Next steps towards improved care for twin anemia polycythemia sequence

This thesis deals with various aspects of twin anemia polycythemia sequence (TAPS). TAPS is a condition that can develop due to unbalanced feto-fetal blood transfusion through minuscule vascular placental anastomoses in monochorionic twin pregnancies, causing the donor twin to become anemic and the recipient twin to become polycythemic.  In this thesis we show that a difference in middle cerebral artery peak systolic velocity (MCA-PSV) > 0.5 Multiples of the Median (MoM) has a high diagnostic accuracy for the the antenatal diagnosis of TAPS. For postnatal diagnosis of the condition, inspection of the color of the maternal side of the placenta can be of great value. Furthermore, we present the results of a large international registry, and report on outcomes after different treatment options for TAPS. As the best treatment for TAPS is unclear, we propose the protocol of The TAPS Trial (a multicenter open-label international RCT) to investigate the potential beneficial effect of fetoscopic laser surgery for the outcome in TAPS twins . In the last chapters of this thesis we discuss short- and long-term outcome and show that TAPS donors show significantly higher rates of perinatal mortality and long-term neurodevelopmental impairment than their recipient co-twins. Afdeling Kindergeneeskunde, Afdeling Verloskunde LUMC, LUMC, Mieke Kerfhof, Nederlandse Vereniging voor Ouders van Meerlingen, Stichting Oranjekliniek, Stichting IDB.LUMC / Geneeskund

Fetal anemia in monochorionic twins: a review on diagnosis, management, and outcome

Introduction: Monochorionic twins may develop fetal anemia when blood is unequally distributed via the placental vascular anastomoses. This review focuses on the causes of fetal anemia in complicated monochorionic twins and highlights the differences in management and outcome. Areas: coveredFetal anemia can occur in the context of twin anemia polycythemia sequence (TAPS), chronic twin-twin transfusion syndrome (TTTS) and acute peripartum TTTS, and in cotwins after single fetal demise. Diagnosis of fetal anemia is based on abnormal Doppler ultrasound measurements. Management options include fetoscopic laser surgery, intrauterine blood transfusion, or expectant management, depending on the type of complication and the severity of the disease. In all complications, fetal anemia may lead to perinatal mortality, neonatal morbidity, severe cerebral injury, and long-term neurodevelopmental impairment. In TAPS specifically, anemic donors may also show bilateral deafness.Expert opinion: Knowledge on the diagnosis and optimal treatment in TTTS is nowadays widespread, but caregivers often fail to distinguish TAPS from acute peripartum TTTS at birth. A full blood count including reticulocyte count is required, and placental dye injection is extremely helpful to reach the correct diagnosis and establish the optimal management.</p

Twin anemia polycythemia sequence in a dichorionic twin pregnancy leading to severe cerebral injury in the recipient

Twin anemia polycythemia sequence (TAPS) is a form of chronic imbalanced feto-fetal transfusion through minuscule placental anastomoses leading to anemia in the TAPS donor and polycythemia in the TAPS recipient and has been reported only in monochorionic twins. We report a very unusual case of TAPS which developed in a dichorionic twin pair, born at a gestational age of 33(+2). Twin 1 (recipient) was polycythemic and had a hemoglobin value of 22.4 g/dL, whereas twin 2 (donor) was anemic with a hemoglobin value of 9.8 g/dL and an increased reticulocyte count (72 parts per thousand). Color dye injection of the placenta revealed the presence of a deep-hidden small veno-venous anastomosis. Dichorionicity was confirmed on histologic examination. Aside from respiratory distress syndrome, the donor twin had an uncomplicated neonatal course. The recipient twin developed a post-hemorrhagic ventricular dilatation requiring treatment with a ventriculoperitoneal shunt and Rickham reservoir. This report shows that in dichorionic twins, placental anastomoses can be present, which can lead to the development of TAPS with severe consequences. Therefore, when a pale and plethoric dichorionic twin pair is born, a complete diagnostic work-up is required, including a full blood count with reticulocytes and placental injection, to investigate the presence and nature of potential underlying feto-fetal transfusion. Once the diagnosis of TAPS has been established, cerebral ultrasound, hearing screening, and long-term follow-up are strongly recommended as these twins have increased risk for severe cerebral injury, hearing loss, and long-term neurodevelopmental impairment.Research into fetal development and medicin

Twin anemia polycythemia sequence: knowledge and insights after 15 years of research

Twin anemia polycythemia sequence (TAPS) is a chronic form of unbalanced feto-fetal transfusion through minuscule placental anastomoses in monochorionic twin pregnancies, leading to anemia in the donor twin and polycythemia in the recipient twin. TAPS can occur spontaneously in up to 5% of monochorionic twins or can arise in 2%-16% of cases after incomplete laser surgery for twin-twin transfusion syndrome. TAPS can develop across the entire second and third trimester. Antenatal diagnosis for TAPS is reached via Doppler measurement of the fetal middle cerebral artery peak systolic velocity, showing an increased velocity in the donor, combined with a decreased velocity in the recipient. Treatment options for TAPS include expectant management, preterm delivery, intrauterine blood transfusion with or without a partial exchange transfusion, fetoscopic laser surgery and selective feticide. The best treatment option is unclear and is currently being investigated in an international multicenter randomized trial (the TAPS trial). Spontaneous fetal demise occurs in 5%-11% of TAPS twins, more often in donors (8%-18%) than in recipients (2%-5%). Severe long-term neurodevelopmental impairment is seen in 9% of TAPS twins, with donors having an increased risk for cognitive impairment and hearing problems (15%).Research into fetal development and medicin

Large hemoglobin differences at birth in monochorionic twins with a placental chorangioma and delayed cord clamping

We report a case of a monochorionic diamniotic twin with an uncomplicated pregnancy, but with an unexpected large intertwin hemoglobin (Hb) difference at birth. Twin 1 was delivered vaginally and had an uneventful neonatal course. The umbilical cord of Twin 1 was clamped approximately 5 min after birth. After the birth of Twin 1, Twin 2 developed severe bradycardia and showed limited cardiac output on ultrasound, for which an emergency cesarean section was performed. A full blood count revealed an Hb of 20.1 g/dL for Twin 1 and 10.2 g/dL for Twin 2 (intertwin difference 9.9 g/dL). Reticulocyte counts were similar, 40 parts per thousand and 38 parts per thousand, respectively. Placental examination revealed 10 vascular anastomoses, including one arterio-arterial anastomosis with a diameter of 1.4 mm. Additionally, a large chorangioma was present on the placental surface of Twin 2. There was no color difference on the maternal side of the placenta. Based on the reticulocyte count ratio and the placental characteristics, twin anemia polycythemia sequence was ruled out as the cause of the large intertwin Hb difference. In this report, we discuss the various potential causes that could explain the large intertwin Hb difference including the role of delayed cord clamping in Twin 1, and the role of a large chorangioma, which may have attracted blood from the fetal circulation of Twin 2.OV

Next steps towards improved care for twin anemia polycythemia sequence

This thesis deals with various aspects of twin anemia polycythemia sequence (TAPS). TAPS is a condition that can develop due to unbalanced feto-fetal blood transfusion through minuscule vascular placental anastomoses in monochorionic twin pregnancies, causing the donor twin to become anemic and the recipient twin to become polycythemic.  In this thesis we show that a difference in middle cerebral artery peak systolic velocity (MCA-PSV) > 0.5 Multiples of the Median (MoM) has a high diagnostic accuracy for the the antenatal diagnosis of TAPS. For postnatal diagnosis of the condition, inspection of the color of the maternal side of the placenta can be of great value. Furthermore, we present the results of a large international registry, and report on outcomes after different treatment options for TAPS. As the best treatment for TAPS is unclear, we propose the protocol of The TAPS Trial (a multicenter open-label international RCT) to investigate the potential beneficial effect of fetoscopic laser surgery for the outcome in TAPS twins . In the last chapters of this thesis we discuss short- and long-term outcome and show that TAPS donors show significantly higher rates of perinatal mortality and long-term neurodevelopmental impairment than their recipient co-twins. </p

Intermittent absent and reversed umbilical artery flows in appropriately grown monochorionic diamniotic twins in relation to proximate cord insertion: A harmful combination?

Objective To compare the prevalence of intermittent absent or reversed end-diastolic flow (iAREDF) in the umbilical artery in appropriately grown monochorionic diamniotic (MCDA) pregnancies with and without proximate cord insertion (PCI), and to evaluate pregnancy outcome. Methods The prevalence of iAREDF in MCDA pregnancies with PCI (n = 11) was compared with a control group without PCI (n = 33). PCI was defined as a distance between the cord insertions below the fifth percentile. Placental sharing, number, and diameter of anastomoses were assessed by placental examination. Pregnancy outcome was evaluated. Results iAREDF was present in 7/11 PCI pregnancies, compared with 0/33 in the control group (P <= .01). All PCI pregnancies and 94% of controls had arterioarterial (AA)-anastomoses (P= .56), the diameter was larger in the PCI group, respectively 3.3 vs 2.1 mm (P= .03). Three cases with iAREDF had adverse outcome, two resulted in fetal death of which one with brain damage in the co-twin, another underwent early premature emergency section for fetal distress. Conclusion iAREDF occurs in a large proportion of MCDA pregnancies with PCI and is related to the diameter of the AA anastomosis. We hypothesize that iAREDF in appropriately grown MCDA twin pregnancies reflects an unstable hemodynamic balance with an increased risk for fetal deterioration. Whether outcome in these pregnancies can be improved by altered management requires further investigation.Research into fetal development and medicin

TAPS TRIAL: Fetoscopic laser surgery versus care as usual for twin anaemia polycythaemia sequence: An open-label randomized controlled trial

Research into fetal development and medicin

The prognostic value of tumor-stroma ratio in tumor-positive axillary lymph nodes of breast cancer patients

Surgical oncolog

The Impact of Selective Fetal Growth Restriction or Birth Weight Discordance on Long-Term Neurodevelopment in Monochorionic Twins: A Systematic Literature Review

Research into fetal development and medicin