Paediatric Chest Conditions

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Reuben GRECH

29th April 2009


Outline of Lecture:

Anatomy

CXR

Respiratory System
Congenital Disorders
Acquired Disorders

Cardiovascular System

Thoracic Neuroblastoma

Non Accidental Injury


Anatomy
Thoracic Wall

1. Chest Wall

Ribs – 12 pairs (1st , 11th and 12th ribs atypical)


Costal cartilages
Intercostals – 3 pairs (external, internal, innermost)
Vertebrae – Th1 – Th12
Sternum – 3 parts (manubrium, body, xiphoid)

2. Diaphragm (Fibromuscular Structure)

Central Tendon
Muscular Fibres Peripherally

3. Thoracic Inlet
Thoracic Cavity

Lungs
Rt lung (3 lobes – 10 bronchopulmonary segments)
Lt lung (2 lobes – 10 bronchopulmonary segments)

Pleura
Parietal
Visceral

Mediastinum
Superior (Great Vessels, Trachea, Nerves)
Anterior (Thymus)
Middle (Pericardium, Heart)
Posterior (Descending Aorta, Oesophagus, Thoracic Duct, Azygos, Thoracic Sympathetic Trunk)
Thoracic Cavity
Lungs
Heart and Large Vessels
Normal CXR
Abnormal findings

Pleura
Effusion
Plaque

Lungs
Consolidation
Reticuloar Shadowing
Nodular Shadowing
Cystic Lesion
Collapse
Pneumothorax
Atelactasis

Heart
Cardiomegaly
Pericardial Effusion

Mediastinum

Extra Thoracic

Bones
Imaging in Paediatrics

Difficulties in acquiring Images

Uncooperative patient
Movement
Phase of respiration

Difficulties in Interpretation

Anatomical Variation
Thymus
Thymus

• Can be very prominent on radiography up to


5 years of age

• Begins to become smaller relative to the


chestby the end of the 1st decade

• Shape can be very variable

• Ultrasonography demonstrates echogenic


septae, which can be helpful in identifying
ectopic thymus

• Differential diagnosis
(i.e. other causes of anterior mediastinal masses)

1.Lymphoma (commonest)
2.Teratoma
3.Thymic Cyst
4.LCH
Congenital Respiratory Disorders
Pulmonary Agenesis / Hypoplasia
Bronchial Atresia
Congenital Diaphragmatic Hernias
CCAM
Congenital Lobar Emphysema
TEF / OE
Sequestration
Bronchogenic Cyst
Congenital Diaphragmatic Hernias
• Incidence = 1:3000
• M : F = 1:1
• L : R = 5-9:1
• Presents with severe respiratory distress in neonates, scaphoid
abdomen, less severe cases may present later in life or incidentally on
radiography
• Associated with other anomalies in 20%
• Types: Bochdalek, Morgagni, Septum Transversum defect, Hiatal
hernia, Eventration
• Usually a posterior defect in the diaphragm (Bochdalek)
• Complicaions: Bilateral pulmonary hypoplasia, Persistent fetal
circulation
• Radiography : Appearance depends on hernia contents
Bowel loops in the chest
Contralateral shift of mediastinum
Compressed Lug
Decreased bowel gas in abdomen
Abnormal position of NGT
Congenital Cystic Adenomatoid Malformatio n

• Cause: arrest of normal bronchoalveolar differentiation with


overgrowth of terminal bronchioles
• Incidence: 25% of congenital lung disorders
• M:F = 1:1
• Presents with respiratory distress and sever cyanosis, recurrent
infections
• Equal frequency in all lobes
•Imaging appearance depends on type:
Type 1: 1 or more large (2-10cm) cysts
Type 2: numerous small cysts of uniform size
Type 3: appears solid on gross inspection and imaging but
has microcysts
• Most CCAMs confined to one lobe
•Cysts typically contain air – communicate with bronchi at birth
• Can appear as a solid mass: CCAM type 3, Fluid not yet cleared from
cysts
Congenital Lobar Emphysema

• Idiopathic, congenital, progressive over-distension of one/multiple


pulmonary lobes
• Classic imaging appearance: hyperlucent, hyperexpanded lobe
• Lobar prediliction: Left upper lobe in 43%
• Presents with respiratory distress and progressive cyanosis
• M:F = 3:1
• Initially after birth lobe may appear radiodense
• causes contrlateral mediastinal shift
• Pulmonary vessels may appear attenuated
• Can be multilobar
Tracheo Eosophageal Fistula / Eosopahgeal Atresia

• Disorder in the formation and separation of the primitive foregut into


trachea and oesophagus

• 5 types
- EA without Fistula (type A)
- EA with Fistula (types B, C, D)
- TOF without atresia (type E - H-shaped fistula)

• Radiograph:
1.Air filled distended pharangeal pouch
2.Food impaction
3.Unsuccessfully passed NGT – coiled NGT
4.With TEF – gas in stomach and bowel
5.Without fistula – no distal bowel gas
6.Signs of other congenital anomalies (VACTERL)
Sequestration

• Congenital malformation consisting of:


1. Non functioning lung segment
2. No communication with tracheobronchial tree
3. Systemic arterial supply

•2 types:
1. Intralobar (75%) – eclosed by visceral pleura of affected
pulmonary lobe
2. Extralobar - accessory lobe with its own pleural sheath, which
prevents collateral air drift resulting in an airless round mass

On imaging :
•round well defined solid homogenous mass near the diaphragm with
mass effect
•Contrast enhancement of sequestration at the same time as thoracic
aorta
Bronchogenic Cyst
• Developmental lesion (a foregut duplication cyst)Best
• Do not communicate with bronchial tree
• Do not contain air unless infected
• Present with recurrent infections, stridor(from airway compression)
• May be an incidental finding
• Best imaging clue: well defined, soft tissue density mass in middle
mediastinum or central lung
• May be mediastinal or lung parencymal
• Almost always solitary
• Do not communicate with the airway
Acquired:
Respiratory Distress Syndrome
Meconium Aspiration Syndrome
Bronchial FB
Pneumothorax
Pneumomediastinum
Pulmonary Interstitial Emphysema
Croup
Epiglottitis
Exudative tracheatis
Retropharyngeal Abcess
Enlarged Tonsils
Glossoptosis

Viral Disease
Bacterial Pneumonia
Round Pneumonia
Cavitatory Pneumonia
Respiratory Distress Syndrome (RDS)

= Surfactant deficiency disorder (SDD) – immature type 2 pneumocytes


•M>F
• Most common cause of death in newborns
• Affects mostly PREMATURE infants
• Onset <2-5hrs after birth (normal film at 6 hours excludes RDS
• Increase in severity from 24 – 48hrs
• Gradual Improvement after 48 – 72hrs
• Presents with tachpnoea, nasal flaring, expiratory grunting, cyanosis
• Radiograph:
• Low lung volumes due to micro-collapse
• Generalised consolidation of variable severity
• Effacement of normal pulmonary vessels
• prominent AIR BRONCHOGRAMS
• Pleural effusions very uncommon (if present think GBS pneumonia)
• Complications include pneumothorax, PIE, pneumomediastinum
Meconium Aspiration Syndrome

= respiratory distress that occurs secondary to intrapartum or


intrauterine aspiration of meconium
• Most commonly occurs in full term or postmature infants
• Most common cause of neonatal respiratory distress in full term infants
• Aspirated meconium causes obstruction of small airways secondary to
its tenacious nature
• Leads to asymmetric areas of hyperinflation and consolidation
•Presents with respiratory distress, rarely cyanosis
• Radiographs:
1.Areas of asymmetric, patchy consolidation, atelactasis
2.Hyperinflation with areas of emphesema
3.NO AIR BRONCHOGRAMS
4.Rope like perihilar densities
5.Pleural effusion
6.Rapid clearing usually within 48hrs
Bronchial FB

= aspiration of a FB that lodges in the bronchus leading to bronchial


obstruction
• May have ball valve effect leading to hyperinflation or complete
obstruction leading to collapse
•Presentation: typically in infants/toddelrs, wheezing, cough,
sometimes fever
• Best imaging clue: static lung volume at different phases of the
respiratory cycle
• Radiograph:
1.Volume of affected lung may be normal, increased or decreased
2.Hyperinflation
3.Oligaemia
4.Atelactasis
5.Lung consolidation
Rarely (3%) aspirated FB is radio-opaque
Pneumothorax

• Presence of air within the pleural space

• Spontaneous pneumothorax
1.Primary (occurring in persons without clinically or radiologically
apparent lung disease)
2.Secondary(in which lung disease is present and apparent)
• Traumatic pneumothorax
1.Resulting from direct (blunt) chest trauma
2.Resulting from penetrating chest trauma
• Iatrogenic pneumothorax
1.Resulting from biopsy procedure
2.Resulting from therapeutic procedures

CX: pneumomediastinum and pneumopericardium , bronchopleural


fistula , tension pneumothorax, re-expansion pulmonary edema
Pulmonary Interstitial Emphysema

• Abnormal location of pulmonary air within the interstitium and


lymphatics secondary to barotrauma
• Best imaging clue: bubble-like and linear lucencies along
bronchovascular structures (often radiate from hilum)
• typically presents on routine radiographs prior to symptoms
• Serves as a warning sign for other pending air block
complications : pneumothorax/pneumomediastinum
• Occurs during the first days of life
• usually transient
• may be focal (one lobe) or diffuse and bilateral
• involved lung usually non compliant
Viral Infection of the Lower Respiratory Tract

• Evaluation of potential LRTI is one of the commonest


indications for imaging in children
• Etiology of LRTI: Viruses (commonest cause),
Mycoplasma Pneumoniae, Strep. Pneumoniae
•Presents with cough, fever, often symptoms of URTI
• Radiograph: major goal is to differentiate from bacterial
pneumonia
• Best imaging clues:
1.Lack of focal lung consolidation (hallmark for bacterial
infection)
2.Increased peribronchial markings
3.Hyperinflation
4.Subsegmental Atelactasis
5.Hilar lymphadenopathy

•RX: supportive (no need for antibiotics)


Round Pneumonia

• Bacterial pneumonia with a very round, well defined appearance on


chest radiography, simulating a mass
• Only seen up to approximately 8 years of age
• Typically caused by Stept. Pneumoniae
• Classic imaging appearance: round lung opacity with well defined
borders (+ air bronchograms)
• Presents with cough and fever
• May progress to lobar pneumonia
Cavitatory Pneumonia

• Dominant area of necrosis of consolidated lobe associated with a


variable number of thin walled cysts
• Classic appearance on CT: lack of normal lung architecture,
decreased lung enhancement, thin walled cysts
• Most commonly seen with Strep. Pneumoniae (nowadays)
• Children exhibit persistent or progressive symptoms despite
antibiotics
• Radiograph: cystic lesions develop within an area of lung
consolidated with pneumonia
• RX: needs intensive support
Cardiac Conditions
Tetralogy of Fallot
Ebstein’s Anomaly
Truncus Arteriosus
TAPVR
Transposition
Lt to Rt Shunts
Scimitar Syndrome
Hypoplastic Lt Heart Syndrome
Coarctation
Pulmonary Atresia
Kawasaki Disease
Cardiomyopathies
Rhabdomyoma
Double Aortic Arch
Pulmonary Sling
Manifestations of CHD

1. Cyanosis

2. Cardiomegaly

3. Pulmonary vasculature

4. Thymic atrophy
Cyanosis

• a clinical sign – peripheral vs central

• seen in:
1.Tetralogy of Fallot
2.Ebstein Anomaly
3.Truncus Arteriosus
4.TAPVR
5.Complete transposition of the great arteries
6.Hypoplastic left heart syndrome
7.CoArctation of the Aorta
8.Pulmonary Atresia
9...others...
Cardiomegaly

• Cardio-Thoratic Ratio
• Only on the PA view
• AP projection leads to magnifcation of heart and mediastinum

• Paediatric Conditions leading to Cardiomegaly:


1.Ebstein Anomaly
2.Truncus Arteriosus
3.TAPVR (types 1 & 2)
4. transposition of the great arteries
5.Lt to Rt shunts
6.Hypoplastic left heart syndrome
7.Vein of Galen
8.Pulmonary Atresia
9.Cardiomyopathies
10....others...
Pulmonary Vasculature
1. Increased (ex: Truncus Arteriosus)

2. Decreased (ex: Fallot’s, Ebstein)

3. Pulmonary oedema (ex: type 3 TAPVR)

4. Irregular (ex: MAPCAs in Pulmonary Atresia)


Tetralogy of Fallot

• most common cyanotic heart lesion


• Presents with clubbing, dyspnoea on exertion, episodic spells,
cyanosis by 3-4months of age

• TETRAD
1.Rt ventricular outflow tract obstruction
2.Large VSD
3.Over riding aorta
4.Rt ventricle hypertrophy

•Radiograph
1.Normal heart size
2.Rt sided aortic arch in 25%
3.Boot-shaped Heart
4.Decreased Pulmonary vasculature
Ebstein Anomaly

• Dysplastic inferiorly displaced tricuspid valve with ventricular division


into:
a) A large superior atrialized portion with thin ventricular wall
b) A small inferior functional chamber
• Associated with ASD, PFO, VSD, PDA
• Presentation: cyanosis, CHF, systolic murmur, hydrops
•M=F

• Radiograph
1. severe Rt sided cardiomegaly – box shaped heart
2.Small vascular pedicle
3.Calcification of tricuspid valve may occur
Truncus Arteriosus

= single outlet of the heart (failure of separation of the conotruncus)


• One great artery arises from the heart, large VSD
• Associated with Rt sided aortic arch, forked ribs, and DiGeorge
syndrome
• Presents with moderate cyanosis, severe CHF, systolic murmur

• Radiograph:
1.Cardiomegaly
2.Wide mediastinum
3.Concave pulmonary segment
4.Increased pulmonary blood flow
5.Waterfall / hilar comma sign
Coarctation of the Aorta

• LOCALISED Form (post-, juxta- ductal, Adult form)


-Short narrowing close to ligamentum arteriosum(most common type)
-Coexisting cardiac anomalies uncommon
-Ductus usually closed
• TUBULAR Form (preductal, Infantile form, diffuse type)
-Hypoplasia of a long segment of aortic arch
- patent ductus arteriosus
-CHF in 50%
-Coexistent cardiac anomalies

• M:F = 4:1
• Collateral circulation: via subclavian artery and its branches

•Radiograph:
•Rib notching (above age 5 years)
•Post stenotic dilatation of proximal descending aorta – figure 3 sign
•LV hypertrophy: rounded apex
Total Anomalous Pulmonary Venous Return

• entire pulmonary venous return directed to RA


•Associated with asplenia, ASD, PFO, CCAM, sequestration

A. Supradiaphragmatic TAPVR (82%)


-Type 1: Supracardiac – drainage into Lt brachiocephalic vein, SVC, azygos vein
- Type 2: Cardiac – drainage into coronary sinus, RA
B. Infradiaphragmatic TAPVR (12%)
- Type 3: drainage into portal vein, IVC, L gastric vein. >90%obstructed

Presentation: <1yr with cyanosis, systolic murmur, Rt ventricular


heave
• Radiograph:
1.Cardiomegaly (types 1& 2) vs small heart (type 3)
2.Pulmonary plethora (types 1& 2) vs pulmonary oedema (type 3)
3.Wide mediastinum (type 1 – snowman heart ) vs narrowed (type 2 &
3)
4.Lt vertical vein often visible in Type 1
Lt to Rt shunts

• Low pressure shunts - ASD


• High pressure shunts – VSD, AVSD, PDA

• Most common congenital cardiac lesion


• Presents with CHF without cyanosis
• Most small muscular VSDs close spontaneously
• Untreated large shunts may lead to Eisenmanger‘s syndrome

• Radiograph
1.Cardiomegaly
2.Convex pulmonary artery segment
3.Interstitial fluid with CHF
4.Hyperinflation due to bronchial compression by dilated pulmonary
arteries
Non Accidental Injuries (Child Abuse)
Rib fractures

• Classic imaging appearance: posterior rib fractures in the region of the costovertebral
joints, rib fractures of different ages
• Unfortunately common
• 30% of fractures in infants are due to abuse
• Secondary to thorax being squeezed by abuser
• Most children are less than 1 year at presentation
•May be subtle prior to callous formation

OTHER CHEST FINDINGS


•Scapular fractures
•Spinous process fractures
•Sternal fractures
•Lung contusions on CT

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