Non-immune hydrops fetalis and bilateral pulmonary hypoplasia in a newborn infant with extralobar pulmonary sequestration

Extralobar pulmonary sequestration was found in a newborn premature infait that presented with non-immune hydrops fetalis, massive bilateral hydrothorax and polyhydramnios in utero. The baby died of severe respiratory insufficiency 15 h after birth. Postmortem examination revealed distended lymphatic vessels in the sequestered lung tissue probably due to impeded lymph drainage. We suggest that not extralobar pulmonary sequestration itself but a subsequent massive unilateral hydrothorax due to severe obstruction of lymph drainage was the cause of the non-immune hydrops fetalis, pulmonary hypoplasia and polyhydramnios. If these symptoms are diagnosed before delivery, a search for extralobar pulmonary sequestration is indicated.