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.