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INTRODUCTION cartilage superiorly (at about the level of the sixth cervical
vertebra) to the carina inferiorly.1 The location of the lower
The trachea is a flexible cylindrical tube composed of end of the trachea varies with body posture, and with
cartilaginous rings, connected by a fibromuscular membrane inspiration and expiration, and in deep inspiration the carina
and lined internally by mucosa. The trachea facilitates the may descend to the level of the sixth thoracic vertebra.2
passage of air between the larynx and the lungs. It is The length of the trachea ranges from 10 to 13 cm, averaging
positioned midline in the neck and courses slightly to the approximately 11 cm.2 Viewed laterally, the trachea as-
right in the upper thorax. It extends from the cricoid sumes an oblique course, running from superoanterior to in-
1700
feroposterior. In severely kyphotic patients, the trachea may lumen shape. During rapid deep inspiration, the thoracic
have a horizontal orientation. portion of the trachea widens and the cervical portion
The degree to which the laryngeal glottis (true vocal narrows. The opposite pattern occurs during expiration; the
folds) is open significantly affects airflow patterns in the extrathoracic tracheal lumen increases in size during
trachea. The glottis has the smallest, yet the most variable, coughing, Valsalva maneuver, or forced expirations,
cross-sectional area of the upper respiratory tract. Air whereas the intrathoracic portion decreases.2
currents in the upper trachea are typically turbulent during The trachea is lined by pseudostratified columnar
inspiration, with a transition to more laminar flow in the epithelium that sits on an elastic lamina propria. Goblet
middle and distal trachea. The velocity of the air stream mucous cells and small subepithelial glands that secrete onto
in the distal trachea during quiet respiration is approximately the luminal surface are interspersed among the ciliated
1 mm/sec.2 During forced expiration or coughing, intra- columnar cells.3 There has been recent interest in studying
thoracic pressure becomes positive, transmural pressure the small intercellular bridges (tight junctions) situated
differences increase, and narrowing of the intrathoracic between the ciliated cells.5 These junctions may explain the
trachea occurs. This narrowing accelerates airflow and observation that cilia in the respiratory tract move in a
facilitates expectoration. The airflow in the central trachea synchronous and coordinated manner. The cilia beat about
during coughing has been estimated to be approximately 1000 times per minute, propelling the mucous lining upward
250 km/hr.3 Airflow patterns in the trachea are significantly toward the pharynx, from which it can be coughed up
more complicated in cases with tracheal stenosis or tracheo- several times a day. Normally, the amount of broncho-
malacia. tracheal secretions expelled is quite small, averaging 10 cc
over a 24 hour period.3 Mechanical irritation by endotra-
cheal tubes or suction devices, however, can increase this
Gross Anatomy/Histology volume 10-fold. During disease states, the amount and
quality of the periciliary fluid and mucous can change,
The skeleton of the trachea is composed of 16 to 20 interfering with the drainage and protective functions of the
incomplete hyaline cartilaginous rings that are bound in a mucosa.
tight elastic connective tissue oriented longitudinally.1 Each tracheal cartilage has a perichondrium, continuous
These cartilage rings may calcify with age and two or more with a dense fibrous membrane that is between adjacent
cartilages may unite, either partially or fully. Rarely, they cartilages and within the posterior membranous wall. The
may bifurcate posteriorly. The cartilage forms about two perichondrium and membrane are composed primarily of
thirds of the circumference of the trachea. Because the collagen with some elastin fibers. Smooth muscle fibers
posterior border of the trachea is formed by fibromuscular (trachealis muscle) are in the membrane posteriorly. Most of
membrane, the cross-sectional shape of the trachea is that of these muscle fibers are transverse, attaching to the free ends
the letter D, with the flat side posterior. The first and last of the tracheal cartilages and providing alteration in the
cartilaginous rings differ from the rest of the tracheal rings. tracheal cross-sectional area. There are also longitudinal
The first ring, located approximately 1.5 to 2 cm below the fibers. The trachealis muscle can diminish the caliber of the
true vocal cords, is partly recessed into the broader ring of tracheal lumen or prevent overdistention of the trachea when
the cricoid cartilage and is the broadest of all the cartilage there is abdominal straining prior to coughing.
rings. The first ring is sometimes merged to the cricoid
cartilage or to the second tracheal ring. The second, third,
and fourth rings are surrounded anteriorly and laterally by Innervation and Blood Supply
the thyroid gland.2 The last tracheal ring is thicker and
broad, and on its lower border there is a triangular process The trachea is innervated by parasympathetic tracheal
that curves downward and backward between the origins of branches of the vagus nerve, by the recurrent laryngeal
the bronchi.4 The mucosal portions of the posterior trachea nerve, and by sympathetic nerves. The parasympathetic
are separated from the esophagus by a thin layer of preganglionic nerve fibers send their axons to local ganglia
connective tissue. This region is often referred to as the in the tracheal wall. The sympathetic postganglionic supply
common party wall, as it separates the trachea in front from comes from both the cervical ganglia and the second through
the esophagus behind. fourth thoracic ganglia. The parasympathetic postganglionic
The cartilaginous rings allow the tracheal lumen to retain as well as the sympathetic fibers innervate seromucous
its patency, even in the extreme circumstances of coughing glands, smooth muscles, and blood vessels.6 Tracheal
and forced expiration. The diameter of the tracheal lumen, smooth muscle, controlled by these autonomic nerves,
like its length, depends on the height, age, and gender of contracts to narrow and stabilize the tracheal lumen,
the individual. In men, tracheal diameter ranges from 13 to preventing excessive invagination of the dorsal membrane.
25 mm in the coronal plane and 13 to 27 mm in the sagittal Branches of the recurrent laryngeal nerve perforate the
plane. Tracheal diameter is slightly less in women, ranging lateral tracheal wall, providing a rich supply of sensory
from 10 to 21 mm in the coronal plane and 10 to 23 mm in afferents to the inner tracheal mucosa. The trachea has a
the sagittal plane.1 Cross-sectional area correlates most segmental blood supply, shared largely with the esophagus
closely with height in children. With increasing age, the and derived from multiple branches of the inferior thyroidal
transverse section of the tracheal lumen successively and bronchial arteries. The inferior thyroidal arteries, as well
assumes the following shapes: round, lunate, flattened, and as the extreme right intercostal artery, supply the superior
roughly elliptical. Respiratory cycle, certain maneuvers, and portion of the trachea; branches from both the bronchial
body position also contribute significantly to the variation in arteries and the third intercostal artery supply the inferior
portion of the trachea. Tracheal venous drainage is to the anterior to the trachea and can cause an indentation,
inferior thyroid venous plexus. particularly in infants. The azygos vein crosses the distal
dorsal trachea toward the right tracheobronchial angle, and
rarely produces an indentation. In infancy, the innominate
Lymphatic Drainage vein lies anterior to the lower cervical trachea; in adulthood,
this vein is intrathoracic.9, 10
Numerous lymph nodes surround the trachea, most
notably in the carinal region. The major lymph node groups
are the inferior tracheobronchial nodes and the right and left RADIOGRAPHIC EVALUATION OF THE
superior bronchial nodes. The lymphatic drainage from the TRACHEA
proximal two thirds of the trachea passes through to the
pre- and paratracheal lymph nodes (level VI), eventually The radiologic evaluation of the trachea includes plain
draining into the lower jugular nodes (level IV). films (both lateral neck and anteroposterior high-kilovoltage
The pattern of tracheal lymph node drainage is important views), tomograms, computed tomography (CT), and mag-
for the radiologist to be aware of for the purpose of lung netic resonance (MR) imaging.11 CT is of great value in as-
cancer staging. For example, left apical lung cancers tend sessing the spectrum of disease that affects the trachea
to drain to aortopulmonary window nodes rather than to and has largely become the imaging modality of choice, es-
hilar and paratracheal nodes. Lower-lobe cancers are more pecially now that two-dimensional and three-dimensional
likely to drain to hilar nodes, followed in frequency by reformatted views can be routinely obtained through image
subcarinal and paratracheal region nodes. Staging of lung postprocessing. Airway fluoroscopy, barium studies of the
cancer, according to the American Thoracic Association, is esophagus, angiography of vascular lesions, and conven-
based largely on knowledge of the nodal stations of the tional tomography all may play a part in the evaluation of
mediastinum—particularly the paratracheal nodes.7 tracheal pathology.
FIGURE 31-1 Large cell carcinoma. Posteroanterior (A) and lateral (B) views of the chest demonstrate a large,
broad-based endoluminal mass arising from the right lateral tracheal wall (arrow in A).
densities, as well as vascular enhancement of tumors or T1-weighted sequences demonstrate the anatomy of the
aneurysms. Abnormal collapsibility of the trachea (i.e., trachea and surrounding structures in detail, whereas
tracheomalacia) can be identified by paired inspiratory/ T2-weighted sequences add to the characterization of lesion
expiratory CT scans. CT is also useful in directing surgical signal differences, such as differentiation of cystic from
or fine needle aspiration (FNA) biopsy of more aggressive solid masses. MR imaging is particularly useful in cases of
lesions. Finally, spiral CT angiography with 3D reconstruc- tracheal compression by vascular rings and slings or by
tion methods (Fig. 31-2) may reveal vascular anomalies other mediastinal masses.
affecting the tracheal lumen.
Congenital lesions affecting the trachea typically require
a chest X-ray as a screening examination, followed by MR
imaging or spiral CT to identify vascular anomalies or Virtual Bronchoscopy
mediastinal masses. Inspiratory and expiratory CT can be
used to investigate tracheal stenosis. CT is also the imaging Three-dimensional reconstruction of helical CT data
modality of choice to investigate tracheal stenosis due to permits navigation through the tracheobronchial tree via
intubation or tracheotomy tubes. Finally, spiral CT is real-time simulated bronchoscopy. Recent advances in
considered a valuable tool in the investigation and treatment computer technology allow faster imaging times with a
planning of all lesions that will undergo endoscopic decrease in radiation dose and motion artifact. Unlike
therapy.12 conventional fiberoptic bronchoscopy, virtual broncoscopy
(VB) permits visualization of the relationship between an
airway lesion and the surrounding mediastinal structures as
well as airway patency beyond a stenosis. In addition, the
MR Imaging viewer can repeatedly navigate through the airway without
adverse consequences to the patient. The level of obstruction
MR imaging can be utilized in the assessment of tracheal and the width and length of an airway stenosis can easily be
stenosis and tumors. However, the current role of MR measured. This information can be used in preoperative
imaging is quite limited, as its spatial resolution does not planning for resection of tracheal tumors or for palliative
permit adequate evaluation of the segmental bronchi and the stent placement. VB also appears to be useful in imaging
longer scan times allow for respiratory motion image pediatric disorders such as vascular rings, tracheomalacia,
degradation. MR imaging, however, can depict a moderate and congenital tracheoesophageal fistula.
degree of anatomic detail without employing ionizing Despite initial enthusiasm, however, the practical appli-
radiation or iodinated intravenous contrast medium.13 cation of volume rendering techniques remains in question.
FIGURE 31-2 Postpneumonectomy syndrome. A 28-year-old female with congenital hypoplasia of the right
lung who underwent right pneumonectomy following recurrent pulmonary infections. Several months after surgery,
the patient developed severe shortness of breath. A, Contrast-enhanced axial CT scan of the chest shows right
mediastinal shift, with herniation of the left lung into the right hemithorax (upper row). Note the compression of the
proximal left mainstem bronchus between the transverse aorta and the vertebral body (arrow, left upper row). The
mediastinal shift was corrected by insertion of saline breast implants (short arrows, lower rows) into the right
pneumonectomy space. Following this, the trachea was midline and the compression of the left mainstem bronchus
was resolved. B, Three-dimensional reconstructions from the dataset in A before and after placement of saline
implants.
Conventional axial CT and virtual images are equally occur as an isolated phenomenon or in conjunction with a
accurate in estimating the maximal luminal diameter and second abnormality.
cross-sectional area of the central airways. Sufficient clinical
data are not yet available to judge whether VB offers a true
benefit to patients over that of the already high predictive Tracheomalacia
values of conventional CT. Although VB may not offer Tracheomalacia is defined as collapse of the trachea on
improved diagnostic accuracy, it is a promising technique expiration, resulting in more than 10% to 20% obstruction of
that tends to enhance the overall understanding of a disease the airway. It is characterized by abnormal flaccidity of the
process, and it will likely prove useful in surgical planning trachea during the respiratory cycle, leading to abnormal
and communication of imaging results to referring clinicians collapse of the thoracic tracheal segment on expiration.
(Figs. 31-2B, 31-3, and 31-4).14, 15 Weakness of the tracheal wall results from softening of the
supporting cartilage, with abnormal widening of the
posterior membranous (noncartilaginous) wall. Frequently,
PATHOLOGIC CONDITIONS
Clinical detection of tracheal disease is notoriously diffi-
cult. With healthy lungs, occlusion of more than 75% of the
tracheal lumen is necessary to cause symptoms of airway
obstruction at rest.1 Occlusion of about 50% of the cross-
sectional tracheal airway is needed to produce symptoms of
dyspnea on exertion. As a result, early identification of
tracheal pathology is unusual. Symptoms such as hemopty-
sis, wheezing, or coughing may not manifest until disease is
advanced. Radiographically, the trachea is often a blind spot
on conventional X-rays, with lesions not identified until they
are quite large. CT is the standard method for imaging a
patient who has clinical symptoms suggesting tracheal
obstruction.
Congenital Abnormalities
The trachea can be affected by an intrinsic anomaly
isolated to the trachea or by an anomaly of the contiguous FIGURE 31-3 Squamous cell carcinoma. Three-dimensional virtual
soft tissues that causes external pressure on the airway. bronchoscopic images demonstrate intraluminal extension of tumor above
Some abnormalities, such as stenosis or tracheomalacia, can the carina.
the anterior and posterior tracheal walls move closer Airway fluoroscopy, combined with a barium swallow,
together during respiration and coughing, reducing the can demonstrate evidence of vascular compression. Rigid
intraluminal diameter (Figs. 31-5 and 31-6). The unsup- bronchoscopy is helpful for evaluating the degree of
ported posterior wall may balloon anteriorly into the airway. tracheobronchial tree collapse, as well as for documenting
Patients complain of inspiratory and/or expiratory stridor, the presence of associated congenital airway anomalies.
wheezing, barking cough, or recurrent respiratory infections. Cine CT imaging and 3D imaging of the trachea during
The triad of an anterior bulging posterior tracheal wall, inspiration and expiration are newer modalities that are
narrowed anteroposterior tracheal luminal diameter, and likely to prove useful.16 In cases of suspected vascular
widened posterior membranous tracheal wall is typical. compression or cardiac anomalies contributing to tracheo-
Tracheomalacia is the major cause of respiratory difficulty malacia, MR imaging and echocardiography can also be
following repair of a tracheoesophageal fistula. performed.
Patients with tracheomalacia may present with minimal Mild cases of primary tracheomalacia often do not
symptoms or with severe, life-threatening airway obstruc- require treatment, because they often resolve within the first
tion. In children, the tracheobronchial tree is more collaps- 1 to 2 years of life. In cases of severe airway obstruction
ible than in adults. Hence, the classic expiratory stridor of with tracheal collapse, tracheostomy with long-term positive
tracheomalacia may be present at birth. Tracheomalacia can airway pressure ventilation may be indicated. Surgical
be categorized into either primary intrinsic or secondary decompression of the trachea may lead to significant
extrinsic forms. Primary tracheomalacia is caused by an improvement in cases of vascular compression. Different
inherent defect of the tracheal cartilaginous rings. In surgical procedures have been developed for the treatment
secondary tracheomalacia, airway collapse on expiration of tracheomalacia, with various degrees of success. These
may be due to extrinsic tracheal compression by a mass or procedures include placement of internal and external stents,
vascular structure, as well as by conditions causing intrinsic segmental resection, and cartilage grafting to the trachea.
weakness of the tracheobronchial structures. Secondary
tracheomalacia may be associated with conditions such as Congenital Stenosis (Subglottic/Tracheal)
tracheoesophageal fistula, laryngotracheal esophageal cleft, Congenital stenosis is an uncommon abnormality that
cardiac abnormalities, or vascular compression. Localized usually is associated with other major or minor anomalies of
tracheomalacia is associated with tracheotomy. the respiratory tract skeleton or other organ systems.
Commonly, there is an association with the H-type focal or affect the entire trachea, extending into the
tracheoesophageal fistula, pulmonary hypoplasia, and a mainstem bronchi. Cartilages can be complete rigid rings.
vascular sling. The affected segment of trachea has rigid Clinically, presentation occurs within the first weeks or
walls with a narrowed lumen. The stenotic segment can be months of life. Although segmental resection has been
reported, it is rarely feasible due to the length of the stenotic
segment. Most cases are treated conservatively and the
patient may live with the stenosis, the tracheal lumen
growing larger as the patient ages. However, approximately
one half of these patients die either of the stenosis and
associated pulmonary infections or of the major congenital
anomalies associated with it.
Tracheal Bronchus
The term tracheal bronchus refers to any well-defined
airway that arises from the trachea above the level of the
carina. Although this anatomic configuration is normal for
many mammals, it represents an anomaly in humans and is
found in only 0.25% to 1% of the population.17 Although the
anomalous bronchus typically arises from the tracheal wall
less than 2 cm above the tracheal bifurcation, it may arise as
high as 6 cm above the carina. The tracheal bronchus
originates more frequently from the right, rather than from
FIGURE 31-6 Tracheomalacia. A 62-year-old female with an the left, in which case it supplies a variable portion of the
increasingly loud, uncontrollable cough. Inspiratory and expiratory axial medial or apical right upper lobe (Fig. 31-7). The incidence
CT images of the upper thorax demonstrate a 44% reduction in the
anteroposterior tracheal diameter on expiration. Both flexible and rigid of this anomaly is greatest in patients with other coexisting
bronchoscopy revealed a flattened, lax trachea with a redundant congenital malformations such as congenital tracheobron-
membranous wall. chial stenosis, aberrant pulmonary artery, Down’s syn-
anomalies involving the cardiovascular, gastrointestinal, site, usually resolves by the age of 2 years, and is best
renal, or central nervous system may also be present.22 diagnosed by collapse of the airway structures on direct
Patients with normal birth weight and no other congenital fluoroscopy. Patients with esophageal atresia and both
malformations have the highest rates of survival. The overall proximal and distal TEFs (rare) may have gaseous disten-
survival rate is approximately 80%.2 tion of the gastrointestinal tract with aspiration pneumonia.
H-type TEF, without esophageal atresia, is a less This diagnosis can be confirmed by contrast studies. Barium
common type of TEF that has more subtle clinical swallow with fluoroscopy is typically employed to diagnose
manifestations and is characteristically associated with H-type fistulas. Cine-esophagography (cinefluorography or
gastroesophageal reflux. Symptoms may include excessive fluoroscopy with videotaping) can assist in differentiat-
oral secretions, respiratory distress, or immediate regurgita- ing TEF from simple laryngeal aspiration. Combined
tion of feedings; intermittent dysphagia and choking may bronchoscopy and esophagoscopy have also been useful in
also be present. The diagnosis of this type of TEF may be increasing the ability to diagnose TEF. Surgery is the
delayed until adulthood. Patients with H-type TEF usually treatment of choice.23
have no associated congenital anomalies or can have
congenital tracheal stenosis. Vascular Rings and Slings
TEF with esophageal atresia is easily diagnosed by the Vascular anomalies of the great vessels include ‘‘rings’’
failure to pass a radiopaque nasogastric tube into the and ‘‘slings’’11, 24, 25 (see also Chapter 29). Airway com-
stomach. The tube coils in the proximal esophageal pouch pression by vascular anomalies may be congenital or
and can be seen on routine chest radiography or after the acquired. In children, vascular impressions on the trachea
instillation of a small amount of water-soluble contrast are generally congenital and may cause a spectrum of
material (Fig. 31-10B). Associated aspiration pneumonia symptoms, from minimal to severe airway compression
may be present. Tracheomalacia may develop at the fistula requiring intervention. Vascular compression syndromes in
adults are usually secondary to acquired aneurysmal anomalies may lead to significant morbidity and occasional
dilatation of the great vessels. The radiologic evaluation of mortality.26 However, vascular rings often cause more
children with respiratory symptoms possibly due to vascular severe airway symptoms than slings.23 In the pulmonary
compression should include barium study of the esophagus, artery sling, the left pulmonary artery originates from the
which is often affected along with the trachea by vascular right pulmonary artery and then passes between the trachea
rings. Contrast-enhanced CT angiography with 3D recon- and esophagus to reach the left lung. The trachea is
structions and multiplanar reformatting is an excellent compressed from posteriorly, and there is an impression on
method of evaluating patients with complex great vessel the anterior esophagus seen at barium swallow.
anomalies. Alternatively, MR imaging can provide images Anterior impression on the trachea by the innominate
of the vessels and the surrounding soft tissues. artery in infants and children is much more common than
Vascular rings are anomalies that completely encircle the compression by an aberrant left subclavian artery.27
trachea and esophagus, causing airway compression. The Although most patients develop symptoms during the first
most common vascular ring is a double aortic arch (Fig. 31- year of life, some reported cases have presented as late as 15
12).26 Patients with double aortic arch typically present early years of age. Symptoms include expiratory stridor, recurrent
in life with airway obstruction and often require early surgi- cough, apnea, and recurrent infection. Rigid bronchoscopy
cal intervention. Another, less constricting, vascular ring is a may establish the definitive diagnosis. In severe cases of
right aortic arch with descending right aorta associated with airway obstruction, surgical intervention is warranted.
an aberrant left subclavian artery and persistent ligamentum
arteriosum. In both types of vascular ring, there is circumfer-
ential tracheal and esophageal indentation. Other findings in- Diffuse Disorders of the Trachea
clude left-sided and anterior displacement of the distal tra- (Nonneoplastic)
chea. Barium studies show a characteristic dorsal and left-
sided impression on the esophagus. Various infections and inflammations involve the tra-
Vascular slings are noncircumferential vascular anoma- cheal wall. They may cause infiltration with narrowing or,
lies that may also cause airway compromise. These less commonly, widening of the trachea.
FIGURE 31-10 Tracheoesopheagal atresia. A, Multiple attempts to pass a nasogastric tube were unsuccessful in
this full-term infant with esophageal atresia. The level of the nasogastric tube tip marks the level of the atresia. B,
Chest X-ray from a different infant demonstrates a coiled nasogastric tube in the cervical esophagus, with contrast
filling the proximal esophageal pouch (arrow). This premature infant presented with copious oral secretions and
noisy respiration. Gas within the bowel indicates a distal communication between the tracheal bronchial tree and the
gastrointestinal tract.
Tuberculosis
Tuberculous tracheitis is rare and is usually associated
with active pulmonary tuberculosis.32, 33 The infection
typically involves the distal trachea, where inflammation
results in irregular circumferential tracheal wall thickening,
leading to narrowing or even obstruction. Areas of necrosis
and ulcerations may develop. Mediastinal and hilar lymph-
adenopathy are usually present. Fibrosis from chronic
inflammation can lead to segmental stenosis of the trachea
and bronchi. Erosion into adjacent mediastinal structures
can lead to the development of a tracheoesophageal fistula or
to severe hemorrhage from vascular invasion. Erosion of
calcified lymph nodes into the airway can lead to
expectoration of broncholiths.34
tuberculosis, intraluminal extension is associated with lower respiratory tracts and may result in tracheal stenosis. It
hemoptysis and expectoration of broncholiths. typically occurs in adults, with a peak incidence in the fourth
Fibrosing mediastinitis is a fibroinflammatory infiltrative and fifth decades of life.42 Two or more organ systems may
process most commonly attributed to histoplasmosis.25, 35–38 be affected simultaneously. Pulmonary and renal involve-
Large masses of fibrous tissue can form in the mediastinum. ment together occurs in more than two thirds of cases.43 The
The accumulated fibrous tissue can squeeze the trachea or kidneys are frequently affected, as are other organs such as
bronchi, resulting in long areas of airway narrowing. The the skin, nervous system, eyes, ears, and heart. Tracheal
patient can present with cough, hemoptysis, or difficulty involvement typically manifests as diffuse or focal tracheo-
breathing. The pulmonary arteries and veins can also be bronchial narrowing.44 Although destruction of cartilage
narrowed, resulting in significant vascular compromise. The may result, malacia is not typical. Dyspnea and progressive
lesions usually calcify and can have low signal intensity on airway obstruction develop over months or years following
T2-weighted images. These features may help differentiate the initial diagnosis of the disease.
the lesion from an invasive malignancy. Fibrosing medias- CT features include circumferential wall thickening with
tinitis has also been associated with tuberculosis, lymphatic narrowing of the tracheal lumen. Enlarged, abnormally
obstruction, and methylsergide. calcified tracheal cartilage may also be present.44 Mediasti-
CT is the best single technique for diagnosing and nal and hilar lymphadenopathy are rare thoracic manifesta-
assessing the severity of mediastinal fibrosis, as well as for tions of Wegener’s granulomatosis.45
evaluating tracheal luminal narrowing. CT studies per-
formed both prior to and following the administration of Sarcoidosis
intravenous contrast may be helpful in differentiating Sarcoidosis is a systemic granulomatous disease of
calcified lymph nodes from enhancing vessels. unknown etiology. Involvement of the lungs, mediastinal
Mediastinal granuloma is closely related to fibrosing lymph nodes, eyes, and skin is common. On rare occasion,
mediastinitis. This term describes a well-demarcated mass the trachea can be involved.46 Sarcoidosis can lead to diffuse
of lymph nodes located in the mediastinum. It is commonly narrowing of the trachea and main bronchi and can cause
discovered as a mass lesion on routine chest radiography, upper-airway obstruction (Fig. 31-13). Dyspnea and cough
typically in the right paratracheal or hilar regions. Mediasti- usually result from extensive fibrosis, which causes distor-
nal granuloma is often encapsulated and asymptomatic. If tion and narrowing of the airway. Bronchial compression by
symptoms are present, they relate to compression of various mediastinal lymph nodes and airway hyperreactivity, pre-
mediastinal structures, including the major veins, tracheo- sumably due to inflammation, contribute to the symptoms.
bronchial tree, esophagus, recurrent laryngeal nerves, While obstruction of the lower airway by granulomatous
pulmonary arteries, and heart. Narrowing of the tracheo- infiltration and chronic pulmonary fibrosis is common,
bronchial tree typically involves the major bronchi, with less tracheal stenosis is rare.
frequent involvement of the distal trachea. Surgery is often
indicated for diagnostic reasons (e.g., to exclude lymphoma) Amyloidosis
or for relief of specific symptoms.39 Amyloid infiltration of the airways may occur as an
isolated phenomenon or as part of a diffuse systemic disease.
Relapsing Polychondritis The focal form of amyloidosis, in which amyloid deposits in
Relapsing polychondritis is a rare systemic disease a tumor-like fashion, is usually limited to one organ or organ
characterized by recurrent episodes of inflammation, frag- system. This form can be found in approximately 10% of
mentation, and fibrosis of cartilage tissue including the cases.47 Primary solitary amyloidosis of the trachea is very
ears, nose, joints, and airways. Its incidence peaks in the rare and has no features to differentiate it from other
fifth decade of life, and there is no sex predilection.39 tumors.48 Deposition of amyloid in the submucosal and
The upper airways (larynx and trachea) are affected in muscular layers of the trachea and bronchi can lead to
more than half of the patients. Tracheal involvement occurs tracheal narrowing and ulceration.
late in the course of the disease. Diffuse tracheal inflamma- Tracheobronchial amyloidosis can involve the bronchial
tion is limited to the cartilage and perichondrium, with tree either as a single pseudotumorous mass or in a diffuse
sparing of the mucosa and submucosa.40 Respiratory form.49 The diffuse form is more frequent and is character-
manifestations are often severe and may be life- ized by extensive subepithelial amyloid deposits. The
threatening.41 Cardiovascular manifestations are present in elevated mucosa, which forms ridges and nodules (approxi-
one third of patients. mately 0.5 to 5 cm in diameter) that may calcify, can narrow
CT usually shows tracheal narrowing with diffuse wall the tracheal lumen.2 Plain film radiographs and CT scanning
thickening limited to the anterior and lateral tracheal walls, demonstrate focal or diffuse narrowing of the trachea and
sparing of the posterior membrane, and narrowing of the bronchi (Fig. 31-14). The nodular form of tracheobronchial
lumen.1 When calcification is seen within the tracheobron- amyloid may appear on CT as a focal mass, partially or
chial wall, the cartilage may appear thicker than normal. completely occluding the airway. There is often infiltration
Both the inner and outer borders of the thickened tracheal of adjacent peritracheal or bronchial tissue, frequently with
wall are smooth, and areas of tracheomalacia may coexist. calcified foci. In this form, it may mimic the appearance of
Fiberoptic bronchoscopy can be useful for both direct both benign and malignant tracheal tumors. Depending on
visualization and biopsy. the size and calcification of the deposition, hemoptysis or
airway obstruction may occur, ultimately resulting in
Granulomatous Disease of the Trachea atelectasis or recurrent pulmonary infection. Resection with
Wegener’s granulomatosis is a hypersensitivity necrotiz- a carbon dioxide laser and repeated bronchoscopic resection
ing granulomatous vasculitis that can involve the upper and are successful methods of treatment.39
Diffuse Conditions Causing Widening of the Trachea connective tissue disorders, such as Ehlers-Danlos syn-
Tracheobronchomegaly (Mounier-Kuhn Syndrome) drome and acquired cutis laxa.
This rare condition, characterized by marked dilatation of The diagnosis of tracheobronchomegaly is made primar-
the trachea and mainstem bronchi, is associated with atrophy ily by radiologic evaluation. On plain chest radiographs the
of the cartilaginous, muscular, and elastic components of the trachea appears enlarged, and bronchiectasis is present. CT
tracheal wall. Patients commonly have associated bronchi- findings include tracheomegaly, with thinning of the
ectasis, with a history of recurrent pneumonia. The disease is tracheal wall, and a diameter of more than 3 cm below the
most commonly diagnosed in males in their third to fourth level of the aortic arch or more than 2 cm above the level of
decades of life. Patients typically have a long-standing, the arch (Fig. 31-16). Tracheal scalloping or frank diverticu-
unusually loud, productive cough, with hoarseness and losis can also be present, although difficult to appreciate on
dyspnea. There is an association with other primary cross-sectional imaging. Three-dimensional reconstructions
FIGURE 31-14 Amyloid. A, Chest radiograph of a 65-year-old man demonstrates a diffusely thickened tracheal
wall on the posteroanterior view (arrows). B, T1-weighted MR image through the lower cricoid shows diffuse
concentric soft tissue (arrow) narrowing the subglottic airway. The arrowhead indicates the position of the inner
cortex of the cricoid cartilage. C, Axial T1-weighted image through the upper trachea shows the narrowing leaving a
very narrowed airway (arrow). T, thyroid gland. D, T2-weighted axial image at the level of the lower cricoid shows
that the abnormality has very low signal (arrow).
Illustration continued on following page
may be used to display this deformity. Another common tracheal mucosa. Mechanical internal injuries are invariably
association is marked tracheomalacia. There can be a due to iatrogenic causes, such as laryngotracheal intubation.
significant decrease in the diameter of the trachea on External traumatic tracheal injuries are more commonly
expiration, even to the point of airway closure. due to blunt rather than penetrating trauma. The spectrum of
injury includes small lacerations, tears of the posterior
Secondary Tracheomegaly membranous wall, fracture of the cartilaginous rings, and
Chronic coughing coupled with ongoing chronic inflam- complete separation. Penetrating trauma is most often the
mation has been blamed for diffuse acquired enlargement of result of stab wounds or projectile injuries. Tracheal injuries
the tracheal lumen. This has been described in cigarette most often occur in association with other severe injuries.
smokers and in patients with cystic fibrosis. Motor vehicle collisions are the most common cause of
blunt tracheal injuries. Although rupture of the trachea in
itself is a life-threatening injury, mortality is typically
Trauma caused by damage to other surrounding vital organs.
Radiologic clues to tracheobronchial traumatic injury
Tracheal trauma may result from either internal or include pneumomediastinum, subcutaneous emphysema,
external injury. Internal tracheal injuries are the result of and pneumothorax. Other signs of tracheal injury include
inhalation of noxious fumes, gas, or steam or of aspiration. elevation of the hyoid bone and distortion of the normal
Edema, followed by rapid laryngotracheal airway obstruc- laryngotracheal air column.53 Anteroposterior and lateral
tion, may result from chemical or thermal injury to the plain films of the neck are useful in identifying free air and
in evaluating for the presence of associated cervical spine the diameter of the tracheal lumen to minimize airway
trauma. Plain chest radiographs are essential in the resistance. Inflated tube cuffs are relatively easy to
evaluation of tension pneumothorax or hemothorax. Clinical recognize on chest X-ray; their position should be checked
findings include new onset of stridor, endobronchial whenever a chest X-ray is obtained. The inflated cuff should
bleeding, and respiratory failure. Direct bronchoscopy is fill, but not bulge, the tracheal lumen. Rigid low-volume
often required for definitive diagnosis.47
Posttracheostomy/Postintubation Complications
The increasing use of cuffed endotracheal tubes and
tracheostomy for long-term ventilation has led to character-
istic injuries of the trachea. Complications from translaryn-
geal intubation may lead to acute airway obstruction due to
postextubation glottic edema or laryngospasm. Alterna-
tively, symptoms may develop months or even years after
injury due to glottic or tracheal stenosis.54
A portable chest radiograph should be obtained following
laryngotracheal intubation to ensure proper positioning of
the endotracheal tube. Ideally, the tip of the endotracheal
tube should be placed well above the carina, with the head
and neck in a neutral position. It is important to recognize
that flexion and extension of the neck will cause movement
of the endotracheal tube of approximately 2 cm in either
direction. In addition, rotation of the head can cause a 1 to
2 cm ascent of the tube. Unlike endotracheal tubes,
tracheostomy tubes do not change position with head or
FIGURE 31-16 Tracheomegaly. Nonenhanced axial CT scan, ob-
neck movement, because tracheostomy tubes are not tained at three levels in the upper thorax, demonstrates marked dilatation of
anchored to the nose or mouth. the trachea in this patient with recurrent pneumonia. Note that above the
Ideally, endotracheal tube lumens should be two thirds level of the aortic arch, the tracheal diameter is greater than 2 cm.
cuffs have been replaced by high-volume, low-pressure difficulty in clearing of secretions, or, if persistent, erosion
cuffs, which are significantly more compliant and therefore or perforation of the tracheal wall. Anterior perforation may
adapt better to the variable cross-sectional shape of the result in trachea-innominate artery fistula, whereas posterior
tracheal lumen.2 Most importantly, these newer cuffs perforation may lead to tracheoesophageal fistula.55
provide sufficient sealing at approximately one tenth of the Late tracheostomy and postintubation stenosis can occur
pressure required for rigid cuffs (about 20 to 30 mm Hg). at the tracheostomy opening (stoma), at the balloon cuff site,
Tracheal narrowing at the cuff site has dramatically or, rarely, where the tip of the tube abuts the tracheal
decreased in frequency since the introduction of these high- mucosa. Most long-term complications are due to pressure
volume, low-pressure cuffs. Immediate complications fol- necrosis at the cuff site.44 When cuff pressure exceeds
lowing laryngotracheal intubation can result from insertion capillary pressure, the blood supply to the tracheal mucosa is
of the tube into the right mainstem bronchus or positioning compromised and ischemic necrosis results.44 At first, the
of the tube tip too close to the carina. The risk of aspiration is mucosa overlying the tracheal cartilages undergoes pressure
significantly increased if the cuff is malpositioned in the necrosis. If the cuff is removed at this point, cicatricial
vocal cords or within the pharynx. In addition, overdisten- healing usually results in an anterior tracheal web/scar. The
tion of a malpositioned cuff may lead to vocal cord necrosis. flexible posterior wall is uninvolved. However, with greater
Early complications of tracheostomy are usually associated cuff pressure, the posterior wall is also affected and a
with abnormal anterior, posterior, or lateral angulation of the circumferential scar is produced. Usually this type of
tube. This can result in increased airway resistance, tracheal damage is limited to one or two tracheal rings at the
level of the cuff-related injury (Figs. 31-17 to 31-22).
Radiographically, the stenosis has a smooth edge. Typically,
symptoms of stenosis develop 2 to 6 weeks postextubation
and, in some patients, months or even years later.44 Stenosis
at the tracheostomy stoma may be prevented by improved
FIGURE 31-25 Tracheal stent. A 30-year-old male who suffered from an accidental pistol discharge was
intubated for 20 days. Approximately 1 week following extubation, he developed difficulty breathing and was found
to have granulation tissue causing tracheal stenosis. A covered tracheal stent was placed, providing relief of
symptoms. A, Pre-stent AP chest X-ray shows narrowing of the subglottic airway above the level of the
tracheostomy (arrow). B, Anteroposterior view of the chest following stent placement demonstrates the covered
tracheal stent extending from the subglottic trachea to approximately 3 mm above the carina.
Adult tracheal tumors occur between the third and fifth and the carina are the most common locations (Fig. 31-27).58
decades of life, with no sex predominance. Most occur at At the time of presentation, the tumor is frequently locally
the distal third of the trachea.58 Symptoms associated advanced, with extension into the major bronchi and
with tracheal tumors include dyspnea, cough, hemoptysis, mediastinum.58, 62 Malignant tracheoesophageal fistula can
new-onset asthma or wheezing, dysphagia, stridor, change result from such extension. Lymph node metastases are
in voice and/or hoarseness, recurrent pneumonia, and generally seen in later disease stages, with distant metastases
emphysema. Approximately one third of patients with uncommon (Fig. 31-28). Patients may present with cough,
tracheal tumors are initially mistakenly treated for asthmatic dyspnea, hemoptysis, wheezing, stridor, and recurrent
bronchitis.44 pneumonia.2 The development of hoarseness usually indi-
cates infiltration of the recurrent laryngeal nerve. The
Malignant Tumors prognosis of patients with SCC is poor, with 5-year survival
The most common malignant tracheal tumor in adults is reported to be between 10% and 30%.2
squamous cell carcinoma, followed by adenoid cystic Radiologic findings include focal and/or circumferential
carcinoma. Together, these two neoplasms account for up to irregular thickening of the tracheal wall with luminal
86% of all malignant adult tracheal tumors.1 Secondary narrowing (Fig. 31-3). CT is useful in demonstrating the
malignant tumors are due to either metastases or direct primary tumor, as well as extension of tumor into
tracheal invasion by adjacent mediastinal neoplasms. The surrounding structures.44
most common primary carcinomas that spread directly to the
trachea are from the lung, thyroid, or esophagus (Fig. Adenoid Cystic Carcinoma
31-26).1 The most common carcinomas to metastasize Adenoid cystic carcinoma (ACC) is the second most
hematogenously to the trachea are breast, gastrointestinal, common primary tracheal malignancy. Unlike SCC, there is
melanoma, lymphoma, renal cell carcinoma, or squamous no definite association with cigarette smoking, and the
cell carcinomas of the head and neck.60, 61 tumor occurs with equal frequency in males and females.
The peak incidence of ACC is about 10 years earlier than in
Squamous Cell Carcinoma patients with SCC.58 ACC arises from the tracheobronchial
Squamous cell carcinoma (SCC) is the most common mucous glands and is usually located on the posterolateral
primary malignancy of the trachea. It typically affects wall of the trachea.1 It is composed of small epithelial cells
middle-aged male smokers (mean age, 50 to 60 years).2 SCC arranged in sheets, with fenestrations that produce a
is associated with other malignant neoplasms of the cribriform pattern. The tumor is a low-grade malignancy
respiratory and digestive tracts in one third of patients. The that tends to invade insidiously the submucosal plane of the
relatively high incidence of synchronous or metachronous trachea.44 Occasionally, circumferential involvement of the
tumors, found most frequently in the larynx, lung, or trachea and bronchus without a distinct mass can be seen.
esophagus, makes comprehensive evaluation and follow-up ACC infiltrates through the fibrous membrane between
imaging of the entire respiratory tract essential. Although adjacent cartilage plates and into surrounding tissues in the
SCC may arise at any level in the trachea, the lower trachea neck and mediastinum.63 There may be perineural, intra-
neural, or pericardial great vessel involvement.60 Indeed, patients are free of disease 5 years after therapy, but late
perineural spread of tumor is a distinctive characteristic of recurrence, or survival with locally recurrent tumor for
ACC. ACC spreads to cervical and mediastinal lymph periods of 10 to 15 years, is common.64, 65
nodes in 10% of patients.58 Involvement of the larynx or the
recurrent laryngeal nerve may result in restriction of vocal Mucoepidermoid Carcinoma
cord motion. Hematogenous metastases spread most com- Mucoepidermoid carcinoma is a rare tumor that can
monly to the lungs, with occasional spread to the brain or occur as either a low-grade or a high-grade malignancy. The
bony skeleton.63 tumor arises from the minor salivary glands lining the
ACC appears on radiographs as either a broad-based or tracheobronchial tree and consists of mucus-secreting cells,
pedunculated polypoid lesion. There may be smooth or squamous cells, and intermediate cells that have no
nodular thickening of the tracheal wall, with associated particular differentiating characteristics.
luminal narrowing.44 Barium swallow may demonstrate Mucoepidermoid carcinoma has been reported in patients
extrinsic esophageal compression or invasion. CT is useful ranging in age from 4 to 78 years, but nearly 50% of patients
in demonstrating the primary tumor and its extent (Fig. are under the age of 30.66 The most common presenting
31-29). However, because ACC may grow in the submuco- symptoms relate to airway irritation (cough or hemoptysis)
sal planes without producing a distinct mass, CT may or obstruction (wheezing or pneumonia).66 In its low-grade
underestimate the true extent of tumor. form, mucoepidermoid carcinoma grows slowly, is noninva-
The treatment of ACC involves surgical resection and sive, and therefore rarely metastasizes.67 Completely resect-
anastomosis when possible. When surgical resection is able low-grade tumors have an excellent prognosis, and
performed, frozen section pathologic evaluation of the these patients rarely have tumor recurrence. Even in its
biopsy margins is recommended. Radiation therapy is used high-grade form the tumor is slow-growing and has a better
when surgery is not indicated. ACC has the best prognosis prognosis than do the more common bronchogenic carcino-
among the malignant tracheal neoplasms.2 About 75% of mas. In one study,68 only 15% of high-grade mucoepider-
FIGURE 31-26 Carcinoma of the thyroid. Anterior (A) and lateral (B) conventional X-ray tomographic
projections, as well as lateral plain film view of the neck (C), demonstrate a focal impression on the anterior left
lateral wall of the trachea caused by an extrinsic thyroid carcinoma (B, arrow). The tracheal lumen is reduced to less
than half of its normal width in the anteroposterior dimension.
FIGURE 31-27 Recurrent squamous cell carcinoma at the carina. A 48-year-old male after pneumonectomy for
SCC of the lung presented with cough and sputum production. Contiguous contrast-enhanced axial chest CT images
demonstrate soft-tissue infiltration anterior to and to the left of the distal trachea extending to the carina (arrows).
Irregularity of the anterior tracheal wall, carina, and left mainstem bronchus is indicative of neoplastic invasion.
Postpneumonectomy changes are noted.
FIGURE 31-29 Adenoid cystic carcinoma. A, Chest radiograph demonstrates a large, broad-based endoluminal
mass arising from the right tracheal wall (arrows) and partially obstructing the tracheal lumen. B, Axial
nonenhanced CT scan demonstrates this large, lobulated, low-attenuation endoluminal mass arising from the right
tracheal wall at the level of the thoracic inlet (arrow). Tumor is seen along the outer wall of the calcified cartilage as
well, indicating infiltration either through or between the tracheal cartilages.
FIGURE 31-30 Glomus tumor. An 86-year-old male with episodic shortness of breath, cough, and hemoptysis.
Rigid bronchoscopy and biopsy revealed multiple tracheal nodules and tracheal thickening. Biopsy demonstrated
glomus tumor. Sagittal reconstruction of a contrast-enhanced CT scan demonstrates a tracheotomy tube that extends
to the level of the transverse aorta (upper left corner). Diffusely thickened soft tissue with nodular components
encases the trachea (remaining panels).
occasionally hemoptysis. These tumors nearly always particularly after surgical resection of pedunculated tumors.
manifest stippled calcification, although clear-cut calcifica- Other methods of treatment, such as fulguration, cryother-
tion is detected on plain films in 10% to 15% of patients in apy, and laser ablation, seem to reduce recurrence rates more
most series.74 If calcification is of the typical ‘‘popcorn’’ than local bronchoscopic resection.
configuration of cartilage calcification, the diagnosis of
harmatoma is virtually certain. The contrast resolution of Benign Mixed Cell Tumors
CT scanning improves the ability to identify calcium and fat Benign mixed cell tumors (pleomorphic adenomas) of the
within these lesions. Surgical excision is the treatment of trachea are histologically similar to benign mixed tumors of
choice. the major salivary glands. These contain a mixture of
epithelial cells, cartilaginous elements, and mesenchymal
Carcinoid Tumor cells. Although these lesions are less commonly found in
Carcinoid tumors of the trachea characteristically infil- tracheal or bronchial minor salivary glands than in major
trate the tracheal cartilage, growing slowly in a dumbbell salivary glands, they constitute the fifth most common type
configuration. The age range at presentation is variable, but of benign tracheal mass. Treatment consists of complete
the peak incidence is in the fifth to sixth decades. The excision; recurrence is unusual.
atypical form of bronchial carcinoid has cellular and clinical
features intermediate between those of typical carcinoid and
SCC.75 All types of carcinoid tumors are of neuroendocrine Tumor-Like Conditions
origin and are thought to form a spectrum of varying degrees
of malignancy. Because these tumors are vascular, hemopty- Many nonneoplastic diseases can present as tracheal or
sis is a common presenting complaint. Other presenting bronchial masses. Infectious diseases such as tuberculosis,
symptoms include cough, pneumonia, atelectasis, and systemic histoplasmosis, or even coccidioidomycosis can
wheezing. The tumor may be predominantly intraluminal, form intraluminal granulomas that may simulate primary
assuming a polypoid configuration, or predominantly tumors. Traumatic injury of the trachea can also incite
extraluminal (an ‘‘iceberg’’ lesion). Calcification and/or granuloma formation. Occasionally, aspirated foreign bod-
ossification within the tumor may occur.76 Cushing’s ies may adhere to the tracheal wall and simulate tumor.
syndrome may develop due to secretion of corticotropins by Cysts and mucoceles in children must be differentiated from
tumor cells, and carcinoid syndrome uncommonly occurs common tumors such as hemangiomas and fibromas.
as a complication of carcinoid tumor.77 CT scanning Ectopic thyroid tissue in the trachea, although rare, can
provides superb anatomic localization of both the intralumi- simulate neoplastic disease. Ectopic thyroid tissue is found
nal and extraluminal components of the tumor. CT also may in 1 of 7000 to 18,000 patients who undergo thyroid gland
identify calcification and enhancement within the tumor. surgery (with a female predominance). It may come to
Recurrence of carcinoid tumor is a significant problem, clinical attention due to growth of normal thyroid tissue,
FIGURE 31-31 Squamous papilloma. Lateral chest radiograph (A) and noncontrast axial chest CT (B)
demonstrate a polypoid endoluminal mass (B, short arrow, A, long arrow) protruding from the posterior left lateral
wall of the midtrachea just above the level of the aortic arch.
FIGURE 31-32 Tracheal papillomatosis. A, Anteroposterior chest radiograph demonstrates multiple exophytic
masses projecting into the tracheal lumen (arrow). B, Coronal plain film tomography demonstrates multiple
polypoid masses arising from the tracheal wall (arrowheads ). C, Axial contrast-enhanced CT scan through the
midtrachea demonstrates multiple pedunculated and broad-based exophytic polypoid masses arising from the
tracheal wall (arrows).
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