Cystic adenomatoid malformation of the lung causing hydrops fetalis: case report and review of the literature.

Objective: Fetal congenital cystic adenomatoid malformation (CCAM) is a rare lung abnormality with a highly variable prognosis depended on the presence of fetal hydrops and the size of the cysts. In case of fetal hydrops the prognosis is fatal without intervention.

Methods And Design: Case report and literature review.

Setting: We report on the ultrasound and pathological findings of a hydropic fetus due to a CCAM Type II at 22 weeks of gestation.

Conclusions: Congenital cystic adenomatoid malformation is a rare fetal lung disease with an excellent prognosis in the absence of fetal hydrops. CCAM associated with fetal hydrops carries a grave prognosis but survival rates of 70% can be achieved by thoraco-amniotic drainage in those with macrocystic lesions.