Congenital cystic adenomatous malformation of the lung (CCAM) is a rare lung lesion easily diagnosed on prenatal scan. The pathology of fetal lesions differs from postnatal lesion, hence the need for separate classifications during the different stages of development. Fetuses with CCAMs and hydrops have a poor prognosis and may be candidates for prenatal intervention where available. Most prenatally diagnosed CCAM have a favourable outcome. Early surgery is required for symptomatic babies. Management of prenatally diagnosed asymptomatic lesions remain controversial with the option of conservative management with CT scan surveillance or surgical excision. Surgical excision is favoured by many centres because of the risk of infection and malignant transformation if the CCAM remains in situ. Surgical outcomes are excellent.
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