Outcomes of haemoglobin Bart's hydrops fetalis following intrauterine transfusion in Ontario, Canada.

OBJECTIVES: With improved access to intrauterine transfusion (IUT), more fetuses with haemoglobin Bart's hydrops fetalis (HBHF; homozygous α0-thalassaemia) will survive. DESIGN: To evaluate the long-term outcome of affected fetuses with and without IUT in Ontario, Canada, we retrospectively collected data on IUTs and pregnancy outcomes in all cases of HBHF, from 1989 to 2014. Clinical outcome and neurocognitive profiles of long-term survivors were also collected and compared with data from 24 patients with transfusion-dependent ß-thalassaemia (TDT-ß). RESULTS: Of the 99 affected pregnancies (93 prenatally diagnosed), 68 resulted in miscarriage or elective termination of pregnancy. Twelve mothers (12%) continued their pregnancies without IUT, and none of those newborns survived the first week of life. All 13 fetuses that received IUT(s) were live-born, but 3 died due to severe hydrops at birth and 1 died due to infection. The remaining nine survivors, in comparison with TDT-ß patients, had earlier iron overload requiring iron chelation therapy. Endocrinopathies and short stature were more frequent in these patients. Neurocognitive outcome was not significantly affected in five patients who were assessed, and none were diagnosed with intellectual impairment. In three patients, MRI studies demonstrated brain white matter changes in keeping with 'silent' ischaemic infarcts. CONCLUSIONS: In patients with HBHF, IUT is associated with improved survival. While acceptable neurocognitive outcome can be expected, these patients have more clinical complications compared with their TDT-ß counterparts. The clinical and neurocognitive outcomes of HBHF should be discussed in detail when counselling and offering IUT for patients.

Isolated hypoplasia of the abdominal wall associated with fetal parvovirus B19 infection.

We report a rare case of a preterm infant with a diagnosis of hydrops fetalis, associated with parvovirus B19 infection. At birth, the infant had severe ascites. She recovered and was discharged in later good condition. In follow-up at 10 years of age, she still had severe isolated hypoplasia of the abdominal muscles. Isolated hypoplasia of the abdominal muscles after parvovirus B19 infection appears to be a separate entity, which should be differentiated from other abdominal wall anomalies.

Nonimmune hydrops fetalis caused by G6PD deficiency hemolytic crisis and congenital dyserythropoietic anemia.

We present a case of a female neonate who had a nonimmune hydrops fetalis and severe hemolytic anemia due to a rare combination of glucose-6-phosphate dehydrogenase (G6PD) deficiency and congenital dyserythropoietic anemia. We conclude that in severe cases with persistent anemia one should search after delivery for a second reason other than G6PD deficiency alone.

Neonatal outcome of fetuses receiving intrauterine transfusion for severe hydrops complicated by Rhesus hemolytic disease.

OBJECTIVE: To evaluate neonatal outcomes among a homogeneous group of fetuses with severe hydrops treated with intrauterine transfusion (IUT). METHODS: In a prospective study at Dokuz Eylul University School of Medicine, Izmir, Turkey, outcomes were compared for 35 IUTs carried out between 2005 and 2010 in 19 pregnancies that were complicated by Rhesus D hemolytic disease with severely hydropic fetuses. RESULTS: There was no correlation between the number of IUTs and the duration of phototherapy or number of exchange transfusions. After delivery, 36% (7/19) of neonates tested positive in a direct Coombs test and their requirement for exchange transfusion was higher than that of neonates who tested negative. The neonatal survival rate was 73.7%. Admission to the neonatal intensive care unit was 78%, and the median duration of neonatal unit stay was 4 days (range, 1-77 days). Only 1 newborn had hearing impairment. CONCLUSION: IUT is a unique, gold standard treatment for severely hydropic fetuses. When treated optimally with IUT, fetuses with severe hydrops showed no increased risk of neurodevelopmental abnormalities. Factors affecting the survival of hydropic fetuses after IUT, and whether the number of IUTs performed affects the number of exchange transfusions required remain unclear.

A case of severe Rh (D) alloimmunization treated by intensive plasma exchange and high-dose intravenous immunoglobulin.

BACKGROUND: In extremely severe Rh (D) alloimmunization, during pregnancy, early diagnosis and treatment is essential to avoid hydrops fetalis. Intrauterine transfusion (IUT) is of utmost importance in the prevention of fetal anemia but it is usually feasible only after 20 weeks of pregnancy. Therefore, additional treatment options in early pregnancy are needed. STUDY DESIGN AND METHODS: A 27-year-old severely D+C immunized woman was admitted at 8 weeks of gestation in her fifth pregnancy with an extremely high concentration of anti-D. Her first pregnancy was uneventful but resulted in D+C alloimmunization. The next two pregnancies were unsuccessful, because of hydrops fetalis resulting in fetal death in pregnancy week 20 and 24, respectively, despite treatment with high-dose intravenous immunoglobulin (IVIG) and IUT treatment. A fourth pregnancy was terminated with legal abortion. The patient was eager and persistent to accomplish a successful pregnancy. Therefore, a combination of treatments consisting of plasma exchange (PE) three times/week and IVIG 100g/week was started in pregnancy week 12. PE was performed 53 times and totally 159L of plasma was exchanged. RESULTS: The anti-D concentration was 12mug/mL (IAT titer 2000) before start of treatment by PE and IVIG in pregnancy week 12. The concentration of anti-D was gradually reduced to approximately 3mug/mL after only two weeks of treatment and was maintained at that level until pregnancy week 22. In pregnancy week 26 and 27, signs of hydrops were detected by ultrasonography and IUT were performed at each occasion. Sectio was inevitable at pregnancy week 28+1 and a male baby was born: Hb 58g/L (cord sample) and 68g/L (venous sample); weight 1385g; Apgar score=4-5-7; Bilirubin 56-150mmol/L (4h). Exchange transfusion was performed on day two and day five. Phototherapy was also implemented for eight days. The newborn's recovery thereafter was uneventful and complete. CONCLUSION: A combination of PE and IVIG may be an efficient treatment possible to start in early pregnancy in patients with extremely severe Rh (D) alloimmunization, with a history of hydrops fetalis in previous pregnancies.

La transfusión intrauterina por cordocentesis: Una alternativa terapéutica con expectativa de vida / Intrauterine transfusion by cordocentesis: A therapuetic alternative with life expectancy

La enfermedad hemolítica por anti D es una grave problemática de nuestro medio que es reconocido como causa de muerte perinatal. Conociendo que las pacientes con enfermedad hemolítica severa, las posibilidades de viabilidad fetal son nulas a pocas semanas de gestación donde otros tratamientos no fueron suficientes, nuestro trabajo demuestra que la transfusión intrauterina por cordocentesis (TIUPC) es una técnica que logra alta expectativa de vida ya que permite no solo reabsorber el hidrops fetal alcanzando embarazos a término con fetos viables, sino también evolución de los recién nacidos sin necesidad de internaciones prolongadas, con menor costo, en relación a los gastos ocasionados por el manejo multidisciplinario que requerían los neonatos sin este tratamiento. (AU)

La transfusión intrauterina por cordocentesis: Una alternativa terapéutica con expectativa de vida / Intrauterine transfusion by cordocentesis: A therapuetic alternative with life expectancy

La enfermedad hemolítica por anti D es una grave problemática de nuestro medio que es reconocido como causa de muerte perinatal. Conociendo que las pacientes con enfermedad hemolítica severa, las posibilidades de viabilidad fetal son nulas a pocas semanas de gestación donde otros tratamientos no fueron suficientes, nuestro trabajo demuestra que la transfusión intrauterina por cordocentesis (TIUPC) es una técnica que logra alta expectativa de vida ya que permite no solo reabsorber el hidrops fetal alcanzando embarazos a término con fetos viables, sino también evolución de los recién nacidos sin necesidad de internaciones prolongadas, con menor costo, en relación a los gastos ocasionados por el manejo multidisciplinario que requerían los neonatos sin este tratamiento.

Maternal ABO-mismatched blood for intrauterine transfusion of severe hemolytic disease of the newborn due to anti-Rh17.

BACKGROUND: Clinically significant antibodies to high-incident antigens present a challenge in hemolytic disease of the newborn. Antigen-negative blood may be difficult to obtain for intrauterine transfusion (IUT). In these instances, maternal blood is de facto compatible regardless of an ABO mismatch. CASE REPORT: A group B/D-- woman with a history of hemolytic disease of the newborn due to anti-Rh17 (titer 256) presented to the obstetrical clinic at 12 weeks gestation for management of her third pregnancy. She consented to donate blood for possible IUT. STUDY DESIGN AND METHODS: Washed maternal packed cells were suspended in saline to 75 percent Hct and irradiated before transfusion. The fetus was transfused via the intrahepatic vein. RESULTS: Ultrasound examination at 19 weeks indicated a hydropic fetus. The fetal blood group was O Rh+, direct antiglobulin test 4+, and hemoglobin 22 g per L. A total of 368 mL of maternal blood was transfused during seven procedures. Labor was induced at 38 weeks, and a 2560-g male infant was delivered by Caesarian-section due to fetal distress. The infant grouped as B Rh+, direct antiglobulin test negative. No group O red blood cells were detected. The hemoglobin level was 143 g per L rising to 209 g per L at discharge 3 days later. The indirect bilirubin was 55 micromol/L and remained stable during the hospital stay. Phototherapy was discontinued after 1 day, and the infant was discharged without an exchange or top-up transfusion. CONCLUSIONS: Maternal ABO-mismatched blood is an alternate source for IUT in instances when antigen-compatible allogenic blood is unavailable.

Hidrops fetal no inmune asociado a talasemia familiar: transfusión intravascular / Non-immune fetal hidrops associated to familiar thalasemia: intravascular transfussion

Se presenta un caso de hidrops fetal no inmune provocado por una anemia hemolítica severa secundaria a una talasemia familiar pesquisada a las 28 semanas y que revierte luego de transfusión intravascular, TIV, practicada en nuestro servicio. Se analiza el diagnóstico, manejo prenatal y posterior evolución

[Ultrasonic monitoring of intravascular transfusion in severe Rhesus incompatibility. Diagnostic and therapeutic aspects after fetal relaxation]. / Die intravasale Transfusion unter Ultraschallkontrolle bei schwerer Rhesus Inkompatibilität. Diagnostische und therapeutische Aspekte unter Einfluss der intrauterinen Retardierung.

Since October 1985 47 ultrasound-guided intrauterine intravascular transfusions were performed in 14 patients at the Department of Obstetrics and Gynecology of the University of Ulm. All newborns were delivered in good condition. The successful diagnostic and therapeutic management of severe erythroblastosis in a twin pregnancy is described. The twin with severe signs of erythroblastosis had an initial hematocrit of 12% at 26 weeks of gestation. Intravascular transfusion of 60 ml packed red cells raised the hematocrit to 41%. The ascites disappeared completely ten days after the first transfusion. Two further transfusions were performed at 29 and 33 weeks of gestation. The intrauterine intravascular relaxation with Norcuron (Vecuroniumbromid) has proved advantageous in preventing interfering fetal movement. Both newborns were delivered in good condition at 36 weeks of gestation.