Congenital cystic adenomatoid malformation is a lung bud lesion characterize by dysplasia of respiratory epithelium caused by overgrowth of distal bronchiolar tissue. Prenatally diagnosed CCAM prognosis depends on the size of the lung lesion and can cause: mediastinal shift, hypoplasia of normal lung tissue, polyhydramnios, and fetal hydrops (cardiovascular shunt). Classified in two types based on ultrasound findings: macrocystic (lobar, > 5 mm cysts, anechoic, favorable prognosis) and microcystic (diffuse, more solid, echogenic, lethal). Occurs as an isolated (sporadic) event with a low rate of recurrence. Survival depends on histology. Hydrops is caused by vena caval obstruction, heart compression and mediastinal shift. The natural history is that some will decrease in size, while others disappear. Should be follow with serial sonograms. Prenatal management for impending fetal hydrops has consisted of thoraco-amniotic shunts (dislodge, migrate and occlude), and intra-uterine fetal resection (technically feasible, reverses hydrops, allows lung growth). Postnatal management consist of lobectomy.
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