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CONGENITAL ANOMALIES (CONGENITAL CYSTIC ADENOMATOID MALFORMATION (usually…
CONGENITAL ANOMALIES
CONGENITAL CYSTIC ADENOMATOID MALFORMATION
rare form of diffuse hamartoma (neoplasm of various tissues)
normal tissues of lung just not arranged in appropriate way for functioning
in lungs of premature or stilborn infants
usually in one lobe
enlarged
white
firm
fibrous
small cysts
microcystic or macrocystic
honeycomb lung
TYPES:
unilateral agenesis
congenital pulmonary hypoplasia
pulmonary sequestration
congenital cysts
congenital cystic adenomatoid malformation (CCAM/CPAM)
PULMONARY SEQUESTRATION
portion of lung doesn't have proper bronchial and vascular connections
Blood supply by abnormal systemic arteries
TYPES:
intralobar
extralobar
2ry fragment of lobe with abnormal blood and bronchial supply
CONGENITAL CYSTS
possibly communicate with bronchial tree
Bronchial/bronchogenic cysts
lined by bronchial epithelium
walls contain cartilage, smooth muscle, bronchial glands
Enterogenous cysts
derived from foregut
lined by gastric / intestinal epithelium
CONGENITAL PULMONARY HYPOPLASIA
Aetiology
compression of lungs
by uterus in oligohydramnios
intrathoracic masses (eg diaphragmatic hernia)
deformities of thorax or neuromuscular abnormalities
congenital abnormalities of blood vessels
renal & urinary tract anomalies causing oligohydramnios
chronic amniotic fluid leak
unilateral
agenesis
may have accompanying malformations