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Residents Section Pat tern of the Month

Cantin et al.
Bronchiectasis

Residents Section
Pattern of the Month
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Residents

inRadiology Bronchiectasis
Luce Cantin1 Bronchiectasis is a relatively frequent condition in the United States, with an estimated
Alexander A. Bankier prevalence of 4.2 per 100,000 persons 1834 years old and 272 per 100,000 persons 75 years
Ronald L. Eisenberg or older. It is defined as an irreversible localized or diffuse dilatation, usually resulting from
chronic infection, proximal airway obstruction, or congenital bronchial abnormality. On
Cantin L, Bankier AA, Eisenberg RL chest radiographs, bronchiectasis manifests as tram tracks, parallel line opacities, ring opaci-
ties, and tubular structures. However, chest radiographs lack sensitivity for detecting mild or
even moderate disease. CT is substantially more sensitive than chest radiography for showing
bronchiectasis, which is characterized by lack of bronchial tapering, bronchi visible in the
peripheral 1 cm of the lungs, and an increased bronchoarterial ratio producing the so-called
signet-ring sign. According to appearance and severity, bronchiectasis can be classified as
cylindric, varicose, or cystic (Fig. 1). The wide differential diagnosis of bronchiectasis can be
substantially narrowed by considering both the anatomic location and the distribution of this
pathology (Fig 2).

Keywords: airway obstruction, bronchiectasis

DOI:10.2214/AJR.09.3053

Received May 15, 2009; accepted after revision


May 26, 2009.

1
All authors: Department of Radiology, Beth Israel
Deaconess Medical Center, Harvard Medical School, 330
Brookline Ave., Boston, MA 02215. Address correspon-
dence to R. L. Eisenberg (rleisenb@bidmc.harvard.edu). A B
WEB Fig. 1Categories of bronchiectasis.
This is a Web exclusive article.
AD, Normal bronchus (arrow) (A), cylindric bronchiectasis with lack of bronchial tapering (arrow) (B), varicose
bronchiectasis with string-of-pearls appearance (arrow) (C), and cystic bronchiectasis (arrow) (D).
(Fig. 1 continues on next page)
AJR 2009; 193:W158W171

0361803X/09/1933W158

American Roentgen Ray Society

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Bronchiectasis
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C D
Fig. 1 (continued)Categories of bronchiectasis.
AD, Normal bronchus (arrow) (A), cylindric bronchiectasis with lack of bronchial tapering (arrow) (B), varicose
bronchiectasis with string-of-pearls appearance (arrow) (C), and cystic bronchiectasis (arrow) (D).

Detection of bronchiectasis

Focal bronchiectasis Diffuse bronchiectasis

Congenital bronchial atresia Peripheral Central


Extrinsic compression predominance predominance
Endobronchial malignancy
Foreign body
Broncholithiasis
Airway stenosis

Upper lung Lower lung Right middle lobe and Allergic bronchopulmonary aspergillosis
predominance predominance lingula predominance Mounier-Kuhns syndrome
Williams-Campbell syndrome

Cystic fibrosis Idiopathic Atypical mycobacterial infection (MAI)


Sarcoidosis Postinfectious Immotile cilia syndrome
Postradiation fibrosis Repeated aspiration
Fibrotic lung disease
Posttransplant rejection
Hypogammaglobulinemia

Fig. 2Flowchart shows algorithm for evaluation of bronchiectasis. MAI = Mycobacterium avium-intracellulare.

Focal Bronchiectasis
Any cause of airway obstruction can lead to focal bronchiectasis (Fig. 3). In contrast to
diffuse bronchiectasis, focal bronchiectasis requires diagnostic bronchoscopy in almost all
patients.

Bronchial Atresia
The most common cause of congenital focal bronchiectasis is bronchial atresia, character-
ized by obliteration of a bronchus with distal bronchiectasis, mucoid impaction, and air trap-
ping that is most commonly seen in the left upper lobe (Fig. 4). In this rare lesion, the bron-
chial tree peripheral to the point of obliteration is patent and the lung parenchyma is overin-
flated because of collateral air drift.

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Cantin et al.

Fig. 3Focal
bronchiectasis
(idiopathic) in left lower
lobe (arrow).
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A B
Fig. 4Bronchial atresia.
A, Transverse image of focal bronchiectasis (arrow) distal to bronchial atresia associated with hyperlucency
and hyperexpansion of left lung.
B, Coronal image shows proximal mucoid impaction (arrow), distal bronchiectasis (arrowhead), and widespread
air trapping of left lung.

Extrinsic Compression
Extrinsic compression is an acquired cause of focal bronchiectasis, most commonly caused
by lymphadenopathy, usually from previous granulomatous exposure. Less frequent causes
include sarcoidosis, hilar mass, and metastatic lymphadenopathy.
Endoluminal Obstruction by Tumor
Most carcinoid tumors are primarily endobronchial lesions, occurring in the central, main,
or segmental bronchi (Fig. 5). Some small tumors are located entirely within the lumen. How-

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A B

C D
Fig. 5Carcinoid. This predominantly endobronchial tumor, arising before bifurcation of left upper and lower
lobe bronchi, causes distal bronchiectasis.
A and B, Transverse images of tumor (arrow, A) and distal bronchiectasis (arrows, B).
C and D, Coronal oblique image (C) and volume-rendering reformation (D) in similar orientation as A and B show
central carcinoid tumor (arrows) and distal bronchiectasis (arrowheads, C).

ever, some display a dominant extraluminal component with only a small part of the tumor
lying within the airway (iceberg lesion). A variety of other benign and malignant neoplasms
can also result in obstruction leading to focal bronchiectasis.
Foreign Body
Aspirated foreign material can result in focal bronchiectasis. Persistence of a noncalcified
foreign body, such as a vegetable fiber, within a bronchus for a prolonged period of time can
serve as a nidus for calcium deposition.
Broncholithiasis
Calcified or ossified material within the bronchial lumen can cause focal bronchiectasis.
By far the most common cause of broncholithiasis is erosion by and extrusion of a calcified

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A B
Fig. 6Broncholithiasis.
AC, Calcified left upper lobe endobronchial
broncholithiasis (arrow) from previous tuberculosis
exposure is seen on transverse image (A), minimum-
intensity-projection reformation in coronal oblique
plane (B), and volume-rendering reformation (C)
in similar orientation. In C, arrow points to distal
bronchiectasis.

C
adjacent lymph node, usually associated with a long-standing focus of necrotizing granu-
lomatous lymphadenitis, especially after tuberculosis (Fig. 6). Nevertheless, the frequency of
broncholithiasis complicating granulomatous infection is quite low. The most common sites
are the proximal right middle lobe bronchus and the origin of the anterior segmental bronchus
of the upper lobes because of airway anatomy and lymph node distribution.

Airway Stenosis
Airway stenosis causing focal bronchiectasis (Fig. 7) can result from a broad spectrum of
entities including infection, intubation stricture, healing of a tracheostomy stoma, tracheo-
bronchopathia osteochondroplastica, amyloidosis, relapsing polychondritis, sarcoidosis, and
fibrosing mediastinitis.

Diffuse Bronchiectasis: Upper Predominance


Cystic Fibrosis
The most common cause of congenital upper-lung-predominant bronchiectasis is cystic
fibrosis, commonly associated with enlarged lung volumes and interstitial alterations (Fig. 8).
An autosomal recessive genetic disorder causing ineffective clearance of secretions, cystic
fibrosis presents with recurrent pneumonias, sinusitis, pancreatic insufficiency, and infertility.
Milder forms of cystic fibrosis, however, can remain unrecognized until adulthood (Fig. 9).

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A B

C
Fig. 7Congenital stenosis of left mainstem bronchus.
A and B, Transverse images show stenosis (arrow, A), distal bronchiectasis, and mucoid impaction (arrows, B).
C, Coronal reformation image shows bronchial stenosis (arrow).

Sarcoidosis
Parenchymal involvement by sarcoidosis can lead to upper and mid lung fibrosis and trac-
tion bronchiectasis, typically associated with multiple nodules in a perilymphatic distribution
(Fig. 10). Mediastinal and bilateral symmetric lymphadenopathy is common, although it can
regress as the interstitial disease worsens.

Postradiation Fibrosis
Another important cause of upper-lung-predominant bronchiectasis is postradiation fibro-
sis, in which traction bronchiectasis is usually limited to the radiation port (Fig. 11). A straight
interface between the irradiated field and normal lung is often seen. This does not respect
anatomic borders, such as fissures and lobes. Postradiation fibrosis may also be bilateral after
mediastinal radiation for lymphoma.

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A
Fig. 8Cystic fibrosis.
A and B, Transverse (A) and coronal (B) images show
upper lobe predominance of cystic bronchiectasis
(arrows) and volume loss, enlarged lung volumes, and
diffuse heterogeneous attenuation.

Fig. 9Adult cystic fibrosis. In this milder case, there is upper lobe predominance of cylindric bronchiectasis
(white arrows) and bronchiolitis (black arrows).

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B
Fig. 10Sarcoidosis.
A and B, Transverse images show fibrosis and traction bronchiectasis (arrows, B) that predominantly involve
upper lobes.

Diffuse Bronchiectasis: Lower Predominance


Lower-lung-predominant bronchiectasis, which is the most frequent pattern, is most com-
monly idiopathic (Fig. 12). However, some cases have known causes.
Recurrent Childhood Infections
Postinfectious bronchiectasis is a frequent cause of lower-lung-predominant bronchiecta-
sis. It is less seen today because of better control of tuberculosis, earlier treatment of pneumo-
nia, and immunization. However, recurrent infections still remain a frequent cause of bron-
chiectasis in immune-suppressed patients.
Aspiration
Any predisposition for repeated aspiration is associated with an increased risk of develop-
ing bibasilar bronchiectasis. Common causes include a large hiatal hernia with gastroesopha-
geal reflux, scleroderma (Fig. 13) and other causes of a patulous esophagus, and esophageal
motility disorders.

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A B
Fig. 11Postradiation fibrosis.
A and B, Right paramediastinal fibrotic changes, which developed after treatment of lung cancer, are
associated with traction bronchiectasis (arrows).

Fig. 12Lower lobe predominance of bronchiectasis.


A, Subtle idiopathic bibasilar cylindric bronchiectasis
shows signet-ring sign (arrows).
B, In another patient, there is marked idiopathic left
lower bronchiectasis with volume loss, bronchial wall
thickening, and diffuse opacity.
B

Fibrotic Lung Disease


Bronchiectasis predominantly involving lung bases is a common finding in fibrotic lung
disease. In usual interstitial pneumonia (UIP), coarse reticulation, honeycombing, parenchy-
mal distortion, and traction bronchiectasis are typically predominant in a subpleural and

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Fig. 13Scleroderma. There is patulous esophagus (white arrow), recurrent aspiration with subsequent
bibasilar bronchiectasis, and chronic ground-glass opacities. Black arrow points to bronchus visible in
peripheral 1 cm of lung.

Fig. 14Usual interstitial pneumonia. Bibasilar and subpleural reticulation and traction bronchiectasis are
seen in areas of fibrosis (arrows).

bibasilar distribution with geographic heterogeneity (Fig. 14). Although often idiopathic, UIP
occurs in asbestosis, drug toxicity, and collagenvascular disease. Nonspecific interstitial
pneumonia has almost the same differential diagnosis but typically occurs in younger patients
and carries a better prognosis. Ground-glass opacity is more frequent than in UIP and honey-
combing usually remains minimal.

Rare Causes
Bronchiolitis obliterans from posttransplantation rejection is a rare cause of bronchiectasis
associated with patchy mosaic perfusion, air trapping, and bronchiolar obstruction (Fig. 15).
Hypogammaglobulinemia can also be a cause of lower-lung-predominant bronchiectasis,
most often caused by recurrent infections.

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A B
Fig. 15Bronchiolitis obliterans after lung transplantation.
A and B, Transverse images of right lung in deep inspiration (A) and end expiration (B) show subtle basilar
cylindric bronchiectasis (arrows, A) and widespread air trapping (arrows, B).

Fig. 16Mycobacterium avium-intracellulare infection. Bronchiectasis (arrows) predominantly involves right


middle lobe and lingula.

Diffuse Bronchiectasis: Middle Lobe and Lingula Predominance


Nontuberculous Mycobacterial Infection
The most common acquired cause of bronchiectasis predominantly involving the right
middle lobe and lingula is nontuberculous mycobacterial infection, such as Mycobacterium
avium-intracellulare (MAI). This infection, typically seen in women over 60 years old, pres-
ents with chronic infection, bronchiectasis, mucoid impaction, and bronchiolitis (Fig. 16).
The disease often has an indolent but progressive course requiring long-term antibiotics.

Immobile Cilia Syndrome


This rare congenital cause of bronchiectasis, which primarily involves the middle lung, is
characterized by ineffective clearing of secretions, causing bronchiectasis, recurrent pneumo-
nias, sinusitis, and infertility. In 50% of cases, total situs inversus is present, a condition
known as Kartageners syndrome (Fig. 17).

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A B
Fig. 17Kartageners syndrome.
A, Chest radiograph shows cardiomegaly, dextrocardia, left middle lobe bronchiectasis, and volume loss.
Arrow points to wrong-sided left marker.
B, Transverse CT image confirms dextrocardia (asterisk is in left ventricle) and bronchiectasis (arrows) that
predominantly affects midportion of lungs.

Fig. 18Allergic bronchopulmonary aspergillosis.


A, Chest radiograph shows central bronchiectasis
and mucoid impaction, so-called finger-in-glove
appearance (arrows).
B, Transverse CT image shows central
bronchiectasis, mucoid impaction (large arrow), and
distal bronchiolitis (small arrow).
A

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B
Fig. 19Mounier-Kuhns syndrome.
A, Enlarged trachea (arrow).
B, Enlarged mainstem bronchi (black arrows) and distal bronchiectasis (white arrows).

Fig. 20Williams-Campbell syndrome. There is mostly varicose and cystic central bronchiectasis (arrows).

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Diffuse Bronchiectasis: Central Predominance


Allergic Bronchopulmonary Aspergillosis
Allergic bronchopulmonary aspergillosis is an immune reaction to Aspergillus species that
damages the bronchial wall, causing central bronchiectasis and mucous plugs that contain
fungus and inflammatory cells. On chest radiography, this produces a characteristic finger-in-
glove appearance. On CT, central bronchiectasis and mucoid impactions are often associated
with areas of peripheral bronchiolitis, manifesting as bronchiolar nodules or tree-in-bud
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opacities (Fig. 18).

Cartilage-Deficiency Disorders
Several rare disorders characterized by cartilage deficiency can cause bronchiectasis that
primarily involves the central regions of the lung. One is tracheobronchomegaly, also known as
Mounier-Kuhns syndrome, which manifests as diffuse enlargement of the trachea and main-
stem bronchi with more distal bronchiectasis (Fig. 19).
An even rarer cartilage deficiency disorder is the Williams-Campbell syndrome, in which
central bronchiectasis, mimicking the appearance of parenchymal cysts, is associated with
widespread air trapping (Fig. 20).

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3. Hansell DM, Bankier AA, MacMahon TC, McLoud TC, Mller NL, Remy J. Fleischner Society: glossary
of terms for thoracic imaging. Radiology 2008; 246:697722
4. Javidan-Nejad C, Bhalla S. Bronchiectasis. Radiol Clin North Am 2009; 47:289306
5. Kim JS, Mller NL, Park CS, Grenier P, Herold CJ. Cylindrical bronchiectasis: diagnostic findings on thin-
section CT. AJR 1997; 168:751754
6. ODonnell AE. Bronchiectasis. Chest 2008; 134:815823
7. Quast TM, Self AR, Browning radiofrequency: diagnostic evaluation of bronchiectasis. Dis Mon 2008;
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8. Reid LM. Reduction in bronchial subdivision in bronchiectasis. Thorax 1950; 5:233247

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