Clinical Imaging 73 (2021) 61–72

Contents lists available at ScienceDirect

Clinical Imaging
journal homepage: www.elsevier.com/locate/clinimag

Cardiothoracic Imaging

Pulmonary sequestration: What the radiologist should know

Michela Gabelloni, Lorenzo Faggioni *, Sandra Accogli, Giacomo Aringhieri, Emanuele Neri
Diagnostic and Interventional Radiology, Department of Translational Research, University of Pisa, Via Roma, 67, 56126 Pisa, Italy

A R T I C L E I N F O A B S T R A C T

Keywords: Pulmonary sequestration consists of a nonfunctioning mass of lung tissue, either sharing the pleural envelope of
Intralobar pulmonary sequestration the normal lung (intralobar) or with its own pleura (extralobar), lacking normal communication with the
Extralobar pulmonary sequestration tracheobronchial tree and receiving its arterial supply by one or more systemic vessels. It is the second most
Diagnostic imaging
common congenital lung anomaly according to pediatric case series, but its real prevalence is likely to be
Therapeutic embolization
underestimated, and imaging plays a key role in the diagnosis and treatment management of the condition and
its potential complications. We will give a brief overview of the pathophysiology, clinical presentation and
imaging findings of intra- and extralobar pulmonary sequestration, with particular reference to multidetector
computed tomography as part of a powerful and streamlined diagnostic approach.

1. Introduction
Pulmonary sequestration (PS) is a malformation of the lower respi­
ratory tract, consisting of a nonfunctioning mass of lung tissue that lacks
normal communication with the tracheobronchial tree and receives its
blood supply from one or more aberrant systemic arteries [1]. Despite
being a relatively rare condition (accounting for 0.15% to 6.4% of all
congenital pulmonary malformations [2]), it is the second most common
congenital lung malformation. Its estimated incidence is 0.15–1.8%,
according to reviews of pediatric surgical thoracic practice, pediatric
postmortem and prenatal ultrasound studies. Such numbers are likely to
be underestimated because some adults have been reported to develop
complications as a result of PS, and small lesions may remain asymp­
tomatic and go undetected [3,4].
Although the condition was first described by Huber in as early as
1777, the term “pulmonary sequestration” was introduced by Pryce in
1946 [3–5] and refers to a congenital anomaly of the primitive foregut
that is supposed to result from the formation of an accessory supernu­
merary lung bud below the normal lung bud [6,7]. Based upon the
pleural investment of the sequestered lung parenchyma, PS can be
classified as either intralobar or extralobar. Intralobar sequestration
(ILS, accounting for 75% of cases of PS) is characterized by sharing the
same visceral pleural lining of the native lung and has a venous drainage
into the pulmonary veins. Conversely, extralobar sequestration (ELS,
making up for the remaining 25% of cases) has its own visceral pleural
investment outside the normal lung and drains into a systemic vein,
forming an accessory lobe (called a “Rokitansky lobe”). However, a clear
differentiation between ILS and ELS is often difficult due to the presence
of a mixed type of venous drainage to both pulmonary and systemic
circulations. In an effort to avoid confusion, the “sequestration spec­
trum” concept has been developed referring to any case of abnormal
connection between one or more of the main components of lung tissue,
including airways, lung parenchyma, arterial supply and venous
drainage [2,3,6,8].
Our purpose is to summarize the pathophysiology, clinical presen­
tation and imaging findings of ILS and ELS, with particular reference to
multidetector computed tomography (CT) and magnetic resonance im­
aging (MRI), based on a systematic review of the existing literature.
Special attention will be paid to the correlation between imaging pat­
terns that should be recognized in the imaging workup of PS and their
pathological underpinnings, and to the relative strengths and weak­
nesses of one imaging modality over another. A brief overview of the
current treatment options of PS will also be provided, highlighting the
role of interventional radiology in the overall therapeutic strategy.

Abbreviations: CBPFM, congenital bronchopulmonary foregut malformation; CPAM, congenital pulmonary airway malformation; CT, computed tomography; ELS,
extralobar pulmonary sequestration; ILS, intralobar pulmonary sequestration; MIP, maximum intensity projection; MRI, magnetic resonance imaging; PS, pulmonary
sequestration; VR, volume rendering.
* Corresponding author.
E-mail address: lfaggioni@sirm.org (L. Faggioni).

https://doi.org/10.1016/j.clinimag.2020.11.040
Received 17 June 2020; Received in revised form 13 November 2020; Accepted 24 November 2020
Available online 3 December 2020
0899-7071/© 2020 Elsevier Inc. All rights reserved.
M. Gabelloni et al.
2. Intralobar and extralobar sequestration: pathophysiology
and clinical features
2.1. Intralobar sequestration
The most established pathogenetic theory of PS sees ELS and ILS as
different pathways sharing a common congenital origin, with ELS
occurring if the accessory supernumerary lung bud continues to migrate
caudally with the esophagus, whereas ILS takes place if the lung bud
arises before the development of pleura [6,7,9]. While such theory is still
generally accepted for ELS, the etiology of ILS is controversial. The
congenital etiology of ILS is supported by its association in a minority of
cases (12%) with other congenital anomalies (including esophago­
bronchial diverticula, diaphragmatic hernias, skeletal deformities, car­
diovascular defects, and renal anomalies) [2,10,11], by its arising at an
early stage of fetal development in some cases [12] and by the fact that
the simultaneous occurrence of both ILS and ELS, occasional bilaterality,
and the occurrence of ILS in twins have been described [13–15].
On the other hand, there is growing evidence in the literature that ILS
has usually an acquired origin caused by chronic pulmonary infection,
leading to the proliferation of aberrant arterial vessels [16–20]. Such
hypothesis is supported by the fact that ILS lacks its own pleural enve­
lope due to its forming within normal lung parenchyma [10] and by its
higher prevalence late in childhood or in adolescents with recurrent
pneumonia in a localized segment of the lung, with 60% of cases pre­
senting before the age of 20 years. Although ILS is rarely found after 50
years old, it is the predominant variant of PS in adults [7,21]. This makes
imaging a pivotal tool for diagnosis and marks a key clinical difference
from ELS, which instead presents more commonly in newborns with
respiratory distress, cyanosis and infection [3,11,16,22,23]. While ILS is
rarely a problem in pediatric patients younger than 2 years old, it can
rarely be discovered at prenatal ultrasound and spontaneous regression
during pregnancy has been reported [3,24–26].
ILS has an almost equal gender distribution. Nearly half of adults
with ILS are asymptomatic, yet only 15% of patients with ILS are
asymptomatic at the time of diagnosis, and more than 50% of those who
develop symptoms are over 20 years old. The clinical presentation most
frequently includes recurrent infection with persistent cough or exer­
tional shortness of breath, sputum production, back pain and hemop­
tysis, which can be massive [27]. Although ILS is, in itself, characterized
by a lack of communication with the tracheobronchial tree, communi­
cation of the lesion through the pores of Kohn may spread the infection
to the neighboring normal lung parenchyma and complicate with ab­
scess formation, erosion and tracheobronchial fistulization [7,10].
Pseudomonas aeruginosa is the most commonly identified agent in ILS-
related infections, with other agents including tuberculosis, Nocardia,
or Aspergillus [7,28,29].
Lower lobe infection or chronic inflammation (typically associated
with bronchiectasis) can result in cystic lung disease with proliferation
of systemic arteries entering the lung through the pulmonary ligament
or across the pleura [10,11]. This reflects the macroscopic appearance of
ILS, consisting of multiple cysts of variable size with thickening of the
overlying pleura, which is tightly adherent to the mediastinum and
diaphragm. Microscopically, the lung parenchyma is replaced by
chronic inflammation and fibrosis, with remnants of bronchi and
bronchioles surrounded by dense fibrous connective tissue with lym­
phocytic infiltration [3].
In most cases, the arterial supply to ILS stems from small systemic
pulmonary ligament arteries that arise from the thoracic aorta, feed the
esophageal arterial plexus, and cross the pulmonary ligament branching
in the visceral pleura of the lower lobes of the lung. Stocker and Malczak
have proposed that these arteries may become parasitized to supply the
infected portion of a lower lobe if the normal pulmonary arterial supply
is compromised [11,30]. However, unlike congenital ILS, these acquired
lesions have an intact bronchial connection, and the arteries in neo­
vascularity are thin and irregular, in contrast to the large and
62
Clinical Imaging 73 (2021) 61–72
structurally regular arteries of ILS. Such acquired conditions that can
mimic PS have been termed pseudo-sequestration [31].
ILS affects the lower pulmonary lobes in as many as 98% of cases and
is more typically located in the medial or posterior basal segments of the
left lower lobe. In general, the left side of the chest is affected in about
60% of cases [3,10,22]. An exception to the left-sided predominance of
ILS is the association with partial anomalous pulmonary venous return
(scimitar syndrome), in which right-sided ILS is commoner [7,32].
2.2. Extralobar sequestration
Unlike ILS, ELS forms separately from the rest of the lung and hence
has its own pleural envelope [10]. Although several etiological mecha­
nisms have been hypothesized, the most widely accepted theory sug­
gests that ELS could result from the development of an accessory lung
bud caudal to the normal lung buds. Since the primitive bronchial tree
begins as a ventral diverticulum of the foregut at 3 weeks, and definitive
lung lobes develop between weeks 5 and 8 of gestation, ELS is supposed
to arise between weeks 4 and 8 of gestation. The high incidence of
congenital diaphragmatic hernia in patients with ELS points to a defect
occurring prior to week 6 of gestation [3,33].
As opposed to ILS, ELS has a 3:1 to 4:1 male predominance in most
reported prenatal series, with 65% of cases occurring on the left side [1].
More than 60% of patients with ELS present in the first six months of life
with respiratory distress, feeding difficulty and malabsorption due to
shunting of blood from the gastrointestinal tract, high output congestive
heart failure due to right-to-left shunt, and occasional spontaneous
pulmonary or pleural hemorrhage due to high pressure from the sys­
temic arterial supply. ELS infection is rare because ELS is separated from
the tracheobronchial tree by its pleural investment. However, ELS can
also be diagnosed in childhood or adulthood, and approximately 10% of
ELS are found incidentally in asymptomatic individuals [1,7].
ELS is often located between the diaphragm and the lower lobes and
rarely infra- diaphragmatically, with 63–77% of cases occurring in the
posterior costodiaphragmatic sulcus between the lower lobe and left
hemidiaphragm, and only 10–15% below the diaphragm [2]. In spite of
its overall male prevalence, intra-abdominal ELS is most often found in
females (75%), usually as a left suprarenal mass [34,35]. Very rarely,
intrapericardial ELS has also been reported [36–38].
In adulthood, accompanying symptoms of ELS are less common than
in infancy [7,23,39], although severe clinical presentations such as
recurring massive pleural effusion [40], massive hemothorax [41,42],
and hemorrhagic infarction associated with torsion of the vascular
pedicle [43–46] have been described.
About 50% to 60% of patients with ELS have another congenital
anomaly, including congenital diaphragmatic hernia (most commonly),
airway malformation [type II pulmonary airway malformation (CPAM)
of the lung having been reported in 15% to 25% of patients with ELS],
pulmonary hypoplasia, bronchogenic and pericardial cyst, congenital
heart disease, diaphragmatic eventration, esophageal achalasia, esoph­
agobronchial diverticula, tracheoesophageal fistula, congenital mega­
colon, skeletal deformities and renal conditions [7,10,23,47].
Rare cases of ELS communicating with the gastrointestinal tract
through a patent bronchus-like structure have been reported, known as
congenital bronchopulmonary foregut malformations (CBPFMs)
[3,7,23,47–50]. In a 30-year case overview by Srikanth et al., CBPFMs
were classified into four anatomical groups, of which about 70%
communicated with the lower esophagus. Most CBPFMs present by 8
months of life (of which 43% in the first week of life) as a result of
pneumonia, pulmonary abscess or respiratory distress exacerbated by
feeding [3,49,51,52]. Less common presentations include gastroesoph­
ageal reflux, hematemesis, anemia and cardiac failure. Concurrent ab­
normalities are present in 40% of cases, the most common being rib or
vertebral malformations (20–40%) and esophageal atresia/fistula (16%)
[3,49,53,54].
M. Gabelloni et al.
Fig. 1. Color Doppler prenatal ultrasound shows feeding artery (arrows) to intra-abdominal ELS arising from the abdominal aorta (AO). ST = stomach.
Reproduced from [62] under the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/).
3. Imaging findings
3.1. Ultrasound
Ultrasound is recommended as the first diagnostic modality for
evaluating a suspected supradiaphragmatic chest mass in childhood and
is the main imaging modality for prenatal imaging [3]. It has several
advantages over other imaging techniques in that it is fast, easily
accessible and relatively inexpensive, makes no use of contrast material
and ionizing radiation, can be performed at the patient’s bedside, and
lesions adjacent to the diaphragm or liver are usually well imaged with
ultrasound [15,55]. However, it also has downsides that should be kept
in mind by diagnosticians, including a limited reproducibility due to
variable operator skillfulness, a small field of view, poor image quality
due to the anatomy under investigation (including the bony thorax,
normally aerated lung, or air-filled cysts), and in the case of prenatal
examinations, ultrasound beam attenuation in the mother’s fat tissue
[3,4,56,57].
Overall, PS accounts for as many as 23% of prenatally detected lung
lesions [3,56,58]. Its typical appearance at prenatal ultrasound is that of
a solid, homogeneous, well-circumscribed echogenic mass, although
cystic components may be detected within hybrid lesions. ILS can be
found exclusively in the chest (with a predominance for the left medial
costophrenic sulcus), may have a rounded appearance demarcated by a
small rim of normal neighboring lung tissue and may undergo a widely
variable degree of cystic degeneration following advanced chronic
63
Clinical Imaging 73 (2021) 61–72
inflammation, ranging from a single cyst to multiple cysts of variable
size [11,12,59]. In contrast, ELS is more often triangular in shape and
can have an extrapulmonary location, including the suprarenal upper
abdomen [58,60–62], or in a few cases, the pericardial sac [36–38].
Depending on the size, the sequestration can compress or displace the
heart and the rest of the lungs, and large lesions can produce a shift of
mediastinal structures.
An ipsilateral pleural effusion can be seen in 6–10% of cases diag­
nosed at prenatal ultrasound (possibly related to dilation of subpleural
lymphatics or torsion around the connecting vessels and fibrous pedicle)
[3,56,58] and can be massive [63] or recur despite successful antenatal
treatment [64]. Of interest, Kitano et al. reported that ample flow in the
aberrant vein of fetal ELS at Doppler ultrasound may indicate a benign
clinical behavior, whereas difficulty in detecting aberrant venous flow
may be correlated with the development of massive pleural effusion,
supporting the hypothesis that pleural effusion is associated to venous
obstruction [65].
PS may manifest with ultrasonographic findings of polyhydramnios
[7,66], with or without fetal hydrops. Polyhydramnios may result from
esophageal compression and diminished swallowing of amniotic fluid by
the fetus, or from excessive fluid secretion by the sequestration, whereas
fetal hydrops may occur due to venous compression by the mass, along
with consequent edema, ascites, and pleural effusion [58,60,67].
Color and power Doppler ultrasound can play a key role in the
diagnosis of PS, as they can allow identifying the systemic arterial
supply to the lesion (a pathognomonic feature of PS, more typically
M. Gabelloni et al.
Fig. 2. ILS in a 61-year-old woman with history of recurrent right-sided pneumonia, appearing on chest radiographs [(a) posteroanterior view, (b) lateral view] as a
mass-like, smoothly marginated paravertebral consolidation located in the right lower lobe (asterisk).
branching from the thoracic descending or subdiaphragmatic aorta)
(Fig. 1), which should therefore be searched for carefully. Internal ves­
sels can be depicted inside the sequestration and both feeding arteries
and draining veins (either into the systemic or the pulmonary circula­
tion) can be displayed, although the venous drainage can be more
difficult to identify. In this latter case, the duplex Doppler flow pattern
can be useful to differentiate between the arterial and venous systems,
with the former showing a pulsatile flow and the latter a continuous
slow flow [59,68]. Moreover, the Doppler ultrasound finding of a sys­
temic arterial supply can be useful in the differential diagnosis between
PS and other conditions (such as type II CPAM) that lack such supply
[69].
Because of its location, the differential diagnosis of PS includes
several conditions including CPAM, bronchogenic cysts, enteric dupli­
cation cysts, neuroblastoma, adrenal hematoma, hemolymphangioma
and teratoma [3,34,35].
3.2. Chest radiography
Uncomplicated ILS may be seen as a homogenous opacity (appearing
as a solitary nodule or uniformly dense mass with smooth or lobulated
contours) or a patchy consolidation with irregular margins that is typi­
cally located in the posterobasal aspect of the left lower lobe, with the
right lower lobe being affected in about one third of cases (Fig. 2). As a
result of chronic inflammation, cystic spaces may develop, and single or
multiple cavitations of varying size may be detected that contain air-
fluid levels (about one third of all cases of cystic ILS) or are
completely air-filled (Fig. 3). Complete air filling can also be due to a
fistulous bronchial communication, which may reflect chronic inflam­
mation and does not necessarily indicate active infection. Pneumothorax
may occur when cysts rupture into the pleural cavity, and rarely the
lesion can appear as an area of radiolucency. Occasionally, the only
radiographic finding is a tubular or branching opacity representing the
anomalous systemic artery, and localized hyperinflation with air trap­
ping of the lung surrounding the sequestered segment may be seen
[2,11]. After antibiotic treatment, the radiographic abnormality may
diminish or change significantly, and a small, irregular area of opacity or
a multicystic lesion may remain.
In 1.5% of cases, ILS may also be seen as an irregular lesion
mimicking a malignant tumor. Pleural effusion may rarely occur (4% of
cases), and other findings such as focal bronchiectasis, subsegmental
atelectasis, decreased lung volume, mediastinal shift, and prominence of
64
Clinical Imaging 73 (2021) 61–72
the pulmonary hilum have also been described [2,11,22,57].
ELS most commonly manifests as a well-defined pyramidal, oval or
round mass, or a small bump in the pleural space along the poster­
omedial aspect of the hemidiaphragm. ELS may also be located outside
the pleural space in the mediastinum, embedded in the diaphragm, or in
the upper abdomen or retroperitoneum. Because of its separate pleural
investment from aerated lung, the development of cystic areas inside
ELS is rare, so that air within the lesion suggests a communication with
the gastrointestinal tract (i.e. the esophagus or stomach). In this latter
instance, barium esophagography can be useful to identify the presence
of a fistula and provide indirect signs of any associated thoracic vascular
anomalies [1,2,22,57].
3.3. Cross-sectional imaging: multidetector CT and MRI
Multidetector CT and MRI play a pivotal role in the diagnosis of PS,
owing to their ability to accurately depict its anatomical location and
structure and to identify its arterial supply and venous drainage. To this
latter purpose, both techniques can replace digital subtraction angiog­
raphy for diagnostic purposes, which is currently reserved for selected
cases (where the clinical and radiological suspicion for PS is high, but a
supplying systemic vessel cannot be shown using CT or MR angiog­
raphy) and to guide treatment [2,70–72].
As known, MRI is a reasonable alternative to CT in many pediatric
scenarios and should be preferred especially in children due to its lack of
exposure to ionizing radiation and iodinated contrast material, and su­
perior soft tissue resolution. Fetal MRI with the use of state-of-the-art
ultrafast sequences can be complementary to US for prenatal detection
of fetal abnormalities, offering advantages such as a higher spatial res­
olution (due to the use of dedicated multichannel coils), a large field of
view (allowing a panoramic assessment of the entire fetal chest and
beyond), and good tissue characterization. PS usually appears as a well-
defined mass with homogeneous, relatively high signal intensity on T2-
weighted images, higher than the normal lung but lower than free am­
niotic fluid. Steady-state free precession sequences can provide a
simultaneous depiction of both the morphology and vascular supply of
the lesion [56,73].
Other strengths of MRI include distinguishing between solid and
cystic components of a PS, helping in complex cases in which an asso­
ciated anomaly (e.g. CPAM or a congenital diaphragmatic hernia) is
present, and aiding differentiation of PS from other conditions (e.g. a
neuroblastoma or adrenal hemorrhage versus subdiaphragmatic ELS)
M. Gabelloni et al. Clinical Imaging 73 (2021) 61–72

Fig. 3. Incidentally detected ILS in a 58-year-old woman candidate to surgery for colon cancer. (a, b) Chest radiographs show a partially air-filled mass-like
consolidation located in the posterior aspect of the left lower lobe, adjacent to the diaphragmatic dome (arrow). (c, d) Multidetector CT examination shows a well
demarcated, air-filled paravertebral cystic mass in the posterior segment of the left lower lobe, associated with consolidation and bronchiectasis of the neighboring
lung parenchyma. A feeding artery arising from the descending thoracic aorta can be seen (dashed arrow).

65
M. Gabelloni et al. Clinical Imaging 73 (2021) 61–72

Fig. 4. 26-year-old woman with ILS undergoing multidetector CT staging for non-Hodgkin lymphoma. (a, b) Chest radiographs acquired before the diagnosis showed
a mass-like, rounded retrocardiac opacity (arrow) that is better seen on the lateral view (b). (c, d) Multidetector CT examination shows an area containing enlarged
vessels in the left lower lobe and a large anomalous artery arising from the descending thoracic aorta (dashed arrow). (e) Maximum intensity projection (MIP) and (f)
VR reconstructions show that the lesion receives its arterial supply from the above-mentioned aberrant aortic branch and drains into the left lower pulmonary vein.

[56,74]. The multiparametric nature of MRI can also be harnessed for
the detection of hemorrhagic infarction as a possible complication of PS,
e.g. due to trauma [75] or torsion of vascular pedicle in ELS [76]. A
recent article by Kellenberger et al. illustrated a dedicated lung MRI
protocol for a comprehensive post-natal structural and perfusion
assessment of CBPFMs (including PS), showing a perfusion defect of all
lesions at peak pulmonary enhancement and a delayed peak of non-
cystic lesions compared to normal lung tissue [77].
Compared to CT, MRI is less accurate for the assessment of the lung
parenchyma, has longer acquisition times (usually requiring sedation in
younger children, with delays in imaging and potential cardiorespira­
tory compromise and neurotoxicity), and may not be as immediately
available in the community [70,78]. Multidetector CT performed with
state-of-the-art equipment allows a fast, comprehensive assessment of
the airways, lung parenchyma, blood vessels and surrounding structures
with substantially smaller amounts of ionizing radiation compared with
older generation technology, allowing a panoramic evaluation of the
relevant parenchymal and vascular anatomy by means of high quality
two- and three-dimensional image processing techniques [19,79,80]. In
this latter context, multiplanar reformations and volume rendering (VR)
reconstructions can be helpful to increase the diagnostic confidence and
overall accuracy, aiding treatment planning and improving communi­
cation with both clinicians and patients [16,19,81]. Modern dual energy
CT scanners can deliver a radiation dose that is equivalent or less
compared with conventional single-energy CT equipment and allow
optimizing tissue and vessel contrast, avoiding true precontrast scans,
assessing functional parameters such as pulmonary perfusion, and
improving the detection of perfusion within vascular malformations
either before or after embolization [82,83].
Multidetector CT can easily provide a direct assessment of paren­
chymal findings, which can be variable and reflect the various patterns
depicted on conventional radiography, including a nodular lesion, a
homogenous or heterogenous solid mass with or without cystic areas, or
consolidation (Fig. 4). This latter pattern may be found (although not
necessarily) in infected PS, as well as ground glass opacities (possibly a
hallmark of alveolar hemorrhage following hemoptysis) or cavitation
with or without air/fluid levels. In particular, the presence of an air/
fluid level or air alone inside cysts is suggestive of a communication with
the tracheobronchial tree following recurrent infections and can mimic
other conditions, including a lung abscess, necrotizing pneumonia,
fungal or mycobacterial infection, a cavitating tumor or empyema.
Contrast-enhancement of the surrounding lung parenchyma may be seen
in infected PS, and during episodes of active inflammation the thin walls
of cysts may become indistinct and obscured by consolidation [11,84].
Emphysematous changes at the transition between the lesion and the
neighboring normal lung tissue can be seen due to collateral air drift
[22].
Irregular cystic spaces and bronchiectasis can also be found as
sequelae of recurrent PS infection [85,86]. In a retrospective study by
Long et al. involving 43 patients with pathologically proven PS, 16 cases
(37.2%) presented as mass lesions, 14 (32.6%) as cystic lesions and 7
(16.3%) as pneumonic lesions, whereas 4 (9.3%) and 2 (4.6%) showed
cavitation and bronchiectasis, respectively [87]. In particular, with ILS
the bronchovascular bundles of the remaining functional lung may be
peripherally displaced by the lesion, and more rarely, a dense branching
pattern due to mucus-impacted ectatic bronchi or ipsilateral pleural
effusion can be seen. A large ELS can also be accompanied by ipsilateral
pleural effusion due to poor lymphatic drainage through dilated lymph

66
M. Gabelloni et al. Clinical Imaging 73 (2021) 61–72

Fig. 5. 48-year-old man with right-sided ILS. (a, b) Source axial multidetector CT images show an elongated, fluid-filled structure in the posterior segment of the
right lower lobe, focally reaching the paravertebral pleural lining (arrow). A large vessel originating from the anterior aspect of the distal descending thoracic aorta
can also be seen (dashed arrow). (c) MIP reconstruction allows displaying the course of the aberrant aortic branch feeding the lesion. (d) VR reconstruction depicts
both the arterial supply and the venous drainage of the lesion into the right lower pulmonary vein.

vessels in the lesion and may produce significant mass effect on medi­
astinal structures [60].
An isolated emphysematous bleb with no functional lung tissue in an
atypical location is also a reported presentation [7,11]. Parenchymal
hyperlucency with air trapping can be found in ILS, supporting the hy­
pothesis of collateral pathways between the lesion and the adjacent
normal lung through pores of Kohn, probably located in the lung pa­
renchyma around the vascular pedicle of the lesion [88,89]. Moreover,
CT can readily depict calcifications, an unusual feature in ILS, which
may be diffuse or peripheral and have been observed within the
sequestration and in the anomalous systemic artery, where they are
related to premature atherosclerosis [11].
Multidetector CT and MR angiography play a key role for the
assessment of the vascular supply of a suspected PS. As mentioned
above, the finding of a systemic arterial supply is essential to differen­
tiate PS from other pulmonary conditions, such as CPAMs (especially
small cyst ones), which may overlap with PS and share histologic fea­
tures of it in so-called hybrid lesions [90–94]. Most frequently, ILS has a
single arterial supply originating from the descending thoracic aorta
(73% of cases) and reaching the lesion after passing through the ipsi­
lateral inferior pulmonary ligament [7,11] (Fig. 5). A multiple systemic
arterial supply has been reported in 16% of ILS (Fig. 6). The supplying
artery may also arise from the abdominal aorta (6.9–31.6% of cases),
splenic artery (21% of cases), celiac axis (20%), intercostal arteries
(3–4%) [1,11,95,96], or more rarely from the renal, subclavian, internal
thoracic, pericardiophrenic, left gastric or coronary arteries
[11,23,28,97–102]. The mean diameter of the feeding artery is 6.3–6.6
mm, but it can be as large as 2.5 cm and can approach the size of the
aorta in newborns, and aneurysms may occur, although unusual. Rarely
the systemic artery is very thin (1 mm or less) or absent and is not dis­
played at cross-sectional imaging, warranting the use of digital sub­
traction angiography for diagnosis [11,22,101,103].
The venous drainage of ILS is most commonly to the left atrium via
the pulmonary veins (about 95% of cases), but it can also be to the
systemic circulation through the azygos, hemiazygos or intercostal
veins, or the superior or inferior vena cava towards the right atrium. In
rare cases, ILS may drain into a branch of the pulmonary artery, joining
antegrade arterial flow into normal lung parenchyma [11,22,98].
The blood supply of ELS typically comes from systemic arteries,
arising directly from the thoracic or abdominal aorta in approximately
80% of cases. The feeding vessel is typically single and sized between 5
mm and 20 mm in diameter. In approximately 15% of cases, ELS is
supplied by smaller feeders from the celiac, splenic, gastric, subclavian,
and intercostal arteries (Fig. 7). There is often a single anomalous artery
arising from the thoracic or abdominal aorta, and the supplying artery
may be not be detectable in cases of infarction. Approximately 20% of

67
M. Gabelloni et al. Clinical Imaging 73 (2021) 61–72

Fig. 6. 44-year-old woman with history of left sided lung infection related to ILS. (a, b) Source axial multidetector CT images show a cystic-like, fluid filled lobulated
mass extending into the posterior segment of the left lower lobe (arrow). An area of homogeneous consolidation borders the anterior aspect of the lesion (dashed
arrow), and there are mild ground glass opacities in the surrounding lung parenchyma. (c) VR reconstruction displays a multiple arterial supply from aberrant
branches stemming from the descending thoracic aorta and a single venous drainage to the left lower pulmonary vein.

lesions are supplied by multiple arteries, whereas in 5% of cases, the
lesion is supplied by branches of the pulmonary artery or by both the
pulmonary and systemic circulations [1,60].
As opposite to ILS, the venous drainage of ELS is usually systemic
(80%) through the azygos system, the hemiazygos system, or the vena
cava to the right atrium. In approximately 25% of cases, the venous
drainage of ELS is partly through the pulmonary veins [104], and less
common routes of drainage include the portal, intercostal, suprarenal,
and other abdominal veins. An ELS supplied by pulmonary arteries is
more likely to have a pulmonary venous drainage [1,60]. Due to the
potential finding of a suprarenal vascular supply to ELS, image acqui­
sition in multidetector CT and MRI examinations should span the chest
and the upper abdomen down to the renal vessels [19,70].
4. Treatment options
Surgical resection with isolation and division of anomalous systemic
feeding arteries is the treatment of choice for PS, and is recommended
because of the likelihood of recurrent infection, the need for larger
resection if the sequestration becomes chronically infected, the risk of
hemorrhage and the reported development of malignancy, but also in
asymptomatic patients to avoid infection [7,23,95,105,106]. Lobectomy
is usually performed, although wedge resection can be preferred for
smaller lesions that are distant from the pulmonary hilum and with a
significant portion of surrounding normal lung tissue [107]. Resection
can be achieved by open thoracotomy or video-assisted thoracoscopy,
which can be a viable option in patients with localized PS without
adhesion to the thoracic cavity or the hilum [108,109].
Endovascular occlusion of the arterial supply can reduce blood flow
to the sequestered tissue, leading to necrosis, fibrosis and progressive
involution, and hence can represent a useful presurgical approach to
minimize the risk of intraoperative bleeding, as well as an alternative to
surgery in selected cases, including small PS (<3 cm) and complications
such as acute hemoptysis [7,110–115]. Embolizing agents include
polyvinyl alcohol particles, microcoils, N-butyl cyanoacrylate glue,
Amplatzer occlusion devices, alcohol, and gelatin [7,112,116]. Micro­
coils are preferred in infants as an embolization agent because they can
be delivered through smaller diameter catheters [7,117], and self-
expandable cylindrical mesh devices like the Amplatzer Vascular Plug
can be used when large and high-flow vessels are present, involving the
risk of coil migration or requiring the use of multiple coils [118–120].
Exclusion of the arterial supply by placement of a thoracic endograft has
also been reported in presence of a large and tortuous aberrant feeding
artery, where it is easier and more effective to stop blood flow by
covering the feeding artery orifice than through direct embolization
[115].
Endovascular treatment is associated with a recurrence rate ranging
from 25% to 47%. Recurrence can be due to several factors, including
incomplete closure of embolized vessels, PS reperfusion due to opening
of collaterals or formation of shunts, and displacement of the embolic
agent. A potential complication of PS embolization as a standalone
treatment is lung infection with abscess formation and/or recurrent
hemoptysis following massive necrosis of the devascularized unresected
PS. For this reason, although no established guidelines have been pro­
duced so far for PS treatment, hybrid operation consisting of emboli­
zation followed by surgical resection can be a promising approach with
potentially higher safety and effectiveness than single resection or
embolization, especially in complex cases with high risks of hemorrhage

68
M. Gabelloni et al. Clinical Imaging 73 (2021) 61–72

Fig. 7. 42-year-old woman with left-sided subdiaphragmatic ELS. (a) Coronal single-shot fast spin-echo T2-weighted MR image and (b) axial fat-suppressed fast spin-
echo T2-weighted image show a hyperintense, smoothly marginated subdiaphragmatic mass containing a small cystic area (arrow). (c, d) MR angiography displays a
thin aberrant vessel arising from the celiac artery and supplying the lesion. A large aneurysm of the distal splenic artery is also visible. (e) Multidetector CT ex­
amination shows small calcifications inside the lesion (double arrow). (f, g) Multidetector CT angiography confirmed the finding of a thin arterial supply from the
distal celiac artery. Neither multidetector CT nor MR angiography was able to identify the venous drainage.

69
M. Gabelloni et al.
and recurrence (e.g. those with multiple or large feeding vessels and/or
large PS) [7,110,121]. Other complications of PS embolization include
post-embolization syndrome (characterized by fever, pain, hyperten­
sion, cough within some days of the procedure), thrombosis at puncture
site and migration of embolization material to nontarget arteries, and
have been reported more frequently in the pediatric population
[113,121,122].
Although no recommendations exist on the timing and indications of
follow-up imaging after endovascular treatment of PS, this can be useful
to monitor treatment outcome by confirming persistence of lack of
lesion perfusion. To this purpose, MRI and especially multidetector CT
can provide a comprehensive post-treatment assessment by displaying
occlusion of the feeding vessels with no contrast enhancement of the PS,
which may undergo gradual involution compared to pre-treatment im­
aging [123–125].
Declaration of competing interest
None.
Acknowledgment
The authors wish to thank Dr. Julia Gray for her kind assistance with
the English editing of this manuscript.
References
[1] Walker CM, Wu CC, Gilman MD, Godwin 2nd JD, Shepard J-Ao, Abbott GF. The
imaging spectrum of bronchopulmonary sequestration. Curr Probl Diagn Radiol
2014;43:100–14. https://doi.org/10.1067/j.cpradiol.2014.01.005.
[2] Abbey P, Das CJ, Pangtey GS, Seith A, Dutta R, Kumar A. Imaging in
bronchopulmonary sequestration. J Med Imaging Radiat Oncol 2009;53:22–31.
https://doi.org/10.1111/j.1754-9485.2009.02033.x.
[3] Corbett HJ, Humphrey GME. Pulmonary sequestration. Paediatr Respir Rev 2004;
5:59–68. https://doi.org/10.1016/j.prrv.2003.09.009.
[4] Guibaud L, Filiatrault D, Garel L, Grignon A, Dubois J, Miron MC, et al. Fetal
congenital diaphragmatic hernia: accuracy of sonography in the diagnosis and
prediction of the outcome after birth. AJR Am J Roentgenol 1996;166:1195–202.
https://doi.org/10.2214/ajr.166.5.8615269.
[5] Pryce DM. Lower accessory pulmonary artery with intralobar sequestration of
lung; a report of seven cases. J Pathol Bacteriol 1946;58:457–67.
[6] Sade RM, Clouse M, Ellis Jr FH. The spectrum of pulmonary sequestration. Ann
Thorac Surg 1974;18:644–58. https://doi.org/10.1016/S0003-4975(10)64417-7.
[7] Chakraborty RK, Modi P, Sharma S. Pulmonary sequestration. StatPearls,
Treasure Island (FL): StatPearls Publishing; 2020.
[8] Clements BS, Warner JO. Pulmonary sequestration and related congenital
bronchopulmonary-vascular malformations: nomenclature and classification
based on anatomical and embryological considerations. Thorax 1987;42:401–8.
https://doi.org/10.1136/thx.42.6.401.
[9] Andrade CF, Ferreira HP da C, Fischer GB. Congenital lung malformations. J Bras
Pneumol 2011;37:259–71. https://doi.org/10.1590/s1806-
37132011000200017.
[10] Kalimi R, Penfield Faber L. Clinical scenarios in thoracic surgery. Lippincott
Williams & Wilkins; 2004.
[11] Frazier AA, Rosado de Christenson ML, Stocker JT, Templeton PA. Intralobar
sequestration: radiologic-pathologic correlation. Radiographics 1997;17:725–45.
https://doi.org/10.1148/radiographics.17.3.9153708.
[12] Walford N, Htun K, Chen J, Liu YY, Teo H, Yeo GSH. Intralobar sequestration of
the lung is a congenital anomaly: anatomopathological analysis of four cases
diagnosed in fetal life. Pediatr Dev Pathol 2003;6:314–21. https://doi.org/
10.1007/s10024-001-0194-z.
[13] Yamamura Y, Hida Y, Kaga K, Kawada M, Niizeki H, Ichinokawa M, et al.
Simultaneous resection of bilateral intralobar and extralobar pulmonary
sequestrations with video-assisted thoracoscopic surgery. Ann Thorac Surg 2009;
87:1939–41. https://doi.org/10.1016/j.athoracsur.2008.11.008.
[14] Jeanfaivre T, Afi M, L’hoste P, Tuchais E. Simultaneous discovery of bilateral
intralobar and extralobar pulmonary sequestrations. Ann Thorac Surg 1997;63:
1171–3. https://doi.org/10.1016/S0003-4975(97)00070-2.
[15] Felker RE, Tonkin IL. Imaging of pulmonary sequestration. AJR Am J Roentgenol
1990;154:241–9. https://doi.org/10.2214/ajr.154.2.2105007.
[16] Lee EY, Boiselle PM, Cleveland RH. Multidetector CT evaluation of congenital
lung anomalies. Radiology 2008;247:632–48. https://doi.org/10.1148/
radiol.2473062124.
[17] Berrocal T, Madrid C, Novo S, Gutiérrez J, Arjonilla A, Gómez-León N. Congenital
anomalies of the tracheobronchial tree, lung, and mediastinum: embryology,
radiology, and pathology. Radiographics 2004;24:e17. https://doi.org/10.1148/
rg.e17.
70
Clinical Imaging 73 (2021) 61–72
[18] al-Salem AH, Adu-Gyamfi Y, Grant CS. Congenital lobar emphysema. Can J
Anaesth 1990;37:377–9. https://doi.org/10.1007/BF03005595.
[19] Tomà P, Rizzo F, Stagnaro N, Magnano G, Granata C. Multislice CT in congenital
bronchopulmonary malformations in children. Radiol Med 2011;116:133–51.
https://doi.org/10.1007/s11547-010-0582-4.
[20] Lanza C, Bolli V, Galeazzi V, Fabrizzi B, Fabrizzi G. Cystic adenomatoid
malformation in children: CT histopathological correlation. Radiol Med 2007;
112:612–9. https://doi.org/10.1007/s11547-007-0166-0.
[21] Montjoy C, Hadique S, Graeber G, Ghamande S. Intralobar bronchopulmonary
sequestra in adults over age 50: case series and review. W V Med J 2012;108:
8–13.
[22] Bolca N, Topal U, Bayram S. Bronchopulmonary sequestration: radiologic
findings. Eur J Radiol 2004;52:185–91. https://doi.org/10.1016/j.
ejrad.2004.03.005.
[23] Wani SA, Mufti GN, Bhat NA, Baba AA. Pulmonary sequestration: early diagnosis
and management. Case Rep Pediatr 2015;2015:454860. https://doi.org/
10.1155/2015/454860.
[24] Vijayaraghavan SB, Rao PS, Selvarasu CD, Rao TMS. Prenatal sonographic
features of intralobar bronchopulmonary sequestration. J Ultrasound Med 2003;
22:541–4. https://doi.org/10.7863/jum.2003.22.5.541.
[25] Pinto RM, Araujo Júnior E, Augusto LC, Costa JIF, Dias DA, Aguiar LB, et al.
Spontaneous regression of intralobar pulmonary sequestration during the
pregnancy: report of two cases through relationships between mass and fetal
biometry and review of the literature. J Matern Fetal Neonatal Med 2016;29:
1720–4. doi:https://doi.org/10.3109/14767058.2015.1063608.
[26] Bermúdez C, Pérez-Wulff J, Bufalino G, Sosa C, Gómez L, Quintero RA.
Percutaneous ultrasound-guided sclerotherapy for complicated fetal intralobar
bronchopulmonary sequestration. Ultrasound Obstet Gynecol 2007;29:586–9.
https://doi.org/10.1002/uog.3944.
[27] Mohapatra M, Mishra S, Jena P. Massive hemoptysis in a case of intralobar
pulmonary sequestration associated with pulmonary hypoplasia and meandering
right pulmonary vein: diagnosis and management. Case Rep Pulmonol 2012;
2012:960948. https://doi.org/10.1155/2012/960948.
[28] Sun X, Xiao Y. Pulmonary sequestration in adult patients: a retrospective study.
Eur J Cardiothorac Surg 2015;48:279–82. https://doi.org/10.1093/ejcts/ezu397.
[29] Berna P, Cazes A, Bagan P, Riquet M. Intralobar sequestration in adult patients.
Interact Cardiovasc Thorac Surg 2011;12:970–2. https://doi.org/10.1510/
icvts.2010.263897.
[30] Stocker JT, Malczak HT. A study of pulmonary ligament arteries. Relationship to
intralobar pulmonary sequestration. Chest 1984;86:611–5. https://doi.org/
10.1378/chest.86.4.611.
[31] Do KH, Goo JM, Im JG, Kim KW, Chung JW, Park JH. Systemic arterial supply to
the lungs in adults: spiral CT findings. Radiographics 2001;21:387–402. https://
doi.org/10.1148/radiographics.21.2.g01mr06387.
[32] Pikwer A, Gyllstedt E, Lillo-Gil R, Jönsson P, Gudbjartsson T. Pulmonary
sequestration—a review of 8 cases treated with lobectomy. Scand J Surg 2006;95:
190–4. https://doi.org/10.1177/145749690609500312.
[33] Luck SR, Reynolds M, Raffensperger JG. Congenital bronchopulmonary
malformations. Curr Probl Surg 1986;23:251–314. https://doi.org/10.1016/
0011-3840(86)90013-4.
[34] Laje P, Martinez-Ferro M, Grisoni E, Dudgeon D. Intraabdominal pulmonary
sequestration. A case series and review of the literature. J Pediatr Surg 2006;41:
1309–12. https://doi.org/10.1016/j.jpedsurg.2006.03.049.
[35] Chouikh T, Berteloot L, Revillon Y, Delacourt C, Khen-Dunlop N. Extralobar
pulmonary sequestration with combined gastric and intradiaphragmatic
locations. Pediatr Pulmonol 2014;49:512–4. https://doi.org/10.1002/
ppul.22891.
[36] Yanagisawa S, Maeda K, Tazuke Y, Baba K, Tuji Y, Kawahara I, et al.
Intrapericardial extralobar pulmonary sequestration detected as an intrathoracic
cystic mass by using prenatal ultrasonography: case report and review of the
literature. J Pediatr Surg 2012;47:2327–31. https://doi.org/10.1016/j.
jpedsurg.2012.09.056.
[37] de Vreede I, Bilardo CM, van Rijn RR, Clur SAB, Heij HA. Intrapericardial
extralobar pulmonary sequestration presenting as a prenatal intrathoracic mass.
Pediatr Cardiol 2008;29:980–2. https://doi.org/10.1007/s00246-007-9147-2.
[38] Wax JR, Pinette MG, Landes A, Blackstone J, Cartin A. Intrapericardial extralobar
pulmonary sequestration-ultrasound and magnetic resonance prenatal diagnosis.
Am J Obstet Gynecol 2002;187:1713–4. https://doi.org/10.1067/
mob.2002.125890.
[39] Liu L, Han P, Zhu Y, Gong J, Xu Y, Wei X, et al. Intra-abdominal pulmonary
sequestration: a case report and literature review. Urol Int 2012;88:121–4.
https://doi.org/10.1159/000331688.
[40] Davoli F, Turello D, Valente G, Rena O, Roncon A, Baietto G, et al. Extralobar
pulmonary sequestration presenting with recurring massive pleural effusion in a
young woman: a challenging case. Heart Lung Circ 2016;25:e13–5. https://doi.
org/10.1016/j.hlc.2015.09.006.
[41] Avishai V, Dolev E, Weissberg D, Zajdel L, Priel IE. Extralobar sequestration
presenting as massive hemothorax. Chest 1996;109:843–5. https://doi.org/
10.1378/chest.109.3.843.
[42] Okubo Y, Hamakawa H, Ueda H, Imai Y, Takahashi Y. Extralobar sequestration
presenting as sudden chest pain due to hemothorax. Ann Thorac Surg 2016;101:
e27. https://doi.org/10.1016/j.athoracsur.2015.10.025.
[43] Nakano T, Tetsuka K, Yamamoto S, Endo S. Strangulation of aberrant artery in
extralobar pulmonary sequestration on video imaging. Interact Cardiovasc
Thorac Surg 2014;19:324–5. https://doi.org/10.1093/icvts/ivu103.
M. Gabelloni et al.
[44] Uchida DA, Moore KR, Wood KE, Pysher TJ, Downey EC. Infarction of an
extralobar pulmonary sequestration in a young child: diagnosis and excision by
video-assisted thoracoscopy. J Laparoendosc Adv Surg Tech A 2010;20:399–401.
https://doi.org/10.1089/lap.2009.0217.
[45] Choe J, Goo HW. Extralobar pulmonary sequestration with hemorrhagic
infarction in a child: preoperative imaging diagnosis and pathological correlation.
Korean J Radiol 2015;16:662–7. https://doi.org/10.3348/kjr.2015.16.3.662.
[46] Pinto Filho DR, Avino AJG, Brandão SLB. Extralobar pulmonary sequestration
with hemothorax secondary to pulmonary infarction. J Bras Pneumol 2009;35:
99–102. https://doi.org/10.1590/s1806-37132009000100015.
[47] Sulhyan KR, Ramteerthakar NA, Gosavi AV, Anvikar AR. Extralobar sequestration
of lung associated with congenital diaphragmatic hernia and malrotation of gut.
Lung India 2015;32:381–3. https://doi.org/10.4103/0970-2113.159585.
[48] Bratu I, Flageole H, Chen MF, Di Lorenzo M, Yazbeck S, Laberge JM. The multiple
facets of pulmonary sequestration. J Pediatr Surg 2001;36:784–90. https://doi.
org/10.1053/jpsu.2001.22961.
[49] Srikanth MS, Ford EG, Stanley P, Mahour GH. Communicating
bronchopulmonary foregut malformations: classification and embryogenesis.
J Pediatr Surg 1992;27:732–6. https://doi.org/10.1016/S0022-3468(05)80103-
4.
[50] Gerle RD, Jaretzki 3rd A, Ashley CA, Berne AS. Congenital bronchopulmonary-
foregut malformation. Pulmonary sequestration communicating with the
gastrointestinal tract. N Engl J Med 1968;278:1413–9. https://doi.org/10.1056/
NEJM196806272782602.
[51] Heithoff KB, Sane SM, Williams HJ, Jarvis CJ, Carter J, Kane P, et al.
Bronchopulmonary foregut malformations. A unifying etiological concept. AJR
Am J Roentgenol 1976;126:46–55. https://doi.org/10.2214/ajr.126.1.46.
[52] Leithiser Jr RE, Capitanio MA, Macpherson RI, Wood BP. “Communicating”
bronchopulmonary foregut malformations. AJR Am J Roentgenol 1986;146:
227–31. https://doi.org/10.2214/ajr.146.2.227.
[53] Pan G, Singleton E, Nihill M, Harberg F. A case of bilateral gastric
bronchopulmonary-foregut malformation. Pediatr Radiol 1989;19:463–4.
https://doi.org/10.1007/BF02387659.
[54] Murray ME, Given-Wilson RM, Christopher JA, Jeffrey IJ. Bilateral
communicating bronchopulmonary foregut malformations in an infant with
multiple congenital anomalies. Pediatr Radiol 1994;24:128–30. https://doi.org/
10.1007/BF02020170.
[55] Merli L, Nanni L, Curatola A, Pellegrino M, De Santis M, Silvaroli S, et al.
Congenital lung malformations: a novel application for lung ultrasound?
J Ultrasound 2019. https://doi.org/10.1007/s40477-019-00406-0.
[56] Dhingsa R, Coakley FV, Albanese CT, Filly RA, Goldstein R. Prenatal sonography
and MR imaging of pulmonary sequestration. AJR Am J Roentgenol 2003;180:
433–7. https://doi.org/10.2214/ajr.180.2.1800433.
[57] Ko SF, Ng SH, Lee TY, Wan YL, Liang CD, Lin JW, et al. Noninvasive imaging of
bronchopulmonary sequestration. AJR Am J Roentgenol 2000;175:1005–12.
https://doi.org/10.2214/ajr.175.4.1751005.
[58] Adzick NS, Harrison MR, Crombleholme TM, Flake AW, Howell LJ. Fetal lung
lesions: management and outcome. Am J Obstet Gynecol 1998;179:884–9.
https://doi.org/10.1016/S0002-9378(98)70183-8.
[59] Riccabona M. Ultrasound of the chest in children (mediastinum excluded). Eur
Radiol 2008;18:390–9. https://doi.org/10.1007/s00330-007-0754-3.
[60] Rosado-de-Christenson ML, Frazier AA, Stocker JT, Templeton PA. From the
archives of the AFIP. Extralobar sequestration: radiologic-pathologic correlation.
Radiographics 1993;13:425–41. https://doi.org/10.1148/
radiographics.13.2.8460228.
[61] Yildirim G, Güngördük K, Aslan H, Ceylan Y. Prenatal diagnosis of an extralobar
pulmonary sequestration. Arch Gynecol Obstet 2008;278:181–6. https://doi.org/
10.1007/s00404-008-0569-8.
[62] Xu G, Zhou J, Zeng S, Zhang M, Ouyang Z, Zhao Y, et al. Prenatal diagnosis of
fetal intraabdominal extralobar pulmonary sequestration: a 12-year 3-center
experience in China. Sci Rep 2019;9:943. https://doi.org/10.1038/s41598-018-
37268-1.
[63] Mallmann MR, Geipel A, Bludau M, Matil K, Gottschalk I, Hoopmann M, et al.
Bronchopulmonary sequestration with massive pleural effusion: pleuroamniotic
shunting vs intrafetal vascular laser ablation. Ultrasound Obstet Gynecol 2014;
44:441–6. https://doi.org/10.1002/uog.13304.
[64] Divjak N, Vasseur Maurer S, Giannoni E, Vial Y, de Buys Roessingh A,
Wildhaber BE. Bronchopulmonary sequestration with morbid neonatal pleural
effusion despite successful antenatal treatment. Front Pediatr 2017;5:259.
https://doi.org/10.3389/fped.2017.00259.
[65] Kitano Y, Sago H, Hayashi S, Kuroda T, Honna T, Morikawa N. Aberrant venous
flow measurement may predict the clinical behavior of a fetal extralobar
pulmonary sequestration. Fetal Diagn Ther 2008;23:299–302. https://doi.org/
10.1159/000123617.
[66] Becmeur F, Horta-Geraud P, Donato L, Sauvage P. Pulmonary sequestrations:
prenatal ultrasound diagnosis, treatment, and outcome. J Pediatr Surg 1998;33:
492–6. https://doi.org/10.1016/s0022-3468(98)90095-1.
[67] Hernanz-Schulman M, Stein SM, Neblett WW, Atkinson JB, Kirchner SG,
Heller RM, et al. Pulmonary sequestration: diagnosis with color Doppler
sonography and a new theory of associated hydrothorax. Radiology 1991;180:
817–21. https://doi.org/10.1148/radiology.180.3.1871300.
[68] Deeg KH, Hofbeck M, Singer H. Diagnosis of intralobar lung sequestration by
colour-coded Doppler sonography. Eur J Pediatr 1992;151:710–2. https://doi.
org/10.1007/BF01957580.
[69] Sepulveda W. Perinatal imaging in bronchopulmonary sequestration.
J Ultrasound Med 2009;28:89–94. https://doi.org/10.7863/jum.2009.28.1.89.
71
Clinical Imaging 73 (2021) 61–72
[70] Romberg EK, Tang ER, Chandra T, Podberesky DJ, Epelman M, Iyer RS.
Applications of pediatric body CT angiography: what radiologists need to know.
AJR Am J Roentgenol 2020;214:1019–30. https://doi.org/10.2214/
AJR.19.22274.
[71] Deguchi E, Furukawa T, Ono S, Aoi S, Kimura O, Iwai N. Intralobar pulmonary
sequestration diagnosed by MR angiography. Pediatr Surg Int 2005;21:576–7.
https://doi.org/10.1007/s00383-005-1454-1.
[72] Lehnhardt S, Winterer JT, Uhrmeister P, Herget G, Laubenberger J. Pulmonary
sequestration: demonstration of blood supply with 2D and 3D MR angiography.
Eur J Radiol 2002;44:28–32. https://doi.org/10.1016/s0720-048x(01)00413-2.
[73] Hubbard AM, Adzick NS, Crombleholme TM, Coleman BG, Howell LJ,
Haselgrove JC, et al. Congenital chest lesions: diagnosis and characterization with
prenatal MR imaging. Radiology 1999;212:43–8. https://doi.org/10.1148/
radiology.212.1.r99jl3143.
[74] Recio Rodríguez M, Martínez de Vega V, Cano Alonso R, Carrascoso Arranz J,
Martínez Ten P, Pérez Pedregosa J. MR imaging of thoracic abnormalities in the
fetus. Radiographics 2012;32:E305–21. https://doi.org/10.1148/rg.327125053.
[75] Chong NV, Polley Jr TZ, Geiger JD. Infarction of extralobar pulmonary
sequestration after blunt trauma. J Pediatr Surg 2007;42:1127–9. https://doi.
org/10.1016/j.jpedsurg.2007.01.069.
[76] Yang L, Yang G. Extralobar pulmonary sequestration with a complication of
torsion: a case report and literature review. Medicine 2020;99:e21104. https://
doi.org/10.1097/MD.0000000000021104.
[77] Kellenberger CJ, Amaxopoulou C, Moehrlen U, Bode PK, Jung A, Geiger J.
Structural and perfusion magnetic resonance imaging of congenital lung
malformations. Pediatr Radiol 2020;50:1083–94. https://doi.org/10.1007/
s00247-020-04658-5.
[78] Kamat PP, Kudchadkar SR, Simon HK. Sedative and anesthetic neurotoxicity in
infants and young children: not just an operating room concern. J Pediatr 2019;
204:285–90. https://doi.org/10.1016/j.jpeds.2018.08.039.
[79] Nievelstein RAJ, van Dam IM, van der Molen AJ. Multidetector CT in children:
current concepts and dose reduction strategies. Pediatr Radiol 2010;40:1324–44.
https://doi.org/10.1007/s00247-010-1714-7.
[80] Greenwood TJ, Lopez-Costa RI, Rhoades PD, Ramírez-Giraldo JC, Starr M,
Street M, et al. CT dose optimization in pediatric radiology: a multiyear effort to
preserve the benefits of imaging while reducing the risks. Radiographics 2015;35:
1539–54. https://doi.org/10.1148/rg.2015140267.
[81] Lee EY, Tracy DA, Mahmood SA, Weldon CB, Zurakowski D, Boiselle PM.
Preoperative MDCT evaluation of congenital lung anomalies in children:
comparison of axial, multiplanar, and 3D images. AJR Am J Roentgenol 2011;
196:1040–6. https://doi.org/10.2214/AJR.10.5357.
[82] Siegel MJ, Ramirez-Giraldo JC. Dual-energy CT in children: imaging algorithms
and clinical applications. Radiology 2019;291:286–97. https://doi.org/10.1148/
radiol.2019182289.
[83] Machida H, Tanaka I, Fukui R, Shen Y, Ishikawa T, Tate E, et al. Dual-energy
spectral CT: various clinical vascular applications. Radiographics 2016;36:
1215–32. https://doi.org/10.1148/rg.2016150185.
[84] Lee EY, Siegel MJ, Sierra LM, Foglia RP. Evaluation of angioarchitecture of
pulmonary sequestration in pediatric patients using 3D MDCT angiography. AJR
Am J Roentgenol 2004;183:183–8. https://doi.org/10.2214/ajr.183.1.1830183.
[85] Intralobar pulmonary sequestration in right lower lobe with secondary infection
in an adult male. Egypt J Chest Dis Tuberc 2014;63:273–5. https://doi.org/
10.1016/j.ejcdt.2013.10.011.
[86] Gompelmann D, Eberhardt R, Heussel C-P, Hoffmann H, Dienemann H,
Schuhmann M, et al. Lung sequestration: a rare cause for pulmonary symptoms in
adulthood. Respiration 2011;82:445–50. https://doi.org/10.1159/000323562.
[87] Long Q, Zha Y, Yang Z. Evaluation of pulmonary sequestration with multidetector
computed tomography angiography in a select cohort of patients: a retrospective
study. Clinics (Sao Paulo) 2016;71:392–8. https://doi.org/10.6061/clinics/2016
(07)07.
[88] Ferretti GR, Jouvan FB, Coulomb M. MDCT demonstration of intralobar
pulmonary sequestration of the right upper lobe in an adult. AJR Am J
Roentgenol 2005;185:1663–4. https://doi.org/10.2214/AJR.05.0155.
[89] Stern EJ, Webb WR, Warnock ML, Salmon CJ. Bronchopulmonary sequestration:
dynamic, ultrafast, high-resolution CT evidence of air trapping. AJR Am J
Roentgenol 1991;157:947–9. https://doi.org/10.2214/ajr.157.5.1927813.
[90] Biyyam DR, Chapman T, Ferguson MR, Deutsch G, Dighe MK. Congenital lung
abnormalities: embryologic features, prenatal diagnosis, and postnatal radiologic-
pathologic correlation. Radiographics 2010;30:1721–38. https://doi.org/
10.1148/rg.306105508.
[91] Schuster B, Holland-Cunz SG, Zimmermann P. Intradiaphragmatic hybrid lesion:
surgical decision-making and value of minimal invasive surgery. BMJ Case Rep
2017;2017. https://doi.org/10.1136/bcr-2016-218527.
[92] Olivieri C, Nanni L, Busato G, Rindi G, Marano R, Pintus C. Intradiaphragmatic
hybrid lesion in an infant: case report. J Pediatr Surg 2012;47:e25–8. https://doi.
org/10.1016/j.jpedsurg.2012.04.010.
[93] Thacker PG, Rao AG, Hill JG, Lee EY. Congenital lung anomalies in children and
adults: current concepts and imaging findings. Radiol Clin North Am 2014;52:
155–81. https://doi.org/10.1016/j.rcl.2013.09.001.
[94] Abbey P, Narula MK, Anand R. Congenital malformations and developmental
anomalies of the lung. Curr Radiol Rep 2014;2:71. https://doi.org/10.1007/
s40134-014-0071-y.
[95] Alsumrain M, Ryu JH. Pulmonary sequestration in adults: a retrospective review
of resected and unresected cases. BMC Pulm Med 2018;18:97. https://doi.org/
10.1186/s12890-018-0663-z.
M. Gabelloni et al.
[96] Irodi A, Cherian R, Keshava SN, James P. Dual arterial supply to normal lung:
within the sequestration spectrum. Br J Radiol 2010;83:e86–9. https://doi.org/
10.1259/bjr/30458107.
[97] Tsitouridis I, Tsinoglou K, Cheva A, Papapostolou P, Efthimiou D, Moschialos L.
Intralobar pulmonary sequestration with arterial supply from the coronary
circulation. J Thorac Imaging 2005;20:313–5. https://doi.org/10.1097/01.
rti.0000179616.29035.0e.
[98] Hilton TC, Keene WR, Blackshear JL. Intralobar pulmonary sequestration with
nutrient systemic arterial flow from multiple coronary arteries. Am Heart J 1995;
129:823–6. https://doi.org/10.1016/0002-8703(95)90336-4.
[99] Bertsch G, Markert T, Hahn D, Silber RE, Schanzenbächer P. Intralobar lung
sequestration with systemic coronary arterial supply. Eur Radiol 1999;9:1324–6.
https://doi.org/10.1007/s003300050841.
[100] Rao DS, Barik R. Rare presentation of intralobar pulmonary sequestration
associated with repeated episodes of ventricular tachycardia. World J Cardiol
2016;8:432–5. https://doi.org/10.4330/wjc.v8.i7.432.
[101] Savic B, Birtel FJ, Tholen W, Funke HD, Knoche R. Lung sequestration: report of
seven cases and review of 540 published cases. Thorax 1979;34:96–101. https://
doi.org/10.1136/thx.34.1.96.
[102] Xie D, Xie H, You X, Chen C, Jiang G. Pulmonary sequestration with aberrant
arteries arising from the renal artery and the internal thoracic artery. Ann Thorac
Surg 2013;96:e131. https://doi.org/10.1016/j.athoracsur.2013.08.018.
[103] Janssen DP, Schilte PP, De Graaff CS, Van Dijk HA. Bronchopulmonary
sequestration associated with an aneurysm of the aberrant artery. Ann Thorac
Surg 1995;60:193–4.
[104] Garcia-Peña P, Coma A, Enríquez G. Congenital lung malformations: radiological
findings and clues for differential diagnosis. Acta Radiol 2013;54:1086–95.
https://doi.org/10.1177/028418511305400901.
[105] Laberge J-M, Bratu I, Flageole H. The management of asymptomatic congenital
lung malformations. Paediatr Respir Rev 2004;5:S305–12. https://doi.org/
10.1016/S1526-0542(04)90055-3.
[106] Belchis D, Cowan M, Mortman K, Rezvani B. Adenocarcinoma arising in an
extralobar sequestration: a case report and review of the literature. Lung Cancer
2014;84:92–5. https://doi.org/10.1016/j.lungcan.2014.01.025.
[107] Zhang N, Zeng Q, Chen C, Yu J, Zhang X. Distribution, diagnosis, and treatment of
pulmonary sequestration: report of 208 cases. J Pediatr Surg 2019;54:1286–92.
https://doi.org/10.1016/j.jpedsurg.2018.08.054.
[108] Sakuma T, Sugita M, Sagawa M, Ishigaki M, Toga H. Video-assisted thoracoscopic
wedge resection for pulmonary sequestration. Ann Thorac Surg 2004;78:1844–5.
https://doi.org/10.1016/j.athoracsur.2003.07.028.
[109] Wang L-M, Cao J-L, Hu J. Video-assisted thoracic surgery for pulmonary
sequestration: a safe alternative procedure. J Thorac Dis 2016;8:31–6. https://
doi.org/10.3978/j.issn.2072-1439.2016.01.25.
[110] de Lagausie P, Bonnard A, Berrebi D, Petit P, Dorgeret S, Guys JM. Video-assisted
thoracoscopic surgery for pulmonary sequestration in children. Ann Thorac Surg
2005;80:1266–9. https://doi.org/10.1016/j.athoracsur.2005.02.015.
[111] Zener R, Bottoni D, Zaleski A, Fortin D, Malthaner RA, Inculet RI, et al.
Transarterial embolization of intralobar pulmonary sequestration in a young
72
Clinical Imaging 73 (2021) 61–72
adult with hemoptysis. J Thorac Dis 2017;9:E188–93. https://doi.org/10.21037/
jtd.2017.02.82.
[112] Healy J, Healey A, Kitley C. Embolization of symptomatic intralobar pulmonary
sequestration - a minimally invasive treatment option. Radiol Case Rep 2019;14:
759–62. https://doi.org/10.1016/j.radcr.2019.03.029.
[113] Ellis J, Brahmbhatt S, Desmond D, Ching B, Hostler J. Coil embolization of
intralobar pulmonary sequestration - an alternative to surgery: a case report.
J Med Case Reports 2018;12:375. https://doi.org/10.1186/s13256-018-1915-5.
[114] Zhang S-X, Wang H-D, Yang K, Cheng W, Wu W. Retrospective review of the
diagnosis and treatment of pulmonary sequestration in 28 patients: surgery or
endovascular techniques? J Thorac Dis 2017;9:5153–60. https://doi.org/
10.21037/jtd.2017.10.145.
[115] Chen Y, Liu B, Shao J, Liu D, Zheng Y. Endovascular treatment of pulmonary
sequestration with thoracic endograft: two case reports. Medicine 2019;98:
e16666. https://doi.org/10.1097/MD.0000000000016666.
[116] Vaidya S, Tozer KR, Chen J. An overview of embolic agents. Semin Intervent
Radiol 2008;25:204–15. https://doi.org/10.1055/s-0028-1085930.
[117] Leoncini G, Rossi UG, Ferro C, Chessa L. Endovascular treatment of pulmonary
sequestration in adults using Amplatzer® vascular plugs. Interact Cardiovasc
Thorac Surg 2011;12:98–100. https://doi.org/10.1510/icvts.2010.246546.
[118] Kim JH, Kim SS, Ha KS, Bae J, Park Y. Anomalous arterial supply to normal basal
segment of the right lower lobe: endovascular treatment with the amplatzer
vascular plug. Tuberc Respir Dis 2014;76:295–8. https://doi.org/10.4046/
trd.2014.76.6.295.
[119] Rossi M, Rebonato A, Greco L, Stefanini G, Citone M, Speranza A, et al. A new
device for vascular embolization: report on case of two pulmonary arteriovenous
fistulas embolization using the amplatzer vascular plug. Cardiovasc Intervent
Radiol 2006;29:902–6. https://doi.org/10.1007/s00270-005-0160-7.
[120] Marine LM, Valdes FE, Mertens RM, Bergoeing MR, Kramer A. Endovascular
treatment of symptomatic pulmonary sequestration. Ann Vasc Surg 2011;25:696.
e11-5. https://doi.org/10.1016/j.avsg.2010.08.012.
[121] He B, Sun M-S, Niu Y, Zhang J-B, Nie Q-Q, Zheng X, et al. Hybrid and
endovascular treatment of pulmonary sequestration: two case reports and
literature review. Ann Vasc Surg 2020. https://doi.org/10.1016/j.
avsg.2020.06.066.
[122] Lee BS, Kim JT, Kim EA-R, Kim K-S, Pi S-Y, Sung K-B, et al. Neonatal pulmonary
sequestration: clinical experience with transumbilical arterial embolization.
Pediatr Pulmonol 2008;43:404–13. https://doi.org/10.1002/ppul.20799.
[123] Borzelli A, Paladini A, Giurazza F, Tecame S, Giordano F, Cavaglià E, et al.
Successful endovascular embolization of an intralobar pulmonary sequestration.
Radiol Case Rep 2018;13:125–9. https://doi.org/10.1016/j.radcr.2017.10.003.
[124] Chien K-J, Huang T-C, Lin C-C, Lee C-L, Hsieh K-S, Weng K-P. Early and late
outcomes of coil embolization of pulmonary sequestration in children. Circ J
2009;73:938–42. https://doi.org/10.1253/circj.cj-08-0914.
[125] Lee K-H, Sung K-B, Yoon H-K, Ko G-Y, Yoon CH, Goo HW, et al. Transcatheter
arterial embolization of pulmonary sequestration in neonates: long-term follow-
up results. J Vasc Interv Radiol 2003;14:363–7. https://doi.org/10.1097/01.
rvi.0000058412.01661.f0.