Hemodynamics in neonates with polycythemia before and after partial exchange transfusion: an observational study.

Introduction: The current recommendations for the management of neonatal polycythemia are that partial exchange transfusion (PET) should be performed if the hematocrit is >70% in an asymptomatic neonate, or if the haematocrit is >65% in a symptomatic neonate. The hemodynamic effects of PET for neonatal polycythemia have not been well researched. Objectives: To evaluate the hemodynamic effects of PET in neonates with polycythemia. Methodology: Prospective observational study conducted in a neonatal intensive care unit of a tertiary care teaching hospital enrolling 21 neonates with polycythemia who underwent PET. Hemodynamic and echocardiographic parameters were obtained prior to PET and 6 h after procedure. Results: The mean gestational age of neonates with polycythemia was 35.08 ± 2.35 weeks with a mean birth weight of 1,929 ± 819.2 g. There was a significant improvement noted in heart rate and oxygen saturation post PET procedure (p < 0.05). Right ventricular systolic function parameters showed significant improvement (Tricuspid annular plane systolic excursion, fractional area change, right ventricular output) (p < 0.05). Left ventricular function parameters showed significant improvement (Fractional shortening, left ventricular output, E:A ratio) (p < 0.05). Resolution of symptoms was noted after PET procedure with no adverse events associated with PET. Conclusion: PET maybe effective in improving heart rate and oxygen saturation levels in polycythemic neonates. It has good short-term hemodynamic stability with improvement in right ventricular systolic, as well as left ventricular systolic and diastolic function. It is a safe and effective procedure with minimal adverse effects. Further studies with larger sample size and a control group would be required to corroborate our findings.

A Rare Dichorionic Triamniotic Triplet Pregnancy with Spontaneous Twin Anemia-Polycythemia Sequence between Two Dichorionic Fetuses.

INTRODUCTION: This report presents a rare case of spontaneous twin anemia-polycythemia sequence (TAPS) between two dichorionic fetuses in a spontaneous, homozygotic, dichorionic, triamniotic, triplet pregnancy treated with multiple intrauterine blood transfusions (IUTs) and partial exchange transfusions (PETs). CASE PRESENTATION: The pregnancy was diagnosed with stage IV TAPS at gestational week 25+1. The patient was treated with laser surgery combined with multiple IUTs and PETs. The triplets were delivered at a planned caesarean section at gestational week 28+1 with postnatal hemoglobin values of 18.21, 26.43, and 11.92 g/dL in triplet 1, 2, and 3, respectively. At 4 years of age, triplet 1 is considered healthy, triplet 2 is diagnosed with mild mental retardation, and triplet 3 with profound mental retardation and dystonic cerebral palsy. DISCUSSION: This is an extremely rare case of TAPS between dichorionic fetuses in a triplet pregnancy, and routine surveillance with measurement of middle cerebral artery peak systolic velocity in dichorionic pregnancies may contribute to the detection of similar cases in the future. Furthermore, this case contributes with rare long-term follow-up data of children treated for high-stage TAPS with multiple IUTs and PETs.

Serum Endocan, Neuron-Specific Enolase and Ischemia-Modified Albumin Levels in Newborns with Partial Blood Exchange Transfusion.

BACKGROUND: Hyperviscosity of blood secondary to polycythemia results in increased resistance to blood flow and decrease in delivery of oxygen. OBJECTIVE: To evaluate whether serum endocan, NSE and IMA levels can be compared in terms of endothelial injury/ dysfunction and neuronal damage in term neonates with polycythemia who underwent PET. METHODS: 38 symptomatic polycythemic newborns having PET and 38 healthy newborns were included in the study. Blood samples for endocan, NSE and IMA were taken at only postnatal 24 hours of age in the control group and in polycytemia group just before PET, at 24 and 72 hours after PET. RESULTS: The polycythemia group had higher serum endocan(1073,4 ± 644,8 vs. 378,8 ± 95,9ng/ml; p<0.05), IMA(1,32 ± 0,34 vs.0,601 ± 0,095absorbance unit; p<0.05) and NSE(44,7 ± 4,3 vs. 26,91 ± 7,12µg/l; p<0.05) levels than control group before the PET procedure. At 24 hours after PET, IMA(0,656 ± 0,07 vs. 0,601 ± 0,095absorbance unit; p<0.05) and endocan(510,9 ± 228,6 vs. 378,8 ± 95,9ng/ml; p<0.05) levels were closer to the control group, being still statistically significant higher. NSE levels decreased to control group levels having no difference between the PET and control groups at 24 hours after PET (28,98 ± 6,5 vs. 26,91 ± 7,12µg/l; p>0.05). At 72 hours after PET the polycythemia and control groups did not differ statistically for IMA, endocan and NSE levels (p>0.05). CONCLUSION: Serum endocan and IMA levels can be used as a biomarker for endothelial damage/ dysfunction and tissue hypoxia in infants with symptomatic polycytemia.

Perinatal Management of Bart's Hemoglobinopathy: Paradoxical Effects of Intrauterine, Transplacental, and Partial Exchange Transfusions.

We describe a fetus at 24 3/7 weeks' gestation that showed ultrasound evidence of anemia, hydrops, and severe growth restriction. Both parents were known to be cis heterozygous carriers for SEA α-thalassemia deletion (αα/-). Cordocentesis confirmed fetal anemia and homozygous α-thalassemia (-/-) in the fetus. Fetal intrauterine transfusions corrected the anemia, treated the hydrops, and improved fetal growth. The postnatal course was complicated by hypoxic respiratory failure and persistent pulmonary hypertension of the newborn, which resolved only after partial volume exchange transfusion. This case report is presented to point out the potential unintended outcomes with transplacental transfusion via delayed cord clamping and cord milking at delivery in the setting of congenital Bart's hemoglobinopathy, and demonstrates that partial exchange transfusion of the newborn may optimize oxygen delivery due to the more favorable oxygen affinity of transfused adult hemoglobin compared with the Bart's hemoglobin.

Intrauterine Interventions for the Treatment of Twin Anemia-Polycythemia Sequence: A Systematic Review.

BACKGROUND: Twin anemia-polycythemia sequence (TAPS) is a complication of monochorionic, multiple gestation pregnancies in which blood shunting through placental anastomoses results in chronic anemia in one fetus and chronic polycythemia in another. The outcomes of different treatment modalities for TAPS are not well known. OBJECTIVE: To determine the outcomes of the intrauterine interventions used to treat TAPS. STUDY DESIGN: A systematic literature search of MEDLINE, EMBASE, and CENTRAL was performed in June 2016. Primary outcomes were mortality, morbidity, and adverse perinatal outcomes. Data were summarized in the form of weighted means, and statistical difference was determined. RESULTS: Twenty-one articles were identified for inclusion in this review and were composed of 105 cases of TAPS. In the cases presented in the literature, there was no statistically significant difference in mortality, morbidity, or emergent Caesarean section rates between expectant management, intrauterine transfusion (IUT), and laser ablation therapy. Laser ablation therapy and IUT were found to have a significantly lower rate of adverse perinatal outcomes when compared to expectantly managed cases. CONCLUSIONS: The literature looking into the treatment of TAPS is very limited, with no randomized controlled trials and only one includable comparative study. Based on the data in the case report and case study literature, there is no mortality difference between any of the treatment modalities. Expectant management may be associated with an increase in adverse perinatal outcomes when compared to laser therapy and IUT. More comparative studies are needed to assist clinicians in adopting an evidence-based approach to the treatment of TAPS.

In utero Partial Exchange Transfusion Combined with in utero Blood Transfusion for Prenatal Management of Twin Anemia-Polycythemia Sequence.

Monochorionic twin pregnancies are at risk of unique complications due to placental sharing and vascular connections between placental territories assigned for each twin. Twin anemia-polycythemia sequence (TAPS) is an infrequent but potentially dangerous complication of abnormal placental vascular connections. TAPS occurs due to very-small-caliber (< 1 mm) abnormal placental vascular connections which lead to chronic anemia in the donor twin and polycythemia in the recipient twin. TAPS may occur spontaneously or following fetoscopic laser photocoagulation of communicating placental vessels for twin-twin transfusion syndrome. One of the hallmarks of TAPS is the absence of polyhydramnios and oligohydramnios. The postnatal diagnosis is based on significant hemoglobin discrepancy between the twins. Middle cerebral artery peak systolic velocity Doppler ultrasound allows for the prenatal diagnosis of TAPS. The optimal prenatal treatment and intervention timing has not been established. Here, we report 3 spontaneous TAPS cases diagnosed and managed in the prenatal period with a combination of in utero blood transfusion for the anemic twin (donor) and in utero partial exchange transfusion for the polycythemic twin (recipient). These cases contribute to the limited outcome data of this underutilized method for the management of TAPS.

Neonatal polycythaemia.

Neonatal polycythaemia has multifactorial causes, and can be designated as active (increased foetal erythropoiesis) or passive (red blood cell transfusion) polycythaemia. Hematocrit estimated from capillary blood (regularly obtained through "heel sticks" in newborns) is normally the principal laboratory feature facility by which polycythaemia is recognszed. An unusually high proportion of haematocrit builds the risk of hyperviscosity, microcirculatory hypoperfusion, and in the long run multisystem organ dysfunction. A report enclosed in this short communication gives a brief review of neonatal polycythaemia, its causes, management and complications.

Hematological disorders at birth in complicated monochorionic twins.

INTRODUCTION: Monochorionic twins are at risk of severe complications including twin-twin transfusion syndrome (TTTS), twin anemia-polycythemia sequence (TAPS) and acute peripartum TTTS. The pathophysiology is based on inter-twin blood transfusion through placental vascular anastomoses. Areas covered: This review focuses on the incidence, management and outcome of neonatal hematological complications at birth in TTTS, TAPS and acute peripartum TTTS. Expert commentary: Hematological disorders are often present at birth in monochorionic twins and include acute or chronic anemia, polycythemia and thrombocytopenia. Routine measurement of complete blood counts in all complicated monochorionic twins is strongly recommended. Increased awareness on these disorders and correct diagnostic tests will lead to prompt and adequate management at birth.

Antenatal management of twin-twin transfusion syndrome and twin anemia-polycythemia sequence.

INTRODUCTION: Twin-twin transfusion syndrome (TTTS) and twin anemia polycythemia sequence (TAPS) are severe complications in monochorionic twin pregnancies associated with high mortality and morbidity risk if left untreated. Both diseases result from imbalanced inter-twin blood transfusion through placental vascular anastomoses. AREAS COVERED: This review focuses on the differences in antenatal management between TTTS and TAPS. Expert commentary: The optimal management for TTTS is fetoscopic laser coagulation of the vascular anastomoses, preferably using the Solomon technique in which the whole vascular equator is coagulated. The Solomon technique is associated with a reduction of residual anastomosis and a reduction in post-operative complications. The optimal management for TAPS is not clear and includes expectant management, intra-uterine transfusion with or without partial exchange transfusion and fetoscopic laser surgery.

Intrauterine transfusion combined with partial exchange transfusion for twin anemia polycythemia sequence: modeling a novel technique.

INTRODUCTION: Twin anemia-polycythemia sequence (TAPS) is a newly described disease in monochorionic twin pregnancies, characterized by large inter-twin hemoglobin differences. Optimal management for TAPS is not clear. One of the possible treatment modalities is intrauterine blood transfusion (IUT) in the donor with or without combination of partial exchange transfusion (PET) in the recipient. METHODS: We applied a computational model simulation to illustrate the mechanism of IUT with and without PET in TAPS occurring after laser surgery for twin-twin transfusion syndrome (TTTS). Model simulations were performed with the representative anastomotic pattern as observed during laser intervention, and after placental dye injection. RESULTS: The model was tested against different cases where IUT was combined with PET for the treatment of post-laser TAPS. Model simulations using the observed anastomotic pattern showed a significant reduction of hyperviscosity in the recipient after IUT/PET compared to IUT without PET. DISCUSSION: In this model simulation we show that the addition of PET to IUT reduces the severity of polycythemia in the recipient. PET may thus be important to prevent complications of hyperviscosity. CONCLUSION: This model simulation shows the beneficial effect of PET for the recipient in TAPS cases treated with IUT.

A pilot study of manual chronic partial exchange transfusion in children with sickle disease.

Objective Red cell exchange transfusion is frequently used in the management of patients with sickle cell disease (SCD) either electively or chronically to maintain hemoglobin S (HbS) <30%. The purpose of this retrospective study was to evaluate the results of manual chronic partial exchange transfusion (MCPET) on level of Hb and HbS, on iron load and on the need for chelation, on risk of immunization, monitoring transfusion-transmitted viral infection, and clinical outcome. Methods We reviewed the long-term effect of MCPET in 10 children (six men and four women) with SCD and evaluated the iron balance during a median follow-up of 20 months (range: 6-36) in which 248 exchanges were performed. Results The pre-exchange median Hb value was 9.5 g/dl (range: 7.7-10.9 g/dl) and the median post-exchange value was 9.4 g/dl (range: 8.4-11.1 g/dl).The majority of patients reached an HbS of <50% with a median HbS value of 40.04% (range: 30-54). At start of the MCPET program, the median ferritin was 439 ng/ml (range: 80-1704 ng/ml). In the final evaluation, the median value of ferritin was 531 ng/ml (range: 84-3840 ng/ml). The annual calculated iron balance was 0. 28 ± 0.08 mg/kg/day. MCPET was well tolerated, and adverse effects were limited. Discussion MCPET in children with SCD is safe to prevent iron overload, and is effective and easy to use in our cohort. Conclusion Indication for chronic exchange blood transfusion is essential for patients with SCD with recurrent and frequent crises who do not respond to hydroxyurea. However, there is no consensual study for the period at which chronic transfusion can safely be stopped and further research in large population of patients with SCD will need to clarify this question.

Hyperviscosity Syndrome in Newborn

We experienced Hyperviscosity syndrome in 5 newborn infants during 6 months period from August 1980 to January 1981. Diagnosis was made on the basis of characteristic clinical symp-toms along with polythemia. The following results were obtained. Sex in 5 affected infants showed male in 2 and female in 3. And gestational age showed preterm in 3 and full term in 2 cases. Predisposing factors were intertwin transfusion in 1, placental insufficiency with maternal to-xemia in 2(one of which was accompained with interwin transfusion) and small for gestational age in 1. Signs and symptoms associated with hyperviscosity syndrome were cyanosis in 3, dyspnea in 2, lethargy in 2, plethora in 1, hypocalcemia in 4, hypoglycemia in 2 and hyperbilirubinemia in 2 cases. Partial exchange transfusion was done in all cases, resulting in improvement of polycythem-ia and clinical condition, except 1 expired case.