Aim: To investigate the school performance and behavioral difficulties in children with hemolytic disease of the fetus and newborn (HDFN) treated with intrauterine transfusion (IUT) compared to... Show moreAim: To investigate the school performance and behavioral difficulties in children with hemolytic disease of the fetus and newborn (HDFN) treated with intrauterine transfusion (IUT) compared to Dutch norm data.Study design: Cros-sectional cohort study. Subjects: Children who received one or multiple IUTs for severe Rh-or K (Kell)-mediated HDFN between January 2008 and January 2015 at the LUMC.Outcome measures: School performance reports were assessed as well as behavioral difficulties as assessed with the Dutch child behavioral checklist (CBCL) by parents and caregivers and the Teacher Report Form (TRF) completed by teachers.Results: A response rate of 56% (70 children, aged 5-12 years) was obtained. Grade repetition occurred in 13 cases (19%), 16 children (23%) received some form of additional help, most often support by a speech therapist (n = 8), but also support for dyslexia (n = 4), physical therapy (n = 2) and social-emotional support (n = 2). None of the children in our study group attended special-needs education. School performance levels for reading comprehension, spelling and mathematics according to the Dutch National Pupil Monitoring System were similar for the study population and Dutch norm data. The incidence of behavioral problems as reported by parents was similar to the Dutch norm data, teachers reported less behavioral difficulties in the study group. Conclusion: This study shows favorable and reassuring school development in children treated with IUT in an experienced fetal-therapy center. A normal distribution in school and behavioral development is to be expected for children with HDFN treated with IUTs. Show less
Witlox, R.S.G.M.; Lopriore, E.; Rijken, M.; Klumper, F.J.C.M.; Oepkes, D.; Klink, J.M.M. van 2019
Fetal thoracic abnormalities such as congenital cystic adenomatoid malformation of the lung (CCAM), bronchopulmonary sequestration (BPS) and fetal pleural effusions (FPE) can lead to fetal hydrops... Show moreFetal thoracic abnormalities such as congenital cystic adenomatoid malformation of the lung (CCAM), bronchopulmonary sequestration (BPS) and fetal pleural effusions (FPE) can lead to fetal hydrops due to their space occupying effect. In these cases the risk of fetal demise is very high when left untreated. Different treatment modalities are available to reduce the intrathoracic pressure either by directly reducing the mass of the lesion (e.g. by laser coagulation of the feeding vessel of BPS) or by shunt placement in FPE or macrocystic CCAM. These treatments reduce perinatal mortality. Infants born after fetal therapy are at increased risk of neonatal morbidity and mortality. The rates of respiratory failure at birth and postnatal demise are highest in the group treated for CCAM with hydrops. In patients with BPS and hydrops the rates of neonatal morbidity and demise are lower. The postnatal course of hydropic fetuses treated with thoracoamniotic shunt for isolated hydrothorax is often complicated by respiratory failure and persistent pleural effusions. Neonatal survival is good provided delivery occurs at or after 32 weeks’ gestation. In these groups of patients severe neurodevelopmental impairment (NDI) at long-term follow-up was detected in 15% of cases. Show less
Kosinska-Kaczynska, K.; Lipa, M.; Szymusik, I.; Bomba-Opon, D.; Brawura-Biskupski-Samaha, R.; Kozlowski, S.; ... ; Lopriore, E. 2018
An increasing number of fetal diseases are being detected prior to birth due to major improvements in prenatal ultrasound examinations and the wide implementation of screening programs. For various... Show moreAn increasing number of fetal diseases are being detected prior to birth due to major improvements in prenatal ultrasound examinations and the wide implementation of screening programs. For various diseases, fetal therapy may be a life-saving option or an alternative to postnatal treatment, to prevent permanent organ damage. A major breakthrough in fetal therapy was the introduction of intrauterine blood transfusion for severe fetal anemia in the early 1960s. Since then, fetal therapy has gradually evolved resulting in a dramatic increase in overall survival in several fetal diseases. In the Netherlands, fetal surgical interventions are concentrated in one center, the LUMC, a tertiary medical center which serves as the national referral center for fetal therapy. Although an increasing number of children are being born alive after fetal therapy, reliable data on the long-term neurodevelopmental outcome remain scarce. Follow-up studies are of paramount importance to increase our knowledge on the quality of long-term survival and to identify potential risk factors for adverse outcome. In this thesis, studies on the long-term neurodevelopmental outcome after fetal therapy for various fetal diseases are presented including intrauterine transfusion for fetal anemia, fetoscopic laser surgery for twin-twin transfusion syndrome and selective reduction in complicated monochorionic pregnancies. Show less
In this thesis fetal fluid and protein dynamics are investigated to gain insight in fetal (patho-)physiology. Studies were performed in fetuses with severe anemia and/or hydrops fetalis.... Show moreIn this thesis fetal fluid and protein dynamics are investigated to gain insight in fetal (patho-)physiology. Studies were performed in fetuses with severe anemia and/or hydrops fetalis. Measurements were performed in fetal blood or amniotic fluid, obtained before or during intrauterine transfusion. The severity of anemia can be predicted by measurement of bilirubin in amniotic fluid. We showed that this concentration is based on bilirubin in fetal blood and on albumin concentrations. Albumin in amniotic fluid is most probably not of fetal however of membrane or maternal origin. Thus, bilirubin seems to exchange between albumin in fetal blood and amniotic fluid over the intramembraneous pathway. Low albumin concentration in fetal blood seems to be a secondary effect of hydrops fetalis. Fetuses with severe anemia were found to maintain their total blood volume. Thus, the decrease in red cell volume is compensated by an increase in plasma volume. This could explain the decrease in albumin concentration. During intrauterine transfusion, part of the plasma volume leaves the circulation. It is expected that this process continues after transfusion, thus further increasing the hematocrit. Acquired insights and differences between fetuses and neonates are discussed. Finally, implications for current practice and future research are described. Show less
