RESUMO
Congenital Cystic Adenomatoid Malformations (CCAM) of the lung are a relatively rare anomaly. The majority of cases do not require any antenatal or perinatal interventions. However, selected cases need special and intensified management. This article discusses a case of a CCAM of the left pulmonary lobe covering 2/3 to 3/4 of the thoracic volume, causing mediastinal shift and hydrops. The enormous size required the planning of an EXIT (Ex Utero-Intrapartum Treatment) procedure, which was performed when the trial of intraoperative ventilation of the lungs was unsuccessful. A thoracotomy and resection of the left upper lobe were performed with the fetus on feto-placental circulation. After resection and closure of the thoracotomy, improvement of lung ventilation allowed the delivery of the child. The child and mother were discharged in a healthy condition. We conclude that the EXIT procedure is a feasible and potentially life-saving method for select cases of prenatally detected CCAM.