Background/purpose: Most babies born with idiopathic nonimmune hydrops fetalis (NIHF) suffer generalized cardiopulmonary collapse and die despite maximal medical therapy. With reported survival rates of less than 10%, many centers consider NIHF an unsalvageable situation and the babies who have this condition, untreatable. In this study, the authors questioned if the aggressive use of extracorporeal life support (ECLS) could salvage this condition and improve the chances of survival for babies born with NIHF.
Methods: The Extracorporeal Life Support Organization's (ELSO) neonatal registry was searched for all available information on babies treated for hydrops fetalis. The ELSO records of all hydropic babies were then reviewed to exclude those babies who had identifiable causes of hydrops. Survival statistics were then calculated for the remaining core group of idiopathic NIHF babies before separating them into two groups based on survival. A detailed analysis comparing the survivors with nonsurvivors was then performed.
Results: A total of 28 hydropic babies were identified in the ELSO registry. Four babies were excluded from analysis because of identifiable causes of hydrops (two with congenital diaphragmatic hernia, one with Rh incompatibility, and one with fetal anemia). Of the remaining 24 babies who had NIHF, 54% (13 babies) survived the neonatal period and were discharged from the hospital. Analysis comparing the survivors with the nonsurvivors in our study showed that the groups were similar in their gestational ages, birth weights, Apgar scores and the time to initial intubation. The most distinguishing factor of survival in our study was that the survivors, on average, received ECLS support 3 days sooner than nonsurvivors (mean, 17.5 +/- 1.3 hours of life for survivors v 105 +/- 36.6 hours for nonsurvivors, P < or = .05).
Conclusion: Idiopathic NIHF should no longer be considered an untreatable condition but a new indication for ECLS that, when begun early, may significantly improve the chances of survival for these babies previously considered "unsalvageable."