R e s i d e n t s ’ S e c t i o n • R ev i ew

Murphy et al.
Imaging of Cystic Fibrosis and Pediatric Bronchiectasis

Residents’ Section
Review
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Residents

Imaging of Cystic Fibrosis and

inRadiology
Pediatric Bronchiectasis
Kevin P. Murphy 1,2 Key Points
Michael M. Maher 1,2 1. CT is superior to pulmonary function tests and chest radiography for the assessment and
Owen J. O’Connor 1,2 monitoring of cystic fibrosis (CF)–related lung disease and, also, of pediatric bronchiectasis
not caused by CF (hereafter referred to as non-CF bronchiectasis).
Murphy KP, Maher MM, O’Connor OJ 2. Low-dose CT protocols that impart radiation doses similar to those used in chest radiog-
raphy are feasible for the surveillance of patients with bronchiectasis.
3. Chest radiography is still most commonly used as the first-line imaging examination of
choice for the assessment of acute complications related to bronchiectasis.
4. Pulmonary MRI, with or without the use of inhaled hyperpolarized gas, can be per-
formed to obtain functional information, and, in dedicated centers, it may yield imaging re-
sults comparable to those obtained by CT.
5. Gastrointestinal and pancreaticobiliary manifestations of CF are observed with greater
frequency in adults, because of increased life expectancy.

horacic manifestations of cystic considerably. It is estimated that individuals

T fibrosis (CF) and pediatric bron-

Keywords: bronchiectasis, cystic fibrosis,
high-­resolution CT, low-dose CT, pulmonary MRI
DOI:10.2214/AJR.15.14437
Received January 25, 2015; accepted after revision
May 20, 2015.
1
Department of Radiology, Cork University Hospital,
Wilton, Cork, Ireland. Address correspondence to
O. J. O’Connor (oj.oconnor@ucc.ie).
2
Department of Radiology, University College Cork,
Cork, Ireland.
AJR 2016; 206:448–454
0361–803X/16/2063–448
© American Roentgen Ray Society
chiectasis not caused by CF
(hereafter known as “non-CF
bronchiectasis”) are commonly encountered
by radiologists. In addition, abdominal mani-
festations (e.g., hepatic steatosis, cirrhosis,
intussusception, and meconium ileus) are
well-recognized as being associated with CF,
and with improved survival of patients with
CF, manifestations previously thought to be
very unusual (e.g., development of carcinoma
or lymphoma) are being encountered with
somewhat increased frequency. Non-CF
bronchiectasis is increasingly diagnosed in
patients at a younger age, and these individu-
als consequently undergo imaging more of-
ten over a longer period. We provide a brief
review of the imaging approaches and find-
ings for these patient cohorts.
Disease Epidemiologic Profile
CF is the most common autosomal reces-
sive disease among white individuals, with
a frequency of approximately 1 in 2400 live
births [1]. Respiratory disease remains the
primary cause of morbidity and mortality, be-
ing responsible for more than 80% of deaths.
As a result of improved therapies, the life ex-
pectancy of patients with CF has improved
born since the year 2000 will have a medi-
an life expectancy in excess of 50 years [1].
Non-CF bronchiectasis in childhood occurs
less commonly in the developed world, with a
frequency of approximately 87 cases per mil-
lion population and accounting for 9.6% of
new referrals to a tertiary pediatric respira-
tory clinic in one reported series [2, 3].
Pathophysiologic Basis of
Cystic Fibrosis
CF arises from an inherited defect in the
CF transmembrane conductance regulator
(CFTR) protein, which is located on chromo-
some 7. When defective, this protein leads to
reduced transport of chloride by the trans-
membrane. The δ-F508 deletion is the cause
of approximately 70% of cases of CF [1, 4,
5]. Affected patients have thickened viscous
and, sometimes, inspissated secretions at the
mucosal surfaces, leading to lung destruc-
tion, gastrointestinal malfunction, and exo-
crine insufficiency. CF typically presents in
the neonatal period with respiratory compro-
mise caused by thickened secretions or with
bowel obstruction secondary to meconium
ileus. Patients with less severe manifesta-
tions may present with recurrent respiratory
infections in childhood and failure to thrive.

448 AJR:206, March 2016

 Imaging of Cystic Fibrosis and Pediatric Bronchiectasis
Non-CF bronchiectasis develops through
a cycle of events known as the Cole vicious
cycle [6]. It is believed that a respiratory in-
jury, which most commonly is infection and
which sometimes occurs in a susceptible
host, leads to non-CF bronchiectasis through
impaired mucociliary clearance, chronic in-
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fection, exaggerated inflammatory response,
and airways destruction [6]. The principal
causes of non-CF bronchiectasis include pre-
vious pneumonia, immunodeficiency, aspira-
tion, and ciliary dyskinesia, with upward of
30% of cases remaining idiopathic [7]. Pa-
tients typically are seen with recurrent low-
er respiratory chest infections or asthmalike
wheezing and dyspnea.
Imaging Strategies
CT
The use of CT for the evaluation of CF and
non-CF bronchiectasis has many advantages.
For patients with normal results of pulmo-
nary function tests, CT frequently can depict
many features of CF, the severity of which
can also be scored on CT. Disease severity
scores determined by CT have been shown
to be more predictive of future lung disease
severity than have those determined by spi-
rometry or radiography [8, 9]. This finding
has important implications for patient care.
Similarly, compared with CT, radiography
is less sensitive for the diagnosis of allergic
bronchopulmonary aspergillosis (ABPA),
because many of the observations that can be
made on radiography are nonspecific and are
commonly present in patients without this
complication of CF [10] (Figs. 1A and 1B).
CT, on the other hand, is more specific for
ABPA, particularly unenhanced CT, when
hyperdense material is observed in patients
with dilated airways.
The European Cystic Fibrosis Society rec-
ommends that patients with stable CF under-
go an annual chest radiographic examination
[11]. The British Thoracic Society does not rec-
ommend routine imaging surveillance for as-
ymptomatic patients with non-CF bronchiec-
tasis, except when humoral immunodeficiency
is present [12]. For patients with deteriorating
pulmonary function, CT is widely considered
to be the best imaging modality and is recom-
mended by the aforementioned guidelines for
the assessment of bronchiectasis and the depic-
tion of associated complications [11, 12].
The most common CT finding in patients
with CF is air trapping, followed by bronchi-
ectasis, mucous plugging, consolidation, and,
uncommonly, cysts or bullae [8] (Fig. 2). In
AJR:206, March 2016 449
non-CF bronchiectasis, the predominant find-
ing is cylindric bronchiectasis [13], whereas
cylindric bronchiectasis and cystic bronchi-
ectasis are seen in association with CF [1]. In
CF, bilateral bronchiectasis with disease pre-
dominant in the upper lobe is routinely noted
(reportedly, bronchiectasis of the right lung is
slightly worse than that of the left lung, albeit
for unknown reasons); however, other chang-
es do not result in the observation of a simi-
lar gradient [8, 14, 15]. Non-CF bronchiecta-
sis has a variable and often localized pattern
of lung involvement, depending on its cause
(Fig. 3). ABPA is classically central. Bronchi-
ectasis related to immunodeficiency is typi-
cally more global, whereas bronchiectasis re-
lated to a foreign body is localized. Features
of mild bronchiectasis, such as the absence
of bronchial tapering, a bronchoarterial ratio
greater than 1, or visibility of bronchi within
1 cm of the pleural surface, may be the only
findings [16].
A number of CF CT scoring systems exist,
but the Bhalla and Brody systems are com-
monly used [8, 17]. Most scoring systems use
the presence, severity, and extent of bron-
chiectasis, bronchial wall thickening, mu-
cus plugging, consolidation or atelectasis, or
hyperinflation to calculate a severity score.
The presence of abscess, air trapping (Fig. 4),
pleural effusion, and bullae may also be in-
cluded [18].
Potential complications of CF and non-CF
bronchiectasis include bacterial, fungal, or
mycobacterial infection and mycetoma (Fig.
5), ABPA, pleural effusion, pneumothorax,
hemoptysis, and pulmonary hypertension.
Appearances indicative of infection on CT
include air-space disease (Fig. 3), tree-in-bud
opacification (Fig. 6), ground-glass opacifi-
cation (Fig. 7A), patchy nodular opacification
(Figs. 7A and 7B), and cavitation or abscess
formation.
It is worth remembering, however, that
bronchiectasis is a risk factor for atypical
pulmonary infection, just as it is for the de-
velopment of hemoptysis. Angiogenesis and
bronchial artery hypertrophy commonly
occur in association with CF, as a result of
long-term inflammation. Hemoptysis gener-
ally is caused by exacerbations of infectious
disease caused by CF. Although bronchos-
copy and CT angiography are often used to
assess and localize the source of bleeding
in patients without CF who have hemopty-
sis, this approach is debated for patients with
CF [19] (Fig. 8). Percutaneous endovascular
embolization of abnormally large (≥ 3 mm)
or tortuous vessels thought to be responsible
for bleeding or an active bleeding source are
used in the management of major hemopty-
sis (expectoration of 30–300 mL of blood) or
massive hemoptysis (expectoration of > 300
mL of blood) [20] (Fig. 9). Physicians debate
whether embolization is required for non-
massive hemoptysis, because most cases are
self-limiting. In addition, embolization of an
actively bleeding vessel or pseudoaneurysm
only versus embolization of abnormal ves-
sels is also debated [20, 21].
Increased use of CT for assessment and
monitoring of CF, combined with an in-
creased life expectancy of patients with CF,
has resulted in increased cumulative radia-
tion exposures [22]. Over the past 15 years,
there has been almost a sixfold increase in
the use of CT for patients with CF [22]. Pa-
tient age at the time of the first CT exami-
nation has also decreased, from a mean age
of 20 years for individuals born before 1980,
to a mean of 1.9 years for those born after
1997 [23]. Dose reduction strategies that are
not specific to the imaging of bronchiectasis,
such as z-axis automated tube current modu-
lation and iterative reconstruction techniques,
are being used, in addition to low-dose proto-
cols that have been optimized for use in the
follow-up of bronchiectasis. Thoracic CT is
particularly suited for use in dose-optimized
protocols, because of the high inherent con-
trast between lung parenchyma and vessels
or bronchi and because of the lower attenu-
ation of the CT beam, compared with CT of
the abdomen. Thin-section nonhelical CT
consisting of six or seven images is a feasible
and effective method for performing a diag-
nostic CT examination at an effective radia-
tion dose of 0.14 mSv, which approximates
the dose used in posteroanterior and lateral
chest radiographs [24]. Newer iterative re-
constructive techniques will likely allow he-
lical CT of the entire chest to be performed
with radiation doses similar to those used in
chest radiography.
Chest Radiography
Radiography is inferior to CT for the as-
sessment of patients with known bronchiec-
tasis or those with suspected disease. Nev-
ertheless, radiography remains a useful
modality for assessing the pulmonary com-
plications associated with bronchiectasis,
because of its low cost, availability, low ra-
diation dose, and speed of acquisition [20].
Several radiographic scoring systems for CF
exist, with the Chrispin-Norman and Bras-

field systems among the most commonly
used [18, 25]. The parameters assessed are
similar to those assessed by CT scoring sys-
tems. Bronchiectasis and, in particular, cy-
lindric bronchiectasis classically appear as
tram-track opacification when observed lon-
gitudinally or as a signet ring when viewed
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en face on radiography (Fig. 10).
MRI and Other Alternatives
Although CT is considered the reference
standard for assessing bronchiectasis, pul-
monary MRI is increasingly being used [26,
27]. MRI is less sensitive for the depiction of
small airways disease but can be used to as-
sess functional changes, such as alterations
in pulmonary ventilation, which have led
many researchers to advocate the use of MRI
for the follow-up of morphologic changes in
CF and non-CF bronchiectasis. Routine T1-
and T2-weighted turbo spin-echo sequences
can be combined with inhaled agents (in par-
ticular, hyperpolarized helium) for imaging
purposes [28] (Figs. 11A and 11B). The use
of inhaled agents, such as hyperpolarized he-
lium or xenon, helps yield functional infor-
mation about gas exchange. The most notable
disadvantages of MRI are the increased time
and costs associated with image acquisition,
in addition to its limited usefulness in pa-
tients receiving ventilation support. The use
of higher-field MRI for patients with non-
CF bronchiectasis has been evaluated, with
MRI findings having good correlation with
CT scores [29].
FDG PET and FDG PET/CT have been
used in research to quantify inflammatory
changes related to exacerbations of CF and
responses to antibiotic therapy [30]. Stan-
dardized uptake values were positively cor-
related with CT scores for CF and were neg-
atively correlated with forced expiratory
volume in 1 second [30]. This approach can
delineate areas of active infection and can
also help differentiate CF from fibrosis. Lim-
ited access, high cost, and radiation dose are
clear constraints that prohibit widespread use
of FDG PET and FDG PET/CT.
Abdominal Imaging in Patients With
Cystic Fibrosis
Abdominal complications related to gas-
trointestinal or pancreaticobiliary disease re-
main common in patients with CF [31]. Neo-
natal meconium ileus occurs in 10–15% of
patients with CF and can be diagnosed on the
basis of clinical, radiologic, or sonographic
findings. Meconium ileus is characterized by
450 AJR:206, March 2016
Murphy et al.
intestinal obstruction resulting from inspis-
sated contents and occurs most commonly in
the terminal ileum [31]. The small bowel ap-
pears echogenic on prenatal ultrasound im-
ages, and meconium mixed with gas creates
a bubbly appearance on radiograph, possi-
bly in association with proximal small bow-
el dilatation or with postperforation perito-
neal calcification. Microcolon may be seen
on images generated during a contrast-en-
hanced enema examination. Distal intestinal
obstruction syndrome (previously known as
“meconium ileus equivalent”) occurs outside
the neonatal period and has characteristics
similar to those of meconium ileus [32]. Pa-
tients with CF are also susceptible to intus-
susception, appendiceal thickening, hepatic
steatosis, cirrhosis, microgallbladder, chole-
lithiasis, biliary dilatation, fatty replacement
(steatosis) and atrophy of the pancreas (Fig.
12), pancreatitis, and nephrolithiasis [31–33].
Of interest, the frequency of acute appendi-
citis is lower in patients with CF than in the
general population (1–2% vs 7%) [32]. With
an increased life expectancy for patients with
CF, abdominal manifestations and compli-
cations of CF are being seen more often in
adults [32, 33]. There is an increased inci-
dence of colonic, small intestinal, pancreat-
ic, and hepatocellular cancer in patients with
CF, compared with the age-matched popula-
tion [34, 35].
Conclusion
CT is the imaging method of choice for
the diagnosis, assessment, and surveillance
of bronchiectasis and its associated compli-
cations and findings in patients with CF and
other pediatric conditions. Modified low-
dose CT protocols should be considered, giv-
en the potential for high cumulative exposure
to radiation in this cohort. Chest radiography
is commonly used, particularly for monitor-
ing acute complications of CF, but it provides
less information than does CT. The use of
MRI with inhaled hyperpolarized gas yields
additional functional information, compared
with CT; however, this type of imaging ex-
amination is not widely available. Abdomi-
nal manifestations of CF are increasingly be-
ing encountered as a result of increased life
expectancy for patients with CF.
Acknowledgment
We thank Casey Sams (Division of Pediat-
ric Imaging, Department of Radiology, Uni-
versity of North Carolina) for providing MR
images.
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Fig. 1—53-year-old woman with asymmetric

bronchiectasis secondary to cystic fibrosis (CF) who
underwent contrast-enhanced CT for monitoring
of chronic progressive deterioration of pulmonary
function.
A, Posterior thoracic coronal CT image displayed on
lung windows shows advanced CF bronchiectasis
with volume loss in left lower lobe. There is less
severe cylindric bronchiectasis (arrows) in right
lower lobe.
B, Chest radiograph shows asymmetric cystic and
cylindric bronchiectasis (arrowheads), left lung
volume loss, and small effusion (straight arrow).
Chest port (curved arrow) is also shown.

A B

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 Murphy et al.
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Fig. 2—24-year-old man with cystic fibrosis who was undergoing annual
surveillance contrast-enhanced CT. Midthoracic axial CT image on lung window
setting shows typical findings of advanced bronchiectasis: advanced cylindric
bronchiectasis (arrows), which is most marked in right upper lobe, with associated
volume loss and bronchial wall thickening (arrowheads). Chest port is shown in
right anterior chest wall.
Fig. 5—27-year-old woman with advanced bronchiectasis secondary to
cystic fibrosis. Contrast-enhanced CT was performed to investigate subacute
exacerbation of respiratory symptoms. Axial CT image displayed on lung window
shows mycetoma (arrows) just below carinal level in superior segment of right
lower lobe.
452 AJR:206, March 2016
Fig. 3—2-year-old boy with bronchiectasis secondary to
hypogammaglobulinemia-related immunodeficiency who presented with
complicated respiratory infection. Lower thoracic axial contrast-enhanced CT
image displayed on lung windows shows varicoid bronchiectasis (curved arrow)
involving anterior segment of right upper lobe with associated air-space disease
(straight arrows).
Fig. 4—22-year-old man with cystic fibrosis complicated by acute deterioration
in symptom severity. Axial unenhanced CT image acquired at level of upper lobes
and displayed on lung windows shows mosaic attenuation (arrows) resulting from
small airways disease with central cylindric bronchiectasis (arrowheads) and
adjacent patchy air-space disease. Left-sided lung parenchymal volume loss is
also shown.
Fig. 6—16-year-old boy with cystic fibrosis with clinical symptoms of lower
respiratory chest infection. Lung basal axial unenhanced CT image displayed on
lung windows shows tree-in-bud opacities (straight arrows) and ground-glass
opacification (curved arrows) in peripheral right lower lobe. Mild lower lobe
bronchiectasis (arrowheads) is also evident.
 Imaging of Cystic Fibrosis and Pediatric Bronchiectasis
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A B
Fig. 7—26-year-old woman with cystic fibrosis who underwent imaging because of clinical deterioration suggestive of infection.
A, Axial contrast-enhanced CT image of midthorax displayed on lung windows shows multiple peripheral peribronchovascular nodular
opacities (arrows) in addition to mosaic attenuation (arrowheads). These opacities were confirmed to be secondary to atypical mycobacterial
infection after bronchoscopy.
B, Chest radiograph shows same nodular opacities (arrows) seen on CT, although opacities are less clear on radiographic image. Chest port
(arrowhead) is also shown.

Fig. 8—42-year-old man with bronchiectasis caused by cystic fibrosis who was
admitted to hospital with significant hemoptysis. Axial contrast-enhanced CT
image of upper lobes displayed on soft-tissue windows shows markedly enlarged
bronchial arteries (arrowheads) in addition to air-space disease and ground-
glass opacification (arrows) from hemorrhage in right upper lobe. Successful
embolization of branches of right bronchial artery was performed.

Fig. 9—18-year-old woman with bronchiectasis not caused by cystic fibrosis who was admitted to hospital
with moderate volume hemoptysis. Digital subtraction angiogram of thoracic aorta and branches shows mildly
enlarged intercostobronchial artery (arrow) in right upper lobe and abnormal blush caused by extravasation of
contrast medium (arrowheads). This artery was selectively embolized with resultant symptom cessation.

AJR:206, March 2016 453

 Murphy et al.

Fig. 10—27-year-old woman with cystic fibrosis who underwent chest radiography for assessment of subacute
deterioration of pulmonary status. Radiographic image shows tram-track opacities (arrowheads) due to
cylindric bronchiectasis predominantly in upper lobe. Chest port (arrow) is also shown.
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Fig. 11—20-year-old man with cystic fibrosis

with subacute deterioration of pulmonary status.
(Courtesy of Sams C, University of North Carolina,
Chapel Hill, NC)
A, Chest radiograph shows lung hyperexpansion
and mild cylindric bronchiectasis (arrows) but no
evidence of pneumonia.
B, Contemporaneous coronal spin-echo MR
image confirms left midzone scarring and
atelectasis (curved arrow), bronchial wall
thickening (arrowheads), and upper zone cylindric
bronchiectasis with some plugging (straight arrows).

A B

Fig. 12—21-year-old woman with cystic fibrosis who underwent contrast-

enhanced CT of thorax for evaluation of pulmonary disease. Lowermost axial
upper abdominal CT image displayed on soft-tissue windows shows steatosis
of pancreas (straight arrows) and liver nodularity secondary to cirrhosis (curved
arrows), in addition to splenomegaly and venous collaterals (arrowheads) resulting
from portal hypertension.

454 AJR:206, March 2016