Nonimmune hydrops fetalis, pulmonary sequestration, and favorable neonatal outcome

Abstract

BACKGROUND: 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. CASES: 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. CONCLUSION: 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.