P06.07: Prenatal Diagnosis and Outcome of Congenital Cystic Adenomatoid Malformation of the Lung: a Report of 9 Cases

To describe the antenatal ultrasound findings and outcome of fetuses with congenital cystic adenomatoid malformation of the lung (CCAM). We used the classification of Adzick for describe the CCAM. The gravity of the pathology has been established according to the type, localization and size of the lesion, the CCAM volume ratio (CVR), and the presence of fetal complications (hydrops, mediastinal shift, polyhydramnios). For differential diagnosis between CCAM and pulmonary sequestretion was performed color Doppler. Cases with CVR > 1.6 and associated with fetal complications or associated with fetal malformations at the time of diagnosis were interpreted as cases with severe evolution. In these situations we discussed with patients about elective termination. In cases with favorable evolution we practiced serial scans. We have not practiced in-utero percutaneous pulmonary drainage in fetuses. Starting with 2012 we practiced prenatal maternal treatment with betamethasone in cases with microcystic CCAM. Newborns have conducted chest X-ray scan and surgical clinical examination. In cases with elective termination was practiced necropsy. Between 03.01.2005-31.03.2013 we diagnosed 9 cases with CCAM at median gestational age of 23 (range, 18–30) weeks. Severe CCAM was identified in 6 cases, in which the elective termination of pregnancy was practiced. Necropsy was practiced in five cases and confirmed prenatal diagnosis. In three of the 9 cases the lesions was decrease, of which a case has been with maternal prenatal treatment with betamethasone. To all those 3 newborn prenatal diagnosis has been confirmed by chest X-ray scan. None of them required postnatally surgical treatment. In severe macrocystic CCAM without surgical treatment (in-utero percutaneous pulmonary drainage) the fetal prognosis remains reserved. In microcystic CCAM maternal prenatal treatment with betamethasone may improve the fetal and neonatal prognosis.