Download - FlashPath - Lung - Congenital Pulmonary Airway Malformation

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Page 1: FlashPath - Lung - Congenital Pulmonary Airway Malformation

FLASHPATHH A Z E M A L I

Page 2: FlashPath - Lung - Congenital Pulmonary Airway Malformation

CONGENITAL PULMONARY

AIRWAY MALFORMATION

H A Z E M A L I

Page 3: FlashPath - Lung - Congenital Pulmonary Airway Malformation

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

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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

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GROSS• More common types are usually Cystic lesions

• All these types have good prognosis EXCEPT type 2

Type 1 Type 2 Type 4Number S i n g l e o r m u l t i p l e c y s t s

Size Large(up to 10 cm)

Small(up to 2 cm)

Large(up to 10 cm)

Wall M o s t l y t h i n w a l lContent C l e a r or m u c i n Location W i t h i n t h e l u n g

Communication with

tracheobronchial tree

P r e s e n t

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GROSS

Type 1 Type 2

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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 bulky mass– Can involve entire lung– Can cause mediastinal shift to opposite side and compress the other lung

• Both types have poor prognosis

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MICROSCOPYType 1 Type 2 Type 4

Size Large cysts Small cysts, back-to-back Large cystsOutline Bronchial-like Bronchiolar-like

“Dilated and irregular”Alveolar-like

Lining Pseudostratified columnar ciliated epithelium, with Interspersed mucous cells

Single cuboidal to columnar ciliated epithelium

Flattened epithelium “Type I & II pneumocytes”

Sub-epithelium

• Fibro-vascular stroma• Smooth muscle fibers• No/rare cartilage islands

• Fibro-vascular stroma• Smooth muscle fibers• No cartilage• Interspersed

alveolar ducts• Skeletal muscle

fibers could be seen

Fibro-vascular stroma

Other features

Lepidic growth within cysts and adjacent lung• Risk for Bronchioalveolar

carcinoma

Rare subtype called Rhabdomyomatous dysplasia

Can simulate Pleuropulmonary blastoma

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MICROSCOPY

Type 1

Type

2

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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

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DIFFERENTIAL DIAGNOSISO t h e r c y s t i c l u n g d i s e a s e s :• Congenital:

– Bronchogenic cysts– Congenital pulmonary cysts– Pulmonary sequestration “Extra-lobar”– Congenital lobar emphysema

• Acquired:– Emphysema– Healed abscess– Honeycombing

• Mixed:– Cystic fibrosis

• Located outside the lung• Has a systemic rather than a

pulmonic blood supply• Does not communicate with the

tracheobronchial tree

• Sub-epithelial mucous glands and cartilage

• Does not communicate with the tracheobronchial tree

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