Long-term neurodevelopmental outcome and brain volume after treatment for hydrops fetalis by in utero intravascular transfusion.

Objective: We tested the hypothesis that long-term neurodevelopmental outcomes of successfully treated fetuses with immune hydrops are similar to their unaffected siblings according to a protocol that addresses the underlying pathophysiologic condition.

Study Design: Sixteen of 18 consecutive hydropic fetuses (89%) who were treated in a dedicated fetal medicine unit between July 1985 and October 1995 survived. The transfusion protocol used a 2-step correction over a 2 to 4 day interval, combined with umbilical venous pressure measurements to avoid over transfusion and bicarbonate administration to assure a posttransfusion UV pH of >7.30. Survivors were evaluated at a mean age of 10 years. Statistical analyses included t-test, Wilcoxon rank-sum test, Fisher's exact test, and Pearson coefficients.

Results: Overall, death or major neurologic morbidity occurred in 4 of 18 of the fetuses (22%) who were treated (2/16 of survivors [12.5%]). Among the survivors, the children with immune hydrops had physical, neurologic, and cognitive outcomes statistically similar to their siblings, except for a measure of visual attention. Two of the children (12%) had major neurologic sequelae. Brain volumes were statistically smaller than unrelated control subjects by 8.8%, but these control subjects were not matched for height at testing or gestational age at birth. Both groups had brain volumes within the normal range.

Conclusion: Intravascular transfusion of fetuses with profoundly anemic immune hydrops results in high survival rates and favorable long-term neuropsychological outcomes.