Congenital Cystic Adenomatoid Malformation of the Lung

Abstract A congenital cystic adenomatous malformation is a developmental lung malformation consisting of abnormal hamartomatous or dysplastic lung tissue and bronchoalveolar structures. The diagnosis can be made during fetal life through an ultrasound examination of the fetal thorax that identifies a hyperechoic cystic or solid-cystic lung mass with blood supply originating in the pulmonary artery. The prognosis is usually good but depends on the lung mass size, the degree of pulmonary compression, and the presence of fetal fluid effusions or hydrops. In those cases with poor prognosis (at risk of perinatal death), different fetal interventions have demonstrated potential benefits in improving the survival rate. However, such procedures are not curative, and therefore all cases require postnatal surgical resection of the lung mass by toracoscopy or toracotomy.