Congenital

Lung
Disease
PULMONARY LUNG DISEASE
PULMONARY SEQUESTRATION
CCAM/CPAM
CLE
Neurogenic cyst
 Congenital pulmonary airway
malformation (CPAM) spectrum

CCAM (Congenital BPS

(Bronchopulmonary CLE (Congenital
cystic adenomatoid
lobar emphysema)
malformation) sequestration)
 What is CCAM?

 Rare pulmonary developmental hamartmatous

abnormality

 Comprised of pulmonary tissue with abnormal bronchial

proliferation

 The fundamental pathologic feature of the lesion is

adenomatoid proliferation of bronchioles that form cysts
at the expense of normal alveoli
 25% of all congenital lung masses

 1 in 25000 live births

 >95% are limited to 1 lobe or segment

 2-3% are B/L

 Pathogenesis

Pulmonary insult during development of

bronchial tree

Failure of bronchial maturation & lack of

normal alveoli

Absence of bronchial cartilage & tubular

glands

Terminal bronchiolar structures without

alveolar differentiation
 Types
Type I Type II Type III

Features 2-10 cms cyst < 2 cms cysts <0.5 cm cysts

Variable sized Uniform cysts Appear solid

cysts

ciliated ciliated cuboidal shows

pseudostratified or columnar adenomatoid
epithelium epithelium elements
consistent with
distal airway
Stage of Gestatio Description Typ
development nal age e
Embryonic 26days- 6 ‘Lung bud’ derived from primitive foregut
weeks and branches dichotomously to form the
early tracheobronchial tree

Pseudoglandul 6- 16 Airways develop to the level of terminal III

ar weeks bronchioles but end blindly within primitive
stroma

Canalicular 16-28 Multiple alveolar ducts arise from II

weeks respiratory bronchioles and are lined by
Type II pneumocytes

Saccular 28-34 Increase in number of terminal sacs, I

weeks thinning of interstitium, proliferation of
capillary bed

Alveolar 34-36 Mature alveoli are composed exclusively of

weeks alveolar lining cells, basement membrane,
cap endothelium
 Additional types

Type 0 Type IV

Acinar dysplasia or agenesis Large peripheral cyst of the

distal acinus lined predom with
alveolar type cells

Associated with malignancy,

specifically pleuropulmonary
blastoma.
 Prenatal classification of CCAM based on USG

Macrocystic Microcystic

Single or multiple cysts >/= <5 mm diameter cysts

5mm diameter

Seen on USG as cysts, difficult to Appear as a solid

identify normal lung on same hyperechogenic mass bec of
side numerous acoustic interfaces

Favourable prognosis Poorer prognosis

 Imaging of CCAM

Pre natal Post natal

 Antenatal USG  CXR

 MRI  CT scan (once air

filled)

 Post natal USG

 Imaging – Prenatal USG

 Earliest diagnosis made at 16 weeks. The diagnosis is

usually made in the 2nd trimester, but sometimes in the
3rd trimester when referred for investigation of
polyhydramnios.

 USG:
 identifies the location of the lung abnormality by its
appearance
 evaluate the blood supply & venous drainage by
doppler
 determine changes in posn of other lung lobes, med &
cardiac structures
 Solid, echogenic lung mass
OR
 Mixed solid cystic mass
OR
 Sometimes, only a single large cyst

 Color doppler- vascular flow to the lesion from a

branch of the pulmonary artery.
 To predict outcome

 CCAM volume- The CCAM volume is estimated using the

formula for a prolate ellipse
CCAM= (Length x Height x Width

x 0.52 )

 A CVR is obtained by dividing the CCAM volume (cc) by

the head circumference (cm) to correct for differences in
the fetal gestational age.
 If the CVR is < 1.6 - favorable prognosis. The risk of developing
hydrops is less than two percent in these cases. The only exceptions
are malformations with a “dominant cyst.”
 Dominant cysts are those that comprise greater than one-third of
the entire volume of the CCAM.
 These lesions can enlarge acutely, do not follow a predictable
pattern of growth and hence must be followed closely.

 Those congenital cystic adenomatoid malformation with a CVR

>
1.6 are at high risk for the development of hydrops and fetal demise
( up to 80 percent of cases).
 Such malformations should be followed with twice-weekly
ultrasound scans so that fetal surgery can be undertaken at the
earliest signs of hydrops.
• All congenital cystic adenomatoid malformation should be
followed once in 2 weeks for measurement of CCAM volume and
CVR until the growth of CCAM reaches a plateau.
•Type 1 c Cystic Adenomatoid malformation. 5-day-old girl. a) Chest X-ray.
Radiolucent image of round morphology and thin wall in the right lung. b
and c) CT scan of chest, axial and coronal reconstruction. Cystic lesion in
the middle lobe, lobed contours and thick wall.
Adenomatoid Cystic malformation type 2. Child 4 days old. a)
Chest X-ray. Thin-walled radiolucent lesions in the right lung. b)
Chest CT, axial. Upper and middle lobe mass consisting of
multiple cystic-looking, thin-walled lesions.
•Type 3 cystic adenomatoid malformation. 2-year-old girl.
CT scan of chest, axial, mediastinal window and pulmonary
window. Mass in the lower right lobe, broad pleural base,
solid, with pointed cystic areas.
Longitudinal section of thorax showing a
large cystic lesion
Cross-section view.The large cystic
lesion is seen in the right hemithorax.
The heart is compressed and
deviated to the left (arrow). Skin
oedema and polyhydramnios
Type 2 congenital cystic adenomatoid malformation (CCAM). A,
Sagittal image of a fetus at 24 weeks with Type 2 CCAM located in the
posterior chest (arrows). B, Transverse image with measurements
showing the inferior extent of the lesion. The mass is multicystic and
located inferior and posterior to the heart. Arrowhead indicates the
stomach.
Transverse image of a fetus at
20 weeks with Type 3 congenital cystic
adenomatoid malformation demonstrating
its position posterior and to the left of the
heart. The heart is displaced to the right.
 Associated findings/ Complications

Mediastinal shift

Polyhydramnios Determine
prognosis &
Mx
Hydrops
 Complications

If large CCAM

Cardiac compression

Altered hemodynamics &

increased CVP

Hydrops- mortality- 100% if

untreated
 Associated anomalies

 Usually isolated and rarely associated with chromosomal

defects.

 Associated anomalies include

 Renal
 Cardiac (truncus arteriosus & TOF)
 CNS & spinal defects
 Abdominal wall defects.
 Type II CCAM is more commonly associated with other
anomalies.

 Elevated alpha-feto protein has been reported with type III

CCAM.
 MRI

 Type I & II- very high SI on T2

- equal to amniotic fluid
- higher than surrounding normal lung

 Type III- moderately high SI

- homogenous
- as they regress- SI drops
- asso with pleural effusion
Coronal T2-weighted MR image shows a well-
circumscribed area of T2 hyperintensity (arrow) in the left
upper lobe.
Oblique coronal T2-weighted MR image shows a well-
circumscribed area of low T2 signal, in a fetus with a
typical, resolving CCAM.
 Prognosis

 If no hydrops by 26 weeks  Good. Therefore, surveillance done every

2 weeks during the 2nd trimester

 Unilateral type I CCAM (macrocystic lesion) in the absence of hydrops

and polyhydramnios - Good prognosis

 The following features are suggestive of poorer prognosis in unilateral

lesions:-
 large size cysts
 mediastinal shift
 fetal hydrops
 polyhydramnios
 associated anomalies
 Bilateral CCAM is lethal

 Type 0 CCAM is considered lethal.

 Resection of Type 1 CCAM is considered to be curative and outcomes are

excellent.

 Outcomes for Type 2 CCAM depend largely on the presence of associated

anomalies.

 The risk of pulmonary hypoplasia is highest with Type 3 CCAM, given its
tendency for growth and mass effect & earlier development of hydrops and
polyhydramnios. Pulmonary hypoplasia cannot, at this time, be predicted
antenatally.
 The natural history of CCAM is near exponential
growth, from 20 weeks gestation until the plateau is
reached which is around 26 weeks

 CCAMs tend to regress during 3rd trimester (30-

40%). As they regress, they become isoechoic to lung,
eventually becomes inapparent in later gestation

 In half of the cases there is no change in the size of

the lesion, while it may enlarge in 10% of the cases.
 Post natal manifestations

 40% with prenatal diagnosis are symptomatic at birth-

acute progressive respiratory distress occurring shortly
after birth with cyanosis, grunting, retractions, and
tachypnea.

 Require intervention or respiratory support (ECMO)

with NICU admission

 Even asymptomatic masses are removed d/t

1. Risk of secondary infection
2. Hemorrhage
3. Carcinoma
4. Prevents development of normal lung
 Patients may present in early infancy with
symptoms of respiratory distress,
vomiting, failure to thrive, and recurrent
pneumonia.
Patients may also present in childhood and adulthood
with recurrent infection localized in the involved lobe
 Post natal (neonatal) imaging-CXR

 Type I lesions -Chest radiographs typically show a

unilateral, airfilled, multicystic lesion in the thorax.
 Homogeneous fluid-opacity pulmonary mass may present
and evolve to demonstrate an air-filled cystic radiographic
appearance.
 The lesions may be very large and may occupy the entire
hemithorax, producing mediastinal shift and mass effect on the
ipsilateral hemidiaphragm.
 The abnormally expanded lung may be herniated across the
midline.
 The uninvolved ipsilateral and contralateral portions of the lung
may be atelectatic or hypoplastic due to compression.
 Type II lesions may appear as heterogeneous areas of
uniform small cysts

 Type III lesions are usually large and homogeneous,

having the appearance of parenchymal consolidation
or a mass rather than that of a cystic lesion
Initial anteroposterior radiograph of
On the second day of life (same patient as in
the chest in a patient with congenital
previous image), an anteroposterior
cystic adenomatoid malformation on
radiograph shows physiologic fluid
the first day of life, with dense lungs
resorbed from an area of congenital cystic
and a suggestion that the right lung is
adenomatoid malformation and replaced
slightly more voluminous than the left
with an air-containing cystic area occupying
lung.
the right upper lung.
Type I lesion in an asymptomatic male
newborn. (a) Frontal chest radiograph obtained for
evaluation of right-sided cardiac sounds shows multipIe
air-filled cysts occupying the left hemithorax. The
cyst walls are thin. There is marked mass effect on the
mediastinum, with displacement to the right. (b) Axial
computed tomographic (CT) image of the thorax
shows multiple thin-walled, air-filled cysts of variable
sizes in the left lung. The lesion produces mass effect
on the mediastinum
 CT Scan

 Areas of small cysts (< 2 cm in diameter) or multiple large

cysts appearing with other abnormalities (a larger cystic
area, consolidation, or low attenuation) are the most
frequent findings.

 Low-attenuation areas are clusters of microcysts.

 Air-fluid levels can be seen in some cysts. These lesions

may be predominantly type I, type II, or a combination of
both.
Computed tomography scan
of the neonate, performed
on day of life 1. There is a 0.9x 0.9 cm
mass with several cysts within the medial
segment of the right lower lobe. No
systemic vessels can be seen supplying
the mass. Findings are consistent with a
Type 2 congenital cystic adenomatoid
malformation.
CT scan of the chest demonstrating a multiseptated
cystic lesion in the right upper lobe consistent with
localized congenital cystic adenomatoid
malformation.
Chest radiographs obtained on day 2 and 3
of life show an expanding, air-filled cystic
lesion (white and yellow arrows) in the
right lower lobe. The newborn also had
hyaline membrane disease.
A CT scan of the same child shows a cystic
lesion in the right lower lobe with
septations (green arrow) and an air-fluid
level (blue arrow).
 Post natal USG

 The complex internal appearance of multiple fluid-

filled areas with internal septations or solid elements
representing the cyst walls can be demonstrated

 Echogenic, solid-appearing thoracic masses may be

seen in patients with type III lesions.
 Post natal management

 In the case of respiratory compromise, resection is indicated and is

curative with minimally invasive surgery

 Elective surgery within few months after birth

 Early resection may allow for compensatory lung development in

the remaining tissue

 Surgical management of CCAM involves lobectomy- suggested for

CCAM because of risks of incomplete resection, which occurs in 15%

 In symptomatic neonates the survival following postnatal

thoracotomy and lobectomy is about 90%.
 D/D of echogenic lesion in fetal thorax
Abnormality Location Distinguishing feature

CCAM U/L (2-3%- B/L) Cystic & solid

Sequestration U/L (left LL-mc) Systemic blood supply

Congenital lobar U/L (UL- most often) Similar to microcystic

emphysema CCAM; enlarged
echogenic lung
with mediastinal shift,
prog lung expansion,
absence of internal linear
opacities

Congenital diaphragmatic U/L (left side more Peristalsis of bowel in

hernia common) chest, stomach above
diaphragm, absence of
part of the diaphragm
Computed tomography scan of a neonate . There is a solid soft
tissue density within the left lower lobe measuring approximately
3 cm 3 2 cm. A small vessel arising from the descending aorta is
seen supplying this solid mass (arrows); findings are consistent
with sequestration. The baby underwent resection of the mass at
3 months of age.
 D/D- Cystic lesion in fetal thorax

Abnormality B/L v/s U/L Distinguishing

features

CCAM U/L (2-3% - B/L) Associated with echogenic

lung mass, typically
multiple cysts

CDH Typically U/L Peristalsis of bowel in

chest, stomach above
diaphragm(abs st bubble)

Cystic Teratoma Mass does not obey lobar

boundaries; may have
calcifications.
Abnormality Distinguishing feature

Neurenteric cyst Adjacent to spine

Bronchogenic cyst Typically single cyst,

direct connection with
upper airway

Esophageal duplication Adjacent to esophagus

Lymphangioma Crosses anatomic

boundaries
Pulmonary Sequestration
• Bronchopulmonary sequestration (BPS) or sequestration is a rare
congenital abnormality of the lower respiratory tract.
• It consists of a non-functioning mass of lung tissue that lacks normal
communication with tracheobronchial tree and receives its arterial
blood supply from systemic circulation.
• Bronchopulmonary sequestrations constitute
approximately 0.15-6.4% of all congenital pulmonary
malformations.
• Anatomically classified as
- Intra lobar Sequestration (ILS) – M/C
- Extra lobar Sequestration (ELS)
• INTRALOBAR SEQUESTRATION (ILS) : - 75%
-Located within the normal lobe and does not have its
own visceral pleura.
• EXTRALOBAR SEQUESTRATION (ELS) : - 25%
-Located outside the normal lung and has its own visceral
pleura.
INTRALOBAR (75%)
Visceral pleura
Adults (recurrent pneumonia)
15% congenital anomalies
associated.
EXTRALOBAR (25%)
Own pleural covering
First six months
50% congenital anomalies associated
(diaphragmatic hernia, cardiac,
hybrid forms CPAM/sequestration)
• Rare types :-
-Hybrid BPS/CPAM lesions :- BPS occurs in combination
with CPAM. These hybrid lesions have histological features of
CPAM and blood supply from a systemic artery.
- Bronchopulmonary-foregut malformation (BPFM)-
A Rare variant of sequestration where sequestrated lung
tissue is connected to the gastrointestinal tract.
 PATHOGENESIS

• The most frequently supported theory of sequestration

involves formation of accessory lung bud that develops
from the ventral aspect of the primitive foregut.
• The pleuripotent tissue from this additional lung bud
migrates in a caudal direction with the normally developing
lung.
• The accessory lung bud receives its blood supply from
vessels that connect to the aorta and cover the
primitive foregut.
• These attachments to the aorta remain to form
the systemic arterial supply of the sequestration.
• Early embryologic development of the accessory lung bud in
pseudoglandular stage (5-17weeks of gestation) results in
formation of the sequestration within normal lung tissue.
This sequestration is encased within the same pleural
covering of normal lung resulting in the Intrapulmonary OR
Intralobar sequestration
• Later development of the accessory lung bud results in
the Extralobar OR Extrapulmonary type that may give rise
to communication with the GI tract.
INTRALOBAR SEQUESTRATION
• ILS are located within a normal lobe and lack their
own visceral pleura.
• Accounts for 75% of BPS.
• Males and females are equally affected.
• Most occur in lower lobes, 60% located in posterior
basal segment of left lower lobe.
• The right lower lobe may be affected in approximately
one- third of cases
• Generally do not have communication with
tracheobronchial tree.
• Intralobar sequestration usually present in late childhood or
in young adults.
• Arterial blood supply is by lower THORACIC AORTA or
upper Abdominal aorta and Venous drainage is usually via
the pulmonary veins
• Infrequently, in 10% - 15%
bronchopulmonary foregut malformations and
skeletal anomalies (like scoliosis and rib
anomalies) may be associated.
• Within visceral pleura
of normal lobe
• Arterial supply by
thoracic aorta
• Venous drainage into
Pulmonary veins
EXTRALOBAR SEQUESTRATION
• Extra pulmonary sequestrations are located outside
the normal lung and have their own visceral pleura,
with a pedicle that contains the vascular connections.
• Accounts for 25% of BPS.
• Male predominance.
• ELS are more common on the left side, m/c between the
left lower lobe and hemi diaphragm (80%).
• They may also be found within or below the diaphragm or
in retroperitoneum.
• They are associated with other congenital malformations
in more than 50% of cases.
• They may connect to the gastrointestinal tract or rarely
to intrapulmonary structures.
• As intrapulmonary connections are uncommon in
ELS, infectious complications are also uncommon.
• Extralobar sequestration is typically supplied by a systemic
artery arising directly from the THORACIC aorta. The
vessel is usually small with low flow.

• Its venous drainage is usually through azygos–

hemiazygos system.
• Accessory lung tissue
surrounded by its own pleura
• Arterial supply by thoracic
aorta
• Venous drainage into systemic
veins
PATHOLOGY
• On cut sections, the INTRALOBAR sequestration
comprises fibrotic and consolidated lung parenchyma
that frequently contains multiple cystic areas.
• The cysts may contain fluid, infected purulent material
or may be predominantly air filled – in which case, the
cysts have partial communication with the
tracheobronchial tree.
• Histologically, intralobar sequestration is characterized by
changes of chronic inflammation and fibrosis with cystic
changes.
• Superimposed changes of acute infection may be
present. The alveoli that border the sequestered lung may
be emphysematous.
Fig. 1. Postoperative histopathology of a patient with intralobar sequestration
(haematoxylin–eosin stain, 40 magnification). The alveolar spaces are
filled with haemorrhage, oedema and chronic inflammatory infiltrate. Few
thick-walled blood vessels and areas of bronchial dilatation are also seen.
PATHOLOG
Y
• EXTRALOBAR SEQUESTRATION is typically a single,
well circumscribed lesion covered by a mesothelial layer
(pleural envelope).
• Its surface may show a reticular pattern due to presence
of dilated subpleural lymphatics.
• On cut section the lesion is firm and homogenous.
• Microscopically, extralobar sequestration resembles normal
lung tissue, except for dilatation of bronchioles and
alveolar ducts and alveoli within the lesion.
• A well formed bronchus is identifiable in approximately
half the cases, typically located at one edge of the lesion.
CLINICAL PRESENTATION
• The clinical features of BPS are variable and depends
upon the type, size and location of the lesion.
• Most infants are asymptomatic at birth.
• If symptomatic, may present with respiratory distress at
birth or in the neonatal period, usually due to large lesions
that limit the volume of normal lung.
• The most common symptomatic presentation after neonatal
period is with pulmonary infection, typically presenting as
fever and cough, sometimes hemoptysis or chest pain, in
pts with ILS.
• Pts with ELS unlikely to develop infection.
• Rarely may present with heart failure due to excessive
flow through aberrant artery.
ASSOCIATED ANOMALIES
• More than half ( 50–65%) of patients with extralobar
sequestration have associated congenital anomalies, the most
common congenital diaphragmatic hernia seen in 20–
30%.
• Various other anomalies have been reported in
association including bronchogenic cyst, foregut
duplication, ectopic pancreas and vertebral anomalies.
• Infants with large BPS may have pulmonary hypoplasia due
to mass effects in utero.
 EVALUATION
PRENATAL :
-on prenatal ultrasound, BPS appears as homogenous
echogenic thoracic mass, usually solid appearing,
triangular and often located in lower hemithorax adjacent
to the diaphragm.
POSTNATAL :
- First step is chest radiograph.
• CHEST RADIOGRAPH :
-Sequestrations typically appear as a uniform dense
mass within the thoracic cavity or lung parenchyma.
-recurrent infections in ILS can lead to cystic areas within
the mass.
- Air-fluid levels in case of bronchial communication.
a) shows a cavitary lesion in right lower zone, in
the retrocardiac location (arrows).
• Computed tomography (CT) :
-Most common appearance is a solid mass that may be
homogenous or heterogenous, sometimes with cystic
changes.
-Less frequently, a large cavitary lesion with an air-fluid
level, a collection of many small cystic lesions with air or
fluid.
-Emphysematous changes at the margin of the lesion ar
characteristic.
The CTaxial image in lung window (b) shows a
large thin-walled cavity with multiple
septations and an air-fluid level in the medical
basal segment of right lower lobe (arrow).
The CT shows presence of a large abnormal artery arising from the
thoracic descending aorta (c) and supplying a portion of the left lower lobe
including the posterior basal segment. Lung window (d)
shows an area of emphysema in the left lower
lobe, around the abnormal vessel.
A 31-year-old man presented with recurrent
chest infection. Chest radiograph shows a subtle nodular opacity in the right lower
zone (arrow in a). Axial CT sections (b,c) show a aberrant systemic artery arising
from the descending thoracic aorta supplying a sequestered segment in right
lower lobe.
 MRI
• This is a safe and non-invasive alternative imaging
method, which may be useful.
• MRI provides an excellent observation of the
supplying systemic arterial anatomy.
• MR imaging can also depict the pulmonary venous return
of the lesion and the relationship of the draining vein to the
cardiac chambers.
 ANGIOGRAPHY
• Traditionally, Angiography has been considered as the gold
standard for diagnosis of pulmonary sequestration by
virtue of its excellent depiction of the arterial and venous
anatomy but now it is replaced by
multidetector CT or MRI angiography
• Invasive catheter angiography may be helpful as a
diagnostic procedure in those select cases where the clinical
and radiological suspicion for sequestration is high, but a
supplying systemic vessel cannot be shown using CT or MR
angiographic techniques.
DSA of the patient shows blush within the sequestered lung segment
on the descending thoracic
aorta run (arrow in f). On selective cannulation of the supplying artery
arising from the thoracic aorta (g), the aberrant vessel is better seen
CT ANGIOGRAPHY
• Multidetector CT angiography has emerged as the imaging
technique of choice for preoperative evaluation of
pulmonary sequestration in both the paediatric and in the
adult population.
• Computed tomography has the advantage of being able to
show the pulmonary parenchymal abnormality as well as
the arterial and venous anatomy all in a single examination.
Computed tomographic angiography images of a 7-year-old male show the origin of an
aberrant systemic artery from the aorta (single arrow in a) supplying a sequestered
segment in the right lower lobe and venous drainage of the lesion through the
pulmonary vein into left atrium (double arrows in b) –classical features of intralobar
sequestration
CT sagital view showing
a feeding artery arising
from the aorta and
irrigating the pulmonary
mass
Maximum intensity projection (a) and surface-shaded display (b) images
of the patient shown in Figure 4; the aberrant vessel (arrow) is
seen arising from the descending thoracic
aorta.
Contrast-enhanced MR angiography maximum intensity projection
image (c) also shows the abnormal systemic artery supplying the
left lower lobe (arrow) with venous drainage into the left
hemiazygos system (arrow in d).
NUCLEAR SCAN
• Radionuclide angiography is a non-invasive technique
that has been used to show the systemic arterial supply to
a pulmonary sequestration.
• The sequestration may be seen as an area of lung having a
relative lack of perfusion during the pulmonary arterial
phase followed by perfusion during the systemic phase
99Tc pertechnetate scan of the patient shown in Figure 9. An aberrant vessel is seen running
parallel to the descending aorta in the blood pool phase of 99Tc pertechnetate scan (arrow
in a). Perfusion scan shows a defect in the posteromedialbasal segment of right lower lobe
(b).
PROTOCOL FOR EVALUATION
• Immediate advanced imaging with CT/MRI for pts with high risk :
- Any symptoms (respiratory distress)
- Large BPS ( >20% of lobe involved)
-B/l or multifocal cysts, pneumothorax or a pleuropulmonary
blastoma
• The purpose of the advanced imaging is to make the diagnosis and
identify the aberrant artery and to help with surgical planning.
• Advanced imaging may help to distinguish among ILS,
ELS and hybrid lesions, but is not completely reliable in
making these distinctions.
• The final definitive diagnosis is made only by
PATHOLOGICAL EXAMINATION after
surgical resection.
DIFFERENTIAL DIAGNOSIS
• Congenital pulmonary airway malformations (CPAM)
- CPAM can be differentiated from BPS by the presence of
connection to tracheobronchial tree and blood supply from
pulmonary circulation.
SUMMARY
• Bronchopulmonary Sequestration is a rare
congenital abnormality of lower respiratory tract.
• It consists of a non-functioning mass of lung tissue that
lacks normal communication with tracheobronchial tree and
receives it blood supply from the systemic circulation.
• An intralobar sequestration (ILS) is located within a normal
lobe and lacks its own visceral pleura whereas An
extralobar sequestration (ELS) is located outside the
normal lung with its own visceral pleura.
• Clinical presentation depends on type, size and location of the
lesion.
• BPS can be detected by prenatal ultrasound & they may regress
during gestation in most cases or may progress.
• The affected newborn is usually asymptomatic, sometimes may
present with respiratory distress.
• On chest x-ray, typically appears as a uniformly dense mass
within the pulmonary parenchyma. Recurrent infections can lead
to cystic areas within the mass, and air-fluid levels if
communicates with a bronchus.
• Infants presenting with respiratory distress are treated
with surgical excision.
• For asymptomatic pts of any age with high risk for
developing complications, surgical resection rather
than observation.
• Without high risk characteristics, conservative management
with observation.
CONGENITAL LOBAR EMPHYSEMA
• Congenital lobar emphysema, is a congenital lung
abnormality that results in progressive overinflation of one
or more lobes of a neonate's lung.

• On imaging, it classically presents on chest radiographs as a

hyperlucent lung segment with overinflation and
contralateral mediastinal shift.
• The incidence of CLE is possibly underestimated, varies between
1:20,000 and 1:30,000 births.
• The male to female ratio is 3:1
• The left upper lobe and the middle lobe are the usual sites, the lower
lobes are rarely affected.
• Respiratory impairment usually occurs in the first few days of life, and
90 % of cases occur before the age of 6 months
Etiology
• Intrinsic airway lumen narrowing due to dysplastic bronchial
cartilage or extrinsic airway lumen narrowing leading to
congenital ball valve effect and hyperinflated lobe
Radiographic features

Predilection for certain lobes:

• left upper lobe: most common, 40-45%

• right middle lobe: 30%
• right upper lobe: 20%
• involving more than a single lobe: 5%
• much rarer in the lower lobes
• CXR on day 1 (above) shows a semi-solid
mass in the right middle lobe obscuring
the right heart border. CXR on day 5
(below) shows the right middle lobe mass
has now cleared of fluid and has filled
with air and is causing mediastinal shift
to the left.
•Congenital lobar emphysema. 7-month-old girl. a) Chest
x-ray. Radiolucency and hypovascularity in the right
pulmonary field. b) CT scan of chest, axial, pulmonary
window. Hyperinflation of the right upper lobe with
contralateral displacement of the mediastinal structures
and passive ate- lectasis of the left pulmonary
parenchyma.
• (a) Axial US at 27 weeks’
gestation demonstrates a large
echogenic mass in the right
hemithorax deviating the heart
to the left. No systemic feeding
vessel was identified.
• (b) Coronal and (c) sagittal
SSFSE T2w MR image at
27 weeks gestation confirms
the presence of a high-signal
mass in the right lower lobe.
• (d) Axial chest CT scan at
5 months of age demonstrates
an emphysematous lobe
consistent with CLH
• Hyperexpanded right
hemithorax compared to the left
hemithorax
Gross pathological
image

• A Distended and
emphysematous right upper
lobe with focal area of
hemorrhage and subpleural
bullae
Histopathological
image
• Dilated but normally formed
alveoli (left) separated by less
expanded alveoli in a small
adjacent lobe (right)
Bronchogenic Cyst
• Bronchogenic cysts (BC) are congenital lesions.
• They are thought to originate from the primitive ventral anterior
bowel and may be mediastinal intrapulmonary.
• Approximately two-thirds are within the mediastinum and one-third
are intraparenchymal.
• The mediastinal location can be paratracheal (generally, on the right
side), carinal, the most frequent, or hiliar. They must be differentiated
from cystic teratomas, thymic cysts or ectopic thyroid glands
•Bronchogenic cyst. 1-year-old girl. a and b) Chest
CT, axial and coronal reconstruction in mediastinal
window. Subcarinal mediastinal mass, cystic, thin-
walled, without enhancement.
Pleuropulmonary Blastoma
• Pleuropulmonary blastoma (PPB) is a rare primary malignant pulmo-
nary tumor that occurs exclusively in pediatric age, especially in
children younger than 6 years of age.
• It belongs to a unique category of tumors observed exclusively in
childhood: dysembriogenic neoplasms, analogous to Wilms’ tumor,
neuroblastoma and hepatoblastoma.
• It is usually located on the periphery of the lung, but may be
extrapulmonary, and affects the parietal pleura, mediastinum, great
thoracic vessels, regional lymph nodes, and diaphragm.
• PPB is of mesodermal origin, classified by Dehner and collaborators in
1994 in three histological types:
• Cystic (type I) the most frequent, affects children under 10 months;
• Mixed (type II) with solid-cystic component, and
• Solid (type III), of poor prognosis, affecting people over 4 years of
age.
• Pleuropulmonary blastoma. 3-year-old girl. a and b) CT scan of chest, coronal,
mediastinal and pulmonary window. Solid-looking mass, lobed contours and
heterogeneous density in the posterior segment of the right upper lobe.
• A type III PPB
Scimitar Syndrome
• Scimitar syndrome is characterized by hypoplasia of the right lung,
dextroposition of the heart, and abnormal drainage of the pulmonary
vein in the inferior vena cava, which produces a curved vascular
shadow along the right edge of the heart.
• The partial anomalous venous connection of the pulmonary vein drains
systemic veins, inferior vena cava and, less commonly, the portal vein,
hepatic veins, right atrium
Three forms of scimitar syndrome

• In the infantile form there is a large shunt between the abnormal artery
that irrigates the lower lobe of the right lung and the subdiaphragmatic
aorta; this is sometimes called hijacking.
• In the adult form there is a small short circuit between the right
pulmonary veins and the inferior vena cava.
• The third form is associated with cardiac and extracardiac
malformations.
Salient Radiographic Features of Scimitar
Syndrome

Enlarged curved vascular structure coursing medially toward the right diaphragm:
This structure enlarges in diameter as it approaches the diaphragm.

Dextroposition of the heart

Hypoplasia of right lung

Figure 8. Scimitar syndrome. 6-year-old girl. Chest CT scan,
coronal MIP reconstruction. Tubular structure in the right
pulmonary field corresponding to anomalous venous drainage
of the right lower lobe.
Congenital Tracheal Stenosis
• The three patterns of congenital tracheal stenosis are
• Diffuse or generalized hypoplasia;
• Diffuse or funnel-like stenosis may be associated with absence of the
posterior membranous wall due to complete ringlike tracheal cartilages.
Patients with complete tracheal rings usually present with respiratory distress
in the first few weeks of life, and this condition is associated with a high
mortality rate. Rarely, the diagnosis is delayed until adulthood
• Funnel-like stenosis (i.e., carrot-shaped trachea), often associated
with an anomalous origin of the left pulmonary artery;
• Segmental stenosis.
• Congenital tracheal stenosis. External three-dimensional
rendering of trachea demonstrates smooth stenosis (white
arrows) of the lower trachea. Round protuberance (black
arrow) above the level of stenosis represents a tracheal
diverticulum viewed en face.
Tracheomalacia
• Tracheomalacia is abnormal collapsibility of the trachea, which is caused by softness or
pliability of the tracheal cartilages.
• It may be primary, associated with a localized absence of the tracheal cartilage, or
secondary, resulting from external compression, such as from an extrinsic mass.
• Tracheomalacia must be differentiated from excessive collapse, which is caused by
abnormal expiratory pressures. For example, collapse of a long segment of the intrathoracic
trachea may occur in late expiration in patients with asthma, chronic bronchitis, and
bronchiolitis.
• Paired inspiratory and dynamic expiratory computed tomography (CT) using state-of-the-art
multidetector-row CT scanners is excellent for evaluating tracheomalacia.
• The accuracy of this technique is comparable to that of bronchoscopy, the gold standard for
diagnosing this condition. A greater than 50% collapse of the tracheal diameter is
considered abnormal, but healthy individuals may sometimes exceed this threshold.
Tracheomalacia is shown on paired inspiratory and dynamic
expiratory axial CT images. A, On inspiration, the trachea is of
normal caliber. B, On expiration, excessive collapsibility of the
trachea can be seen
Congenital Tracheobronchomegaly
• Tracheobronchomegaly (i.e., Mounier-Kuhn syndrome) is characterized by loud,
prolonged chronic cough with ineffective secretions and recurrent bronchitis or
pneumonia.
• Imaging of the trachea demonstrates absence or atrophy of elastic fibers and
thinning of muscle; airway dynamics are abnormal with dilation on inspiration and
collapse on expiration.
• There are frequent saccular bulgings of the intercartilaginous membranes. The
diagnosis can be established by chest radiography when the transverse diameter
of the trachea is greater than 25 mm and the diameters of the right and left main
bronchi are greater than 23 and 20 mm, respectively .
• CT can confirm the increased diameter of the trachea. Frequently, there is
bronchiectasis in the lung parenchyma.
Congenital
tracheobronchomegaly
(Mounier-Kuhn
syndrome)

• A, The posteroanterior chest

radiograph shows marked
tracheal dilation (43 mm)
(arrows) and bronchiectasis in
the right lung.
• B, CT confirms the large tracheal
lumen. C, The dilation extends
into the main bronchi, which
exhibit saccular bulging of their
walls (arrow).
Aberrant Trachiel Bronchus
• Rarely, the right upper lobe bronchus or a segment of the right upper
lobe bronchus may originate in the trachea (i.e., tracheal or pre-
eparterial bronchus).
• This is usually of no clinical consequence. However, in a minority of
affected patients, impaired drainage may result in recurrent infections
• After endotracheal intubation, the balloon may inadvertently
obstruct such a bronchus, causing right upper lobe atelectasis.
• Tracheal bronchus. A, Axial CT shows the anomalous origin of the apical segment of the right upper
lobe bronchus directly from the trachea, with an obstructed lumen (arrow) and postobstructive
pneumonia.

• B, External three-dimensional rendering of the airways shows the anomalous origin of the right
upper lobe apical segment bronchus (arrow) from the trachea.
Tracheoesophageal Fistula
• Congenital tracheoesophageal fistula is invariably a pediatric disease. It occurs
in newborns and is most frequently associated with esophageal atresia.
• However, about 3% of all tracheoesophageal fistulas occur with an otherwise
normal esophagus, and patients may present in these instances in adult life.
• Roughly 75% show communication with the trachea, and the others
communicate with the major bronchi.
• Patients usually have a history of recurrent pneumonias. The chest
roentgenogram may show evidence of bronchiectasis. The diagnosis can be
confirmed by a contrast esophagogram, which often can identify the fistula
and show evidence of contrast material within the tracheobronchial tree and
the lung.
Congenital bronchoesophageal fistula
in a 24-year-old woman with a
history of repeated pneumonias
since childhood. A, The
posteroanterior chest radiograph
shows patchy pneumonia in the
right upper lobe and right base,
with right hilar and paratracheal
adenopathy caused by repeated
infections. B, The barium
esophagogram demonstrates a
fistula between the lower
esophagus and a branch of the
right lower-lobe bronchus
(arrows).
Bronchial Atresia
• This anomaly consists of atresia of a lobar or segmental bronchus with obliteration of the
lumen and preservation of distal structures.
• The most common site is the left upper lobe, particularly the apical posterior segment. The
right middle and upper lobes are less common sites.
• Mucus secreted within the airways distal to the atretic segment cannot pass the stenosis and
accumulates as a mucous plug or mucocele. Collateral air drift keeps the lobe or segment
inflated, and it becomes hyperinflated as a result of expiratory air trapping.
• The chest radiograph shows an area of hyperlucency in the affected portion of the lung . The
mucocele appears as an ovoid or branching structure at the hilar level. CT demonstrates the
mucoid impaction at the site of obstruction, which is associated with lobar or segmental
hyperinflation
• . There may be an accompanying shift of the mediastinum and compression of the
surrounding lung.
 Bronchial atresia. The
anteroposterior radiograph
shows an overinflated left
upper lobe with a slight
mediastinal shift. The left
perihilar opacity represents
mucoid impaction distal to the
atresia (arrow).
• Figure 2-7 Bronchial
atresia. Axial CT shows a
site of mucoid
impaction (arrow) distal
to the atretic left upper
lobe’s apical posterior
segmental bronchus
and surrounding
hyperlucency in the left
upper lobe.
Congenital Bronchiectasis
• Congenital bronchiectasis (i.e., Williams-Campbell syndrome) is rare,
and its existence is controversial. It results from an intrinsic
abnormality of cartilage.
• The cartilaginous deficiency occurs within the fourth- to sixth-order
bronchi and is manifested by cystic bronchiectasis and pulmonary
hyperinflation.
• Bronchiectasis, which is acquired and caused by chronic infection,
may occur early in life as a result of other congenital, developmental,
or genetic disorders.
Williams-
Campbell
syndrome. The
posteroanterior
(A) and lateral (B)
views of a
bronchogram of
the right lung
shows diffuse
cystic
bronchiectasis
involving all lobes.
Congenital Hydrothorax
• Chylothorax
• Secondary Hydrothorax
• Secondary cases of hydrothorax include entities such as anemia, CPAM, BPS,
lymphangiectasia, cardiac anomalies, Turner’s syndrome, trisomy 21, cystic
hygroma, and TORCH infection
Chylothorax
• Chylothorax is a rare but serious condition in which lymph
formed in the digestive system (chyle) accumulates in your
chest cavity. Lymph is a fluid containing white blood cells and
proteins that moves through your lymphatic system and drains
into your bloodstream.
Chylous effusion. (a) Coronal US of the chest demonstrates a large fluid collection in the left hemithorax
compressing the left lung. (b) SSFSE coronal MR image confirms the presence of a large left pleural effusion
with compressed lung parenchyma and no underlying lung mass
Congenital Lung Tumors
• Cystic Pleuropulmonary Blastoma (Cystic PPB)
• Fetal Lung Interstitial Tumor (FLIT)
• Congenital peribronchial myofibroblastic Tumor (CPMT)
• Congenital Fibrosarcoma
FLIT
Fetal lung interstitial tumor (FLIT). (a) Axial US image
at 27 weeks gestation demonstrates an echogenic
mass (arrow) deviated the heart to the right. (b)
Coronal T2w MR image on the same date confirms the
presence of a heterogeneous mass compressing the
diaphragm inferiorly and shifting the mediastinum to
the right. This was thought to be a CPAM prenatally,
but the mass did not involute in the third trimester. (c)
Following delivery, the infant was in respiratory
distress. Coronal reformatted image of the chest
demonstrates pulmonary vessels coursing through a
solid-appearing mass. The mass was resected, and
FLIT was confirmed
Pulmonary Agenesis
• Pulmonary agenesis is a condition with total absence of the lung
parenchyma and the vessels and bronchi distal to the carina.
• Patients with pulmonary aplasia have a rudimentary bronchus, which ends
in a blind pouch without lung tissue or pulmonary vasculature. A decrease in
the number or size of airways, vessels, and alveoli characterizes pulmonary
hypoplasia.
• The lesion may be primary, or it may result from several pathogenetic
mechanisms that may occur during gestation.
• These conditions include decreased pulmonary vascular perfusion,
oligohydramnios, or compression of the lung by a space-occupying mass
within the pleural cavity (e.g., congenital diaphragmatic hernia)
A, PA radiograph of a 16 year-old girl shows complete absence of the right lung and pronounced mediastinal shift
and overinflation of the left lung. B, Coronal multiplanar reformation of a CT angiogram demonstrates absence of
the right lung and marked shift of mediastinal structures into the right hemithorax, which is reduced in size
compared with the left.
Right lung agenesis. (a) Coronal SSFSE and (b) FIESTA T2w images at 21 weeks’ gestation demonstrate shift
of the heart to the right with no right lung parenchyma or right mainstem bronchus identified. (c) Contrast
chest CT after delivery confirms the diagnosis of agenesis of the right lung
Pulmonary Hypoplasia
• Pulmonary hypoplasia is a wide spectrum diagnosis which includes
agenesis, aplasia, as well as hypoplasia.
• Agenesis is the actual absence of lung parenchyma and bronchi
• Aplasia is the absence of lung tissue with rudimentary bronchi.
• Hypoplasia is the presence of alveoli and bronchi that are
underdeveloped with a decreased number of airways and alveoli
resulting in a decrease in size and weight of the lungs. Alveoli and
pulmonary vascularity develop concomitantly, so associated
anomalies of pulmonary vessels are common
Severe pulmonary hypoplasia. A, The posteroanterior chest
radiograph suggests right lung agenesis. B, CT demonstrates
rudimentary lung tissue at the right base that contains
pulmonary vessels (arrow).
Pulmonary hypoplasia
•Coronal SSFSE MR image of the fetal chest
demonstrates small low-signal pulmonary
parenchyma in this fetus with renal anomalies
resulting in oligohydramnios
Pectus Excavatum
• Pectus excavatum, also known as funnel chest or trich-terbrust, is the
most common congenital deformity of the sternum.
• Its incidence is approximately 1 in 400 births, afflicting males more
than females
• A 24-year-old female patient being considered for surgical correction of pectus excavatum. Three
consecutive radiographsdemonstrate subsequent corrective surgeries.
• (a) Pre-operative chest radiograph.
• (b) The patient initially underwent a Nussprocedure. Note the post-operative right-sided
pneumothorax and subcutaneous surgical emphysema.
• (c) She subsequentlyneeded the Nuss bar removed, which was then exchanged for a Ravitch bar
• a) Pre-operative axial CT of the same 24-year-old patient demonstrating
pectus excavatum. Note the sternal depressionand tilting, with leftward
displacement of the heart. (b) Sagittal reconstruction
Pectus Carinatum
• Pectus carinatum is colloquially referred to as “pigeon chest”.Itis the
second most common chest wall congenital deformity,although much
less common than pectus excavatum.
• Its incidence varies in literature, approximately 1 in 1000 with a
predilection for males.
Axial and sagittal CT reconstructions of the samepatient with
pectus carinatum
Pectus Arcuatum
• Pectus arcuatum, or “wave-like chest”, is a rare condition with an
unknown etiology.
• The term is used to describe mixed deformities which contain both
excavatum and carinatum either along the longitudinal or axial axis
and is also known as a pouter pigeon chest
• A 24-year-old female patient with mixed deformity consisting of both
pectus excavatum and carinatum, creating “rolling” appearance.
Poland Syndrom
• Poland syndrome is a non-genetic congenital abnormality and the
etiology is unknown. The most popular theory is that it is due to
hypoplasia of the subclavian artery.
• It occurs in 1 : 7000 to 1 : 100,000 live births.6It is characterized by
partial or total absence of the pectoral muscles and is most
commonly.
Figure 17. Maximum
intensity projection
image of the
samepatient now
demonstrating Poland’s
syndrome. In addition
tothe absence of right
sided pectoral muscles,
there is alsoabnormal
sternal protrusion
Chest Wall Hypoplasia
• There is a wide range of chest wall hypoplasia due to thoracospinal
growth asymmetry and developmental arrest, which causes spinal
scoliosis when focal. Many conditions are eponymously named and are
associated with a range of cardiac defects and distal limb deformities.
With advancements in genetics, there is now a better understanding of
the pattern of inheritance with these conditions. An example of such an
eponymous condition is Jeune syndrome , otherwise known as
asphyxiating thoracic dystrophy.
• It is a very rare symmetrical deformity, recessively inherited with an
incidence of between 1 in 100,000 and 130,000 live births.8 Patients
have either a uniformly narrow thorax or a bell-shaped thorax
• Three-dimensional reconstruction of the bell-shaped thorax of a neonate
with Jeune syndrome
Terima kasih