Nonimmune hydrops fetalis, pulmonary sequestration, and favorable neonatal outcome

Abstract

The association of pulmonary sequestration and nonimmune fetal hydrops reportedly carries a very poor prognosis for survival. We describe three newborns with good outcomes despite the diagnosis of pulmonary sequestration; two cases were associated with hydrops fetalis and one with isolated fetal ascites. Two neonates with severe hydrops fetalis had pulmonary sequestration diagnosed postnatally. A third infant presented early in gestation with marked fetal ascites that regressed spontaneously before delivery; this infant also had pulmonary sequestration. Despite severe respiratory insufficiency requiring aggressive management, all three infants survived after surgical resection of the sequestered lung mass. These cases demonstrate the difficulties associated with antenatal counseling regarding long-term prognosis for infants with nonimmune hydrops and pulmonary sequestration. With optimal care in a tertiary perinatal center, a less pessimistic outlook than previously described in the literature may be appropriate.