Congenital lung abnormalitiesDr Aftab Qadir

1.TRACHEOBRONCHIAL ABNORMALITIES•Tracheal agenesis•Tracheal stenosis•Tracheomalacia•Tracheo-oesophageal fistula (TOF)•Bronchial atresia•Tracheal bronchus (PIG bronchus)

2.PULMONARY UNDER DEVELOPMENT•Lung agenesis•Lobar under development•Scimitar syndrome

3.Bronchopulmonary foregut malformation•Bronchogenic cysts•Enteric cysts•Neuroenteric cysts•CCAM•Pulmonary sequestration

5.DIAPHRAGMATIC ABNORMALITIES•Congenital diaphragmatic hernia•Congenital eventeration of the diaphragm

Tracheal agenesis•Rare •Commonly associated with maternal

polyhydramnios•Presentation is immediate and acute with

severe respiratory distress, absent cry and inability to intubate the airway.

•There are three main forms of tracheal agenesis:

Tracheal stenosis•Congenital stenosis due to complete

cartinogenous rings is rare. •50% focal, 30% generalised ,20% funnel

shaped•90% of affected children present in the

first year of life with biphasic stridor. •CT is useful in assessing the anatomy and

has the added advantage of angiographic capabilities.

•MRI and bronchoscopy

Tracheomalacia•Softening of the tracheal wall, due to

cartilaginous abnormalities. •Commonest type is secondary to

tracheostomy, oesophageal atresia/TOF •Chronic inflammation(associated with

cystic fibrosis, recurrent aspiration, immuno-deficiency)

•Extrinsic compression (vascular rings, slings or aberrancy)

•Neoplasia

Causes expiratory wheeze•Fluoroscopy shows an exaggerated

decrease in the sagital width of the trachea during expiration.

•Dynamic CT can be useful to assess the cross-sectional anatomy and compliance of the trachea.

Tracheo-oesophageal fistula (TOF)•Majority of cases are associated with the

presence of oesophageal atresia. •May present with choking, cyanosis,

coughing at the time of feeding.•Contrast oesophagram is used to

demonstrate the presence of a fine hair-like structure connecting the oesophagus and trachea with linear opacification of the posterior tracheal wall

Bronchial atresia•The upper lobe bronchi are more

frequently affected by congenital atresia of lobar or segmental bronchi.

•May be associated abnormalities such as bronchogenic cyst, intralobar sequestration or cystic adenomatoid malformation.

Tracheal bronchus ( Pig bronchus)•Incidence is 1 % of the normal population•Right upper lobe bronchus arises directly

from the trachea

PULMONARY UNDER DEVELOPMENT•Agenesis, aplasia and hypoplasia.•Agenesis is complete absence of a lung

or lobe with absent bronchi •Aplasia is absence of lung tissue but the

presence of a rudimentary bronchus•Hypoplasia is the presence of both

bronchi and alveoli in an underdeveloped lobe

Lung agenesis•Recognizable with a small opaque

hemithorax, displacement of mediastinal structures towards that side.

•Bronchography or bronchoscopy confirms the absent main stem bronchus

•Angiography shows no pulmonary or bronchial arterial circulation.

Scimitar syndrome• Unique form of lobar agenesis or aplasia• Common feature hypoplasia or aplasia of one or

more lobes of the right lung. • The hemithorax is small, with obscuration of the

heart border and a retrosternal soft-tissue density

• Anomalous vein has the appearance of a Turkish scimitar, which normally drains to the IVC

• The right pulmonary artery may be absent• Systemic vessel arising from the lower thoracic

or upper abdominal aorta supplying the right lower lobe.

Congenital lobar over inflation/emphysema

•Characterised by progressive over distension of a lobe

•Aetiology is unknown in 50% of cases•Male to female ratio is 3:1 •Associated anomalies include the patent

ductus arteriosus, ventricular septal defect and tetralogy of Fallot

•The upper lobes, or right middle lobe, are commonly involved.

Bronchopulmonary foregut malformation•Bronchogenic cysts •Enteric and •Neurenteric cysts•Cystic hamartomatous (adenomatous)

malformation •Pulmonary sequestration.

Bronchogenic cysts•50% of all congenital thoracic cysts •May be intrapulmonary or mediastinal

bronchogenic •Can be paratracheal (usually right sided,

carinal or hilar) •Carinal location is most common. •Bronchogenic cysts do not usually

communicate with the tracheobronchial tree

Enteric cysts•Located in the posterior mediastinum. •If present in the oesophageal wall these

are referred to as oesophageal cysts or duplication cysts.

•Mediastinal uptake of 99 Tc-MDP (pertechnetate)contain gastric mucosa.

Neuroenteric cysts•Present as posterior mediastinal masses

with associated vertebral abnormalities.•MRI is the most useful tool for evaluating

the thoracic and spinal components of neuroenteriec cysts

CCAM•Hamartomatous proliferation of terminal

bronchioles •Composed of both solid and cystic tissue. •Malformations are classified on the basis

of clinical, radiographic and histological features:

•Type 1,2 and 3

Pulmonary sequestration•Congenital mass of aberrant pulmonary

tissue•No normal connection with the bronchial

tree or with the pulmonary arteries.•Usually located in one of the basal

segments of the lower lobe. •Intralobar sequestration (ILS) is

contained within the lung with no separate pleural covering

CHARACTERSTIC

INTRALOBAR

EXTRALOBAR

Incidence More common ( 75 %) Less common( 25 %)

Gender predisposition Equal Men 4: 1

Pleural investment Shares visceral pleura of parent lobe

Separate visceral pleura

Location Posterior basal segments(Approx. 60% on left)

Above, below or withindiaphragm(Approx. 90% on left)

Venous Drainage Pulmonary venous Systemic venous (azygos, IVC, portal)

Presentation Early adulthood with a history of pulmonary infection, chronic cough, or asthma.Asymptomatic mass (15%)

Mostly present during first 6 months of lifedue to respiratory orfeeding problems

RadiographicFeatures

Homogeneousconsolidation withirregular margins oruniformly dense masswith smooth or lobulatedcontours.

Single well defined,homogeneous, triangularshaped opacity in the lowerthorax. May presentelse where in the thoraciccavity.

Pulmonary arteriovenous malformations• Congenital or acquired. • The acquired connections arc called

pulmonary fistulas. • Congenital arteriovenous malformations are

abnormal communications between pulmonary arteries and veins

• No intervening capillary bed and are often clinically silent,

• 60% are in the lower lobes• Typical appearances are of a well-defined

pulmonary mass which is often lobulated.

DIAPHRAGMATIC ABNORMALITIES•Hernia •Eventeration •Agenesis

Congenital diaphragmatic hernia•Boakdalek hernia-Posteropleuoperitoneal

foramen•Causes severe respiratory distress in the

neonate •Involves the left pleuroperitoneal foramen

in 75 % of cases.•The prognosis correlates with the degree

of underlying lung hypoplasia

•Neonatal radiograph shows a left-sided large intrathoracic mass of soft-tissue density

•There is absence of the normal gas-containing bowel in the abdomen.

Congenital eventeration of the diaphragm•Either partial or complete •Often right sided, due to hypoplasia of the

diaphragmatic muscle. •Most eventerations are minor, transitory,

local diaphragmatic elevations found incidentally within the first few years of life

•Disappear with age.

CYSTIC FIBROSIS•Autosomal recessive trait •Prevalence of approximately 1 in 2500. •Chronic respiratory illness

Imaging • Atelectasis• Mucoid impaction• Focal or generalized overinflation• Cylindrical or cystic bronchiectasis• Bronchial wall thickening • Hilar adenopathy• Pulmonary arterial hypertension and cor

pulmonale• Recurrent pneumonias• Hypertrophic osteoarthropathy • Recurrent pneumothorax is common

Few cases

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