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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., (Copyright © 2017 ISUOG. Published by John Wiley & Sons Ltd.)

Objectives: Fetoscopic laser surgery for twin-twin transfusion syndrome is a procedure for which no objective tools exist to assess technical skills. To ensure that future fetal surgeons reach competence prior to performing the procedure unsupervised, we developed a performance assessment tool. The aim of this study was to validate this assessment tool for reliability and construct validity., Methods: We made use of a procedure-specific evaluation instrument containing all essential steps of the fetoscopic laser procedure, which was previously created using Delphi methodology. Eleven experts and 13 novices from three fetal medicine centers performed the procedure on the same simulator. Two independent observers assessed each surgery using the instrument (maximum score: 52). Interobserver reliability was assessed using Spearman correlation. We compared the performance of novices and experts to assess construct validity., Results: The interobserver reliability was high (Rs  = 0.974, P < 0.001). Checklist scores for experts and novices were significantly different; the median score for novices was 28/52 (54%), whereas that for experts was 47.5/52 (91%) (P < 0.001). The procedure time and fetoscopy time were significantly shorter (P < 0.001) for experts. Residual anastomoses were found in 1/11 (9%) procedures performed by experts and in 9/13 (69%) procedures performed by novices (P = 0.005). Multivariable analysis showed that the checklist score, independent of age and gender, predicted competence., Conclusions: The procedure-specific assessment tool for fetoscopic laser surgery shows good interobserver reliability and discriminates experts from novices. This instrument may therefore be a useful tool in the training curriculum for fetal surgeons. Further intervention studies with reassessment before and after training may increase the construct validity of the tool. Copyright © 2015 ISUOG. Published by John Wiley & Sons Ltd., (Copyright © 2015 ISUOG. Published by John Wiley & Sons Ltd.)

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., (Copyright © 2015 ISUOG. Published by John Wiley & Sons Ltd.)

Objective: To determine, by expert consensus, the essential substeps of fetoscopic laser surgery (FLS) for twin-twin transfusion syndrome (TTTS) that could be used to create an authority-based curriculum for training in this procedure among fetal medicine specialists., Methods: A Delphi survey was conducted among an international panel of experts (n = 98) in FLS. Experts rated the substeps of FLS on a five-point Likert-type scale to indicate whether they considered them to be essential, and were able to comment on each substep, using a dedicated online platform accessed by the invited tertiary care facilities that specialize in fetal therapy. Responses were returned to the panel until consensus was reached (Cronbach's α ≥ 0.80). All substeps that were rated  ≥ 4 by 80% of the experts were included in the evaluation instrument., Results: After the first iteration of the Delphi procedure, a response rate of 74% (73/98) was reached, and in the second and third iterations response rates of 90% (66/73) and 81% (59/73) were reached, respectively. Among a total of 81 substeps rated in the first round, 21 substeps had to be re-rated in the second round. Finally, from the initial list of substeps, 55 were agreed by experts to be essential. In the third round, the 18 categorized substeps were ranked in order of importance, with 'coagulation of all anastomoses that cross the equator' and 'determination of fetoscope insertion site' as the most important., Conclusions: A total of 55 substeps of FLS for TTTS were defined by a panel of experts to be essential in the procedure. This list is the first authority-based evidence to be used in the development of a final training model for future fetal surgeons., (Copyright © 2014 ISUOG. Published by John Wiley & Sons Ltd.)

Objectives: To test the validity of the myocardial performance index (MPI) and its components against the more conventional methods of fetal cardiac function assessment: the ejection fraction (EF) for systolic function and the E/A index (ratio of transmitral flow during early (E) ventricular filling to flow during atrial (A) contraction) for diastolic function, both in a normal population and in a population at risk for cardiac failure because of volume overload (recipient fetuses in cases of twin-twin transfusion syndrome (TTTS))., Methods: The MPI was measured prospectively in addition to more commonly used indices of systolic (EF) and diastolic (E/A index) cardiac function in 117 healthy fetuses (gestational age range, 20-36 weeks) and in 14 fetuses suspected of cardiac failure because of the presence of TTTS. Nomograms were constructed for all variables, and correlations between the MPI, EF and E/A index were assessed. The time taken to obtain the measurements as well as the interobserver and intraobserver variability were determined for the MPI and EF., Results: In healthy fetuses, the MPI and EF were independent of gestational age, whereas the E/A index and isovolumetric relaxation time (IRT) increased with gestational age. The MPI correlated inversely with the EF (P<0.001). The IRT showed a trend towards an inverse correlation with the E/A index (P=0.10). The mean+/-SD time needed to measure the MPI and EF was 140+/-65 s and 185+/-187 s, respectively (P=0.43). Interobserver and intraobserver intraclass correlation coefficients for the MPI were 0.98 (95% CI, 0.85-0.99) and 0.82 (95% CI, 0.14-0.95), respectively; those for the EF were 0.58 (95% CI, -0.16 to 0.85) and 0.51 (95% CI, -0.46 to 0.83), respectively; and those for the E/A index were 0.97 (95% CI, 0.88-0.99) and 0.91 (95% CI, 0.66-0.98), respectively. All variables, except ejection time, were significantly different between normal fetuses and those with TTTS., Conclusions: The MPI is an indicator of the systolic component of fetal left ventricular function that can be easily acquired and reproduced. The MPI is strongly correlated with the EF but shows less interobserver and intraobserver variability., (Copyright (c) 2008 ISUOG.)

Objective: To evaluate the impact of fetoscopic laser coagulation of placental anastomoses in severe twin-twin transfusion syndrome on fetal Doppler indices and umbilical vein blood flow volume as calculated with Doppler and two-dimensional ultrasound., Methods: Thirty-two cases of second-trimester severe twin-twin transfusion undergoing laser therapy were examined with serial ultrasound before and 1, 3 and 5 days after therapy. Pulsatility indices in the umbilical artery and ductus venosus were measured. Blood volume flow at the level of the intra-abdominal umbilical vein was calculated by means of Doppler and two-dimensional ultrasound. The development of hydropic signs in donors was recorded. Perinatal outcome in terms of neonatal survival was recorded for all cases., Results: In recipients, ductus venosus pulsatility index decreased progressively after therapy and, by day 5, median pulsatility index was significantly lower than that before therapy (0.97 vs. 0.82, P < 0.0001). Umbilical vein blood flow volume in recipient twins showed no significant variations before and after laser. In donors, umbilical artery pulsatility index decreased significantly by the first day following therapy (2.1 vs. 1.6, P < 0.0001). Previously absent or reverse umbilical end-diastolic flow reappeared after therapy in 46% (7/15) of donors. Ductus venosus pulsatility index in donors increased significantly by day 1 after therapy (0.99 vs. 1.35, P < 0.0001) but, over days 3 and 5, it returned towards preoperative values. Umbilical vein flow volume measurements (mL/min/kg) in the donor increased by approximately 50% the day after treatment (151 vs. 232, P < 0.0001) and remained elevated. Umbilical vein flow volume before laser therapy was significantly lower in donors compared to recipients (151 vs. 260, P < 0.0001), but the difference was non-significant after treatment (240 vs. 267). One or more hydropic signs developed in eight (25%) donors during the 5 days' observation after therapy., Conclusions: Laser therapy induced important changes in fetal hemodynamic parameters, resulting in a reversion of the disturbances associated with severe twin-twin transfusion syndrome. The recipient twin showed a progressive improvement of previous signs of right cardiac overload. The donor experienced a substantial increase in umbilical vein blood volume flow accompanied by a transitory state of relative right overload, which may explain the development of transient hydropic signs in a proportion of donors.

Objective: To evaluate the incidence, characteristics, and impact on perinatal outcome of the development of hydropic signs in the donor fetus after laser coagulation for severe twin-twin transfusion syndrome in the second trimester., Methods: Forty cases of second-trimester severe twin-twin transfusion underwent fetoscopic laser coagulation of placental anastomoses and were followed up with serial ultrasound up to 14 days after therapy. Ultrasound scans were performed just before, and on days 1, 3, 5 and 14 after therapy, and fetuses were monitored for the presence of hydropic signs (skin edema, pleural effusion, ascites and pericardial effusion). Response to therapy and perinatal outcome in terms of neonatal survival were recorded for all cases., Results: Four recipients (10%) and three donors (7.5%) died over the first 72 h after therapy. During the study period, two recipients (5.5%) developed frank hydrops fetalis, and eventually died in utero. Ten donors (27.0%) developed one or more hydropic signs. In nine (90%), signs were mild or moderate and transient (mean, 3.2 days), while in one case hydrops worsened and the fetus eventually died. No donors with transient hydrops died in utero, and two died postnatally due to extreme prematurity. Gestational age at procedure and deepest amniotic fluid vertical pocket were similar between hydropic and non-hydropic donors. Discrepancy in estimated fetal weight at procedure was significantly lower in donors developing hydrops (9.6 (SD, 6.4) vs. 17.8 (14.4)%,P = 0.018). In pregnancies with hydropic donors, gestational age at delivery (33.1 (3.4) vs. 29.7 (3.5) weeks,P = 0.015) and birth weight for recipient (2392 (423) vs. 1631 (576) g, P < 0.001) and donor (1908 (774) vs. 1281 (583) g,P = 0.021) were significantly higher. The development of transient hydrops had no influence on neonatal survival at 28 days nor on the rate of clinical resolution of intertwin transfusion., Conclusions: Transient hydropic signs may develop in a proportion of donor fetuses after laser coagulation for severe twin-twin transfusion syndrome. This phenomenon is normally not associated with a poor prognosis, and possibly indicates an hemodynamic adaptation response following interruption of the transfusion process.

Outcome of monochorionic twin pregnancy with selective fetal growth restriction at 16, 20 or 30 weeks according to new Delphi consensus definition Keywords: definition; discordance; growth restriction; monochorionic twin pregnancy; outcome EN definition discordance growth restriction monochorionic twin pregnancy outcome 821 830 10 12/03/20 20201201 NES 201201 CONTRIBUTION B What are the novel findings of this work? More specifically, a pregnancy with sFGR complicated by TTTS at 19 weeks was included in the 16-week analysis but not in the 20- or 30-week analysis, as this pregnancy would no longer be diagnosed with isolated sFGR. In 44/177 (25%) sFGR pregnancies, the smaller twin had continuous or intermittent absent or reversed end-diastolic umbilical artery flow, which was classified as sFGR Type II in 11 and Type III in 33 pregnancies, with survival rates of 55% (12/22) and 83% (55/66), respectively ( I P i = 0.006). [Extracted from the article]

Objective: The Management of Myelomeningocele Study (MOMS) trial demonstrated the safety and efficacy of open fetal surgery for spina bifida aperta (SBA). Recently developed alternative techniques may reduce maternal risks without compromising the fetal neuroprotective effects. The aim of this systematic review was to assess the learning curve (LC) of different fetal SBA closure techniques.Methods: MEDLINE, Web of Science, EMBASE, Scopus and Cochrane databases and the gray literature were searched to identify relevant articles on fetal surgery for SBA, without language restriction, published between January 1980 and October 2018. Identified studies were reviewed systematically and those reporting all consecutive procedures and with postnatal follow-up ≥ 12 months were selected. Studies were included only if they reported outcome variables necessary to measure the LC, as defined by fetal safety and efficacy. Two authors independently retrieved data, assessed the quality of the studies and categorized observations into blocks of 30 patients. For meta-analysis, data were pooled using a random-effects model when heterogeneous. To measure the LC, we used two complementary methods. In the group-splitting method, competency was defined when the procedure provided results comparable to those in the MOMS trial for 12 outcome variables representing the immediate surgical outcome, short-term neonatal neuroprotection and long-term neuroprotection at ≥ 12 months of age. Then, when raw patient data were available, we performed cumulative sum analysis based on a composite binary outcome defining successful surgery. The composite outcome combined four clinically relevant variables for safety (absence of extreme preterm delivery < 30 weeks, absence of fetal death ≤ 7 days after surgery) and efficacy (reversal of hindbrain herniation and absence of any neonatal treatment of dehiscence or cerebrospinal fluid leakage at the closure site).Results: Of 6024 search results, 17 (0.3%) studies were included, all of which had low, moderate or unclear risk of bias. Fetal SBA closure was performed using standard hysterotomy (11 studies), mini-hysterotomy (one study) or fetoscopy by either exteriorized-uterus single-layer closure (one study), percutaneous single-layer closure (three studies) or percutaneous two-layer closure (one study). Only outcomes for standard hysterotomy could be meta-analyzed. Overall, outcomes improved significantly with experience. Competency was reached after 35 consecutive cases for standard hysterotomy and was predicted to be achieved after ≥ 57 cases for mini-hysterotomy and ≥ 56 for percutaneous two-layer fetoscopy. For percutaneous and exteriorized-uterus single-layer fetoscopy, competency was not reached in the 81 and 28 cases available for analysis, respectively, and LC prediction analysis could not be performed.Conclusions: The number of cases operated is correlated with the outcome of fetal SBA closure, and the number of operated cases required to reach competency ranges from 35 for standard hysterotomy to ≥ 56-57 for minimally invasive modifications. Our observations provide important information for institutions looking to establish a new fetal center, develop a new fetal surgery technique or train their team, and inform referring clinicians, potential patients and third parties. Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd. [ABSTRACT FROM AUTHOR]

Objective: To evaluate the diagnostic accuracy of middle cerebral artery peak systolic velocity (MCA-PSV) Doppler measurements in prediction of hemoglobin levels in twin anemia-polycythemia sequence (TAPS).Methods: This study involved a consecutive cohort comprising monochorionic twin pregnancies complicated by TAPS managed at three European fetal medicine centers between 2005 and 2013. The accuracy of MCA-PSV, measured immediately prior to fetal hemoglobin (Hb) measurement by fetal or cord blood sampling, for prediction of anemia and polycythemia was assessed using 2 × 2 tables.Results: A total of 116 measurements (74 recorded in donors and 42 in recipients) from 43 twin pregnancies complicated by TAPS were available for analysis. MCA-PSV multiples of the median (MoM) values correlated well with Hb levels (r = - 0.86; P < 0.001). The sensitivity of MCA-PSV ≥ 1.5 MoM to predict severe anemia (Hb deficit > 5 SD below the mean) in TAPS donors was 94% (95% CI, 85-98%); specificity was 74% (95% CI, 62-83%); positive and negative predictive values were 76% (95% CI, 65-85%) and 94% (95% CI, 83-98%), respectively. The sensitivity of MCA-PSV ≤ 1.0 MoM to predict polycythemia (Hb level > 5 SD above the mean) in TAPS recipients was 97% (95% CI, 87-99%); specificity was 96% (95% CI, 89-99%); positive and negative predictive values were 93% (95% CI, 81-97%) and 99% (95% CI, 93-100%), respectively.Conclusion: MCA-PSV measurement has high diagnostic accuracy for predicting abnormal Hb levels in fetuses with TAPS. [ABSTRACT FROM AUTHOR]