Grayscale and Doppler sonographic evaluation of response to in utero treatment of hydrops fetalis caused by extralobar pulmonary sequestration

Pulmonary sequestration is defined as nonfunctional lung tissue that lacks communication with the bronchial tree and that is supplied by an anomalous systemic vessel. In comparatively rare cases, pulmonary sequestration may lead to hydrothorax or hydrops fetalis, which is nearly universally fatal. In this report, we describe a case of pulmonary sequestration with hydrops fetalis, which was successfully treated by thoracoamniotic shunting. A sonographic Doppler study in this case suggested that the underlying mechanism of the hydropic change in a fetus with extralobar pulmonary sequestration may have differed from that in fetuses with primary hydrothorax not associated with a structural anomaly.