Diagnostic Imaging of Congenital Pulmonary Abnormalities

1. Chest
Congenital Pulmonary Abnormalities

2. Mohamed Zaitoun
Assistant Lecturer-Diagnostic Radiology
Department , Zagazig University Hospitals
Egypt
FINR (Fellowship of Interventional
Neuroradiology)-Switzerland
zaitoun82@gmail.com

5. Knowing as much as
possible about your enemy
precedes successful battle
and learning about the
disease process precedes
successful management

6. 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

7. 1-Bronchopulmonary Foregut Malformations :
-Is a term that encompases :
1-CCAM
2-Pulmonary Sequestration
3-Foregut Duplication Cysts

8. 1-Congenital Cystic Adenomatoid Malformation
(CCAM) :
a) Incidence
b) Pathology
c) Types
d) Location
e) Clinical Picture
f) Radiographic Features
g) Differential Diagnosis

9. a) Incidence :
-They account for approximately 25% of
congenital lung lesions

10. b) Pathology :
-The condition results from failure of normal
broncho-alveolar development with
hamartomatous proliferation of terminal
respiratory units in a gland-like pattern
(adenomatoid) without proper alveolar
formation

11. c) Types :
1-Macrocystic (Stocker types 1 and 2) :
-Single cyst or multiple cysts >5 mm confined to
one hemithorax ; better prognosis ; common
2-Microcystic (Stocker type 3) :
-Homogeneous echogenic mass without
discernible individual cysts ; closely resembles
pulmonary sequestration or intrathoracic bowel
from a diaphragmatic hernia ; less common

12. d) Location :
-Lesions are usually unilateral and involve a
single lobe
-They appear less frequently in the right
middle lobe

13. e) Clinical Picture :
-The diagnosis is usually either made on
antenatal ultrasound or in the neonatal
period on investigation of progressive
respiratory distress
-If large , they may cause pulmonary
hypoplasia with resultant poor prognosis

14. f) Radiographic Features :
1-Antenatal Ultrasound
2-Plain Radiography
3-CT

15. 1-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

16. 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

17. 2-Plain Radiography :
-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
3-CT :
-The same as Plain Radiography

18. Plain chest radiograph in this newborn shows three
separated cysts in the right hemithorax , CT shows a
large septated cyst containing air and fluid

31. g) Differential Diagnosis :
-General imaging differential considerations
include :
1-Bronchogenic cyst
2-Pulmonary sequestration
3-Congenital diaphragmatic herniation
4-Congenital lobar emphysema

32. 2-Pulmonary Sequestration :
a) Incidence
b) Pathology
c) Types
d) Location
e) Clinical Picture
f) Radiographic Features
g) Differential Diagnosis

33. a) Incidence :
-The estimated incidence is at 0.1%
- The age of presentation is dependent on
the type of sequestration
-ELS more commonly presents in newborns
whereas ILS presents in late childhood or
adolescence

34. b) Pathology :
-Also called (accessory lung)
-Refers to aberrant formation of segmental lung tissue that
has no connection with the bronchial tree
-The anomalous lung tissue has a systemic arterial supply
which is usually a branch of the aorta
-Venous supply is variable and dependant on the type of
sequestration :
1-ILS : Via the pulmonary veins
2-ELS : Through the systemic veins (IVC , Azygos , Portal)
into the right atrium

35. c) Types :
1-Intralobar Sequestration (ILS)
2-Extralobar Sequestration (ELS)

36. 1-Intralobar Sequestration (ILS) :
-Accounts for the majority (75-85% of all
sequestrations
-Present later in childhood with recurrent infections
2-Extralobar Sequestration (ELS) :
-Less common (15-25% of all sequestrations)
-Usually present in the neonatal period with
respiratory distress , cyanosis and / or infection
-More in males , M:F ratio 4:1
-Can be infra diaphragmatic in 10 % of cases

37. d) Location :
-Overall , sequestration 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 can
be sub-diaphragmatic

38. e) Clinical Picture :
-Recurrent pulmonary infections

39. f) Radiographic Features :
1-Ultrasound
2-Plain Radiography
3-CT
4-MRI
5-Angiography

40. 1-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

43. 2-Plain Radiography :
-Will often show an 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

44. 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

45. Homogenous dense opacity (red arrow) in right
cardiophernic angle

48. Well-defined mass in the left lower lobe which determines loss of
volume of the left lung and mediastinal shift to the right , the lesion
corresponded to an extralobar sequestration

50. 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

51. 3-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

52. 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

53. CT+C shows a homogeneous mass in the posterior segment of the left
lower lobe , feeding artery (arrow) is seen arising from the aorta , a
finding that is diagnostic for sequestration

60. ELS

61. 4-MRI :
*T1 :
-The sequestrated segment tends to be of
comparatively high signal to normal lung tissue
*T2 :
-Also tends to be of comparatively high signal
*MRA :
-Can be helpful in demonstrating anomalous
arterial supply

62. 5-Angiography :
-Not part of routine investigation but is the
gold standard in determining arterial
supply

63. g) Differential Diagnosis :
-See CCAM

64. 3-Foregut Duplication Cysts :
a) Bronchogenic Cyst
b) Esophageal Duplication Cyst
c) Neuroenteric Cyst

65. a) Bronchogenic Cyst :
1-Incidence
2-Pathology
3-Location
4-Clinical Picture
5-Radiographic Features
6-Differential Diagnosis

66. 1-Incidence :
-Bronchogenic cysts are rare congenital
lesions accounting for only 5-10%
of pediatric mediastinal masses

67. 2-Pathology :
-Bronchogenic cysts form as a result of
abnormal budding of the bronchial tree
during embryogenesis (between 4th-
6th weeks) and as such they are lined by
secretory respiratory epithelium (cuboid or
columnar ciliated epithelium)

68. 3-Location :
-The most common location is the middle
mediastinum
-subcarinal , right paratracheal and hilar
locations most common

69. 4-Clinical Picture :
-In many instances , bronchogenic cysts are
asymptomatic and are found incidentally
when the chest is imaged
-When large , mass effect may result in
bronchial obstruction leading to air
trapping and respiratory distress
-An alternative presentation may occur
when the cyst becomes infected

70. 5-Radiographic Features :
a) Plain Radiography
b) CT

71. a) 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

72. The chest X-ray showed a non calcified homogeneous opacity lateral to
the trachea with a well defined interface with the lung ,
cervicothoracic sign indicated the posterior localisation of the mass.

73. Azygoesophageal recess reflection , (a) PA chest radiograph shows the
azygoesophageal line (arrowheads) , (b) CT shows the azygoesophageal
recess (white arrow) formed by the esophagus anteriorly (black arrow) and
the azygos vein posteriorly (arrowhead) , the azygoesophageal line
in a represents the interface between this recess and the lung

74. (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

75. (a) PA chest radiograph shows the posterior junction line (arrow)
projecting through the tracheal air column , (b) CT shows the
posterior junction line (arrow) which is formed by the interface
between the lungs posterior to the mediastinum and consists of four
pleural layers

76. (a) PA chest radiograph shows a mass (arrow) obliterating the posterior
junction line , note that the mass extends above the level of the clavicle and
has a well demarcated outline due to the interface with adjacent lung
(arrowhead) , (b) CT helps confirm the posterior location of the mass
(arrow), which proved to be a bronchogenic cyst

77. Bronchogenic cyst , conventional radiograph demonstrates a right
paratracheal mass

78. Bronchogenic cyst , conventional radiograph shows a subcarinal mass

79. Bronchogenic cyst , conventional radiograph demonstrates a thin walled cyst in
the left lower lobe with an air fluid level

83. b) CT :
-Typically appear as 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

85. CT+C : well circumscribed unilocular water attenuation cyst in the
middle mediastinum , the cyst has smooth imperceptible walls with
no enhancement

86. Bronchogenic cyst , CT shows a subcarinal mass with fluid density

87. 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

91. 6-Differential Diagnosis :
-From oesophageal duplication cyst
a) Clinical Picture :
-Asymptomatic bronchogenic cyst
-Symptomatic oesophageal cyst in case of
peptic ulceration

92. b) Plain Radiography :
-Bronchogenic cysts appear at a subcarinal
location (most commonly)
-Oesophageal cysts appear more tubular
and in close relation to the esophagus
c) CT :
-Thin walled bronchogenic cyst
-Thick walled oesophageal cyst (due to
presence of smooth muscle)

93. Bronchogenic Cyst (subcarinal) Esophageal Duplication Cyst ( in close relation to esophagus)

94. Bronchogenic Cyst Esophageal Duplication Cyst

95. b) Oesophageal Duplication Cyst :
1-Incidence
2-Clinical Picture
3-Radiographic Features
4-Differential Diagnosis

96. 1-Incidence :
-It is the second most common GIT
duplication after that of the ileum
-As a congenital abnormality , if
symptomatic , it is usually identified soon
after birth

97. 2-Clinical Picture :
-Presentation of large duplication cysts is usually
in the newborn or infant with symptoms referable
to pressure on the adjacent lung or esophagus
leading to :
1-Respiratory difficulties
2-Dysphagia
3-Vomiting
-Smaller cysts can be asymptomatic and only
found incidentally at any time

98. 3-Radiographic Features :
a) Plain Radiography
b) Barium Swallow
c) CT
d) MRI

99. a) Plain Radiography :
-They are usually seen as rounded fluid /
soft tissue density posterior mediastinal
masses

103. 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)

104. b) Barium Swallow :
-In cystic esophageal duplication , the
oesophagogram shows the esophagus to
be displaced to the side opposite the
mass

106. c) CT :
-Duplication cysts appear as is sharply
marginated masses with homogeneous
fluid density
-No enhanced after intravenous contrast
administration is visible

111. d) MRI :
-MRI demonstrates features of a cyst
*T1 :
-Low signal
*T2 :
-High signal
*T1+C :
-No solid enhancement

112. T1 T2

113. 4-Differential Diagnosis :
-From Bronchogenic cyst
-See before

114. c) Neuroenteric Cyst :
1-Incidence
2-Radiographic Features

115. 1-Incidence :
-Neurenteric cysts are rare posterior
mediastinal lesions that may be connected
to the meninges through a midline defect
in one or more vertebral bodies
-This abnormality may be associated with
vertebral anomalies such as
hemivertebra , butterfly vertebra or spina
bifida

116. 2-Radiographic Features :
a) Plain Radiography
b) CT
c) MRI

117. a) Plain Radiography :
-Well circumscribed rounded mediastinal
mass

118. Neurenteric cyst in a 30 year old female with flank pain , (a) Chest radiograph shows a
well defined round mass (arrow) in the lower thoracic region , (b- c) Both axial T1 and
coronal T2 show a large mass that is of homogenous high signal intensity in the right
paravertebral region (arrow) , the cyst presumably contains proteinaceous fluid

120. b) CT :
-The cyst is seen as hypoattenuating lesion
which may show soft tissue attenuation
c) MRI :
-The appearance depends on the variable
protein content :
*T1 & T2 : of variable signal intensities

121. 2-Congenital Lobar Emphysema :
a) Incidence
b) Pathology
c) Associations
d) Location
e) Clinical Picture
f) Radiographic Features
g) Differential Diagnosis

122. a) Incidence :
-More common in males (M:F = 3:1)

123. b) Pathology :
-Is a congenital lung abnormality that results in
progressive overinflation of one or more lobes of
a neonate lung
-Idiopathic , 50%
-Obstruction of airway with valve mechanism ,
50% :
1-Bronchial cartilage deficiency or immaturity
2-Mucus
3-Web , stenosis
4-Extrinsic compression

124. c) Associations :
-May be associated with congenital heart
defects such as :
1-Ventricular septal defect (VSD)
2-Patent ductus arteriosus (PDA)
3-Tetralogy of Fallot

125. d) 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 hemithorax is the
most common side to be affected

126. e) Clinical Picture :
-Patients typically present with respiratory
distress most commonly in the neonatal
period and usually within the first 6 months
of life

127. f) Radiographic Features :
1-Plain Radiography
2-CT

128. 1-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

129. b) Later Findings :
-Appears as an area of hyperlucency in the
lung with a paucity of vessels
-Mass effect with mediastinal shift and
hemidiaphragmatic depression
-Lateral film may demonstrate posterior
displacement of the heart

130. 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

131. Low density and expanded left lung with important mass
effect (mediastinal shift to the right , contralateral
atelectasis and increased of the left intercostal space)

132. A large hyperlucent area (red arrow) in the left upper , mid
and lower zone with attenuated vascular markings within
the lucency (green arrow)

135. 2-CT :
-Hyperlucent lobe (hallmark)
-As Plain Radiography

138. g) Differential Diagnosis :
1-General Differential Diagnosis
includes:
a) CCAM
b) Pulmonary artery hypoplasia
c) Bronchial atresia

139. 2-Unilateral Hypertransradiant
Hemithorax :
a) Rotation
b) Chest Wall
c) Pleura
d) Lung
e) Pulmonary Vessels

140. a) Rotation :
-The hypertransradiant hemithorax is the side to which the
patient is turned
1-Poor technique
2-Scoliosis
b) Chest Wall :
1-Mastectomy
2-Poliomyelitis
3-Poland’s syndrome (unilateral congenital absence of
pectoral muscles +/- rib defects)
c) Pleura :
-Pneumothorax

141. d) Lung :
1-Compensatory Hyperelaxation :
-Following lobectomy
2-Air way Obstruction :
-Air trapping on expiration results in increased lung volume and shift of
the mediastinum to the contralateral side
-Foreign body , bronchial atresia , endobronchial mass & bronchial
compression
3-Unilateral Bullae , pneumatocele
4-Swyer-James (McLeod) Syndrome
5-Congenital Lobar Emphysema
6-Schimitar Syndrome
e) Pulmonary Vessels :
-Pulmonary embolus to a major pulmonary artery

142. 3-Pulmonary Underdevelopment :
a) Types
b) Radiographic Features

143. 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
-More than 50% of affected fetuses have other
abnormalities involving the cardiovascular (patent ductus
arteriosus , patent foramen ovale) , gastrointestinal
(tracheoesophageal fistula , imperforate anus) ,
genitourinary or skeletal (limb anomalies , vertebral
segmentation anomalies) system

144. 2-Pulmonary Aplasia :
-Blind ended rudimentary bronchus is
present without lung parenchyma or
pulmonary vasculature
3-Pulmonary Hypoplasia :
-Bronchus and rudimentary lung are present
, however the airways , alveoli &
pulmonary vessels are decreased in size
& number

145. -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

146. -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

147. b) Radiographic Features :
1-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 to
the contralateral lung is increased
-CT helps confirm the absence of the lung
parenchyma , bronchus and pulmonary artery on
the involved side

148. Pulmonary Agenesis

149. 2-Pulmonary Aplasia :
-Imaging findings in pulmonary aplasia and
agenesis are similar except for the
presence of a short blind-ending bronchus
in aplasia

150. 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 blind-
ending left main bronchus (arrowhead) with absence of the left lung parenchyma

151. 3-Pulmonary Hypoplasia :
-The ribs may appear crowded with a low thoracic-
to-abdominal ratio
-Films may also show features of the neonate's
underlying condition
-There may be mediastinal shift with a
homogenous density on the involved hypoplastic
side and compensatory herniation of the
contralateral lung across the mediastinum

152. Chest radiograph of a newborn with primary pulmonary hypoplasia of
the right lung showing shift of the mediastinum to the right
hemithorax

153. Radiograph showing left pulmonary hypoplasia

154. Chest Radiograph of a 3-month-old infant with primary pulmonary
hypoplasia of the right lung

155. White left lung with diffuse interstitial opacity and hyperexpansion of the
right lung , the trachea is deviated to the left as is the cardiac
silhouette

156. A chest radiograph of a newborn with diaphragmatic hernia in the right hemithorax shortly
after birth , CT shows the presence of abdominal contents in the right hemithorax ,
note the presence of the left lower bronchus and its main branches (horizontal arrow)
and absence of the right lower lobe bronchus , the liver in the right hemithorax is
indicated by the upper arrow

157. AP and lateral chest radiographs in a patient with skeletal dysplasia
show the short dysplastic ribs and narrowed thorax

158. A chest radiograph of a newborn with achondroplasia and
small chest causing hypoplasia of both lungs

161. 4-Scimitar Syndrome :
a) Pathology
b) Location
c) Associations
d) Radiographic Features

162. a) Pathology :
-Also known as Hypogenetic Lung Syndrome ,
Pulmonary Venolobar Syndrome
-Characterized by a hypoplastic lung that is
drained by an anomalous vein into the systemic
venous system (IVC)
-A combination of pulmonary hypoplasia and
partial anomalous pulmonary venous return
(PAPVR)

164. b) Location :
-It almost exclusively occurs on the right
side

165. c) Associations :
1-Accessory diaphragm , diaphragmatic
hernia
2-Bony abnormalities : hemivertebrae , rib
notching & rib hypoplasia
3-CHD : ASD , VSD , PDA & tetralogy of
Fallot

166. d) Radiographic Features :
1-Plain Radiography
2-CT

167. 1-Plain Radiography :
-Small lung with ipsilateral mediastinal shift and 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
2-CT :
-As Plain Radiography

174. (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 (arrow)

176. 5-Bronchial Atresia :
a) Pathology
b) Location
c) Radiographic Features
d) Differential Diagnosis

177. a) 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

178. b) Location :
-Commonly occurs at the apico-posterior
segment of the left upper lobe

179. c) 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

180. Central mass surrounded by hyperlucent lung (blue arrow)

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

182. 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

183. CXR shows an area of hyperlucency in the left upper lung with branching
serpiginous shadows within (arrows) , CT confirms the unenhanced mucous
plug within the area of hyperlucency , expiratory film confirms air trapping

185. d) Differential Diagnosis :
1-Congenital Lobar Emphysema
2-Allergic Bronchopulmonary Aspergillosis
(ABPA) : for finger in glove appearance

186. 6-Congenital Diaphragmatic Hernia :
a) Incidence
b) Associations
c) Types

187. a) Incidence :
-1 in 2000 to 3000 births
-Mortality rate of isolated hernias is 60%
(with postnatal surgery) and higher when
other abnormalities are present

188. b) Associations :
1-Pulmonary hypoplasia (common)
2-CNS abnormalities :
-Neural tube defects : spina bifida ,
encephalocele
-Anencephaly

189. c) Types :
1-Morgagni Hernia
2-Bochdalek Hernia

190. 1-Morgagni Hernia :
a) Incidence
b) Pathology
c) Location
d) Radiographic Features
e) Differential Diagnosis

191. a) Incidence :
-Is one of the congenital diaphragmatic
hernias
-It is rarer than the Bochdalek hernia and
accounts for approximately 2% of all
congenital diaphragmatic hernias

192. b) Pathology :
-It is characterized by herniation through
the foramen of Morgagni (anterior opening
in the diaphragm that extends between the
sternum medially and the eighth rib
laterally)
-As compared to the Bochdalek hernia , the
Morgagni hernia is rare , small & anterior

193. c) Location :
-This hernia occurs in the anterior midline
(retrosternal or parasternal) through the
foramen of Morgagni with 90% of cases
occurring on the right side
-Morgagni hernias typically occur medially

194. d) Radiographic Features :
-Anterior herniation of bowel loops on a
lateral chest radiograph is the typical
finding
-Other herniated viscera include the liver ,
spleen and omentum

197. Morgagni hernia in a 2 year old child , lateral chest radiograph shows
herniation of a bowel loop (arrows) in a classic location through an
anteromedial defect

201. e) Differential Diagnosis :
-Cardiophrenic angle lesions :
-The cardiophrenic space is usually filled with fat , however
lesions originating above or lower to the diaphragm can
present as cardiophrenic angle lesions :
1-Pericardial fat pad
2-Pericardial cyst
3-Pericardial fat necrosis
4-Morgagni’s hernia
5-Lymphadenopathy : metastases , lymphoma
6-Pericardial lipomatosis

202. 2-Bochdalek Hernia :
a) Incidence
b) Pathology
c) Location
d) Radiographic Features

203. a) Incidence :
-Is one of the congenital diaphragmatic
hernias
-More common than Morgagni hernia
-It is more common in infants (90%)

204. b) Pathology :
-They occur posteriorly and are due to a
defect in the posterior attachment of
the diaphragm when there is a failure of
pleuroperitoneal membrane closure in
utero
-Retroperitoneal structures may prolapse
through the defect , e.g. retroperitoneal fat
, spleen or left kidney

205. c) Location :
-They occur posteriorly
-It occurs most frequently on the left side
with approximately 80% being left-sided
and 20% right-sided

206. d) Radiographic Features :
1-Plain Radiography
2-CT

207. 1-Plain Radiography :
-On conventional radiographs , the hernia
may appear as a lung base soft-tissue
opacity lesion seen posteriorly on lateral
images

210. Intrathoracic kidney

211. 2-CT :
-CT usually demonstrates fat above the
diaphragm and is extremely beneficial in
revealing organ entrapment
-Coronal and sagittal reformatted images
show the defect to best advantage

214. 7-Kartagener’s Syndrome :
a) Pathology
b) Clinical Picture
c) Radiographic Features

215. a) 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

216. b) 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

218. c) Radiographic Features :
-Complete thoracic and abdominal situs
inversus
-Bronchiectasis
-Sinus hypoplasia and mucosal thickening