Patient empowerment improves follow-up data collection after fetal surgery for spina bifida: institutional audit.

OBJECTIVES: To define and grade fetal and maternal adverse events following fetal surgery for spina bifida and to report on the impact of engaging patients in collecting follow-up data. METHODS: This prospective single-center audit included 100 consecutive patients undergoing fetal surgery for spina bifida between January 2012 and December 2021. In our setting, patients return to their referring unit for further pregnancy care and delivery. On discharge, referring hospitals were requested to return outcome data. For this audit, we prompted patients and referring hospitals to provide data in cases of missing outcomes. Outcomes were categorized as missing, returned spontaneously or returned following additional request, by the patient and/or referring center. Postoperative maternal and fetal complications until delivery were defined and graded according to Maternal and Fetal Adverse Event Terminology (MFAET) and the Clavien-Dindo classification. RESULTS: There were no maternal deaths, but severe maternal complications occurred in seven women (anemia in pregnancy, postpartum hemorrhage, pulmonary edema, lung atelectasis, urinary tract obstruction and placental abruption). No cases of uterine rupture were reported. Perinatal death occurred in 3% of fetuses and other severe fetal complications in 15% (perioperative fetal bradycardia/cardiac dysfunction, fistula-related oligohydramnios, chorioamnionitis and preterm prelabor rupture of membranes (PPROM) before 32 weeks). PPROM occurred in 42% of patients and, overall, delivery took place at a median gestational age of 35.3 weeks (interquartile range, 34.0-36.6 weeks). Information provided following additional request, from both centers and patients but mainly from the latter, reduced missing data by 21% for gestational age at delivery, 56% for uterine-scar status at birth and 67% for shunt insertion at 12 months. Compared with the generic Clavien-Dindo classification, the MFAET system ranked complications in a more clinically relevant way. CONCLUSIONS: The nature and rate of severe complications following fetal surgery for spina bifida were similar to those reported in other large series. Spontaneous return of outcome data by referring centers was low, yet patient empowerment improved data collection. © 2023 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.

Application of Automatic Segmentation on Super-Resolution Reconstruction MR Images of the Abnormal Fetal Brain.

BACKGROUND AND PURPOSE: Fetal brain MR imaging is clinically used to characterize fetal brain abnormalities. Recently, algorithms have been proposed to reconstruct high-resolution 3D fetal brain volumes from 2D slices. By means of these reconstructions, convolutional neural networks have been developed for automatic image segmentation to avoid labor-intensive manual annotations, usually trained on data of normal fetal brains. Herein, we tested the performance of an algorithm specifically developed for segmentation of abnormal fetal brains. MATERIALS AND METHODS: This was a single-center retrospective study on MR images of 16 fetuses with severe CNS anomalies (gestation, 21-39 weeks). T2-weighted 2D slices were converted to 3D volumes using a super-resolution reconstruction algorithm. The acquired volumetric data were then processed by a novel convolutional neural network to perform segmentations of white matter and the ventricular system and cerebellum. These were compared with manual segmentation using the Dice coefficient, Hausdorff distance (95th percentile), and volume difference. Using interquartile ranges, we identified outliers of these metrics and further analyzed them in detail. RESULTS: The mean Dice coefficient was 96.2%, 93.7%, and 94.7% for white matter and the ventricular system and cerebellum, respectively. The Hausdorff distance was 1.1, 2.3, and 1.6 mm, respectively. The volume difference was 1.6, 1.4, and 0.3 mL, respectively. Of the 126 measurements, there were 16 outliers among 5 fetuses, discussed on a case-by-case basis. CONCLUSIONS: Our novel segmentation algorithm obtained excellent results on MR images of fetuses with severe brain abnormalities. Analysis of the outliers shows the need to include pathologies underrepresented in the current data set. Quality control to prevent occasional errors is still needed.

Longitudinal MRI Evaluation of Brain Development in Fetuses with Congenital Diaphragmatic Hernia around the Time of Fetal Endotracheal Occlusion.

BACKGROUND AND PURPOSE: Congenital diaphragmatic hernia is associated with high mortality and morbidity, including evidence suggesting neurodevelopmental comorbidities after birth. The aim of this study was to document longitudinal changes in brain biometry and the cortical folding pattern in fetuses with congenital diaphragmatic hernia compared with healthy fetuses. MATERIALS AND METHODS: This is a retrospective cohort study including fetuses with isolated congenital diaphragmatic hernia between January 2007 and May 2019, with at least 2 MR imaging examinations. For controls, we used images from fetuses who underwent MR imaging for an unrelated condition that did not compromise fetal brain development and fetuses from healthy pregnant women. Biometric measurements and 3D segmentations of brain structures were used as well as qualitative and quantitative grading of the supratentorial brain. Brain development was correlated with disease-severity markers. RESULTS: Forty-two fetuses were included, with a mean gestational age at first MR imaging of 28.0 (SD, 2.1) weeks and 33.2 (SD, 1.3) weeks at the second imaging. The mean gestational age in controls was 30.7 (SD, 4.2) weeks. At 28 weeks, fetuses with congenital diaphragmatic hernia had abnormal qualitative and quantitative maturation, more extra-axial fluid, and larger total skull volume. By 33 weeks, qualitative grading scores were still abnormal, but quantitative scoring was in the normal range. In contrast, the extra-axial fluid volume remained abnormal with increased ventricular volume. Normal brain parenchymal volumes were found. CONCLUSIONS: Brain development in fetuses with congenital diaphragmatic hernia around 28 weeks appears to be delayed. This feature is less prominent at 33 weeks. At this stage, there was also an increase in ventricular and extra-axial space volume.

Anatomical and diffusion-weighted imaging of brain abnormalities in third-trimester fetuses with cytomegalovirus infection.

OBJECTIVES: In this study of cytomegalovirus (CMV)-infected fetuses with first-trimester seroconversion, we aimed to evaluate the detection of brain abnormalities using magnetic resonance imaging (MRI) and neurosonography (NSG) in the third trimester, and compare the grading systems of the two modalities. We also evaluated the feasibility of routine use of diffusion-weighted imaging (DWI) fetal MRI and compared the regional apparent diffusion coefficient (ADC) values between CMV-infected fetuses and presumed normal, non-infected fetuses in the third trimester. METHODS: This was a retrospective review of MRI and NSG scans in fetuses with confirmed first-trimester CMV infection performed between September 2015 and August 2019. Brain abnormalities were recorded and graded using fetal MRI and NSG grading systems to compare the two modalities. To investigate feasibility of DWI, a four-point rating scale (poor, suboptimal, good, excellent) was applied to assess the quality of the images. Quantitative assessment was performed by placing a freehand drawn region of interest in the white matter of the frontal, parietal, temporal and occipital lobes and the basal ganglia, pons and cerebellum to calculate ADC values. Regional ADC measurements were obtained similarly in a control group of fetuses with negative maternal CMV serology in the first trimester, normal brain findings on fetal MRI and normal genetic testing. RESULTS: Fifty-three MRI examinations of 46 fetuses with confirmed first-trimester CMV infection were included. NSG detected 24 of 27 temporal cysts seen on MRI scans, with a sensitivity of 78% and an accuracy of 83%. NSG did not detect abnormal gyration visible on two (4%) MRI scans. Periventricular calcifications were detected on two MRI scans compared with 10 NSG scans. While lenticulostriate vasculopathy was detected on 11 (21%) NSG scans, no fetus demonstrated this finding on MRI. MRI grading correlated significantly with NSG grading of brain abnormalities (P < 0.0001). Eight (15%) of the DWI scans in the CMV cohort were excluded from further analysis because of insufficient quality. The ADC values of CMV-infected fetuses were significantly increased in the frontal (both sides, P < 0.0001), temporal (both sides, P < 0.0001), parietal (left side, P = 0.0378 and right side, P = 0.0014) and occipital (left side, P = 0.0002 and right side, P < 0.0001) lobes and decreased in the pons (P = 0.0085) when compared with non-infected fetuses. The ADC values in the basal ganglia and the cerebellum were not significantly different in CMV-infected fetuses compared with normal controls (all P > 0.05). Temporal and frontal ADC values were higher in CMV-infected fetuses with more severe brain abnormalities compared to fetuses with mild abnormalities. CONCLUSIONS: Ultrasound and MRI are complementary during the third trimester in the assessment of brain abnormalities in CMV-infected fetuses, with a significant correlation between the grading systems of the two modalities. On DWI in the third trimester, the ADC values in several brain regions are abnormal in CMV-infected fetuses compared with normal controls. Furthermore, they seem to correlate in the temporal area and, to a lesser extent, frontal area with the severity of brain abnormalities associated with CMV infection. Larger prospective studies are needed for further investigation of the microscopic nature of diffusion abnormalities and correlation of different imaging findings with postnatal outcome. © 2022 International Society of Ultrasound in Obstetrics and Gynecology.

Prevalence of supratentorial anomalies assessed by magnetic resonance imaging in fetuses with open spina bifida.

OBJECTIVES: To determine the prevalence of brain anomalies at the time of preoperative magnetic resonance imaging (MRI) assessment in fetuses eligible for prenatal open spina bifida (OSB) repair, and to explore the relationship between brain abnormalities and features of the spinal defect. METHODS: This was a retrospective cross-sectional study, conducted in three fetal medicine centers, of fetuses eligible for OSB fetal surgery repair between January 2009 and December 2019. MRI images obtained as part of the presurgical assessment were re-evaluated by two independent observers, blinded to perinatal results, to assess: (1) the type and area of the defect and its anatomical level; (2) the presence of any structural central nervous system (CNS) anomaly and abnormal ventricular wall; and (3) fetal head and brain biometry. Binary regression analyses were performed and data were adjusted for type of defect, upper level of the lesion (ULL), gestational age (GA) at MRI and fetal medicine center. Multiple logistic regression analysis was performed in order to identify lesion characteristics and brain anomalies associated with a higher risk of presence of abnormal corpus callosum (CC) and/or heterotopia. RESULTS: Of 115 fetuses included, 91 had myelomeningocele and 24 had myeloschisis. Anatomical level of the lesion was thoracic in seven fetuses, L1-L2 in 13, L3-L5 in 68 and sacral in 27. Median GA at MRI was 24.7 (interquartile range, 23.0-25.7) weeks. Overall, 52.7% of cases had at least one additional brain anomaly. Specifically, abnormal CC was observed in 50.4% of cases and abnormality of the ventricular wall in 19.1%, of which 4.3% had nodular heterotopia. Factors associated independently with higher risk of abnormal CC and/or heterotopia were non-sacral ULL (odds ratio (OR), 0.51 (95% CI, 0.26-0.97); P = 0.043), larger ventricular width (per mm) (OR, 1.23 (95% CI, 1.07-1.43); P = 0.005) and presence of abnormal cavum septi pellucidi (OR, 3.76 (95% CI, 1.13-12.48); P = 0.031). CONCLUSIONS: Half of the fetuses assessed for OSB repair had an abnormal CC and/or an abnormal ventricular wall prior to prenatal repair. The likelihood of brain abnormalities was increased in cases with a non-sacral lesion and wider lateral ventricles. These findings highlight the importance of a detailed preoperative CNS evaluation of fetuses with OSB. © 2021 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.

Complex gastroschisis: a new indication for fetal surgery?

Gastroschisis (GS) is a congenital abdominal wall defect, in which the bowel eviscerates from the abdominal cavity. It is a non-lethal isolated anomaly and its pathogenesis is hypothesized to occur as a result of two hits: primary rupture of the 'physiological' umbilical hernia (congenital anomaly) followed by progressive damage of the eviscerated bowel (secondary injury). The second hit is thought to be caused by a combination of mesenteric ischemia from constriction in the abdominal wall defect and prolonged amniotic fluid exposure with resultant inflammatory damage, which eventually leads to bowel dysfunction and complications. GS can be classified as either simple or complex, with the latter being complicated by a combination of intestinal atresia, stenosis, perforation, volvulus and/or necrosis. Complex GS requires multiple neonatal surgeries and is associated with significantly greater postnatal morbidity and mortality than is simple GS. The intrauterine reduction of the eviscerated bowel before irreversible damage occurs and subsequent defect closure may diminish or potentially prevent the bowel damage and other fetal and neonatal complications associated with this condition. Serial prenatal amnioexchange has been studied in cases with GS as a potential intervention but never adopted because of its unproven benefit in terms of survival and bowel and lung function. We believe that recent advances in prenatal diagnosis and fetoscopic surgery justify reconsideration of the antenatal management of complex GS under the rubric of the criteria for fetal surgery established by the International Fetal Medicine and Surgery Society (IFMSS). Herein, we discuss how conditions for fetoscopic repair of complex GS might be favorable according to the IFMSS criteria, including an established natural history, an accurate prenatal diagnosis, absence of fully effective perinatal treatment due to prolonged need for neonatal intensive care, experimental evidence for fetoscopic repair and maternal and fetal safety of fetoscopy in expert fetal centers. Finally, we propose a research agenda that will help overcome barriers to progress and provide a pathway toward clinical implementation. © 2021 International Society of Ultrasound in Obstetrics and Gynecology.

Outcome of monochorionic twin pregnancy with selective fetal growth restriction at 16, 20 or 30 weeks according to new Delphi consensus definition.

OBJECTIVES: To report the outcome of selective fetal growth restriction (sFGR) diagnosed according to the new Delphi consensus definition, and determine potential predictors of survival, in a cohort of unselected monochorionic diamniotic twin pregnancies. METHODS: This was a retrospective study of monochorionic diamniotic twin pregnancies followed from the first trimester onward, which were diagnosed with sFGR at 16, 20 or 30 weeks' gestation. sFGR was defined according to the new Delphi consensus criteria as presence of either an estimated fetal weight (EFW) < 3rd centile in one twin or at least two of the following: EFW of one twin < 10th centile, abdominal circumference of one twin < 10th centile, EFW discordance ≥ 25% or umbilical artery pulsatility index of the smaller twin > 95th centile. The primary outcomes were the overall survival rate (up to day 28 after birth) and risk of loss of one or both twins. We further determined possible predictors of survival using uni- and multivariate generalized estimated equation modeling. RESULTS: We analyzed 675 pregnancies, of which 177 (26%) were diagnosed with sFGR at 16, 20 or 30 weeks. The overall survival rate was 313/354 (88%) with 146/177 (82%) pregnancies resulting in survival of both twins, 21/177 (12%) in survival of one twin and 10/177 (6%) in loss of both twins. Subsequent twin anemia-polycythemia sequence (TAPS) developed in 6/177 (3%) and twin-twin transfusion syndrome (TTTS) in 17/177 (10%) pregnancies. All TAPS fetuses survived. The survival rate in sFGR pregnancies that subsequently developed TTTS was 65% (22/34), compared with 91% (279/308) in those with isolated sFGR (no subsequent TAPS or TTTS) (P < 0.001). The majority of sFGR cases were Type I (110/177 (62%)) and had a survival rate of 96% (212/220), as compared with a survival of 55% (12/22) in those with Type-II (P < 0.001) and 83% (55/66) in those with Type-III (P = 0.006) sFGR. The majority of sFGR pregnancies (130/177 (73%)) were first diagnosed at 16 or 20 weeks (early onset), with a survival rate of 85% (221/260), as compared with a survival of 98% (92/94) in sFGR first diagnosed at 30 weeks (late onset) (P = 0.04). A major anomaly in at least one twin was present in 28/177 (16%) sFGR cases. In these pregnancies, survival was 39/56 (70%), compared with 274/298 (92%) in those without an anomaly (P < 0.001). Subsequent development of TTTS (odds ratio (OR), 0.18 (95% CI, 0.06-0.52)), Type-II sFGR (OR, 0.06 (95% CI, 0.02-0.24)) and Type-III sFGR (OR, 0.21 (95% CI, 0.07-0.60)) and presence of a major anomaly in at least one twin (OR, 0.12 (95% CI, 0.04-0.34)), but not gestational age at first diagnosis, were independently associated with decreased survival. CONCLUSIONS: Isolated sFGR is associated with a 90% survival rate in monochorionic diamniotic twin pregnancies. The subsequent development of TTTS, absent or reversed end-diastolic flow in the umbilical artery of the smaller twin and the presence of a major anomaly adversely affect survival in sFGR. Copyright © 2020 ISUOG. Published by John Wiley & Sons Ltd.

Laser for twin-to-twin transfusion syndrome: a guide for endoscopic surgeons.

Twin-to-twin-transfusion syndrome (TTTS) is the most important cause of handicap and death in monochorionic twin pregnancies. It is caused by a certain pattern of anastomoses between the two fetal circulations leading to an unbalanced blood and fluid transfer. This leads to severe amniotic fluid discordance and variable degrees of cardiac dysfunction. Untreated, this condition has a very poor survival rate. Fetoscopic laser has been shown to be the best first line treatment, which aims to dichorionise the placenta therefore arresting the inter-twin transfusion. Fetoscopic laser is a causative therapy, which aims to functionally create a dichorionized placenta hence arresting inter-twin transfusion. This is achieved by percutaneous sono-endoscopic coagulation of placental anastomoses. In addition, redundant amniotic fluid is drained. Fetoscopic laser coagulation of chorionic plate anastomoses is safe and effective. There is level I evidence that it is the best treatment modality, in particular when the placental surface is lined along the vascular equator. A recent meta-analysis confirmed an increased fetal survival and decreased risk for neonatal and pediatric neurologic morbidity. Laser therapy is the first line therapy for TTTS. The technique is quite standardized and safe and effective in experienced hands. Herein we describe the technique and current instrumentation used for this procedure.

Prevention of maternal-fetal transmission of cytomegalovirus after primary maternal infection in the first trimester by biweekly hyperimmunoglobulin administration.

OBJECTIVE: To examine the efficacy of biweekly hyperimmunoglobulin (HIG) administration to prevent maternal-fetal transmission of cytomegalovirus (CMV) in women with primary first-trimester CMV infection. METHODS: This was a prospective observational study of women with confirmed primary CMV infection in the first trimester who had the first HIG administration at or before 14 weeks' gestation. All women had biweekly HIG treatment until 20 weeks' gestation at a dose of 200 IU/kg of maternal body weight. Each subject underwent amniocentesis at least 6 weeks after first presentation at about 20 weeks. Primary outcome was maternal-fetal transmission at the time of amniocentesis, and secondary outcome was the frequency of congenital CMV infection at birth. The results were compared with a historic cohort of women with first-trimester CMV infection who did not undergo HIG treatment and who had amniocentesis at about 20 weeks. RESULTS: Subjects were 40 pregnant women with a primary CMV infection, with a median gestational age at first presentation of 9.6 (range, 5.1-14.3) weeks. On average, HIG administration started at 11.1 weeks and continued until 16.6 weeks. Within this interval, HIG was administered between two and six times in each patient. While CMV immunoglobulin-G (IgG) monitoring showed periodic fluctuations during biweekly HIG administration cycles, high CMV-IgG avidity indices remained stable over the whole treatment period. Maternal-fetal transmission before amniocentesis occurred in only one of the 40 cases (2.5% (95% CI, 0-13.2%)). At delivery, two additional subjects were found to have had late-gestation transmission. Considering all three cases with maternal-fetal transmission, the transmission rate was 7.5% (95% CI, 1.6-20.4%) in our 40 cases. All infected neonates were asymptomatic at birth. The matched historical control group consisted of 108 pregnancies. Thirty-eight transmissions (35.2% (95% CI, 26.2-45.0%)) occurred in the control group, which was significantly higher (P < 0.0001) than the transmission rate in the HIG treatment group. CONCLUSION: After a primary maternal CMV infection in the first trimester, biweekly HIG administration at a dose of 200 IU/kg prevents maternal-fetal transmission up to 20 weeks' gestation. Copyright © 2018 ISUOG. Published by John Wiley & Sons Ltd.

Reliability of MR Imaging-Based Posterior Fossa and Brain Stem Measurements in Open Spinal Dysraphism in the Era of Fetal Surgery.

BACKGROUND AND PURPOSE: Fetal MR imaging is part of the comprehensive prenatal assessment of fetuses with open spinal dysraphism. We aimed to assess the reliability of brain stem and posterior fossa measurements; use the reliable measurements to characterize fetuses with open spinal dysraphism versus what can be observed in healthy age-matched controls; and document changes in those within 1 week after prenatal repair. MATERIALS AND METHODS: Retrospective evaluation of 349 MR imaging examinations took place, including 274 in controls and 52 in fetuses with open spinal dysraphism, of whom 23 underwent prenatal repair and had additional early postoperative MR images. We evaluated measurements of the brain stem and the posterior fossa and the ventricular width in all populations for their reliability and differences between the groups. RESULTS: The transverse cerebellar diameter, cerebellar herniation level, clivus-supraocciput angle, transverse diameter of the posterior fossa, posterior fossa area, and ventricular width showed an acceptable intra- and interobserver reliability (intraclass correlation coefficient > 0.5). In fetuses with open spinal dysraphism, these measurements were significantly different from those of healthy fetuses (all with P < .0001). Furthermore, they also changed significantly (P value range = .01 to < .0001) within 1 week after the fetal operation with an evolution toward normal, most evident for the clivus-supraocciput angle (65.9 ± 12.5°; 76.6 ± 10.9; P < .0001) and cerebellar herniation level (-9.9 ± 4.2 mm; -0.7 ± 5.2; P < .0001). CONCLUSIONS: In fetuses with open spinal dysraphism, brain stem measurements varied substantially between observers. However, measurements characterizing the posterior fossa could be reliably assessed and were significantly different from normal. Following a fetal operation, these deviations from normal values changed significantly within 1 week.

Sonographic Development of the Pericallosal Vascularization in the First and Early Second Trimester of Pregnancy.

BACKGROUND AND PURPOSE: Anomalies of the corpus callosum are rare. Routine scanning in midtrimester of the pregnancy often fails to identify defective development. The purpose of the study was to identify the pericallosal artery and all its main branching arteries during early gestation from the first trimester onward, to measure the length of the pericallosal artery during its development, and to establish a normal vascular map for each week of development. MATERIALS AND METHODS: We performed a single-center prospective, longitudinal clinical study in 15 patients between 11 and 22 weeks of gestation. The origin and course of the different blood vessels were identified. RESULTS: There was a linear association among gestational age, the biparietal diameter, and the length of the pericallosal artery. The curvature of the developing pericallosal artery increases linearly with the gestational age and biparietal diameter, and 4 variations of branching of the callosomarginal artery were observed. CONCLUSIONS: The pericallosal artery and its branches can be identified and measured from 11 weeks on, and the pericallosal artery takes its characteristic course. A defective course or an abnormal biometry of the pericallosal artery could be an early sonographic marker of abnormal development of the corpus callosum.

Does site of cord insertion increase risk of adverse outcome, twin-to-twin transfusion syndrome and discordant growth in monochorionic twin pregnancy?

OBJECTIVES: It is not currently well known to what extent the sites of cord insertion influence the risk of complicated outcome in monochorionic twin pregnancy. The objectives of this study were to examine whether the sites of cord insertion, as determined on prenatal ultrasound examination, affect the risks of adverse outcome, twin-to-twin transfusion syndrome (TTTS) and discordant growth, and whether discordance in insertion sites or velamentous insertion in one or both twins best predicts risk. METHODS: This was a retrospective cohort study of monochorionic diamniotic twin pregnancies followed from the first trimester. The cohort was divided into three groups of increasing discordance in cord insertion sites: concordant (normal-normal; marginal-marginal; velamentous-velamentous), intermediate (normal-marginal; marginal-velamentous) and discordant (normal-velamentous). Adverse outcome was defined as fetal or neonatal loss or birth prior to 32 weeks. The associations of adverse outcome, TTTS and discordant growth were assessed using logistic regression analysis with the following predictors: the three groups of insertion sites and velamentous insertion in one or both twins. RESULTS: Included in the analysis were 518 pregnancies. On univariate analysis, both discordant and velamentous insertions in one twin increased the risk of adverse outcome, TTTS and discordant growth. Intermediate insertion only increased the risk of discordant growth. Velamentous insertion in both twins increased the risk of adverse outcome and TTTS, but not of discordant growth. Multivariate logistic regression analysis showed velamentous insertion in one or both twins to independently predict adverse outcome and TTTS. For discordant growth, both intermediate/discordant and velamentous cord insertion in one twin were independent predictors. CONCLUSIONS: Velamentous cord insertion in one or both twins increases the risk of adverse outcome and TTTS, irrespective of discordance in the insertion sites, whereas the risk of discordant growth is determined by both discordance in insertion sites and velamentous cord insertion in one twin. Copyright © 2017 ISUOG. Published by John Wiley & Sons Ltd.

Sonographic detection of central nervous system defects in the first trimester of pregnancy.

The fetal central nervous system can already be examined in the first trimester of pregnancy. Acrania, alobar holoprosencephaly, cephaloceles, and spina bifida can confidently be diagnosed at that stage and should actively be looked for in every fetus undergoing first-trimester ultrasound. For some other conditions, such as vermian anomalies and agenesis of the corpus callosum, markers have been identified, but the diagnosis can only be confirmed in the second trimester of gestation. For these conditions, data on sensitivity and more importantly specificity and false positives are lacking, and one should therefore be aware not to falsely reassure or scare expecting parents based on first-trimester findings. This review summarizes the current knowledge of first-trimester neurosonography in the normal and abnormal fetus and gives an overview of which diseases can be diagnosed.

Intrauterine transfusion for fetal anemia due to red blood cell alloimmunization: 14 years experience in Leuven.

OBJECTIVE: The purpose of this study is to report on the pregnancy and neonatal outcome of intrauterine transfusion (IUT) for red blood cell (RBC-)alloimmunization. MATERIAL AND METHODS: Retrospective cohort study of all IUT for RBC-alloimmunization in the University Hospital of Leuven, between January 2000 and January 2014. The influence of hydrops, gestational age and technique of transfusion on procedure related adverse events were examined. RESULTS: 135 IUTs were performed in 56 fetuses. In none of the cases fetal or neonatal death occurred. Mild adverse events were noted in 10% of IUTs, whereas severe adverse events occurred in 1.5%. Hydrops and transfusion in a free loop were associated with an increased risk of adverse events whereas gestational age (GA) at transfusion after 34 weeks was not. Median GA at birth was 35.6 weeks and 9% was born before 34 weeks. Besides phototherapy 65.4% required additional neonatal treatment for alloimmune anemia. Non-hematologic complications occurred in 23.6% and were mainly related to preterm birth. CONCLUSION: In experienced hands, IUT for RBC-alloimmunization is a safe procedure in this era. Patients should be referred to specialist centers prior to the development of hydrops. IUT in a free loop of cord and unnecessary preterm birth are best avoided.

Simulator training in fetoscopic laser surgery for twin-twin transfusion syndrome: a pilot randomized controlled trial.

OBJECTIVE: To evaluate the effect of a newly developed training curriculum on the performance of fetoscopic laser surgery for twin-twin transfusion syndrome (TTTS) using an advanced high-fidelity simulator model. METHODS: Ten novices were randomized to receive verbal instructions and either skills training using the simulator (study group; n = 5) or no training (control group; n = 5). Both groups were evaluated with a pre-training and post-training test on the simulator. Performance was assessed by two independent observers and comprised a 52-item checklist for surgical performance (SP) score, measurement of procedure time and number of anastomoses missed. Eleven experts set the benchmark level of performance. Face validity and educational value of the simulator were assessed using a questionnaire. RESULTS: Both groups showed an improvement in SP score at the post-training test compared with the pre-training test. The simulator-trained group significantly outperformed the control group, with a median SP score of 28 (54%) in the pre-test and 46 (88%) in the post-test vs 25 (48%) and 36 (69%), respectively (P = 0.008). Procedure time decreased by 11 min (from 44 to 33 min) in the study group vs 1 min (from 39 to 38 min) in the control group (P = 0.69). There was no significant difference in the number of missed anastomoses at the post-training test between the two groups (1 vs 0). Subsequent feedback provided by the participants indicated that training on the simulator was perceived as a useful educational activity. CONCLUSIONS: Proficiency-based simulator training improves performance, indicated by SP score, for fetoscopic laser therapy. Despite the small sample size of this study, practice on a simulator is recommended before trainees carry out laser therapy for TTTS in pregnant women.

Outcome of 12 antenatally diagnosed fetal arachnoid cysts: case series and review of the literature.

OBJECTIVES: To investigate the natural history, associated abnormalities and outcome of 12 fetuses with arachnoid cyst diagnosed antenatally by ultrasound and magnetic resonance imaging and to compare the outcome with cases in the literature. METHODS: A retrospective study of all cases of antenatally detected fetal arachnoid cysts was performed in patients referred to a tertiary unit between 2007 and 2013. Associated abnormalities, pregnancy outcome and postnatal follow-up were analyzed. All papers about prenatally diagnosed arachnoid cysts, of the last 30 years, were evaluated (search terms in Pubmed: "prenatal diagnosis", "Arachnoid Cysts"). RESULTS: Fetal arachnoid cysts were diagnosed in 12 fetuses, 9 were females. The mean gestational age of diagnosis was 28 1/7 (range 19 1/7-34 2/7 weeks). A total of 9 cases were supratentorial, 3 were located in the posterior fossa. In 10 cases a fetal MRI was performed which confirmed brain compression in 4 out of 5 supratentorial arachnoid cyst. MRI did not reveal other malformations nor signs of nodular heterotopia. Only one fetus presented with additional major anomalies (bilateral ventricumomegaly of >20 mm and rhombencephalosynapsis) leading to a termination of pregnancy. Two neonates underwent endoscopic fenestration of the arachnoid cyst in the first week of life with no additional intervention in childhood. All but one (10/11) had a favorable postnatal outcome. This child suffered from visual impairment at autism was diagnosed at the age of 5. One child had a surgical correction of strabismus later in childhood. In one child the infratentorial arachnoid cyst regressed spontaneously on ultrasound and MRI in the postnatal period. CONCLUSIONS: The majority of arachnoid cysts in this series are of benign origin and remain stable. Based on the current series and the review of the literature, in the absence of other associated anomalies and when the karyotype is normal, the postnatal overall and neurological outcome is favorable. Large suprasellar arachnoid cysts however, may cause visual impairment and endocrinological disturbances. Rarely associated cerebral or cerebellar malformations are present. Modern postnatal management of suprasellar arachnoid cyst consists of endoscopic cystoventriculostomy.

Comparison of matching by body volume or gestational age for calculation of observed to expected total lung volume in fetuses with isolated congenital diaphragmatic hernia.

OBJECTIVE: To determine the bias induced by matching fetuses according to gestational age (GA) or fetal body volume (FBV) when calculating the observed to expected total fetal lung volume (o/e TFLV) in cases of isolated congenital diaphragmatic hernia (CDH). METHODS: This was a single-center, retrospective study on archived magnetic resonance (MR) images of fetuses with isolated CDH over a 10-year period. We retrieved the TFLV, GA and o/e TFLVGA , and delineated FBV to obtain TFLVFBV in each case. We evaluated the relationship between o/e TFLVFBV and o/e TFLVGA by Bland-Altman analysis. All outliers were manually identified, and their specific clinical features were retrieved. RESULTS: Records of a total of 377 MR examinations of 225 fetuses were identified and included in the analysis. The mean ( ± SD) time spent on FBV measurement was 16.12 ± 4.95 min. On reproducibility analysis of FBV measurement (n = 10), the intraobserver intraclass correlation coefficient (ICC) was 0.998 and the interobserver ICC was 0.999. FBV was highly correlated with GA (R(2) = 0.899; P < 0.0001). There was good agreement between o/e TFLVGA and o/e TFLVFBV , with a mean difference of -1.10% and 95% limits of agreement of -8.58 to 6.39. There were no outliers in fetuses that had an o/e TFLV < 25%. Discrepancies induced by different methods were more likely in women with a body mass index ≥ 25 kg/m(2) (+16.5%), fetuses with an estimated fetal weight (EFW) ≤ 10(th) centile (+21.3%) or an EFW > 90(th) centile (+14.7%). CONCLUSIONS: Discrepancies in matching by FBV and GA when calculating o/e TFLV are more likely in fetuses with an abnormal EFW or in fetuses carried by overweight women. The clinical relevance of using FBV rather than GA for calculation of the o/e TFLV might be limited, as there was no discrepancy between the two methods in fetuses with small lungs ( < 25%), which is the group of most interest for lung volume assessment.