14th World Congress in Fetal Medicine A case of hemolytic disease of the fetus with ten intrauterine blood transfusions Braga JRS, Pritsivelis C, Andrade RP, Marzano MOC, Pereira MR, Amin Jr J, Cunha AA Maternidade Escola da Universidade Federal do Rio de Janeiro, RIO DE JANEIRO, Brazil Objective The widespread administration of rhesus immunoglobulin has resulted in a marked decrease in the alloimunization caused by the RhD antigen. However, cases continue to occur because of maternal sensitization in the two first trimesters of pregnancy, inadvert omission of RhIG and inadequate dosing after delivery where there has been an excessive fetomaternal haemorrhage. The literature refers to a series of hundreds of cases with a mean of three intrauterine transfusions. Sometimes, they are not enough, demanding some extra transfusions. The objective of this study is to report a case with ten intrauterine blood transfusions. Methods Case report. Results On 07/30/2014, a 29 year old patient, at 20 weeks of gestation was referred to Maternidade Escola da Universidade Federal do Rio de Janeiro, located at Rio de Janeiro, Brazil, with the diagnosis of perinatal hemolytic disease. She had two previous pregnancies, one with a vaginal birth and a caesarean section. The second child had a history of neonatal jaundice and cerebral palsy. She had taken anti-Rh immunoglobulin after the last pregnancy. Her blood type is 0 (-). The Coombs test was 1/256. The red blood cells panel showed anti-D antibodies. On the same day an obstetric ultrasound revealed polyhydramnios and a placental thickness of 32mm. The fetus had biometric measurements compatible with 21 weeks, consistent with her gestational age. Doppler of the middle cerebral artery showed a maximum speed of 63cm/s (2. 25MoM). Intrauterine transfusion was performed on 08/01/2014. The initial hematocrit was 27. 8%. A transfusion of 12ml of red blood cells was performed and the post transfusion hematocrit was 28. 2%. Since then, ten intrauterine transfusions were performed, as summarized in Table 1. Date Gestacional age (weeks) Estimated fetal weigth (g) Coombs test (1: x) Pre transfusion hematocrit (%) Transfused volume (ml) Pos transfusion hematocrit (%) 08/01/14 21 460 256 27. 8 12 28. 2 08/13/14 22+5d 700 256 6. 9 28 28 08/20/14 23+5d 820 256 19 30 30 08/27/14 24+5d 933 256 28. 9 20 33 09/03/14 25+5d 980 256 3 35 32 10/09/14 26+5d 1, 132 512 23 34 33 09/18/14 27+6d 1, 260 512 22 25 30 09/24/14 28+5d 1, 450 1, 024 15. 9 57 33. 3 10/01/14 29+5d 1, 590 1, 024 29. 3 40 39. 9 10/15/14 31+5d 2, 076 2, 048 26. 9 50 37. 9 Table 1. On 10/29/2014, two weeks after the last intrauterine transfusion, at 34+2 weeks of gestation, she underwent a c-section. A boy was born with 2, 540g and Apgar score 7-8. The patient developed respiratory distress, being necessary nasal CPAP for a day. There was significant improvement in respiratory symptoms, staying in room air since then. He was treated with triple phototherapy soon after birth. Umbilical cord blood was tested indicating a hematocrit of 37%, total bilirubin 5. 6mg/dl and indirect bilirubin 4. 8mg/dL. Exchange transfusion was performed with about nine hours of life without complications. Remained in phototherapy for five days. He received immunoglobulin for two days. During the hospital stay, he had six episodes of supraventricular tachycardia. Vagal maneuver was attempted, initially without success. Later, pharmacological treatment with amiodarone was started. The neonatal echocardiography was normal. He was discharged on 11/10/2014, with thirteen days and with 2, 610g weight. On that day, was in exclusive breastfeeding and the hematocrit was 48%. Conclusion In this case, a severe type of hemolytic disease was detected during pregnancy, which was caused by anti-D. Proper antenatal follow up of all pregnant women who are Rh D negative with a positive antibody screen and/or a history of previous babies with signs of HDN is required. Antenatal ultrasound may detect signs of hydrops fetalis. Doppler ultrasound of the middle cerebral artery (MCA) has largely replaced fetal blood sampling as an initial test for the detection of fetal anemia by measuring the peak systolic velocity in MCA. The risk of anemia is high in fetuses with a peak systolic velocity of 1. 50 times the median or higher. According to Mari (2000), an elevated peak MCA velocity of >1. 5 multiples of the median is useful in the timing of the initial intrauterine transfusion in the red cell-alloimmunized pregnancies. Fetuses with values below 1. 50 either do not have anemia or have only mild anemia. The fact that this test does not predict mild anemia well is not clinically important, because no intervention is indicated in fetuses with mild anemia. In this case, the Doppler was helpfull to make the diagnosis of fetal anemia. Surprisingly the hematocrit was very low 12 days after the first transfusion. After this, the intrauterine transfusions were performed weekly. The hematocrit was very low at most of the times, before the transfusions. Making intrauterine transfusions almost every week, the pregnancy ended at the 34th week, when interruption was indicated. A healthy newborn was born, without apparent sequelae..