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... Show moreIntroduction: 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. Show less
In 70% of identical twin pregnancies, the twins share a single placenta in the womb that provides them with nutrients. This shared placenta can be unequaly shared. This means that one of the twins... Show moreIn 70% of identical twin pregnancies, the twins share a single placenta in the womb that provides them with nutrients. This shared placenta can be unequaly shared. This means that one of the twins has a much smaller share of the placenta compared to the other twin, resulting in a large growth discrepancy during pregnancy, also known as 'selective fetal growth restriction'. At birth, a large difference in birth weight ensues. Sometimes one twin can be almost twice the size of the other twin.These twins are considered a vulnerable patient group in which perinatal complications are prevalent. Nevertheless, a large gap in knowledge persists, impeding proper parent counseling and risk assessment by health care providers. Simultaneously, these twins can also be considered a unique natural 'experiment' to uncover the early origins of health and disease following an adverse environment in the womb. The growth-restricted twin can be compared to a genetically identical, normally-grown twin who has been in the same womb of the same mother at the same time.In this thesis, Sophie Groene has investigated the placental mechanisms and the short- and long-term outcomes of this special group of twins. Show less
Background: Psychosocial development in monochorionic (MC) twins born after selective fetal growth restriction (sFGR) has been unreported to date, despite its importance for daily functioning and... Show moreBackground: Psychosocial development in monochorionic (MC) twins born after selective fetal growth restriction (sFGR) has been unreported to date, despite its importance for daily functioning and future relationships. Aims: To investigate psychosocial development, attachment and school functioning in MC twins with sFGR and compare outcomes with the general population and between smaller and larger twins. Study design: Observational cohort study. Subjects: MC twins with sFGR (defined as a birth weight discordance >= 20 %) born between 2002 and 2017 and aged 3-17 years. Outcome measures: Multiple parent report questionnaires: the Child Behavior Checklist (social-emotional devel-opment and behavior), the (Early) Childhood Behavior Questionnaire Very Short Form (temperament), the Attachment Insecurity Screening Inventory (attachment) and a school functioning questionnaire. Results: Median age for the 48 twin pairs was 11 (interquartile range (IQR) 8-13) years. Attachment insecurity for both twins was higher than in the general population for ambivalence/resistance (34 % (21/62) vs. 16 %, p = 0.024) and total attachment insecurity (35 % (22/62) vs. 16 %, p = 0.016). Smaller twins had more internalizing behavioral problems, i.e. negative emotions and behaviors turned inwards (22 % (10/46) vs. 11 % (5/46), p = 0.021) and a higher negative affect, i.e. more likely to experience negative emotions (3.2 (2.9-3.7) vs. 2.9 (2.2-3.2), p = 0.009) than larger twins, as well as a lower secondary school level (p = 0.031). Conclusion: MC twins with sFGR have more ambivalent/resistant attachment insecurity following the compli-cated pregnancy course. Smaller twins have a tendency towards negative emotions and internalizing behaviors compared to larger twins, indicating an increased sensitivity for depression and anxiety. Show less
Groene, S.G.; Tollenaar, L.S.A.; Meeren, L.E. van der; Slaghekke, F.; Verweij, E.J.; Hooper, S.B.; ... ; Lopriore, E. 2021
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... Show moreWe 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. Show less
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... Show moreTwin 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%). Show less
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... Show moreThis 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. Show less
Objective: The aim of this study was to evaluate the differences in leukocyte counts at birth between donors and recipients with twin-twin transfusion syndrome (TTTS) or twin anemia-polycythemia... Show moreObjective: The aim of this study was to evaluate the differences in leukocyte counts at birth between donors and recipients with twin-twin transfusion syndrome (TTTS) or twin anemia-polycythemia sequence (TAPS). Methods: We performed a retrospective cohort study in monochorionic twin pairs with TTTS or TAPS. TTTS and TAPS cases treated with fetoscopic laser surgery were excluded. Primary outcome was the difference in leukocyte levels at birth between donor and recipient twins and the presence of leukopenia (defined as leukocyte count <4 x 10(9)/L). Secondary outcomes included early-onset sepsis, necrotizing enterocolitis, use of antibiotics during admission, and neonatal mortality. Results: We included 99 twins pairs, of which 61 twin pairs were affected by TAPS and 38 twin pairs by TTTS. The mean leukocyte count at birth in donors and recipients was 7.5 x 10(9)/L versus 7.4 x 10(9)/L (p = 0.936), respectively. Leukopenia was significantly more common in donor twins compared to recipient twins (7.1% [7/99] vs. 0% [0/99], p = 0.016). Of the 7 donors with leukopenia, 6 were affected by TAPS and 1 by TTTS. Overall, donors were more often affected by early-onset sepsis than recipients, 23.7% (23/97) versus 13% (13.7/95) (p = 0.049), respectively. Conclusions: Leukocyte counts at birth in twins with TTTS or TAPS are similar between donors and recipients, but TAPS donors are at an increased risk of leukopenia. Overall, TTTS and TAPS donors seem to be at an increased risk of early-onset neonatal sepsis compared to recipient twins. Show less
We report a case of a monochorionic diamniotic twin diagnosed with twin-twin transfusion syndrome (TTTS; stage 3) with co-existing severe cerebral damage in the donor twin at 18 + 4 weeks'... Show moreWe report a case of a monochorionic diamniotic twin diagnosed with twin-twin transfusion syndrome (TTTS; stage 3) with co-existing severe cerebral damage in the donor twin at 18 + 4 weeks' gestation. After counselling, the parents opted for selective foeticide of the donor twin. For the procedure, radiofrequency ablation (RFA) was used. Serial ultrasound examinations at 20 + 1 and 21 + 1 weeks' gestation showed good recovery of the ex-recipient, after which the patient was sent back to the referring hospital. At 29 + 5 weeks' gestation, an unexpected foetal death was diagnosed. On macroscopic placental examination, (iatrogenic) monoamnionicity was detected. In addition, the umbilical cord of the recipient was found to be constricted by the macerated umbilical cord of the ex-donor. This case demonstrates that iatrogenic monoamnionicity can be a serious complication of RFA in monochorionic twins complicated by TTTS, with a subsequent risk for cord entanglement leading to a fatal outcome for the remaining co-twin. Although the actual incidence of iatrogenic monoamnionicity after RFA remains unknown, increased attention to the intactness of the inter-twin membrane even weeks after the RFA may be required. Show less
Kosinska-Kaczynska, K.; Lipa, M.; Szymusik, I.; Bomba-Opon, D.; Brawura-Biskupski-Samaha, R.; Kozlowski, S.; ... ; Lopriore, E. 2018
Monochorionic twins are at increased risk of complications compared to dichorionic twins due to differences in placentas. Nearly all monochorionic twins have placental anastomoses connecting... Show moreMonochorionic twins are at increased risk of complications compared to dichorionic twins due to differences in placentas. Nearly all monochorionic twins have placental anastomoses connecting the blood circulation of both twins, whereas dichorionic twins have always two separate placentas without vascular connections. Vascular anastomoses lead to inter-twin blood transfusion, which is ‘balanced’ in uncomplicated monochorionic twins. In twin-twin transfusion syndrome (TTTS) and twin anemia-polycythemia sequence (TAPS) inter-twin blood transfusion is unbalanced, resulting in high mortality rates if left untreated. However, improved antenatal management has led to an increase in perinatal survival. Attention is now shifting towards postnatal complications in survivors. In this thesis hematological and biochemical complications in twins affected by TTTS or TAPS are evaluated, including albumin levels and short-term postnatal renal function and hemoglobin levels in uncomplicated monochorionic twins and dichorionic twins. Show less
Verbeek, L.; Slaghekke, F.; Favre, R.; Vieujoz, M.; Cavigioli, F.; Lista, G.; ... ; Lopriore, E. 2016
