Congenital Adenomatoid Cystic Malformation: Expectant Management Outcomes

Abstract

Objective: Our purpose was to describe the evolution and perinatal outcome of Congenital Adenomatoid Cystic Malformation (CCAM) according to prenatal sonographic features in expectantly managed fetuses. Materials and Method: A retrospective review of all prenatally diagnosed CCAM cases from 1995 to 2017 at a perinatal referral center in Mexico City was conducted. Results: Twenty-seven cases were identified. Mean gestational age at the time of diagnosis was 26.5 weeks. According to Stocker ́s classification, there were eight type 1 (29.6%), ten type 2 (30%) and nine type 3 (33.4%) cases. No bilateral lesions were found. Hydrops was observed in two cases (7.4%). Lesion dimensions remained stable throughout gestation in most cases (63%), ultrasonographic regression was observed in nine fetuses (33.3%). There were no elective pregnancy terminations. Fetal demise was reported in two cases. Mean gestational age at birth was 37.1 weeks. Most newborns required only usual neonatal care. After birth, two cases showed progressive deterioration which lead to early neonatal death. Conclusion: CCAM is a relatively rare abnormality of lung development, which can be accurately diagnosed by prenatal ultrasound. Conservative management is appropriate in most cases, with a favorable outcome and a chance of regression of up to one-third of the cases.