Post on 19-Jan-2017
transcript
FLASHPATHH A Z E M A L I
CONGENITAL PULMONARY
AIRWAY MALFORMATION
H A Z E M A L I
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
• 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
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 bulky mass– Can involve entire lung– Can cause mediastinal shift to opposite side and compress the other lung
• Both types have poor prognosis
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
MICROSCOPY
Type 1
Type
2
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 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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