Pulmonary sequestration presenting as fetal hydrops

Pulmonary sequestration is a rare developmental anomaly of the lung characterized by nonfunctional pulmonary tissue without communication with the tracheobronchial tree and receiving an aberrant systemic arterial blood supply. Few cases of non-immune hydrops fetalis associated with this entity have been reported. A 2 day old male baby born by cesarean section at 31 weeks gestational age due to fetal hydrops is presented. Autopsy revealed a hydropic baby with extralobar pulmonary sequestration and bilateral pulmonary hypoplasia. The clinicopathologic presentation of this unusual pulmonary developmental anomaly is discussed.