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HAZEM ALI
 CONG ENITAL
P ULM ON AR Y A IRWAY
MA LFORM ATION
HAZEM ALI
CLINICAL
Rare (1 per 25,000 births)

formerly known as congenital cystic adenomatoid malformation

Most cases diagnosed Ante-natally (by U/S)

Many cases show spontaneous regression

Can present Post-natally as:

Stillbirth with generalized anasarca
Infants with respiratory distress (cyanosis, grunting, tachypnea)
Asymptomatic infants
May present later in childhood with recurrent infection, cough or dyspnea
So they need close follow-up and elective surgery for persistent lesions within first year of life
CLINICAL
Show communication with tracheobronchial tree

Five pathological subtypes (0, 1, 2, 3, 4)

Most common is type 1
Can be surgically corrected (good prognosis)

Most rare is type 0

Incompatible for life (poorest prognosis)

Type 2 associates with 50% of cases of extra-lobar pulmonary sequestration

Also can associate other anomalies like renal agenesis, diaphragmatic hernia, and
cardiovascular anomalies
GROSS
More common types are usually Cystic lesions

Type 1 Type 2 Type 4

Number Single or multiple cysts
Size Large Small Large
> 2 cm < 2 cm > 2 cm
Wall Mostly thin wall
Content Clear or mucin
Location Within the lung
Communication Present
with
tracheobronchial
tree
All these types have good prognosis EXCEPT type 2
GROSS

Type 1 Type 2
GROSS
Less common types are usually Solid lesions

Type 0
Also called acinar dysplasia or dysgenesis
Widespread and bilateral
Lungs appear small, firm, with fine nodularity on the surface

Type 3
Large, firm, and solid mass
Can involve entire lung
Can cause mediastinal shift to opposite side and compress the other lung

Both types have poor prognosis

 MICROSCOPY
Type 1 Type 2 Type 4
Size Large cysts Small cysts, back-to-back Large cysts
Outline Bronchial-like Bronchiolar-like Alveolar-like
Dilated and irregular
Lining Pseudostratified columnar Single cuboidal to Flattened epithelium
ciliated epithelium, with columnar ciliated Type I & II
Interspersed mucous cells epithelium pneumocytes
Sub- Fibro-vascular stroma Fibro-vascular stroma Loose mesenchymal
epithelium Smooth muscle fibers Smooth muscle fibers stroma
No/rare cartilage islands No cartilage
Interspersed
alveolar ducts
Skeletal muscle
fibers could be seen

Other Risk for Bronchioalveolar Rare subtype called Uncertainrelationto

features carcinoma Rhabdomyomatous Pleuropulmonary
Lepidic growth within dysplasia Blastoma type I
cysts and adjacent lung
MICROSCOPY

Type 2
Type 1
MICROSCOPY
Type 0
Disorganized bronchial-like structures
Respiratory epithelium
Fibro-muscular connective tissue
Cartilage

Type 3
Disorganized bronchiolo-alveolar structures
Lined with cuboidal epithelium gland-like
Simulate fetal lung
DIFFERENTIAL DIAGNOSIS
Other congenital / cystic lung diseases
Congenital: Sub-epithelial mucous glands and
cartilage
Bronchogenic cysts Does not communicate with the
Congenital pulmonary cysts tracheobronchial tree
Pulmonary sequestration Extra-lobar Located outside the lung
Congenital lobar emphysema Has a systemic rather than a
pulmonic blood supply
Acquired: Does not communicate with the
Emphysema tracheobronchial tree
Healed abscess
Honeycombing
Mixed:
Cystic fibrosis
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HAZEM ALI