Prenatal diagnosis and management of fetal xerocytosis associated with ascites.

OBJECTIVES: To discuss the prenatal diagnosis and management of fetal xerocytosis associated with ascites.

CASE REPORT: A 29-year-old woman with hereditary xerocytosis was found to present a fetus with severe ascites on the 20-week scan. Cordocentesis showed mild anemia and blood transfusion was discarded. Ascites worsened and 2 weeks later a new cordocentesis showed lower hematocrit values. Blood transfusion was performed but ascites remained unchanged. Cordocentesis was repeated at 28 weeks and albumin was transfused. Fetal hemoglobin was within the normal range. Peak systolic velocity of the middle cerebral artery remained normal and correctly predicted mild anemia. Expectant management was followed. An elective cesarean section was performed at 32 weeks because of breech presentation and preterm labor which did not respond to aggressive tocolysis. A female newborn weighing 2,615 g was delivered and required paracenteses and exchange transfusion. The newborn was discharged at 4 weeks of life and at 2 months of age, the ascites resolved completely.

CONCLUSIONS: The mechanism of development of ascites in fetal xerocytosis remains unanswered. As ascites does not seem to be related to fetal anemia or hypoalbuminemia, does not substantially change after blood transfusion and tends to resolve in late gestation, a conservative management is reasonable if fetal anemia is not severe.