congenital lung.pptx

Congenital pulmonary malformations
can be classified in several ways.
A simple classification considers two
groups:
(1) those usually unassociated
with abnormal vessels
(2) those associated with or
consisting of abnormal vessels

TRACHEAL BRONCHUS
 Tracheal bronchus is sometimes referred to as a “pig bronchus” or “bronchus suis” as it is common in pigs and other
cloven-hoofed animals
arises from the right tracheal wall, at or within 2 cm of the tracheal bifurcation
supplies a variable portion of the medial or apical right upper lobe, most often the apical segment
recurrent infection or bronchiectasis may result, since the tracheal bronchus is often slightly narrowed at its origin
narrowed at its origin
Rarely, the entire right upper lobe bronchus arises from the trachea When a right-sided tracheal bronchus is present, the
tracheal bronchus is present, the azygos arch is seen above the tracheal bronchus..
They can be classified into two main types:
 supernumerary: usual bronchial supply to affected lung segment is concurrently present
present
 displaced: usual bronchial supply to affected lung segment is concurrently absent

RADIOGRAPHIC FEATURES - CT
CT is the best modality for assessing the
anatomy and allows direct visualization
and orientation of the anomalous
bronchus.
Coronal multi-planar reconstructions in
"lung window" settings are the most
helpful and is best in depicting this
anomaly.

Coronal CT reconstruction shows a tracheal bronchus (T
Br) arising from the right tracheal wall just above the
carina. The azygos arch (Azygos) is visible above the
bronchus.

CT (1.25-mm slice thickness) shows the origin of the tracheal
bronchus. As in this case, slight narrowing at the origin of the bronchus is
common. The bronchus supplies the apical segment of the right upper lobe

ACCESSORY CARDIAC
BRONCHUS
arises from the medial wall of the bronchus intermedius or right lower lobe bronchus and
extends inferiorly and medially toward the mediastinum or heart
some cases, the cardiac bronchus is a short, blind-ending bronchial stump without associated
alveolar tissue, and it may terminate in the mediastinum
others, a longer branching bronchus is present, associated with rudimentary lung tissue
most cases, this anomaly is an incidental finding; occasionally, chronic infection or hemoptysis is
associated

Coronal CT reconstruction shows an
accessory cardiac bronchus (CB) arising
from the medial aspect of the bronchus
intermedius (BI), to supply a small region
of lung.

Transaxial image shows the cardiac bronchus
(CB) arising from the medial wall of the
bronchus intermedius (BI).

Below (C), the bronchus supplies a small
segment of lung separated from the lower
lobe by an accessory fissure (arrows).

BRONCHIAL ISOMERISM
Bronchial isomerism refers to bilateral symmetry of the bronchi and associated
pulmonary lobes.
It may be isolated or associated with a variety of anomalies, particularly congenital heart
disease.
Bronchial anatomy may be bilaterally right sided (associated with asplenia) or left sided
(associated with polysplenia).

BRONCHIAL ATRESIA
 developmental defect characterized by local narrowing or obliteration of a lobar, segmental, or
subsegmental bronchus
 most common in the left upper lobe, followed by the right upper and right middle lobes; it is less
common in the lower lobes
 usually detected incidentally in adults and is undoubtedly related to some cases of congenital lobar
overinflation (CLO)
 usually have no symptoms, but lung distal to the obstruction may occasionally become infected
 patients with chronic infection, resection may be necessary.

 lobe or segment distal to the bronchial obstruction usually remains aerated because of collateral
ventilation
 air trapping in the distal lung results in decreased perfusion; radiographs and CT show the affected
lung to be hyperlucent and hypovascular
 Affected lung is often increased in volume, resulting in mediastinal shift or shift of a fissure.
mucus accumulates within dilated bronchi distal to the obstruction, resulting in a tubular, branching,
in a tubular, branching, or ovoid density (mucous plug or mucocele) - Mucus within dilated bronchi usually
appears low in attenuation
 Expiratory radiographs or CT scans show air trapping

Chest radiograph in a young patient with
bronchial atresia involving the left
upper lobe.
The left upper lobe is increased in
volume, with mediastinal shift toward
the right side. The lobe is lucent, and
its vascularity is decreased

Transaxial CT shows the left upper
lobe to be increased in volume, with
mediastinal shift to the right,
hyperlucent and hypovascular.
Branches of the left upper lobe
bronchus are dilated, and one branch
contains a mucous plug (arrows).

Coronal reconstruction shows the hyperlucent
and hypovascular left upper lobe with focal
atresia of its apical-posterior segmental
branch (arrow). Distal bronchi are dilated.

A: CT shows a branching mucous plug
(white arrow) in the location of the posterior
segmental bronchus of the right upper lobe.
Lung distal to the bronchus (black arrows) is
hyperlucent and hypovascular. Mediastinal
shift to the left side is
present.
B: Expiratory CT shows air trapping in the
lung distal to the obstructed bronchus.
Normal lung increases significantly in
attenuation relative to (A).

CONGENITAL LOBAR OVERINFLATION
Congenital lobar overinflation (CLO), aka congenital lobar emphysema, is a congenital lung
abnormality that results in progressive overinflation of one or more lobes of a neonate's
lung.
PATHOLOGY :
A lobe (or more) becomes distended and may or may not have an overabundance of alveoli.
Associated with partial or complete bronchial obstruction occurring as a result of
(a) deficient cartilage;
(b) external compression, usually by an anomalous vessel or bronchogenic
cyst; or
(c) luminal abnormalities such as mucosal folds
- Some cases are unassociated with bronchial obstruction.

LOCATION :
◦ -Left upper lobe : most common 40-45%
◦ -Right middle lobe : 30%
◦ -Right upper lobe : 20%
◦ -May involve more than a single lobe in 5%
◦ -Much rarer in the lower lobes
◦ -Therefore despite the left upper lobe being most commonly affected , the right
side to be affected
CLINICAL PICTURE :
◦ Most cases present within the first month of life; symptoms of respiratory distress are
overinflated lobe compresses normal lung.

PLAIN RADIOGRAPHY
a) IMMEDIATE POSTPARTUM PERIOD :
◦ -The affected lobe tends to appear opaque and homogeneous because of fetal
lung fluid or it may show a diffuse reticular pattern that represents distended
lymphatic channels filled with fetal lung fluid
b) LATER FINDINGS :
◦ -Appears as an area of hyperlucency in the lung with oligemia ( paucity of vessels )
◦ -Mass effect with mediastinal shift and hemidiaphragmatic depression
◦ -Lateral film may demonstrate posterior displacement of the heart

Chest radiographs demonstrates increased translucency of the left lung with mild
mediastinal shift to the right side , on the lateral view the hyperlucency is confined to the
left upper lobe, shifting the oblique fissure posteroinferiorly

Hyperexpansion of the left upper
lobe with a portion herniated
midline. There is shift of the
mediastinum to the right,
left hemidiaphragm and some
compression of the left lower
lobe.

CT
-Hyperlucent lobe (hallmark)
•attenuation of vascular structures in affected lobe
•may also show compressive atelectasis of adjacent lobes
•As Plain Radiography

BRONCHOGENIC CYST
Bronchogenic cysts are congenital malformations of the bronchial tree, present as a
mediastinal mass that may enlarge and cause local compression.
PATHOLOGY :
◦ Abnormal budding of the bronchial tree during embryogenesis (between 4th – 6th
weeks) and are lined by secretory respiratory epithelium (cuboid or columnar ciliated
epithelium)
◦ Do not communicate with the bronchial tree ,therefore typically not air-filled. Rather,
they contain fluid (water), variable amounts of proteinaceous material, blood
products, and calcium oxalate . It is the latter three components that result in increased
attenuation mimicking solid lesions. They are rarely multiple.

Bronchogenic cysts may be mediastinal or pulmonary. Mediastinal
bronchogenic cysts are much more common than pulmonary cysts
LOCATION :
-The most common location is the middle mediastinum
-subcarinal , right paratracheal and hilar locations most common
-Pulmonary bronchogenic cysts are most common in the medial lung and the
lower lobes
CLINICAL PICTURE :
-In many instances ,are asymptomatic and are found incidentally
-When large , mass effect may result in bronchial obstruction leading to air trapping
and respiratory distress

Cysts may slowly increase in size; a rapid increase in size is unusual
unless infection occurs. - the outer cyst wall may become less well defined
because of surrounding lung inflammation.
During or after infection, a cyst may contain air or a combination of air
and fluid (withan air-fluid level).

PLAIN RADIOGRAPHY :
-Findings are nonspecific , mediastinal masses should be
evaluated further using CT scanning or MRI to confirm the
presence of fluid
-The cysts usually appear as soft-tissue density rounded
structures
As the cysts may contain calcium oxalate, dependent
layering of calcific density material (milk of calcium) may
The chest X-ray showed a non calcified
homogeneous opacity lateral to the trachea with a
well defined interface with the lung ,

(a) PA chest radiograph demonstrates a subcarinal
abnormality with increased opacity (*) , splaying of
the carina and abnormal convexity of the upper
and middle thirds of the azygoesophageal line
(arrowheads)
(b) Corresponding CT scan helps confirm a subcarinal
mass (arrow) which proved to be a bronchogenic cyst

CT
-well circumscribed spherical or ovoid masses
of variable attenuation
-Approximately 50% are fluid density (0-20 HU) ,
however a significant proportion are of soft tissue
density (>30 HU) or even hyperdense to
surrounding mediastinal soft tissues
-CT is better able to detect calcium oxalate (milk
of calcium) layering dependently
-Usually no solid contrast enhancement
CT+C : well circumscribed unilocular water
attenuation cyst in the middle mediastinum , the cyst
has smooth imperceptible walls with no enhancement

CT with and without contrast media showed a large homogeneous mass of low attenuation
arising in the right paratracheal space extending posteriorly , thin wall and absence of
enhancement suggested the diagnosis of Bronchogenic cyst

Pulmonary bronchogenic cyst.
A sharply marginated round nodular
opacity (arrow) is visible in the right lower
lobe.
This measured 0 HU in attenuation.
This appearance is typical of a fluid-filled
bronchogenic cyst.

Pulmonary bronchogenic cyst.
A thin-walled, sharply marginated, air-
filled
bronchogenic cyst is visible in the left
lung. The presence of air within the cyst
indicates prior infection.

CONGENITAL PULMONARY AIRWAY
MALFORMATION (CONGENITAL CYSTIC
ADENOMATOID MALFORMATION)
CPAM are multicystic masses of segmental lung tissue with abnormal bronchial proliferation
PATHOLOGY :
failure of normal broncho-alveolar development with hamartomatous proliferation of
terminal respiratory units in a gland-like pattern (adenomatoid) without proper alveolar
formation
About 70% present during the first week of life, but 10% are diagnosed after the first year,
been reported.
Lower lobes are most often involved, but any lobe can be affected. CPAM/CCAM can occupy
an entire lobe.
CPAM/CCAM communicates with the bronchial tree and is supplied by the pulmonary artery;

SUBTYPES
Five subtypes are currently classified, mainly according to cyst size:
• TYPE I
• most common
• large cysts - one or more dominant cysts: 2-10 cm in size
• may be surrounded by smaller cysts
• TYPE II
• cysts are <2 cm in diameter
• associated with other abnormalities
• Can be solid
• renal agenesis or dysgenesis
• pulmonary sequestration
• congenital cardiac anomalies

•TYPE III
• microcysts: <5 mm in diameter - typically involves an entire lobe
• has a poorer prognosis
•TYPE IV
• unlined cyst
• typically affects a single lobe - indistinguishable from type I on
•TYPE 0
• very rare, lethal postnatally
• acinar dysgenesis or dysplasia
• represents global arrest of lung development

In infancy, CCAM/CPAM presents as a space-occupying lesion.
Symptoms of respiratory distress are common.
In neonates, CCAM/CPAM usually presents as a solid mass regardless of its type. Types 1 and 2 may
become air filled over a period of days to weeks. They are often associated with progressive air trapping and
mediastinal shift to the opposite side.
In adults, CCAM/CPAM usually presents as an air-filled or air- and fluid-filled cystic or multicystic mass.
Most adults present with recurrent pneumonia, although recurrent pneumothorax has also been associated.
Occasionally, adenocarcinoma may arise in relation to a CCAM/CPAM.

ANTENATAL ULTRASOUND
These lesions appear as an isolated cystic or
solid intrathoracic mass
There can be mass effect where the heart may
appear displaced to the opposite side
Hydrops fetalis or polyhydramnios may develop
Longitudinal prenatal sonogram of the right thorax
obtained at 27 weeks of amenorrhea ,
A large typical type II CCAM (c) is shown in the
right lung
associated with marked ascites (a) ;
(d) indicates diaphragm

PLAIN RADIOGRAPHY & CT
Multiple cystic pulmonary lesions of variable size
The cysts may be completely or partially fluid filled in which case the lesion may appear solid or with air fluid levels
Variable thickness of cyst wall
Chest radiographs in type I and II CPAMs may demonstrate a multicystic (air-filled) lesion
Type III lesions appear solid
Three separated cysts in the right hemithorax , CT shows a large septated cyst containing
air and fluid

TYPE 1 CCAM (CPAM)
contain one or more cysts more than 2 cm in diameter . They usually appear radiographically as
a large, air-filled multicystic lesion, sometimes with air-fluid levels, which may occupy the entire
hemithorax.
A multicystic lesion is visible in the right lower
lobe. This is a typical appearance of type 1
CPAM/CCAM in an adult.

Type 2 CCAM (CPAM)
contain multiple cysts less than 2 cm in diameter. They present radiographically as an air-filled
multicystic mass or a solid mass or area of consolidation This type may be associated with a poor
prognosis because of associated renal and cardiac abnormalities.
A solid mass (black arrow) is visible in the
right lower lobe of a neonate.
An opacified vessel is visible within the mass
(white arrow).
Type II CPAM/CCAM may present as an air-
filled or fluid-filled mass.

The treatment of choice is excision of the affected lobe.
The prognosis of neonates withCCAM/CPAM is adversely affected by large size,
underdevelopment of uninvolved lung (i.e., the presence of associated lung
hypoplasia), and the presence of associated fetal hydrops or other congenital
anomalies.

PULMONARY ARTERIOVENOUS MALFORMATION
 also known as arteriovenous fistula, likely results from deficient formation or abnormal
dilation of pulmonary capillaries due to a developmental defect in the capillary wall
 35% to 67% of cases are associated with Osler-Weber-Rendu syndrome (hereditary
hemorrhagic telangiectasia), in which AVMs are found in the skin, mucous
membranes, and viscera
 Pulmonary arteriovenous fistulas slowly enlarge over time and are usually first
diagnosed in adulthood.
 More than two thirds of AVMs are found in the lower lobes, and they are typically
subpleural in location.
 Fistulas are multiple in 35% of patients and bilateral in 10%.

Simple and Complex Arteriovenous
A simple AVM is a single, dilated vascular sac connecting one artery and one vein. It is
most frequent and accounts for the bulk of cases of AVM. Malformations
Complex AVMs, which have more than one feeding artery, are rare.
Radiographically, a simple fistula appears as a peripheral, well-defined round, oval,
lobulated, or serpentine opacity , almost always in a subpleural location . Large vessels
(feeders) extending centrally toward the hilum are often visiblewith fistulas of significant
size (larger than 1 to 2 cm), the feeders are easily recognizable. In general, the feeders
are about half the diameter of the fistula.
Following the bolus injection of contrast, pulmonary AVMs show rapid contrast
opacification and washout, occurring in phase with opacification and washout of the main
pulmonary artery and right ventricle.
Enlargement of fistulas over a period of months or years is common, and a rapid increase
in size can occur.

Chest radiograph shows a nodule in the
subpleural right lower lobe (large white
arrow) with a large feeding vessel (small
arrows)

CT slice shows a fistula in the subpleural lung
with a feeding vessel

On an arteriogram,
a single feeding artery (large arrow) supplies a
simple malformation drained by a single vein
(small arrows).
As is typical, the fistula is subpleural in
location.

A well-defined, smooth, round, 3-cm
nodule is visible on chest radiograph in
the left lower lobe

Contrast-enhanced CT shows a
densely opacified fistula in the
subpleural lung.
Its feeding artery is also visible
on this slice.

Pulmonary arteriogram shows an
AVM and a single feeding artery.

 AVMs less than 2 cm in diameter are asymptomatic. Single fistulas are less
commonly symptomatic than multiple fistulas
 AVM results in a right to left shunt, and cyanosis may be present, depending on
the size of the shunt
 The most frequent symptoms occurring in patients with AVMs are dyspnea,
palpitation, hemoptysis, and chest pain. Rupture can result in pulmonary
hemorrhage or hemothorax
 Transcatheter occlusion of fistulas using wire coils is the treatment of choice for
simple AVMs and is generally reserved for fistulas having an arterial feeder
greater than 3 mm in diameter. Recanalization of an AVM may occasionally occur
after coil embolization.

The fistula has been
occluded by wire
coils

PULMONARY
UNDERDEVELOPMENT
PULMONARY AGENESIS,
APLASIA,
HYPOPLASIA

PULMONARY AGENESIS AND
APLASIA
Pulmonary agenesis and aplasia are nearly the same, and a distinction is not usually
necessary
Pulmonary agenesis - represents complete absence of a lung, its bronchi, and its vascular
supply.
Pulmonary aplasia - characterized by complete absence of a lung and its vascular supply,
but a rudimentary bronchus is present , ending in a blind pouch
Either side may be affected
associated with a poor prognosis, with few patients surviving to adulthood.
Radiographically, pulmonary agenesis and aplasia result in opacification of a hemithorax
and marked ipsilateral mediastinal shift

CT through the upper chest shows opacification of the right hemithorax and shift of the
trachea to the right side

Just distal to the tracheal carina, the left main bronchus (large arrow) and a small right-sided
bronchus (small arrow) are visible.
There is marked shift of the mediastinum to the right, with left lung herniating across the midline.

At a lower level, the bronchi to the left lung (large arrow) and the small right-sided
bronchus (small arrow) are both visible.
The heart is displaced into the posterior right hemithorax.

PULMONARY HYPOPLASIA
Bronchus and rudimentary lung are present , however the airways , alveoli & pulmonary
vessels are decreased in size & number.The pulmonary artery supplying the hypoplastic lung is
reduced in size or absent
Can be primary or secondary
primary pulmonary hypoplasia in which a cause cannot be elucidated is much less common
than secondary hypoplasia
-The majority of cases of pulmonary hypoplasia are secondary to a process limiting the
thoracic space for lung development which can be either intrathoracic or extrathoracic
-The most common intrathoracic cause is congenital diaphragmatic hernia which is left
sided in 75%–90% of cases
-The most common extrathoracic cause of pulmonary hypoplasia is severe oligohydramnios
skeletal dysplasias in which a small and rigid thoracic cage causes pulmonary hypoplasia

-The ribs may appear crowded with a low thoraco- abdominal ratio
-There may be mediastinal shift with a homogenous density on the involved hypoplastic side and compensatory
herniation of the contralateral lung across the mediastinum
- CT, the size of pulmonary vessels may appear reduced on the affected side.
Chest radiograph of a newborn with primary
pulmonary hypoplasia of the right lung showing shift of
the mediastinum to the right hemithorax
Pulmonary hypoplasia in a baby with congenital
diaphragmatic hernia

CT shows mediastinal shift, opacification of the right hemithorax, and both main
bronchi.
The right lung is airless.

3D MR angiogram shows a normal left pulmonary artery
(arrows). The right pulmonary artery is not clearly seen

HYPOGENETIC LUNG (SCIMITAR OR
VENOLOBAR) SYNDROME
Hypogenetic lung syndrome is characterized by
(a) hypoplasia of the lung with abnormal segmental
or lobar anatomy,
(b)hypoplasia of the ipsilateral pulmonary artery,
(c) anomalous pulmonary venous return to the
inferior vena cava (or right atrium, hepatic veins,
etc.)
(d) anomalous systemic arterial supply to a portion
of the hypoplastic lung, usually the lower lobe
almost always occurring on the right side

1-PLAIN RADIOGRAPHY / CT :
◦ -Small hypoplastic lung with ipsilateral mediastinal shift
◦ in one third of cases the anomalous draining vein may be seen as a tubular structure paralleling the
right heart border in the shape of a Turkish sword (scimitar)
◦ -The right heart border maybe blurred

(a) CXR shows volume loss in the right hemithorax with
rightward mediastinal shift , the right heart border is not well
seen , an anomalous vessel (arrowheads) is seen in the right
cardiophrenic angle , this vessel increases in caliber in the
caudal direction (scimitar sign)
(b) CT+C shows the lower lobe pulmonary vein (scimitar vein)
draining into the intrahepatic IVC (arrows)
(c) Volume-rendered CT clearly depicts the anomalous vein

PROXIMAL INTERRUPTION OF THE
PULMONARY ARTERY
 proximal portion of a main pulmonary artery, usually the right, fails to develop
 closely resemble hypogenetic lung syndrome.
 ipsilateral lung is hypoplastic because of deficient growth but has a normal number of
lobes and segments, and bronchial anatomy is normal
 Vessels within the lung appear small , whereas those on the opposite side are much
larger
 lung vascular supply is derived from hypertrophied bronchial arteries
 often associated with congenital heart disease, most typically tetralogy of Fallot and
septal defects
 usually occurs on the side opposite the aortic arch – Right side

Chest radiograph shows
mediastinal shift to the right with small right pulmonary
vessels and a large left pulmonary artery (arrow).

Contrast-enhanced CT shows the ascending
aorta (Ao) and a large left pulmonary artery
(LPA), but the right pulmonary artery is
absent. Large bronchial arteries (arrows)
supply the right lung.

Lung window scan shows hypoplasia of the right lung and small right lung
vessels.

PULMONARY SEQUESTRATION
Pulmonary sequestration, (aka accessory lung ) - aberrant formation of segmental lung
tissue that has no connection with the bronchial tree or pulmonary arteries.
The anomalous lung tissue has a systemic arterial supply which is usually a branch of the aorta
Types :
◦ 1-Intralobar Sequestration (ILS) -closely connected to the adjacent normal lung and do
pleura , Venous supply via the pulmonary veins
◦ 2-Extralobar Sequestration (ELS) -separated from any surrounding lung by its own
Venous supply through the systemic veins (IVC , Azygos , Portal) into the right atrium

LOCATION :
◦ Preferentially affects the lower lobes
◦ 60% of ILS affect the left lower lobe and 40% the right lower lobe
◦ ELS almost always affect the left lower lobe, however approximately 10% of
ELS canbe sub-diaphragmatic
CLINICAL PICTURE :
◦ -Recurrent pulmonary infections

ULTRASOUND :
-The sequestrated portion of lung is usually more echogenic than the rest of the lung
-ELS may be seen as early as 16 weeks gestation and typically appears as a solid well defined triangular
echogenic mass
-Colour Doppler may identify a feeding vessel (in utero cases) from the aorta
-If the sequestration is sub diaphragmatic , it may appear as an echogenic intra abdominal mass

Longitudinal Transverse
Coronal

PLAIN RADIOGRAPHY :
-Will often show a triangular opacity in the affected segment
-Recurrent infection can lead to the development of cystic areas within the mass
-Both ILS and ELS can rarely have air bronchograms as they may be connected with the GIT
CXR shows opacity in the
posterior segment of the left
lower lobe behind the
cardiac silhouette ,
projecting over the spinal
column and not bordering
the left diaphragm

CT
Large solid mass that may be homogeneous
or heterogeneous , sometimes with cystic
changes (>5 cm) near diaphragm
Air fluid levels if infected
Surrounding pulmonary consolidation
Sequestration may communicate with GIT
3D reconstructions can be particularly helpful
in detecting :
a) Anomalous arterial vessels
b) Concurrent anomalous veins
c) Differentiating between intra-lobar and
extra-lobar sequestrations

CT shows area of lobulated opacity in the posterior segment of the left lower lobe , note in the 2D
reconstruction the thoracic aorta branch directed towards the opacity

CT+C shows an ILS , the yellow arrow
in upper figures shows a hyperdense
region in the left lower lobe of the lung
with small cystic lesions containing
air within it the red arrows in the lower
figures show a contrast enhanced
vessel arising from the aorta and
supplying the area of hyperdensity in
the lung

UNILATERAL PULMONARY VEIN
ATRESIA
 long segments of the pulmonary veins of one lung are congenitally atretic
 The involved lung can be normal in size or hypoplastic, and it often shows increased
interstitial densities and interlobular septal thickening because of venous stasis, edema,
and fibrosis
 The ipsilateral pulmonary artery may appear small
 Angiography shows decreased size of the ipsilateral pulmonary artery, contrast stasis,
and nonvisualization of the pulmonary veins
 Symptoms include hemoptysis and infection.

Scout view from a CT shows the right lung
to be small, with mediastinal shift to the right and
elevation of the right hemidiaphragm and increased
interstitial markings on the right.

Contrast-enhanced CT shows the right
inferior
pulmonary vein to be absent (black arrow).
The right superior pulmonary vein was also
absent.
The right lung is hypoplastic with
mediastinal shift to the right, and the right
pulmonary artery is much smaller than the
left (large arrow).
A small right pleural effusion is present.

High-resolution CT shows interlobular septal thickening in the hypoplastic right lung.

TRACHEAL ATRESIA
congenital absence of the trachea - extremely rare anomaly
ASSOCIATIONS
◦ esophageal atresia with tracheo-esophageal fistula
◦ polyhydramnios
◦ VACTERL association
PATHOLOGY
◦ results either from an obstructing lesion (i.e. cartilaginous bar) or
vascular insult with atresia of the airways which occurs during
intrauterine development.

Floyd’s classification of tracheal agenesis.
• Type I has an atresia of part of the trachea (t), a remaining short trachea and normal bronchi.
A tracheo-oesphageal fistula (f) is present.
• Type II has a complete atresia of the trachea but normal bifurcation and bronchi.
There may, or may not be a tracheo-oesophageal communication.
• Type III has a complete agenesis of the trachea. The bronchi arise directly from the oesophagus (o).

ANTENATAL ULTRASOUND
•may show uniformly increased echogenic enlarged lung +/- pleural effusions +/- fetal
ascites
•markedly dilated fluid-filled bronchi may be seen
•may also show ancillary sonographic features such as the presence of polyhydramnios
•the fetal cardio-thoracic circumference ratio is decreased due to larger lung volumes

Ultrasound of a fetus with CHAOS demonstrating enlarged, homogeneous, and echogenic lungs compressing the cardiac silhouette and
inverting the diaphragms bilaterally. Dilated tubular non-color flow structures were present centrally within the lungs consistent with dilated
bronchi.

Fetal MRI of a fetus with CHAOS, identifying
overinflated but homogeneous lung tissue and dilated
central bronchi.
The fluid within the trachea can be visualized to the
thoracic inlet but was discontinuous with the distal
intrathoracic trachea/carina (arrows).
Intraoperatively, this proved to be edematous fluid
trapped in the tissue planes running from the carina to
the neck.
Severe ascites and soft tissue edema can also be
seen.

Chest X-ray of the infant with CHAOS. The image was obtained after the EXIT
procedure with insertion of a tracheostomy tube directly into the right mainstem
bronchus. Marked extensive anasarca is seen, with abdominal distention,
edematous lung tissue and bilateral pleural effusions. The tracheostomy tube is
seen with the tip in the right main bronchus (arrow). The right lung well inflated with
volume loss in the left lung.
aThe trachea was found to be absent from just
below the cricoid cartilage to an area just above the
carina.
b The position of the endotracheal tube immediately
after the EXIT procedure is depicted, showing its
placement into the right mainstem bronchus

Tracheal agenesis. 4 days old
girl.
Study of the oesophagus.
The sequence shows filling of
a fistula (arrow) from the
oesophagus to the trachea (B)
and gradual filling of a almost
normal bronchial three (C).

CONGENITAL TRACHEO-ESOPHAGEAL
FISTULA
congenital pathological communication between the trachea and
esophagus.
PATHOLOGY
◦ The trachea is an out-budding from the ventral foregut, and tracheo-
esophageal fistulae represent incomplete/abnormal division

ULTRASOUND
Antenatal ultrasound may demonstrate polyhydramnios or even in some
cases a distended proximal blind-ending esophagus

PLAIN RADIOGRAPH
Demonstration of the nasogastric tube curled in the proximal esophagus in a child
where the passage of the tube has been unsuccessful is usually sufficient for
diagnosis. The proximal esophageal stump may be distended with air (types A and
C).
The presence of air in the stomach and bowel in the setting of esophageal atresia
implies that there is a distal fistula.
Often the lungs demonstrate areas of consolidation/atelectasis due to recurrent
aspiration.

TYPE A: ISOLATED ESOPHAGEAL ATRESIA

TYPE B: PROXIMAL FISTULAWITH DISTALATRESIA

TYPE C: PROXIMALATRESIAWITH DISTAL FISTULA
(85% - MOST COMMON)

TYPE D: DOUBLE FISTULA WITH INTERVENING ATRESIA
(1%)

TYPE E: ISOLATED FISTULA(H-TYPE)

FLUOROSCOPY
Barium is usually the contrast medium of choice, a non-ionic iodinated
contrast medium can be used alternatively.
Ionic iodinated contrast medium should not be used as they can cause
chemical pneumonitis.

TYPE E: ISOLATED FISTULA (H-TYPE)

TRACHEAL BRONCHUS
A tracheal bronchus (aka pig bronchus) is an anatomical variant where an
accessory bronchus originates directly from the supracarinal trachea.
The latter term (pig bronchus or bronchus suis) is often given when the entire
upper lobe (usually right side) is supplied by this bronchus .
PATHOLOGY
◦ Tracheal bronchi arise from the right lateral wall of the trachea usually at a
distance of <2 cm from the level of the carina.
◦ They can be classified into two main types:
◦ supernumerary: usual bronchial supply to affected lung segment is
concurrently present
◦ displaced: usual bronchial supply to affected lung segment is concurrently
absent

CONGENITAL PULMONARY ABNORMALITIES
1-Bronchopulmonary Foregut Malformations
2-Congenital Lobar Emphysema
3-Pulmonary Underdevelopment
4-Scimitar Syndrome
5-Bronchial Atresia
6-Congenital Diaphragmatic Hernia
7-Kartagener’s Syndrome

BRONCHOPULMONARY FOREGUT
MALFORMATIONS
Is a term that encompases :
1- CPAM
2-Pulmonary Sequestration
3-Foregut Duplication Cysts

CONGENITAL PULMONARY AIRWAY MALFORMATION
aka CONGENITAL CYSTIC ADENOMATOID
MALFORMATION (CCAM)
CPAM are multicystic masses of segmental lung tissue with abnormal bronchial
proliferation
PATHOLOGY :
failure of normal broncho-alveolar development with hamartomatous
proliferation of terminal respiratory units in a gland-like pattern (adenomatoid)
without proper alveolar formation
LOCATION :
-Lesions are usually unilateral and involve a single lobe
-They appear less frequently in the right middle lobe

DIFFERENTIAL DIAGNOSIS :
General imaging differential considerations include :
1-Bronchogenic cyst
2-Pulmonary sequestration
3-Congenital diaphragmatic herniation
4-Congenital lobar emphysema

FOREGUT DUPLICATION CYSTS
a) Bronchogenic Cyst
b) Esophageal Duplication Cyst
c) Neuroenteric Cyst

OESOPHAGEAL DUPLICATION CYST
Esophageal duplication cysts are a type of congenital foregut duplication cyst.
Pathology:
◦ congenital malformation of the posterior primitive foregut and result
posterior division of the embryonic foregut at 3-4 weeks gestation.
◦ They comprise two outer muscle layers and an inner epithelial lining
include stratified squamous, simple columnar, pseudostratified
ciliated epithelium; there are even reports of intermixed heterotopic
mucosa.

PLAIN RADIOGRAPHY :
-They are usually seen as rounded fluid / soft tissue density posterior mediastinal masses
Esophageal duplication cyst in a
3 year old girl with cough and
dyspnea,
(a) Chest radiograph shows
homogenous opacification of the
right hemithorax (arrows) at the
time of first admission ,
(b) Follow-up CT 3 years later
shows a large cystic
periesophageal mass (arrows)

BARIUM SWALLOW
-In cystic esophageal duplication , the oesophagogram shows the esophagus to be
displaced to the side opposite the mass

CT :
-Duplication cysts appear as is sharply marginated masses with homogeneous fluid density
-No enhancement after intravenous contrast administration is visible

PLAIN RADIOGRAPHY
-Bronchogenic cysts appear at a subcarinal location (most commonly)
-Oesophageal cysts appear more tubular and in close relation to the esophagus
Bronchogenic Cyst (subcarinal)
Esophageal Duplication Cyst ( in
close relation to esophagus)

CT :
-Thin walled bronchogenic cyst
-Thick walled oesophageal cyst (due to presence of smooth muscle)
Bronchogenic Cyst Esophageal Duplication Cyst

PULMONARY UNDERDEVELOPMENT
A) TYPES :
1-PULMONARY AGENESIS :
-Complete absence of the lung parenchyma , bronchus & pulmonary vasculature
-Abnormal blood flow in the dorsal aortic arch during the 4th week of gestation (embryonic
phase) causes pulmonary agenesis

-Postnatal radiography demonstrates
diffuse opacification of the involved hemithorax with ipsilateral mediastinal shift ,
severe volume loss and opacity on the side of agenesis often with close spacing of the ribs ,
the bronchus and PA to the affected lung are absent and blood flow tothe contralateral lung is increased
-CT helps confirm the absence of the lungparenchyma , bronchus and pulmonary artery on the involved side

2-PULMONARY APLASIA :
-Blind ended rudimentary bronchus is present without lung parenchyma or pulmonary vasculature

PULMONARY APLASIA :
-Imaging findings in pulmonary aplasia and agenesis are similar except for the presence of a short
blind-ending bronchus in aplasia
Pulmonary aplasia (a) Chest radiograph depicts the trachea (white arrow) and the right main bronchus
(arrowhead) however , the left main bronchus is not seen , there is leftward mediastinal shift , compensatory
hyperinflation of the right middle lobe extending into the left hemithorax is also noted (black arrow) ,
(b) CT shows a blindending left main bronchus (arrowhead) with absence of the left lung parenchyma

Antenatal ultrasound
may show oligohydramnios
Several sonographic parameters may give indirect clues as to the presence and extent of pulmonary hypoplasia. Such
include:
•fetal lung : head ratio: reduced (ratios <1 usually indicate a poor prognosis)
•fetal chest circumference (or thoracic circumference (TC)) : reduced
In the case of intra-thoracic causes, both these parameters can be normal)
•thoracic : abdominal circumference ratio <0.6
•femur length : abdominal circumference ratio <0.16

4-SCIMITAR SYNDROME
PATHOLOGY :
-Also known as Hypogenetic Lung Syndrome , Pulmonary
Venolobar Syndrome
-Characterized by a hypoplastic lung that is drained by an
anomalous pulmonary vein into the systemic venous system (IVC)
-A combination of pulmonary hypoplasia and partial anomalous
pulmonary venous return (PAPVR)
-Haemodynamically, there is an acyanotic left-to-right shunt. The anomalous
vein usually drains into the:
◦ inferior vena cava: most common
◦ right atrium
◦ portal vein

LOCATION :
◦ -It almost exclusively occurs on the right side
ASSOCIATIONS :
◦ 1-Accessory diaphragm , diaphragmatic hernia
◦ 2-Bony abnormalities : hemivertebrae , rib notching & rib hypoplasia
◦ 3-CHD : ASD , VSD , PDA & tetralogy of Fallot
The diagnosis is made by transthoracic or transesophageal echocardiography, angiography, or by CT or MR
angiography.

5-BRONCHIAL ATRESIA
PATHOLOGY :
◦ -Bronchial atresia is a rare anomaly resulting from focal obliteration of a segmental , subsegmental or
lobar bronchus
◦ -The bronchi distal to the stenosis are dilated and filled with mucus with mild hyperinflation of the
adjacent lung due to air trapping(The lung distal to the atretic bronchus develops normally but is overinflated due to
collateral air drift with air trapping.)
◦ The exact cause of bronchial atresia is not well known - suggested that atresia is probably secondary to a traumatic event
during fetal life rather than a result of abnormal growth and development
LOCATION :
◦ -Commonly occurs at the apico-posterior segment of the left upper lobe followed by the right upper lobe

RADIOGRAPHIC FEATURES : HRCT
-Atretic bronchial stump(s) often become mucus plugged and can give a finger in glove appearance
-Distal lung parenchyma supplied by the atretic segment can be emphysematous due to air trapping
Central mass surrounded by hyperlucent lung (blue arrow)

Bronchial atresia , CT shows mucoid impaction
(arrow) just distal to bronchial atresia in the right
upper lobe , distal air trapping is also noted

CXR in inspiration/expiration show hyperlucency of the left lung
, the heart is displaced towards the right and there is shifting of
the mediastinum towards the right on expiration confirming air
trapping of the left lung , in addition , a tubular structure is
visible in the left lower lung (arrow) , CT shows hyperlucency of
the left lower lung limited by normal upper lung (white arrows) ,
there is a non-enhancing tubular structure in the centre of the
left lower lung (red arrows) , the displacement of the heart
towards the right is due to the expanded left lung plus a
marked pectus excavatum

KARTAGENER’S SYNDROME
PATHOLOGY :
-Kartagener's syndrome (immotile cilia syndrome) is due to the deficiency of the dynein arms of cilia
causing immotility of respiratory , auditory and sperm cilia
CLINICAL PICTURE :
-Kartagener syndrome is characterized by the clinical triad of :
◦ 1-Situs inversus
◦ 2-Chronic sinusitis and / or nasal polyposis
◦ 3-Bronchiectasis
◦ -Other features include :
◦ 1-Telecanthus : widened interpupillary distance by a nasal polyp
◦ 2-Infertility in male
◦ 3-Subfertility in female

RADIOGRAPHIC FEATURES :
-Complete thoracic and abdominal situs inversus
-Bronchiectasis
-Sinus hypoplasia and mucosal thickening

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