Unexpected intrauterine death following resolution of hydrops fetalis after betamethasone treatment in a fetus with a large cystic adenomatoid malformation of the lung

Abstract

Without intervention, the development of hydrops fetalis secondary to cystic adenomatoid malformation of the lung (CAML) implies a 100% mortality rate. Conversely, four CAML cases with in-utero resolution of hydrops fetalis after prenatal betamethasone therapy with good clinical outcome have been reported in the literature. The mechanism is speculated to be the effect of corticosteroid in improving lung maturation. Here we present another CAML case with resolution of hydrops fetalis after prenatal betamethasone therapy but which was followed by later intrauterine death. We speculate that the resolution of the CAML lesion itself may be more important in determining the clinical outcome than the resolution of hydrops. The association between prenatal betamethasone therapy and resolution of hydrops may be related to a different mechanism other than improvement of lung maturation.