be limited by availability of 3He. migratory consolidation or groundglass abnor-
mality, often associated with airway wall
324 Woods & Lynch
Fig.10. Pneumomediastinum in a child with asthma. (A) Chest radiograph shows pneumomediastinum and air in
the left neck (arrows). (B) Radiograph of left arm shows air in the soft tissues.
thickening and septal thickening (see Fig. 11).58 person with breathlessness or wheezing may be
Small nodules and tree-in-bud pattern may indi- assigned the label of asthma. Therefore, the chal-
cate bronchiolitis. The extrapulmonary involve- lenge for the radiologist who reviews the images of
ment is often the main clue to the diagnosis. a patient with ‘‘asthma’’ is to disprove this diag-
Churg-Strauss syndrome is usually more benign nosis. The most common condition to be misdiag-
in its course than Wegener’s granulomatosis. nosed with asthma is vocal cord dysfunction,
a functional condition in which inspiratory or expi-
MIMICS OF ASTHMA ratory stridor is produced by adduction of the
vocal cords.60 Patients with this disorder are often
The aphorism ‘‘all that wheezes is not asthma’’59 is treated with large doses of steroids because of
most important for the radiologist. Any young refractory asthma. The diagnosis of vocal cord
Fig.11. Churg-Strauss syndrome in a 58- year-old man with a long history of asthma, and new fever and skin rash.
(A) CT scan photographed at lung window settings shows bilateral basal predominant consolidation and ground-
glass abnormality, with some peribronchovascular predominance. (B) CT photographed at lung windows at
a lower level shows bilateral pleural effusions and a pericardial effusion. Left ventricle is mildly dilated because
of cardiomyopathy.
dysfunction is made by laryngoscopy. There are granulomatosis, or amyloidosis, or may be caused

no radiologic manifestations of vocal cord by cartilaginous disorders, such as relapsing poly-
dysfunction, but one might suspect this condition chondritis or tracheobronchopathia osteochondro-
when the chest radiograph or chest CT shows plastica. In patients with suspected tracheal
normal bronchial wall thickness in a patient who obstruction, a flow-volume loop shows character-
has severe symptoms. istic blunting of the inspiratory limb of the loop.
Patients with tracheal or carinal obstruction Helical CT scan with multiplanar reformations or
commonly receive the label of asthma, in spite of 3-D rendering can usefully define the surgical ana-
the lack of fluctuation of their symptoms, and the tomy in these patients (see Fig. 12).63
frequent presence of inspiratory stridor (Fig. 12).61 Patients with constrictive bronchiolitis present
The obstructing airway lesion is often visible on the with airway obstruction that is usually refractory
frontal or lateral chest radiograph, unless it is at to bronchodilators. Bronchiolitis in these patients
the carina. Focal lesions causing tracheal obstruc- may be a result of previous infection, collagen
tion include benign and malignant tracheal vascular disease, or may be idiopathic. It may be
neoplasms, tracheal stenosis following intubation, difficult or impossible to distinguish clinically
and vascular rings.62 Diffuse or long-segment between refractory, late-onset asthma and crypto-
tracheal narrowing may be a result of infiltrative genic bronchiolitis obliterans. High-resolution CT
disorders, such as sarcoidosis, Wegener’s scan assists with this distinction by identifying
Fig. 12. Tracheal stenosis in a child with long-standing shortness of breath misdiagnosed as asthma. (A) Chest
radiograph shows moderate tracheal narrowing just above the carina (arrow). A tracheal bronchus is also visible
(arrowhead). (B, C) Coronal CT reconstruction and virtual bronchogram confirm these findings.
326 Woods & Lynch
Fig.13. Bronchiolitis obliterans. (A) Inspiratory CT shows decreased attenuation in the anterior left lung. (B) Expi-
ratory CT shows air trapping in the same anterior distribution.
the sharply demarcated lobular areas of air trap- pneumonitis may closely mimic asthma. The peri-
ping that are characteristic of cryptogenic bron- bronchiolar granulomas of hypersensitivity pneu-
chiolitis obliterans (Fig. 13). In a study of 14 monitis sometimes cause dominant airway
patients with obliterative bronchiolitis and 30 with obstruction rather than restriction. On CT, a pattern
severe asthma, we found that mosaic attenuation of lobular decrease in attenuation and expiratory
on inspiratory images was the best discriminant, air trapping, usually associated with groundglass
found in 50% of those with obliterative bronchioli- abnormality and often with centrilobular nodularity,
tis and only one of those with severe asthma.64 is an important clue to this diagnosis (Fig. 14).68,69
Copley and colleagues65 found that vascular
attenuation and decreased lung attenuation were SUMMARY
more prevalent in obliterative bronchiolitis than in
asthma. Patients with bronchiolitis obliterans Asthma is a common disease with increasing inci-
who have diffuse air trapping, however, may be dence, which manifests radiologically with
indistinguishable from those with asthma. common, but nonspecific findings. The primary
Infiltrative lung diseases that cause airway task of the radiologist is to identify mimics and
obstruction, such as sarcoidosis66 and hypersen- complications of asthma. However, rapid advances
sitivity pneumonitis,67 must be included in the in quantitative imaging using MDCT, hyperpolarized
3
differential diagnosis of asthma. In particular, the He MR, and dual-energy CT or synchrotron radia-
fluctuating symptoms of hypersensitivity tion CT with Xe subtraction all show promise in
Fig. 14. Hypersensitivity pneumonitis in a patient presenting with ‘‘asthma.’’ (A) Inspiratory CT shows patchy
groundglass abnormality, with lobular decrease in lung attenuation (white arrowheads). (B) Expiratory CT shows
multifocal air trapping.
improving the diagnosis and surveillance of the 16. Harding SM, Richter JE, Guzzo MR, et al. Asthma
disease, which in turn should promote earlier recog- and gastroesophageal reflux: acid suppressive
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1996;100(4):395–405.
17. Peterson KA, Samuelson WM, Ryujin DT, et al. The
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Imaging of Airways :
Chronic Obstructive
Pulmonary Disease
Julia Ley-Zaporozhan, MDa,*, Hans-Ulrich Kauczor, MD, PhDb
KEYWORDS
COPD Airways CT MRI Visualization Quantification
Chronic obstructive pulmonary disease (COPD) is extremely useful, PFTs are known to be relatively
one of the leading causes of morbidity and insensitive to both early stages and small changes
mortality worldwide. At present, it is the fourth of manifest disease.
most common cause of death among adults.1 The manifestations of obstructive lung disease
COPD is characterized by airflow limitation that is are not limited to the tracheobronchial tree, but
not fully reversible. The airflow limitation is usually also affect the parenchyma by hyperinflation
progressive and associated with an abnormal and destruction; as well as the vasculature by
inflammatory response of the lung to noxious hypoxic vasoconstriction. Several pathologic
particles or gases. It is caused by a mixture of studies have shown that a major site of airway
airway obstruction (obstructive bronchiolitis) and obstruction in patients with COPD is in airways
parenchymal destruction (emphysema), the rela- smaller than 2 mm of internal diameter.3 The
tive contributions of which are variable.1 Chronic 2-mm airways are located between the fourth
bronchitis, or the presence of cough and sputum and the 14th generation of the tracheobronchial
production for at least 3 months in each of 2 tree. Airflow limitation is closely associated with
consecutive years, remains a clinically and epide- the severity of luminal occlusion by inflammatory
miologically useful term. Pulmonary emphysema is exudates and thickening of the airway walls due
a pathologic term and is defined by the American to remodeling.4
Thoracic Society as an abnormal permanent COPD obviously comprises several subtypes
enlargement of the air spaces distal to the terminal which primarily can be related to the major site
bronchiole, accompanied by the destruction of of involvement and then further differentiated.
their walls.2 In a simplified way, obstructive airflow The subtypes include the airways, mainly obstruc-
limitation leads to air trapping with subsequent tive bronchiolitis, and the parenchyma (ie, emphy-
hyperinflation and later destruction of the lung sema). PFTs, as a global measure, are unable to
parenchyma. categorize these subtypes. With the increasing
For diagnosis and severity assessment pulmo- number of therapeutic options in COPD, particu-
nary function tests (PFT) are the accepted and larly in its advanced stages, there is a high demand
standardized workhorse providing quantitative for a noninvasive imaging test to identify different
measures for forced expiration volume in one phenotypes of the disease according to structural
second (FEV1), FEV1/FVC (forced vital capacity), and functional changes and provide the regional
diffusing capacity for carbon monoxide (DLco) information of such changes to target therapies
and others. However, PFT only provide a global accordingly. For phenotyping, a precise charac-
measure without any regional information not to terization of the different components of the
mention any detail about lung structure. Although disease, such as inflammation, hyperinflation,
a
Department of Diagnostic and Interventional Radiology, University Hospital Heidelberg, Im Neuenheimer
Feld 430, 69120 Heidelberg, Germany
b
Department of Diagnostic and Interventional Radiology, University Hospital Heidelberg, Im Neuenheimer
Feld 150, 69120 Heidelberg, Germany
E-mail address: [email protected] (J. Ley-Zaporozhan).
Radiol Clin N Am 47 (2009) 331–342

doi:10.1016/j.rcl.2008.11.012
332 Ley-Zaporozhan & Kauczor
and so forth, is highly desirable to select the kernel, noisy images might result. The trade-off
appropriate therapy. between high-resolution images, which is what
In contrast to PFT, radiological imaging tech- we are used to from traditional HRCT, and noise
niques might allow for differentiation of the currently poses a major challenge. The detailed
different components of obstructive lung disease visual assessment of small structures, such as
(ie, airways, parenchyma, and vasculature) on peripheral airways in the lung parenchyma, nor-
a regional basis. Computed tomography (CT) is mally requires a high-resolution kernel. At the
a long standing player in this field with emphasis same time, the performance of postprocessing
on structural imaging of lung parenchyma and segmentation and quantitation using dedicated
airways. Magnetic resonance imaging (MR software tools is often hampered by noisy high-
imaging) of the lung has the potential to provide resolution images. The use of images recon-
regional information about the lung without the structed with a regular soft tissue kernel might be
use of ionizing radiation, but is hampered by more appropriate.8,9 As the airways in the lung
several challenges: the low amount of tissue parenchyma are high contrast structures, interme-
relates to a small number of protons leading to diate dose settings are appropriate. In general,
low signal, countless air-tissue interfaces cause a tube voltage of 120 kV together with a tube
substantial susceptibility artifacts as well as respi- current between 50 mAs to 150 mAs is
ratory and cardiac motion.5,6 The strength of MR recommended.
imaging is the assessment of function like perfu-
sion, ventilation, and respiratory dynamics.
Visualization of Large Airways
This overview focuses on the imaging of the
airway component of COPD. Enlarged or thick- The isotropic datasets allow for different postpro-
ened airways can be directly visualized while cessing techniques to be used for evaluation and
pathologies of the small airways are frequently presentation purposes.10,11 Two different tech-
assessed by indirect signs like expiratory air niques are generally used and described later in
trapping. this article: (1) two-dimensional multiplanar refor-
mats (MPR), which allows assessment of the
central airways along their anatomic course and
COMPUTED TOMOGRAPHY
reduction of the number of images to be analyzed;
Technical Aspects
(2) complex three-dimensional segmentations and
At the present time, a state of the art multidetector volume rendering which illustrate the important
computed tomography of the thorax yields a volu- findings in a very intuitive way (eg, a frontal view
metric dataset of images with an isotropic submil- of the patient or a simulation of an intraoperative
limeter resolution (0.5 to 0.75 mm slice thickness). situs) although they are more time consuming.
It covers the complete thoracic cavity in a single Owing to this capability for continuous volumetric
breath hold. CT can characterize anatomic details acquisitions during a single breath hold, multislice
of the lung as small as 200 mm to 300 mm, which CT has developed as the gold standard for visual-
corresponds to approximately the seventh to ninth ization of intra- and extraluminal pathology of the
bronchial generation.7 Since normal centrilobular trachea and the bronchi. A presentation of over-
bronchioles are not visible at thin-section CT, the lapping thin slices in a cine mode allows identifying
recognition of air-filled airways in the lung the bronchial divisions from the segmental origin
periphery usually means that the airways are down to the small airways. This viewing technique
both dilated and thick-walled. The combination helps to identify intraluminal lesions, characterize
of high spatial resolution and volumetric coverage the distribution pattern of any airway disease,
of the lung is recommended as it results in three- and might also serve as a road map for the bron-
dimensional high resolution CT (3D HRCT). For choscopist. The perception of the anatomy of the
multiplanar reformats, and other post processing tracheobronchial tree is further enhanced by
tools, overlapping reconstruction is advanta- MPR and maximal, and especially, minimal inten-
geous. The reconstructed slice thickness should sity projections. Interactive viewing of MPR on
match the collimation and the reconstruction a workstation is the best way to find the appro-
increment and should be 80% of the slice thick- priate plane in which key features of the disease
ness or less. Using a 512 or 768 matrix and a small are displayed. These views allow to confidently
reconstruction field of view targeted onto the identify the main pattern of the disease, any asso-
tracheobronchial tree, the spatial resolution can ciated findings, and the distribution of lesions rela-
be further optimized. Thin slices inherently carry tive to the airways. Tracheobronchial stenoses,
a high level of image noise. Together with image especially in the vertically oriented bronchi, are
reconstruction using a high-spatial resolution often underestimated if evaluated on axial slices
Imaging of Airways in COPD 333
only.12 MPR allow obviating this underestimation Thus, virtual bronchoscopy can be extremely help-
as the craniocaudal extension can be exactly ful in a clinical setting, especially in planning trans-
determined. Beyond the assessment of the extent bronchial biopsies.
of stenosis, MPR are of value in treatment planning
and follow-up as well as quantification of patho- Assessment of Small Airways Disease
logic changes in obstructive lung disease like
To assess the presence of expiratory obstruction,
bronchial wall thickening.13
multislice CT acquisitions in inspiration and
The maximum intensity projection (MIP) tech-
expiration are recommended; especially for the
nique projects the highest attenuation value of
acquisition of the expiratory scan, a low mAs
the voxels on each view throughout the volume
protocol, 40- to 80- mAs, will be sufficient.18
onto a two-dimensional image.14 This method
Regional air trapping reflects the retention of
does not change the high resolution of the thin sli-
excess gas at any stage of respiration as an indi-
ces. Since the bronchiolar walls measure less than
rect sign of peripheral airway obstruction, predom-
0.1 mm in thickness, the small airways are nor-
inantly seen on expiratory images. At expiration,
mally not visible on volumetric high-resolution
the cross section of the lung will decrease,
CT. However, when inflammatory changes are
together with an increase of lung attenuation.
present in the bronchiolar wall and lumen, the
Usually, gravity dependent areas will show
bronchioles may become visible on CT scans, as
a greater increase in lung density during expiration
small centrilobular nodular or linear opacities.
than nondependent lung regions. At the same
The application of the MIP technique with the
time, the cross-sectional area of the airways will
generation of 4- to 7-mm thick slabs may increase
also decrease. Air trapping is defined as the lack
the detection and improve the visualization of
of an increase of lung attenuation and the
these small centrilobular opacities.12,15,16
decrease of the cross-sectional area of lung. Air
The minimum intensity projections (minIP) are
trapping may be depicted in individual lobes in
a simple form of volume rendering to visualize
the dependent regions of the lung. In general,
the tracheobronchial air column into a single
any air trapping involving less than 25% of the
viewing plane. It is usually applied to a selected
cross-sectional area of one lung at a single scan
subvolume of the lung which contains the airways
level can still be regarded as a physiologic finding.
under evaluation. The pixels encode the minimum
Thus, physiologic air trapping will be detected at
voxel density encountered by each ray. Subse-
an expiratory CT in up to 50% of asymptomatic
quently, an airway is visualized because the air
subjects. The frequency of air trapping will
contained within the tracheobronchial tree has
increase with age and is also associated with
a lower attenuation than the surrounding pulmo-
smoking.19 Some publications suggest that the
nary parenchyma. This technique displays only
extent of air trapping is related to smoking history
10% of the data set and is the optimal tool for
independent from the current smoking habits. Air
the detection, localization, and quantification of
trapping has to be considered pathologic when it
ground-glass and linear attenuation patterns.14 In
affects a volume of lung equal or greater than
clinical routine, minIP are rarely used in the evalu-
a pulmonary segment, and not limited to the supe-
ation of the airways because numerous draw-
rior segment of the lower lobe.20 Abnormal air
backs have limited its indications in the
trapping is a hallmark of small airway disease but
assessment of airway disease. The technique is
it may also be seen in a variety of lung diseases,
especially susceptible to variable densities within
including emphysema, bronchiectasis, bronchioli-
the volume of interest and partial volume effects.12
tis obliterans, and asthma.21
Three-dimensional surface rendering tech-
niques and volume rendering techniques are very
Qualitative Evaluation
helpful to enhance the visualization of the anatomy
of the airway tree. CT bronchography consists of Trachea
a volume-rendering technique applied at the level Tracheobronchomalacia (TBM) generally results
of central airways after reconstruction of 3D from weakness of the tracheal or mainstem bron-
images of the air column contained in the airways. chial walls caused by either softening of the sup-
Virtual bronchoscopy provides an internal analysis porting cartilaginous rings, redundancy of the
of the tracheobronchial walls and lumen owing to connective tissue of the posterior membrane due
a perspective rendering algorithm that simulates to a reduction in the size and number of elastic
an endoscopic view of the internal surface of the fibers, or both. In association with COPD, TBM is
airways. In comparison to bronchoscopy, CT usually diffusely distributed affecting the whole
allows for visualization beyond stenoses, which trachea. During exhalation, increasing pleural
supports planning of endobronchial procedures.17 pressure causes the weakened central airways to
narrow (collapse), which may exacerbate obstruc- are categorized in cylindric (mild bronchial dilata-
tive symptoms.22 Symptoms can mimic worsening tion, regular outline of the airway), varicose
asthma or COPD, and it is not generally known (greater bronchial dilatation, accompanied by local
which patients with COPD have TBM contributing constrictions resulting in an irregular outline of the
to their symptoms. Acquired TBM has been re- airway), and cystic or saccular type (ballooned
ported in up to 44% of patients undergoing bron- appearance of the airways, reduced number of
choscopy in the setting of chronic bronchitis.23 bronchial divisions).
By consensus, excessive narrowing (collapse) is Abnormalities of bronchial cartilage are also
defined as a decrease of at least 50% in tracheal frequently present in COPD, associating atrophy
diameter during forced exhalation. For assess- and scarring. This deficiency of bronchial cartilage
ment of TBM cine acquisitions during continuous induces alternated narrowing and dilatation of the
respiration or forced expiration (Fig. 1) by CT are airways in advanced disease. This explains both,
recommended and easy to be performed.24,25 loss of normal proximal to distal tapering of the
airway lumen and the presence of bronchiectasis
in COPD patients. Like the trachea, collapse of
Large airways
segmental or subsegmental bronchi occurs at
Throughout the lung, the bronchi and pulmonary
dynamic maximum forced expiratory maneuver
arteries run and branch together. The ratio of the
due to cartilage deficiency.
size of the bronchus to its adjacent pulmonary
artery is widely used as a criterion for detection
of abnormal bronchial dilatation. In a healthy lung Small airways
it should be the same at any level with the mean Small airways disease on CT can be categorized
ratio being 0.98 [0.14] (with a wide range of 0.53– into visible and indirect patterns of the disease.
1.39).26 The ratio of the internal luminal diameter The tree-in-bud sign reflects the presence of
of the bronchus to the diameter of its accompa- dilated centrilobular bronchioles with lumina that
nying pulmonary artery has been estimated for are impacted with mucus, fluid, or pus; it is often
the healthy subjects to be 0.62 [0.13].27 CT signs associated with peribronchiolar inflammation.29
of bronchiectasis are nontapering, or flaring, of Cicatricial scarring of many bronchioles results in
bronchi, dilatation of the bronchi with or without the indirect sign of patchy density differences of
bronchial wall thickening (signet ring sign), the lung parenchyma, reflecting areas of under-
mucus-filled dilated bronchi (flame and blob ventilation and air trapping and subsequent hypo-
sign), plugged and thickened centrilobular bron- perfusion (mosaic perfusion).
chioles (tree-in-bud sign), crowding of bronchi As a powerful adjunct to inspiratory scans, expi-
with associated volume loss, and areas of ratory acquisitions reveal changes in lung attenu-
decreased attenuation reflecting small airways ation related to air trapping and pulmonary
obliteration (Figs. 2 and 3).28 The bronchiectasis blood volume, and illustrate regional volumetric
Fig. 1. CT images of a COPD patient at inspiration (A) and expiration (B) showing increased collapsibility of the
right main bronchi (arrow) after exhalation due to bronchomalacia.
bronchiectasis, bronchiolitis obliterans, and

asthma.21
Quantitative Evaluation
Manual assessment
Using HRCT images visual assessment bronchial
wall thickness and the extent of emphysema
were the strongest independent determinants of
a decreased FEV1 in patients with mild to exten-
sive emphysema.33 However, visual assessment
of bronchial wall thickening is highly subjective
and poorly reproducible.34 The quantitative anal-
ysis can be performed by manual delineation of
bronchial contours.35 Nakano and colleagues36,37
were the first to perform quantitative measure-
ments of airway wall thickening in COPD patients
and reported a significant correlation between
wall thickness of the apical right upper lobe bron-
Fig. 2. Mucoid impaction in the lumen of the right
lower lobe bronchus (segment 10, arrow). chus and FEV1% predicted. Due to technical limi-
tations of HRCT, neither the generation of the
bronchus measured could be determined nor
changes providing deeper insights into local could measurements be performed exactly
hyperinflation and expiratory obstruction perpendicular to the axis of the bronchus. It was
(Fig. 4).30 Since the severity of emphysema, as also demonstrated that the normalized airway
evaluated by CT, does not necessarily show wall thickness was larger in smokers with COPD
a good correlation with FEV1,30,31 small airway than in smokers or nonsmokers without COPD.38
disease appears to contribute more significantly Unfortunately, the measurements were restricted
to the airflow limitation in COPD. Regional air trap- to bronchi running almost perpendicular to the
ping reflects the retention of excess gas at any transverse CT section. Based on these experi-
stage of respiration as an indirect sign of periph- ences it is obvious that the complexity and size
eral airway obstruction. It is best detected on of the bronchial tree render manual measurement
expiratory CT as areas with abnormally low atten- methods impractical and inaccurate.39
uation.32 Air trapping is highly unspecific as it The dramatically increased acquisition velocity
occurs under physiologic conditions as well as in of MSCT, which reduced motion artifacts, and
a variety of lung diseases, including emphysema, the increasing spatial resolution (especially
Fig. 3. Mild bronchial dilation without wall thickening (A, arrow) and moderate bronchial wall thickening
without dilatation (B, arrow) of subsegmental bronchi, typical for the airway predominant type of COPD.
Fig. 4. Coronal CT images at inspiration (A, B) and expiration (C, D) of a patient with severe COPD (GOLD stage 3)
showing massive air trapping of the lower lobes. The upper lobes and middle lobe show a normal increase in
density after exhalation.
z-axis-resolution), opened the door to quantitative segment the airways down to the sixth genera-
evaluation of airway dimensions down to tion.8 This allows for visualization of the tracheo-
subsegmental bronchial level. Volumetric CT bronchial tree without any overlap by
allows for quantitative indices of bronchial airway surrounding parenchyma and for the reproducible
morphology to be calculated, including lumen and reliable measurements of the segmented
area, airway inner and outer diameters, wall thick- airways.
nesses, wall area, airway segment lengths, airway The simplest segmentation method used
taper indices, and airway branching patterns. a single threshold (cutoff) of pixel values in Houns-
field units (HU). Pixels with HU values lower than
Automatic assessment the threshold were assigned to the lumen (air),
The use of curved MPR from 3D-CT datasets is while surrounding pixels with HU values higher
a simple solution to accurately measure airway than the threshold were assigned to the airway
dimensions regardless of their course with respect wall or other surrounding tissue. Based on this
to the transaxial CT scan (Fig. 5). Airway tree analysis, the 3D region growing algorithm extracts
segmentation can be performed manually, which all voxels that are definitely airway. However, using
is tedious and extremely time consuming.40 a simple global threshold often fails in detecting
On the basis of the volumetric data sets sophis- the wall of smaller airways; thereby such tools
ticated post processing tools will automatically often require manual editing. To enhance this
Fig. 5. Volumetric CT datasets allow for 3D segmentation and skeletonization of the airways. In this example, the
centerline of the left upper bronchi was used to generate a curved MPR, and subsequently, a perpendicular
display of the bronchus. Images were generated using MeVIS software (Bremen, Germany).
basic segmentation process some additional pixels are used as the inner and outer (beginning
(add-on) algorithms were developed, (ie, based and ending) pixels of the airway wall. All pixels
on fuzzy connectivity41 or by wave propagation inside the first FWHM pixel are assigned to a lumen
from the border voxels).8 The luminal segmenta- and all pixels outside the second FWHM pixel are
tion result is condensed to the centerline (or skel- assigned to the lung parenchyma. The measure-
etization) running exactly in the center of the ments with the simple FWHM method result in
airway. These centerline points are the starting unacceptable large errors for wall thicknesses
points for the airway wall detection and quantifica- smaller than 1.0 mm. A new method of integral
tion. Approaches include methods based on the based closed-form solution based on the volume
estimation of the full width at half-maximum conservation property of convolution showed
(FWHM), brightness-area product and pixel- precise results.46 The integral under the profile is
intensity gradient.38,41–44 The most frequently re- kept constant, while the profile itself is trans-
ported method is based on the FWHM approach45 formed, narrowed, and elevated (Fig. 6).
that is explained in more detail; by identifying the Quantitative measurements revealed high corre-
lumen center, the scheme measures pixel values lations between airway luminal area, and to a lesser
along radial rays casting from the lumen center extent for wall thickening, with FEV1% predicted in
outward beyond the airway wall in all directions. patients with COPD. The correlation actually
Along each ray, the boundary between the lumen improved as airway size decreased from the third
and wall is determined by a pixel whose HU value (r 5 0.6 for airway luminal area and r 5 0.43 for
is half the range between the local minimum in the wall thickening) to sixth bronchial generation
lumen and the local maximum in the airway wall; (r 5 0.73 and r 5 0.55, respectively).47
while the boundary between the wall and lung By using integral-based method, the mean wall
parenchyma is determined by a pixel whose HU percentage, mean wall thickness, and median
value is half the range between the local maximum wall thickness in nonsmokers (29.6%; 0.69 mm;
in the wall and the local minimum in the paren- 0.37 mm) was significantly different (P<.001) from
chyma. Along each radial ray, these two FWHM the COPD (smokers, GOLD stage 2 and 3) group
Fig. 6. (A, B) 3D-segmentation of the tracheobronchial tree with the centerline (green line) and branching points
(red dots). Automatic segmentation of the inner and outer diameter of the airways is performed on images
perpendicular to the centerline (C, D). Images were generated using YACTA software (Oliver Weinheimer, Univer-
sity of Mainz, Germany).
(38.9%; 0.83 mm; 0.54 mm). Correlation between this feature is still experimental; however, first
FEV1 and FEV1 % predicted and the wall tests have demonstrated promising results.
percentage for airways greater than 4 mm in diam-
eter was r 5 0.532 and r 5 0.541, respectively.
MAGNETIC RESONANCE IMAGING
Correlation was higher (r 5 0.621 and r 5 0.537)
Technical Aspects
when only airways of 4 mm diameter in total
and smaller were considered.48 Overall, the use of MR imaging for assessment of
Identifying mucoid impacted airways is also airways is limited. Therefore, mainly our experi-
important for the clinical practice. For the auto- ences can be provided.
matic detection the branch points are extrapolated The most frequently used sequences in MR
at each terminal branch along its axis to extract 2D imaging of obstructive lung disease are acquired
cross-sections that are subsequently matched to in a breathhold. For fast T2-weighted imaging,
a model of mucus plugging computed from the single-shot techniques with partial-Fourier acqui-
dimensions of the terminal branch.9 Up until now, sition (HASTE) or ultrashort TE (UTSE) are
recommended. The T2-weighted HASTE

sequence in coronal or axial orientation allows
for the depiction of pulmonary infiltrates, inflam-
matory bronchial wall thickening and mucus
collections. T1-weighted 3D gradient echo
sequences, such as VIBE, are suitable for the
assessment of the mediastinum and common
nodular lesions. The intravenous application of
contrast material markedly improves the diag-
nostic yield of T1-weighted sequences by a clearer
depiction of vessels, hilar structures and solid
pathologies. A major goal in inflammatory obstruc-
tive airway disease is to differentiate inflammation
within the wall from muscular hypertrophy, edema,
and mucus collection which cannot be achieved Fig. 7. MR imaging (axial T1-weighted GE sequence
post contract [VIBE]) showing severe bronchial dilata-
by CT, but can be addressed by the use of
tion especially of the right lower lobe (arrows).
T1- and T2-weighted images and contrast
enhancement.
concentrations (15 L/min) may be risky in patients
Qualitative Evaluation with severe COPD. 3Helium MR imaging is based
on the inhalation of hyperpolarized 3helium gas. It
Trachea allows for direct evaluation of the distribution of
The depiction of airway dimensions and size of the the tracer gas after a breath hold (static) or during
airway walls by MR imaging under physiologic continuous breathing (dynamic) and airspace
conditions is limited to the central bronchi. In the dimensions. The latter is done by diffusion-
authors own experience, the trachea can be best weighted MR imaging. Areas with ventilation
visualized by a T1w 3D volume interpolated defects caused by airway obstruction and emphy-
gradient echo sequence (VIBE). For perception of sema represent the only limitation because they
TBM, MR imaging should be acquired in inspira- cannot be assessed due to lack of the tracer gas
tory and expiratory breath hold. For dynamic entering these areas. Thus, there is almost no
assessment of tracheal instability MR cine acquisi- information about these affected lung regions.
tions during continuous respiration or forced expi- Overall, the high cost of the noble gas 3helium,
ration are recommended.24 For data acquisition, the process of laser-induced hyperpolarization,
time resolved techniques are used which can be and the need for nonproton imaging remain the
based on FLASH or trueFISP sequences. This major drawbacks of this technology on its way to
allows for a high temporal resolution down to 100 broader clinical applications.
ms per frame. Due to the reflex of hypoxic vasoconstriction,
Large airways ventilation defects in COPD largely correspond to
For depiction of the bronchiectasis high spatial perfusion defects in the same areas. Thus, the
resolution is essential (Fig. 7). By using a 3D assessment of pulmonary perfusion makes a lot
volume interpolated gradient echo sequence of sense, especially as perfusion MR imaging is
(VIBE) with a voxel size of approximately 0.9 much easier and more straightforward than venti-
0.88 2.5 mm3 a sensitivity of 79% and a speci- lation MR imaging. The basic principle of
ficity of 98% regarding the visual depiction of contrast-enhanced perfusion MR imaging is
bronchiectasis was shown compared with CT.49 a dynamic acquisition during and after an intrave-
nous bolus injection of a paramagnetic contrast
agent. Perfusion MR imaging of the lung requires
Functional Imaging
a high-temporal resolution to visualize the peak
The major advantage of MR imaging is the func- enhancement of the lung parenchyma. Conse-
tional imaging. Ventilation and perfusion can be quently, contrast-enhanced perfusion MR imaging
directly visualized by MR imaging. Several uses T1-weighted gradient echo MR imaging with
different techniques are available, with oxygen ultra-short TR and TE, such as FLASH. With the
enhancement and inhalation of hyperpolarized introduction of parallel imaging techniques, 3D
noble gases being the most prominent. Oxygen- perfusion imaging, with a high spatial and
enhanced MR imaging requires no special scanner temporal resolution and an improved anatomic
hardware, is easy to use, and the costs for oxygen coverage and z-axis resolution, can be
are low. However, the use of high oxygen acquired.50–52 These data sets are also well suited
Fig. 8. Coronal and sagittal images (5 mm MIP) in a patient with severe emphysema: initial examination (A–D) and
6 months after the placement of the endobrochial valves (circle) in the left upper lobe (E–H). The interlobar
fissure moved slightly upwards after the therapy suggesting a volume reduction of the left upper lobe. By quan-
titative measurement the volume of the treated lung can be assessed, but only a small shift of volume from the
left upper to the lower lobe was found (the volume of the left lung remains the same while the volume of the
upper lobe decreased by 300 mL). The corresponding MR perfusion (50 mm MIP images) shows hypoxic vasocon-
striction in the left upper lobe (arrow) with redistribution of perfusion to ventilated areas. This example demon-
strates nicely the small volume difference after therapy but with a significant functional change.
for high quality multiplanar reformats. Due to high is well suited for dynamic assessment of tracheal
spatial resolution, detailed analysis of pulmonary instability and for gaining functional information
perfusion and precise anatomic localization of on perfusion and ventilation in COPD.
the perfusion defects on a lobar, and even
segmental level, can be performed. By applying
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2009, Vol.47, Issues 2, Imaging of Airway Diseases

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Imaging of Airway Diseases

Radiol Clin N Am 47 (2009) xi

Radiol Clin N Am 47 (2009) 185–201

found. A combination of slabs of various thick- surrounding pulmonary parenchyma

provides an internal rendering of the tracheobron-

first four to six generations that effectively

9. Fraser RS, Muller NL, Colman N, et al. The normal

Radiol Clin N Am 47 (2009) 203–225

DEVELOPMENTAL INTERRUPTION asymptomatic, leading to incidental discovery in

obstructive pulmonary disease, or even as compression from a tracheal congenital divertic-

reported.82,91,92 Bronchiectasis, atelectasis, focal Accessory Cardiac Bronchus

MALFORMATIONS ASSOCIATED blood from the pulmonary veins. Situs anomalies

population. Kartagener syndrome is found in wide number of possible combinations of malfor-

Primary tumors of the trachea and main bronchi CLINICAL PRESENTATION

Radiol Clin N Am 47 (2009) 227–241

Epithelial Neoplasms Mesenchymal Neoplasms

CT BRONCHOSCOPIC CORRELATIONS MALIGNANT TUMORS

It accounts for approximately 0.2% of malignant esophagus results in tracheoesophageal or bron-

most cases, carcinoids are endobronchial but

OTHER PRIMARY MALIGNANT NEOPLASMS

Fig. 3. Adenoid cystic carcinoma in a 50-year-old LYMPHOMA

composed of a mixture of fat, cartilage, fibrous

Focal Infection CT is more sensitive than bronchoscopy by identi-

Radiol Clin N Am 47 (2009) 243–260

bronchoscopy and biopsy to confirm the diag-

RHINOSCLEROMA strictures of the trachea and bronchi. Mediastinal

characterized by recurrent inflammation of the

considerable collapse on expiratory CT images reconstruction may be used to provide long-term

SARCOIDOSIS important, because endobronchial biopsy of an

and conglomerate fibrosis or some combination of interventional bronchoscopic techniques may be

Tracheobronchomalacia (TBM) is a disorder that 3-dimensional images, image interpretation, and

Radiol Clin N Am 47 (2009) 261–269

When interpreting functional CT scans of symptoms, severity and distribution of disease,

Fig. 8. ‘‘Frown sign’’ of TM in 64-year-old man with

Radiol Clin N Am 47 (2009) 271–287

peribronchial lesions in the absence of endobron- CT fluoroscopic guidance

through the working channel of a flexible bron-

Section of Cardiothoracic Imaging, Mallinckrodt Institute of Radiology, Washington University School of

Radiol Clin N Am 47 (2009) 289–306

chronic hypoxemia and hypercarbia in more severe

fact, in two retrospective studies evaluating the

Congenital Airway Wall Abnormality a progression of the bronchiectasis and lung

especially mycobacterium avium intracellulare-

Acquired Wall Abnormalities

cystic fibrosis, and abnormalities of the structure CYSTIC FIBROSIS

Staphylococcus aureus, Haemophilus influenza, thickening. Extensive mucus plugging of the

PRIMARY CILIARY DYSKINESIA PCD tends to be diagnosed relatively late,

Hyper-Immune Response may cough up brown mucus plugs. Clinical diag-

DEFINITION branches; the alveolar ducts, sacs, and alveoli

Radiol Clin N Am 47 (2009) 307–316

good patient effort. The most important confounder INFLAMMATORY BRONCHIOLITIS

Type Based on Imaging Prototype Cause

Imaging of Small Airways Disease

such as can be seen with D-penicillamine therapy. Postinfectious Constrictive Bronchiolitis

Swyer-James or Macleod Syndrome

Fig. 7. Hypersensitivity pneumonitis. Axial thin section Noxious Fume Exposure

Radiol Clin N Am 47 (2009) 317–329

CT A recent prospective study of CT in patients

Fig. 7. Bronchial dilation in asthma. Inspiratory CT

colleagues30 confirmed that bronchial dilation is