The authors report the first successful case of fetal exchange transfusion performed in Rh disease. The patient was a gravida 3, para 1; a first ultrasound guided intrafunicular transfusion was carried out at 28 gestation weeks (Coombs test: 256, Liley’s chart: zone III, fetal hemoglobin: 5.7 g/l). One week later a fetal exchange transfusion was decided because of the appearance of ascites, sinusoidal heart rate pattern, and Manning’s score at 4. At 29 weeks of gestation an ultrasound-guided umbilical cord puncture was performed with a 16-gauge Tuohy needle, a catheter for epidural analgesia (Perifix®) was inserted through the trocar. A total exchange of+ 126/-96 cm3 of 50 hematocrit (Hct) concentrated and irradiated blood without leukocyte and platelet was performed increasing fetal hemoglobin concentration from 3.9 to 11.9 g/l. A 2,040-gram girl was delivered by cesarean section at 33 gestation weeks with Apgar score of 7/9/9 and she was given 4 total exchange transfusions in the Neonatal Intensive Care Unit. Two years later her development is normal. This new procedure seems easier than fetoscopy to achieve umbilical cord puncture and avoid excess blood volume while exact correction of anemia is possible. With more concentrated blood (70 Hct) the duration of the procedure is short
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