Congenital adenomatoid malformation of the lung: When is active fetal therapy indicated?

OBJECTIVE: Although aggressive fetal therapies such as thoracoamniotic shunting can be applied to cystic adenomatoid malformations of the lung diagnosed in utero, there is no clear consensus regarding their indications. Our purpose was to evaluate a management policy in which aggressive fetal therapy was restricted to those cases complicated by major polyhydramnios or hydrops; all other cases were managed conservatively. STUDY DESIGN: A prospective cohort study of 33 cases with a prenatal diagnosis of cystic adenomatoid malformations of the lung was performed. Thoracoamniotic shunting was offered only in nine macrocystic cases with acute polyhydramnios or hydrops. RESULTS: Four cases were diagnosed postnatally as sequestrations. Of 12 cases complicated by acute polyhydramnios or hydrops, 5 survived (1 type III with spontaneous incomplete resolution in utero, 4 type I with substantial volume reduction after shunting). The 17 cases without acute polyhydramnios or hydrops were managed conservatively and survived. CONCLUSION: Conservative management is indicated in cases of cystic adenomatoid malformations of the lung without acute polyhydramnios or hydrops.