Objective To evaluate the neonatal outcome of fetuses with isolated right‐sided congenital diaphragmatic hernia (iRCDH) based on prenatal severity indicators and antenatal management. Methods This was a retrospective review of prospectively collected data on consecutive cases diagnosed with iRCDH before 30 weeks' gestation in four fetal therapy centers, between January 2008 and December 2018. Data on prenatal severity assessment, antenatal management and perinatal outcome were retrieved. Univariate and multivariate logistic regression analysis were used to identify predictors of survival at discharge and early neonatal morbidity. Results Of 265 patients assessed during the study period, we excluded 40 (15%) who underwent termination of pregnancy, two cases of unexplained fetal death, two that were lost to follow‐up, one for which antenatal assessment of lung hypoplasia was not available and six cases which were found to have major associated anomalies or syndromes after birth. Of the 214 fetuses with iRCDH included in the neonatal outcome analysis, 86 were managed expectantly during pregnancy and 128 underwent fetal endoscopic tracheal occlusion (FETO) with a balloon. In the expectant‐management group, lung size measured by ultrasound or by magnetic resonance imaging was the only independent predictor of survival (observed‐to‐expected lung‐to‐head ratio (o/e‐LHR) odds ratio (OR), 1.06 (95% CI, 1.02–1.11); P = 0.003). Until now, stratification for severe lung hypoplasia has been based on an o/e‐LHR cut‐off of 45%. In cases managed expectantly, the survival rate was 15% (4/27) in those with o/e‐LHR ≤ 45% and 61% (36/59) for o/e‐LHR > 45% (P = 0.001). However, the best o/e‐LHR cut‐off for the prediction of survival at discharge was 50%, with a sensitivity of 78% and specificity of 72%. In the expectantly managed group, survivors with severe pulmonary hypoplasia stayed longer in the neonatal intensive care unit than did those with mildly hypoplastic lungs. In fetuses with an o/e‐LHR ≤ 45% treated with FETO, survival rate was higher than in those with similar lung size managed expectantly (49/120 (41%) vs 4/27 (15%); P = 0.014), despite higher prematurity rates (gestational age at birth: 34.4 ± 2.7 weeks vs 36.8 ± 3.0 weeks; P < 0.0001). In fetuses treated with FETO, gestational age at birth was the only predictor of survival (OR, 1.25 (95% CI, 1.04–1.50); P = 0.02). Conclusions Antenatal measurement of lung size can predict survival in iRCDH. In fetuses with severe lung hypoplasia, FETO was associated with a significant increase in survival without an associated increase in neonatal morbidity. © 2020 International Society of Ultrasound in Obstetrics and Gynecology
Objectives To examine the performance of the routine 11–13‐week scan in detecting fetal defects in twin pregnancies and to examine if, in pregnancies with a fetal defect, compared to those with normal fetuses, there is increased incidence of nuchal translucency thickness (NT) ≥ 95th and ≥ 99th percentiles or intertwin discordance in crown–rump length (CRL) ≥ 10% and ≥ 15%. Methods This was a retrospective analysis of prospectively collected data in twin pregnancies undergoing routine ultrasound examination for fetal anatomy, according to standardized protocols, at 11–13 weeks' gestation between 2002 and 2019. Pregnancies with known chromosomal abnormality were excluded. The final diagnosis of fetal defect was based on the results of postnatal examination in cases of live birth and on the findings of the last ultrasound examination in cases of pregnancy termination, miscarriage or stillbirth. The performance of the 11–13‐week scan in the detection of fetal defects was determined. Results The study population of 6366 twin pregnancies with two live fetuses at 11–13 weeks' gestation included 4979 (78.2%) dichorionic (DC) and 1387 (21.8%) monochorionic (MC) twin pregnancies. The main findings were: first, the overall incidence of fetal defects was higher in MC than in DC twins (2.8% vs 1.3%); second, the proportion of defects diagnosed in the first trimester was higher in MC than in DC twins (52.6% vs 27.1%); third, the pattern of defects in relation to detectability at the 11–13‐week scan (always detectable, sometimes detectable and never detectable) was similar to that reported previously in singleton pregnancies; fourth, always‐detectable defects included acrania, alobar holoprosencephaly, encephalocele, pentalogy of Cantrell, exomphalos, body‐stalk anomaly, twin reversed arterial perfusion sequence and conjoined twins; fifth, the incidence of fetal NT ≥ 95th percentile was higher in those with than in those without a defect (16.5% vs 4.5% in DC twins and 19.2% vs 5.9% in MC twins) and this was also true for NT ≥ 99th percentile (8.3% vs 1.0% in DC twins and 15.4% vs 2.0% in MC twins); and sixth, the incidence of CRL discordance ≥ 10% was higher in those with than in those without a defect (20.2% vs 7.9% in DC twins and 33.8% vs 9.3% in MC twins) and this was also true for CRL discordance ≥ 15% (10.1% vs 1.9% in DC twins and 28.2% vs 2.8% in MC twins). Conclusions First, fetal defects are more common in MC than in DC twin pregnancies. Second, first‐trimester detection of fetal defects in DC twin pregnancies is similar to that in singleton pregnancies. Third, first‐trimester detectability of defects in MC twins is higher than in DC twins. Fourth, in twin pregnancies with a fetal defect, there is higher intertwin discordance in CRL and incidence of increased NT, but the predictive performance of screening by these markers is poor. Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd.
CONTRIBUTIONWhat are the novel findings of this work? This study of 6225 twin pregnancies with two live fetuses at 11-13 weeks' gestation and no major abnormalities, first, compares overall survival, fetal loss at < 24 weeks' gestation, perinatal death at ≥ 24 weeks, delivery at < 37 and < 32 weeks, and birth weight < 5 th percentile between dichorionic, monochorionic diamniotic and monochorionic monoamniotic twins, and, second, examines the potential impact of endoscopic laser surgery for severe twin-twin transfusion syndrome and/or selective fetal growth restriction on the outcome of monochorionic diamniotic twins. What are the clinical implications of this work?In twin pregnancy, determination of chorionicity and amnionicity at the routine 11-13-week scan is essential because this defines the subsequent pregnancy outcome and the need for surveillance and intervention. ABSTRACTObjectives To report and compare pregnancy outcome in dichorionic (DC), monochorionic diamniotic (MCDA) and monochorionic monoamniotic (MCMA) twin pregnancies with two live fetuses at 11-13 weeks' gestation and to examine the impact of endoscopic laser surgery for severe twin-twin transfusion syndrome (TTTS) and/or selective fetal growth restriction (sFGR) on the outcome of MCDA twins.Methods This was a retrospective analysis of prospectively collected data on twin pregnancies undergoing routine ultrasound examination at 11-13 weeks' gestation between 2002 and 2019. In pregnancies with no major abnormalities, we compared overall survival, fetal loss at < 24 weeks' gestation, perinatal death at ≥ 24 weeks, delivery at < 37 and < 32 weeks, and birth weight < 5 th percentile between DC, MCDA and MCMA twins. ResultsThe study population of 6225 twin pregnancies with two live fetuses at 11-13 weeks' gestation with no major abnormalities included 4896 (78.7%) DC, 1274 (20.5%) MCDA and 55 (0.9%) MCMA twins. In DC twins, the rate of loss at < 24 weeks' gestation in all fetuses was 2.3%; this rate was higher in MCDA twins (7.7%; relative risk (RR), 3.258; 95% CI, and more so in MCMA twins (21.8%; RR, 9.289; 95% CI,). In DC twins, the rate of perinatal death at ≥ 24 weeks in all twins that were alive at 24 weeks was 1.0%; this rate was higher in MCDA twins (2.5%; RR, 2.456; 95% CI,) and more so in MCMA twins (9.3%; RR, 9.130; 95% CI,. In DC twins, the rate of preterm birth at < 37 weeks' gestation in pregnancies with at least one liveborn twin was 48.6%; this rate was higher in MCDA twins (88.5%; RR, 1.824; 95% CI, 1.760-1.890) and more so in MCMA twins (100%; RR, 2.060; 95% CI, 2.000-2.121). In DC twins, the rate of preterm birth at < 32 weeks was 7.4%; this rate was higher in MCDA twins (14.2%; RR, 1.920; 95% CI,) and more so in MCMA twins (26.8%; RR, 3.637; 95% CI,. In DC twin pregnancies with at least one liveborn twin, the rate of a small-for-gestational-age neonate among all liveborn twins was 31.2% and in MCDA twins this rate was higher (37.8%; RR, 1.209; 95% CI,; in MCMA twins, the rate was not significantly different (33.3%; RR, 1.067; 95...
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