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Objective: To determine the perinatal outcome of monochorionic diamniotic (MCDA) twin pregnancies complicated by selective intrauterine growth restriction (sIUGR), which were classified according to the umbilical artery (UA) Doppler flow pattern of the IUGR twin., Methods: This was a retrospective cohort study of all MCDA twin pregnancies diagnosed with sIUGR and managed at a single tertiary referral center between 2012 and 2018. Cases were classified according to the UA Doppler flow pattern of the IUGR twin as Type I (positive end-diastolic flow), Type II (persistently absent/reversed end-diastolic flow) or Type III (intermittently absent/reversed end-diastolic flow). Patients with Type-II or -III sIUGR were hospitalized at 26-28 weeks of gestation for increased fetal surveillance. Perinatal and immediate and long-term neurodevelopmental outcomes were stratified according to the UA Doppler flow pattern at the final examination. Intact survival rate was defined as the number of infants surviving without neurological impairment, divided by the total number of fetuses. Composite adverse outcome, defined as any mortality, presence of severe brain lesions on postnatal transcranial ultrasound or severe neurological morbidity, was also assessed., Results: Of 88 MCDA twin pregnancies with sIUGR included in the study, 28 underwent selective termination by cord occlusion using radiofrequency ablation, resulting in a perinatal survival rate of 89.3% and a median gestational age (GA) at delivery of 33.8 (interquartile range (IQR), 28.8-38.2) weeks for the large cotwin. Expectant management was employed in 60 cases. In 26 (43.3%) cases in the expectant-management group, the classification according to the UA Doppler flow pattern changed during gestation, resulting in 26 (43.3%) cases of Type-I, 22 (36.7%) cases of Type-II and 12 (20.0%) cases of Type-III sIUGR at the final examination. The perinatal survival rate of both twins with sIUGR Types I, II and III at the final examination was 100%, 81.8% and 75.0%, respectively (P = 0.04). Two cases of double fetal death and one case of single fetal death occurred 1-4 weeks after the Doppler pattern had changed from Type I or Type II to Type III. The median GA at delivery was 34.8 (IQR, 33.1-35.7) weeks in Type I, 30.3 (IQR, 28.6-32.1) weeks in Type II and 32.0 (IQR, 31.3-32.6) weeks in Type III (P < 0.01). The total intact survival rate was 100% for Type I, 77.3% for Type II and 75.0% for Type III (P < 0.001). Multivariate analysis demonstrated that early GA at diagnosis (odds ratio (OR), 0.83 (95% CI, 0.69-0.99); P = 0.04) and the presence of Type II or III vs Type I at the last examination (OR, 13.16 (95% CI, 1.53-113.32); P = 0.02) were associated with preterm birth < 32 weeks' gestation. Early GA at diagnosis was also associated with the composite adverse outcome (OR, 0.60 (95% CI, 0.36-0.99); P = 0.04)., Conclusions: The classification system of MCDA pregnancy complicated by sIUGR, according to the UA Doppler flow pattern of the IUGR twin at final examination, is associated with perinatal outcome. Importantly, the UA Doppler flow pattern can change during gestation, which has an impact on the risk of fetal death. Nevertheless, under strict fetal surveillance, the perinatal outcome of these pregnancies is favorable. © 2020 International Society of Ultrasound in Obstetrics and Gynecology., (© 2020 International Society of Ultrasound in Obstetrics and Gynecology.)

Objective: To explore the outcome of monochorionic twin pregnancies affected by selective intrauterine growth restriction (sIUGR) according to the umbilical artery Doppler pattern of the smaller twin., Methods: An electronic search of MEDLINE, EMBASE, CINAHL and ClinicalTrials.gov databases (2000-2016) was performed. sIUGR was defined as the presence of one twin with an estimated fetal weight and/or abdominal circumference < 10 th or < 5 th percentile and classified according to the umbilical artery Doppler flow pattern of the smaller twin (Type I: persistently positive; Type II: persistently absent/reversed; Type III: intermittently absent/reversed). Primary outcomes were perinatal mortality, intrauterine death, neonatal death and double fetal loss. Secondary outcomes were neonatal morbidity, including abnormal postnatal brain imaging, intraventricular hemorrhage, periventricular leukomalacia, admission to neonatal intensive care unit and respiratory distress syndrome, deterioration of fetal status, gestational age at delivery and degree of birth-weight discordance. A composite adverse outcome, defined as the presence of any mortality or abnormal brain findings, was also assessed. Quality assessment of the included studies was performed using the Newcastle-Ottawa Scale. A random-effects meta-analysis was used to compute the summary odds ratios (ORs), mean differences (MD) and proportions for the different outcomes., Results: Thirteen studies (610 pregnancies) were included. The risk of perinatal mortality was higher in twins affected by Type II compared with Type I sIUGR (OR, 4.1 (95% CI, 1.6-10.3)), whereas there was no difference among the other variants of growth restriction. Risk of abnormal postnatal brain imaging was significantly higher in twins affected by either Type II (OR, 4.9 (95% CI, 1.9-12.9)) or Type III (OR, 8.2 (95% CI, 2.0-33.1)) sIUGR compared with Type I sIUGR. The risk for neonatal intensive care unit admission was higher in Type II compared with Type I sIUGR (OR, 18.3 (95% CI, 1.0-339.7)). Twin pregnancies affected by Type I sIUGR were delivered at a significantly later gestational age compared with Type II (MD, 2.8 (95% CI, 1.83-3.86) weeks) and Type III (MD, 2.1 (95% CI, 0.97-3.19) weeks). The degree of birth-weight discordance was higher in Type II compared with Type I (MD, 21.6% (95% CI, 9.9-33.2%)) and Type III (MD, 9.3% (95% CI, 3.8-14.9%)) sIUGR., Conclusion: Monochorionic twin pregnancies affected by Type II sIUGR are at a higher risk of perinatal mortality and morbidity compared with Type I. The likelihood of an abnormal outcome is usually not significantly different between sIUGR Types II and III, although the latter has an unpredictable clinical course. Copyright © 2016 ISUOG. Published by John Wiley & Sons Ltd., (Copyright © 2016 ISUOG. Published by John Wiley & Sons Ltd.)

Objective: To determine how prenatal ultrasound measurements of dividing membrane thickness correlate with postnatal histological measurements and chorionicity in twin gestations., Methods: This was a prospective, longitudinal cohort study of twin gestations. Dividing membrane thickness was measured by transabdominal ultrasound, with the insonation beam both parallel and perpendicular to the membrane, in the second or third trimester, depending on when care was established. Ultrasound examinations were performed every 4 weeks following initial assessment until delivery. Measurements of membrane thickness from the first ultrasound examination were compared with histological measurements after delivery., Results: A total of 45 twin pregnancies (32 dichorionic, 13 monochorionic) were included. Mean gestational age at initial ultrasound examination was 24.1 ± 7.3 weeks. Parallel ultrasound measurements of membrane thickness were 1.6 ± 0.8 mm for monochorionic and 2.5 ± 0.9 mm for dichorionic gestations (P = 0.001). Perpendicular ultrasound measurements were 1.6 ± 0.3 mm for monochorionic and 2.2 ± 0.8 mm for dichorionic gestations (P = 0.009). Inter- and intraobserver reliability of ultrasound measurements were 0.847 and 0.950, respectively. Parallel and perpendicular ultrasound measurements correlated better with each other (R = 0.807, P < 0.001) than with histological measurements of membrane thickness (R parallel  = 0.538, P < 0.001; R perpendicular  = 0.529, P < 0.001). Receiver-operating characteristics curve analyses to predict histological membrane thickness > 50 th percentile resulted in an area under the curve (AUC) of 0.828 for parallel (P < 0.001) and 0.874 for perpendicular (P < 0.001) measurements with a cut-off value of 1.9 mm for both approaches. The AUCs for parallel and perpendicular measurements to predict dichorionicity were 0.892 (P < 0.001) and 0.823 (P < 0.001) with cut-off values of 1.9 and 1.8 mm, respectively., Conclusion: Prenatal ultrasound measurement of twin dividing membrane thickness is positively correlated with postnatal histological measurement. Dichorionicity can be determined by a magnified dividing membrane thickness ≥ 1.9 mm. Measurements with the ultrasound beam parallel to the dividing membrane may be more accurate than perpendicular measurements. Copyright © 2016 ISUOG. Published by John Wiley & Sons Ltd., (Copyright © 2016 ISUOG. Published by John Wiley & Sons Ltd.)

Objectives: To describe changes in umbilical artery (UA) Doppler flow in monochorionic diamniotic (MCDA) twins affected by selective intrauterine growth restriction (sIUGR), to correlate Doppler findings with pregnancy course and perinatal outcome, and to report postnatal follow-up., Methods: This was a retrospective study of 140 MCDA twins with sIUGR. UA end-diastolic flow, defined as Doppler waveform pattern Type I (persistently positive), Type II (persistently absent or persistently reversed) or Type III (intermittently absent or intermittently reversed), was recorded at first examination and monitored weekly until double or single intrauterine fetal death (IUFD), bipolar cord coagulation or delivery. All neonates had an early neonatal brain scan, magnetic resonance imaging, when indicated, and neurological assessment during infancy. Rates (per 100 person-weeks) and hazard ratios (HR) of IUFD in the IUGR twin in each pregnancy were calculated considering UA Doppler pattern as a time-dependent variable., Results: At first examination, there were 65 cases with UA Doppler waveform pattern Type I, 62 with Type II and 13 with Type III. Of the 65 Type-I cases, 48 (74%) remained stable, while 17 (26%) changed to either Type II absent (14%), Type II reversed (9%) or Type III (3%). Of 62 Type-II cases (47 with absent and 15 with reversed flow), 33 (53%) remained stable (18 with absent and all 15 with reversed flow). The 29 Type-II absent cases which changed became Type II reversed (24/47, 51%) or Type III (5/47, 11%). All 13 Type-III cases remained stable. Compared with Type I, the risk of IUFD (adjusted for estimated fetal weight discordance and amniotic fluid deepest vertical pocket) was highest when the pregnancy was or became Type II reversed (HR, 9.5; 95% CI, 2.7-32.7) or Type II absent (HR, 4.3; 95% CI, 1.3-14.3). Mild neurological impairment was more prevalent in the IUGR twin than in the large cotwin (7% vs 1%, P = 0.02)., Conclusions: Risk stratification based on UA Doppler is useful for planning ultrasound surveillance. However, patterns can change over time, with important consequences for management and outcome. Copyright © 2016 ISUOG. Published by John Wiley & Sons Ltd., (Copyright © 2016 ISUOG. Published by John Wiley & Sons Ltd.)

Twin anemia-polycythemia sequence (TAPS) is recognized increasingly antenatally by the demonstration of an anemic twin and a polycythemic cotwin using the middle cerebral artery peak systolic velocity (MCA-PSV). While the MCA-PSV has been shown to correlate well with anemia in singleton fetuses, the evidence to support its use to diagnose fetal polycythemia appears to be less clear-cut. We aimed to evaluate fetal, neonatal and adult literature used to support the use of MCA-PSV for the diagnosis of polycythemia. Comprehensive literature searches were performed for ultrasound evidence of polycythemia in the human fetus, neonate and adult using key search terms. Only manuscripts in the English language with an abstract were considered for the review, performed in June 2014. Fifteen manuscripts were found for the human fetus, including 38 cases of TAPS. Nine of these defined fetal polycythemia as MCA-PSV < 0.8 multiples of the median (MoM), five used < 1.0 MoM and one used 0.8-1.0 MoM. Only two studies, involving a total of 15 cases, proposed a diagnostic level, acknowledging false-positive and -negative cases, though neither reported sensitivities or specificities. Six neonatal studies (96 neonates) demonstrated evidence of decreased cerebral velocities in polycythemia and a consequent increase with hemodilution. In the adult, five studies (57 polycythemic adults) demonstrated increased flow or velocity with hemodilution. Neither neonatal nor adult studies conclusively defined levels for screening for polycythemia. Despite widespread adoption of a cut-off of < 0.8 MoM in the published literature for the polycythemic fetus in TAPS, this is based upon minimal evidence, with unknown sensitivity and specificity. We recommend caution in excluding TAPS based purely upon the absence of a reduced MCA-PSV., (Copyright © 2015 ISUOG. Published by John Wiley & Sons Ltd.)

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

Objective: Our purpose was to evaluate the antenatal incidence of single umbilical artery (SUA) in twin pregnancies according to chorionicity and to assess its relationship with outcome., Methods: Consecutive twin pregnancies undergoing ultrasound evaluation at our institutions were included. A targeted sonographic evaluation of the umbilical cord and vessels was performed in all cases. Chorionicity was determined according to standard ultrasound criteria., Results: A total of 174 twin pregnancies, 100 dichorionic (DC) and 74 monochorionic (MC), were included in the study. An SUA was identified in 17 (9.8%) pregnancies, and in 18 (5.2%) fetuses. No difference was found in the incidence of SUA in DC and MC twins. Among affected pregnancies, all but one DC twin pregnancy were discordant for SUA. Structural and/or chromosomal abnormalities were present in 27.8% of fetuses with SUA. The prevalence of small-for-gestational-age fetuses and of discordant birth weight (> 20% discordance) was higher in the SUA group than in the rest of the population, although these differences were not statistically significant. Twin pairs discordant for SUA had significantly higher weight discordance than those with normal umbilical cords. The sonographic cross-sectional area of the SUA did not appear to show the typical adaptive dilatation usually seen in singleton pregnancies with SUA., Conclusions: The incidence of SUA in twins is higher than in singletons, with no difference between MC and DC twins. Intrapair discordance for SUA in identical twins provides evidence against an exclusively genetic origin of this anomaly. The apparent failure of compensatory dilatation of the umbilical artery in twins with SUA may explain in part the higher risk for fetal growth restriction in these cases., (Copyright © 2012 ISUOG. Published by John Wiley & Sons, Ltd.)

Objectives: Our objectives were to compare the size and volume of the developing fetal thymus obtained by two-dimensional ultrasound (2D-US) and three-dimensional ultrasound (3D-US), develop normative data for thymus volume (TV), and investigate TV in fetuses with congenital heart disease (CHD) and normal twin gestations., Methods: We studied 321 fetuses (gestational age (GA): 17-39 weeks) including 238 normal singletons, 64 normal twins and 19 singleton fetuses with CHD. We used 2D-US to assess fetal thymus maximum transverse diameter (MTD), maximum transverse area (MTA), anteroposterior diameter (APD) and superoinferior diameter (SID). TV was obtained by 3D-US using virtual organ computer-aided analysis. Measurements were adjusted for estimated fetal weight where appropriate. Linear regression analysis, general linear models and Fisher's Z-transformation were used where appropriate. A nomogram of fetal TV based on singleton gestations was produced according to previously published methods., Results: Ultrasound assessment of the fetal thymus was possible in 95.3% (306/321) of cases. Both 3D-US and 2D-US measurements were significantly correlated with GA (TV r = 0.989; MTA r = 0.918; MTD r = 0.884; APD r = 0.849; and SID r = 0.816; all P < 0.05). After Fisher's Z-transformation, the correlation between the TV and GA was significantly stronger than that between any individual 2D-US measurement and GA (P < 0.05). Normal twin fetuses had TVs similar to those of singletons adjusted for estimated fetal weight and GA (P = 0.85). TV adjusted for estimated fetal weight and GA was significantly lower in fetuses with CHD than in normal singletons (P < 0.05)., Conclusion: 2D-US and 3D-US are useful tools for evaluation of the size and volume of the human fetal thymus through gestation. Fetal TV by 3D-US seems to reflect normal development of the thymus in utero better than do 2D-US measurements. Lower TV should be expected in association with CHDs., (Copyright © 2011 ISUOG. Published by John Wiley & Sons, Ltd.)

Objectives: The aim of this study was to evaluate the use of ultrasound assessment to predict risk of mortality in expectantly managed monochorionic twin fetuses with selective intrauterine growth restriction (sIUGR)., Methods: This was a retrospective study of 101 monochorionic twin pregnancies diagnosed with sIUGR before 26 weeks of gestation. All patients were under expectant management during the observation period. At the initial evaluation, the presence or absence of each of the following abnormalities was documented: oligohydramnios; stuck twin phenomenon; severe IUGR < 3(rd) centile of estimated fetal weight; abnormal Doppler in the umbilical artery; and polyhydramnios in the larger twin. The relationships between these ultrasound findings and mortality of sIUGR fetuses were evaluated using multiple logistic regression analysis., Results: Of 101 sIUGR twins, 22 (21.8%) fetuses suffered intrauterine demise and nine (8.9%) suffered neonatal death; 70 (69.3%) survived the neonatal period. Multiple logistic regression analysis revealed that the stuck twin phenomenon (odds ratio (OR): 14.5; 95% CI: 2.2-93.2; P = 0.006) and constantly absent diastolic flow in the umbilical artery (OR: 29.4; 95% CI: 3.3-264.0; P = 0.003) were significant risk factors for mortality., Conclusions: Not only abnormal Doppler flow in the umbilical artery but also severe oligohydramnios should be recognized as important indicators for mortality in monochorionic twins with sIUGR.

Objectives: To assess the prevalence of cord entanglement and perinatal outcome in a large series of monoamniotic twin pregnancies and to review the recent literature on similar published large series., Methods: Prospective observational study of all prenatally detected cases of monoamniotic twin pregnancies during an 8-year period in a tertiary fetal medicine unit. A Medline database review for publications since 2000 containing five or more cases of monoamniotic pregnancies that showed data on cord entanglement and perinatal outcome was also undertaken., Results: A total of 32 monoamniotic pregnancies were diagnosed during the study period, including three conjoined twins, seven pregnancies with twin reversed arterial perfusion (TRAP) syndrome, three surgical pregnancy interruptions for discordant fetal abnormality and one miscarriage before 16 weeks' gestation. The remaining 18 monoamniotic pregnancies were included in the study analysis. All monoamniotic pregnancies were complicated with antenatal cord entanglement diagnosed by B-mode and color Doppler ultrasound. There were 34 live births and a double intrauterine death diagnosed at 19 + 2 weeks' gestation. There were two late neonatal deaths, one from congenital complete heart block and the other after surgery for transposition of the great arteries. The overall perinatal loss rate was 11.1% after 16 weeks and 5.9% after 20 weeks' gestation. The cumulative rates of cord entanglement and perinatal mortality in the reviewed literature were 74% and 21%, respectively., Conclusions: Umbilical cord entanglement is present in all monoamniotic twins when it is systematically evaluated by ultrasound and color Doppler. Perinatal mortality in monoamniotic twins is mainly a consequence of conjoined twins, TRAP, discordant anomaly and spontaneous miscarriage before 20 weeks' gestation. Expectantly managed monoamniotic twins after 20 weeks have a very good prognosis despite the finding of cord entanglement. The practice of elective very preterm delivery or other interventions to prevent cord accidents in monoamniotic twins should be re-evaluated.

Objective: To compare a laparoscopy-assisted fetoscopic approach with an ultrasound-directed percutaneous approach for laser photocoagulation of placental anastomoses in cases of twin-twin transfusion syndrome (TTTS) with anterior placentation., Method: We performed a retrospective review of all cases that underwent laser ablation of placental anastomoses for TTTS with an anterior placenta at Texas Children's Fetal Center from November 2006 to November 2008. The two cohorts were identified by chart review based on the type of approach: laparoscopy-assisted vs. ultrasound-guided percutaneous uterine entry for fetoscopy. Operative and outcome data were extracted and the groups were compared using statistical methods, taking P < 0.05 as statistically significant., Results: In the 100 cases of TTTS studied, 48 had an anterior placenta. Fifteen (31%) of these underwent laparoscopy-assisted fetoscopy (LAF) while a percutaneous approach was used in the remaining 33 (69%) cases. The total procedure time was longer in the LAF group than in the percutaneous group (96.1 +/- 25 vs. 67.9 +/- 28 min; P < 0.01). There was no difference in the rate of preterm premature rupture of membranes up to 2 weeks and 4 weeks after surgery (7 vs. 15% and 13 vs. 21%, for the LAF group vs. the percutaneous group, respectively; P = 0.7). The gestational ages at delivery were similar: 30.3 +/- 4.5 weeks in the LAF group and 29.2 +/- 4.6 weeks in the percutaneous group (P = 0.32). The overall survival rate at birth was tending towards better survival in the laparoscopic group than in the percutaneous group (80 vs. 61%, respectively; P = 0.06). The neonatal survival rate was better with the LAF approach than with the percutaneous approach (80 vs. 59%, respectively; P = 0.045)., Conclusion: Laparoscopy-assisted entry of the uterus is associated with improved neonatal survival for laser photocoagulation in cases of TTTS with a complete anterior placentation.

Objective: To evaluate the prevalence of and perinatal outcome associated with increased nuchal translucency thickness (NT) > 99(th) percentile in dichorionic and monochorionic twins with normal karyotype., Methods: Two hundred and six consecutive twin pregnancies (166 dichorionic and 40 monochorionic) underwent an NT scan during a 4-year period. In those with NT > 99(th) percentile and normal karyotype, we performed first- and second-trimester anomaly scans, fetal echocardiography at 14-16 and 20-22 weeks, maternal serology and, in monochorionic pregnancies, serial ultrasound examinations every other week. Perinatal outcome was recorded., Results: There were 10 (4.95%) pregnancies in which one of the fetuses had NT > 99(th) percentile and normal karyotype, including five fetuses (1.5%) in dichorionic and five (6.25%) in monochorionic pregnancies. During the same period the prevalence of NT > 99(th) percentile and normal karyotype in singleton pregnancies studied in our center was 2.6%. Among the 10 twins affected, six (60%) were diagnosed with structural abnormalities (three dichorionic and three monochorionic), and two intrauterine fetal demises occurred (one dichorionic and one monochorionic). Thus, the perinatal outcome was normal in only two of the pregnancies, one dichorionic and one monochorionic., Conclusions: The prevalence of NT > 99(th) percentile in dichorionic twins with a normal karyotype is similar to that in singletons. In monochorionic twins we confirmed previous studies reporting an increased prevalence compared with singletons. NT > 99(th) percentile with a normal karyotype in twins was associated with a high rate of fetal abnormalities and/or fetal demise (overall 80%) irrespective of chorionicity.

Primary pulmonary hypoplasia is a rare, usually lethal, condition presenting only after birth without other congenital abnormalities. We describe the first case of fraternal twins diagnosed prenatally with primary pulmonary hypoplasia. Both had diffuse hypoplasia of the pulmonary arteries initially identified by fetal echocardiography and confirmed at autopsy following termination. These cases permit examination of the histopathology of this disease in the fetal stage of development.

Objectives: To assess cardiovascular findings in twin-reversed arterial perfusion (TRAP) sequence pre- and post-therapy and compare these findings to traditional obstetric markers, defined as acardius to pump twin weight ratio and presence of polyhydramnios., Methods: This was a retrospective review of 27 cases of TRAP sequence diagnosed between 2004 and 2008. Echocardiographic data included indexed cardiac output and functional and anatomic parameters. Ultrasound reports were reviewed for acardius to pump twin weight ratio and polyhydramnios. We assessed the relationship between cardiac output and the remaining cardiac/obstetric variables obtained pre- and post-treatment., Results: Twenty-three subjects had complete echocardiographic data sets at initial evaluation (mean gestational age, 20.4 +/- 2.5 weeks) and, of these, post-treatment echocardiographic evaluation was available in 10. Six of seven (86%) pump twins with elevated indexed cardiac output had significant cardiovascular compromise. Most fetuses with abnormal cardiac output or right ventricular dysfunction normalized post-therapy. There was no relationship between cardiac output and obstetric markers., Conclusions: Elevated indexed cardiac output is strongly associated with cardiovascular compromise. Traditional obstetric prognosticators do not correlate with cardiovascular derangements. In pump twins with cardiac compromise, postoperative cardiovascular status improves acutely. Given this analysis, we conclude that assessment of cardiovascular findings should be incorporated into the management and treatment of TRAP sequence., (Copyright (c) 2009 ISUOG. Published by John Wiley & Sons, Ltd.)

Objectives: To study the frequency and clinical significance of crown-rump length (CRL) discrepancy at 11-14 weeks of gestation in twin pregnancies from an unselected population., Methods: This was a retrospective analysis of all twin pregnancies that underwent a routine 11-14-week scan at a large teaching hospital. Fetal loss was defined as fetal demise of one or both twins after 14 weeks., Results: A total of 507 twin pregnancies were studied; 382 (75.3%) were dichorionic and 125 (24.7%) were monochorionic twins. The discrepancy in CRL was expressed as a percentage of the CRL of the larger twin. The 95(th) and 99(th) centile for CRL discrepancy in twins was 12.2% and 19.3%, respectively. The discrepancy in CRLs in monochorionic and dichorionic twins was not significantly different (Mann-Whitney U = 22,406, P = 0.302). In 39 twin pairs, there was subsequent intrauterine death of one or both twins. Fetal loss was more common in monochorionic twins (24/125) than in dichorionic twins (15/382) (chi-square = 30.9, P < 0.001). In monochorionic twins, the discrepancy in CRLs in the 24 cases with subsequent loss was significantly greater than in the 101 twin pairs with no subsequent loss (Mann-Whitney U = 896, P = 0.048). The discrepancy in CRLs in 15 dichorionic twins with subsequent loss was not different from that in the 367 twins with no loss (Mann-Whitney U = 2116.5, P = 0.129). The CRL discrepancy was significantly correlated with birth-weight discordance in twins (Spearman's rho = 0.128, P = 0.006). However, this was due to a significant correlation in dichorionic twins (Spearman's rho = 0.127, P = 0.016) but not in monochorionic twins (Spearman's rho = 0.145, P = 0.14)., Conclusions: Fetal loss is significantly associated with discrepancy in CRL at the 11-14-week scan in monochorionic twins and discordance in birth weights is significantly associated with discrepancy in CRL in dichorionic twins. However, intertwin CRL discrepancy is of limited value in screening for these adverse events.

We report a case of a dizygotic monochorionic twin pregnancy preceded by intracytoplasmic sperm injection treatment including assisted hatching. On ultrasound examination at 25 weeks' gestation the twins, which had been assumed to be monochorionic, were found to be of different sexes. Karyotyping and zygocity determination were performed on amniotic fluid and showed the twins to be dizygotic with normal female and male karyotypes. There were clinical and sonographic signs of twin-twin transfusion syndrome (TTTS), and Cesarean delivery was performed at 32 weeks' gestation. At birth the twins were phenotypically a normal male and a normal female. Histology of the placenta showed it to be monochorionic diamniotic. Blood chimerism was found postnatally as both infants had the karyotypes 46,XX[13]/46,XY[17]. Chimerism was not found in cells from a buccal swab at 6 months of age. This is one of only a few reported cases of dizygotic monochorionic twins. Nearly all of these cases have been conceived after assisted reproductive technology procedures. It is of clinical importance to be aware of this rare phenomenon in relation to TTTS, prenatal screening and parental counseling., ((c) 2008 ISUOG. Published by John Wiley & Sons, Ltd.)

Objectives: To explore the feasibility and added value of three-dimensional (3D) inversion rendering of the developing embryonic/fetal ventricular system, and to apply this display technique to enhance the sonographic examination of holoprosencephaly., Methods: 3D volumes of the embryonic/fetal brain were acquired from four normal fetuses at 8-11 postmenstrual weeks, and four fetuses diagnosed with holoprosencephaly between 9 and 14 postmenstrual weeks. The volumes were then analyzed offline to obtain inversion rendered 3D shapes of the ventricular systems., Results: All inversion rendering displays were successful and provided the opportunity to study normal as well as pathological aspects of the early fetal brain., Conclusions: Inversion rendering of early fetal brain ventricles is feasible and should be attempted if additional information about the early normal or pathological fetal brain is needed. It may have scientific value in embryologic or genetic studies., ((c) 2008 ISUOG. Published by John Wiley & Sons, Ltd.)

Objectives: To evaluate a classification of selective intrauterine growth restriction (sIUGR) in monochorionic (MC) twins based on the characteristics of umbilical artery (UA) Doppler flow in the smaller twin, in terms of association with clinical outcome and with the pattern of placental anastomoses., Methods: One hundred and thirty-four MC twins diagnosed with sIUGR at 18-26 weeks were classified as Type I (UA Doppler with positive diastolic flow, n = 39), Type II (persistent absent or reversed end-diastolic flow, n = 30) and Type III (intermittent absent or reversed end-diastolic flow, n = 65). Perinatal outcome, placental sharing and the pattern of anastomoses were compared with those in 76 uncomplicated MC twins., Results: Mean gestational age at delivery was 35.5 (range, 30-38) weeks in controls, 35.4 (range, 16-38) weeks in Type I, 30.7 (range, 27-40) weeks in Type II (P < 0.0001) and 31.6 (range, 23-39) weeks in Type III (P < 0.0001) pregnancies. Fetal weight discordance was significantly higher in Type II (38%) and Type III (36%) than in Type I (29%) (P < 0.0001) pregnancies. Deterioration of the growth-restricted fetus occurred in 90% of Type II cases, compared with 0% and 10.8% of Types I and III, respectively (P < 0.001). Unexpected intrauterine fetal death of the smaller twin occurred in 15.4% of Type III cases, compared with 2.6% and 0% of Types I and II respectively (P < 0.05). Parenchymal brain lesions in the larger twin were observed in 19.7% of Type III cases and less than 5% in the other groups (P < 0.05). Placental discordance (larger/smaller) was 1.3 in controls, compared with 1.8, 2.6 and 4.4 in Types I, II and III, respectively (P < 0.01). The proportion of cases with arterioarterial anastomoses > 2 mm in diameter was 55% in controls, 70% in Type I, 18% in Type II (P < 0.01) and 98% in Type III (P < 0.01)., Conclusion: sIUGR can be classified on the basis of umbilical artery Doppler into three types that correlate with different clinical behavior and different patterns of placental anastomoses. This classification may be of help in clinical decision-making and when comparing clinical studies.

Objective: To evaluate the outcome of screening for structural malformations in twins and the outcome of screening for twin-twin transfusion syndrome (TTTS) among monochorionic twins through a number of ultrasound scans from 12 weeks' gestation., Methods: Enrolled into this prospective multicenter observational study were women with twin pregnancies diagnosed before 14 + 6 gestational weeks. The monochorionic pregnancies were scanned every second week until 23 weeks in order to rule out early TTTS. All pregnancies had an anomaly scan in week 19 and fetal echocardiography in week 21 that was performed by specialists in fetal echocardiography. Zygosity was determined by DNA analysis in all twin pairs with the same sex., Results: Among the 495 pregnancies the prenatal detection rate for severe structural abnormalities including chromosomal aneuploidies was 83% by the combination of a first-trimester nuchal translucency scan and the anomaly scan in week 19. The incidence of severe structural abnormalities was 2.6% and two-thirds of these anomalies were cardiac. There was no significant difference between the incidence in monozygotic and dizygotic twins, nor between twins conceived naturally or those conceived by assisted reproduction. The incidence of TTTS was 23% from 12 weeks until delivery, and all those monochorionic twin pregnancies that miscarried had signs of TTTS., Conclusion: Twin pregnancies have an increased risk of congenital malformations and one out of four monochorionic pregnancies develops TTTS. Ultrasound screening to assess chorionicity and follow-up of monochorionic pregnancies to detect signs of TTTS, as well as malformation screening, are therefore essential in the antenatal care of twin pregnancies., (Copyright (c) 2007 ISUOG.)

Objective: To describe prenatal ultrasound and autopsy findings in fetuses with OEIS (omphalocele, bladder exstrophy, imperforate anus, spina bifida) complex., Methods: This was a retrospective study of the nine cases with OEIS complex diagnosed at our center using detailed fetal ultrasound during the last 10 years. We summarized the fetal ultrasound findings that led to the diagnosis and compared them with the autopsy results., Results: All affected fetuses were diagnosed using detailed fetal ultrasound after 16 weeks' gestation. The main prenatal findings were omphalocele, skin-covered lumbosacral neural tube defect, non-visualized bladder and limb defects. Prenatal sonography failed to detect the abnormal genitalia, bladder exstrophy and anal atresia. All cases had abnormalities in a 'diaper distribution', which helped in making the prenatal diagnosis. Eight of the nine couples chose to terminate the pregnancies following multidisciplinary counseling. The pregnancy that was continued was a case with dizygotic twins discordant for OEIS, and the affected fetus died in utero., Conclusions: The combination of the following ultrasound findings: ventral wall defect, spinal defect and a non-visualized bladder with or without limb defects, are characteristic of OEIS complex. Diagnosis can be made with confidence as early as 16 weeks' gestation, although earlier diagnosis may be possible., (Copyright 2007 ISUOG. Published by John Wiley & Sons, Ltd.)

Objective: To help develop an evidence-based approach to the best management of twin pregnancies discordant for anencephaly., Methods: We retrospectively examined the management and outcome of 18 pregnancies discordant for anencephaly diagnosed at 11 + 0 to 13 + 6 weeks of gestation in our center. We combined these data with those from other publications. In total, there were 44 dichorionic pregnancies that were managed expectantly (n = 35) or by selective feticide (n = 9) and 19 monochorionic pregnancies that were managed expectantly. We also reviewed the literature to ascertain the outcome of monochorionic twin pregnancies undergoing cord occlusion., Results: In the 35 dichorionic pregnancies that were managed expectantly, 20 (57.1%) developed polyhydramnios at 25-31 weeks; 13 were managed expectantly, five had amniodrainage and two had selective feticide. In 34 of the 35 cases the non-anencephalic twin was liveborn at a median gestation of 36 (range, 28-39) weeks and in six (17.6%) of these it was born before 33 weeks. In the dichorionic pregnancies that had selective feticide, there was one miscarriage and eight (88.9%) live births at a median gestation of 37 (range, 30-40) weeks and in one (12.5%) of these it was born before 33 weeks. In the monochorionic pregnancies, four (21.1%) anencephalic fetuses died at 20-32 weeks and in three of these the normal co-twin also died. In the 16 (84.2%) cases resulting in the live birth of the normal twin, delivery occurred at a median gestation of 33 (range, 27-39) weeks and in six (37.5%) of these it was before 33 weeks. Ultrasound-guided bipolar cord coagulation in 92 pregnancies, mostly complicated by twin reversed arterial perfusion sequence or severe twin-to-twin transfusion syndrome, was associated with a survival rate of 77.2% and early preterm delivery rate of 31.0%., Conclusion: Dichorionic twins discordant for anencephaly are best managed with serial ultrasound examinations for early diagnosis of polyhydramnios, which can then be treated either by amniodrainage or selective feticide. In monochorionic twins it is uncertain whether the best management is expectant or by cord occlusion., (Copyright 2006 ISUOG. Published by John Wiley & Sons, Ltd.)

Objective: To assess the feasibility of the prenatal diagnosis using fetal neurosonography of brain injuries in the surviving fetus after the demise of a monochorionic cotwin., Methods: This was a retrospective observational study in the period 1990-2004 of monochorionic twin pregnancies with a single fetal demise. A detailed sonographic evaluation of the intracranial anatomy of the surviving twin had been performed whenever possible using a multiplanar approach and from 1999, fetal magnetic resonance imaging was offered as well. Postnatal follow-up was obtained in all cases., Results: In six of nine cases, abnormal neurosonographic findings were identified including intracranial hemorrhage, brain atrophy, porencephaly and periventricular echogenicities evolving into polymicrogyria. Prenatal diagnosis of brain lesions was confirmed postnatally and all affected infants who survived had severe neurological sequelae. Two fetuses had normal cerebral structures both on the prenatal neurosonogram and on postnatal imaging and were following normal developmental milestones, one at 1 and the other at 5 years of age. In one case the neurosonographic examination was suboptimal and the infant was found at birth to have a porencephalic cyst. Fetal magnetic resonance imaging was performed in two cases and confirmed the ultrasound diagnosis., Conclusions: Prenatal neurosonography is a valuable tool for the prediction of neurological outcome in fetuses surviving after the intrauterine death of a monochorionic cotwin. Although our experience is limited, we suggest that magnetic resonance imaging should also be offered., (Copyright 2006 ISUOG)

Objective: The aim of this study was to describe the outcome of a case series of fetuses with omphalocele., Methods: Ninety fetuses with omphalocele at the National Center for Fetal Medicine (NCFM) between January 1985 and January 2004 were followed from the time of prenatal diagnosis. Follow-up times ranged from 6 months to 17 years. Omphaloceles were subdivided into epigastric, central and hypogastric types based on their location on the abdominal wall., Results: There were 58 central and 32 epigastric omphaloceles. Abnormal karyotype was found in 40/58 (69%) of the central and in 4/32 (12.5%) of the epigastric omphaloceles. Trisomy 18 was the most frequent abnormality associated with omphalocele. Among the fetuses with normal karyotype, 89% of the central and 71% of the epigastric cases had other anomalies. 38 (66%) of the fetuses with central omphalocele were terminated and 12 (21%) died during pregnancy or after delivery. 11 (34%) of the fetuses with epigastric omphalocele were terminated and eight (25%) died during pregnancy or after delivery. Of the 90 cases followed from the time of diagnosis there were 21 (23%) survivals. Of eight survivals with central omphalocele, only two were considered healthy while six had other anomalies and/or substantially impaired development. Of 13 survivals with epigastric omphalocele, six were considered healthy and seven had other anomalies and/or substantial impairment., Conclusions: Fetal central and epigastric omphaloceles may be different entities: central omphaloceles are more strongly associated with abnormal karyotype (69%) than are epigastric omphaloceles (12.5%). The outcome of fetuses with omphalocele is poor irrespective of the type of omphalocele, with only eight of the 90 (9%) being alive and healthy at present. The results emphasize the importance of identifying both those fetuses with a potentially good prognosis and favorable outcome and those which are likely to have a fatal outcome., (Copyright (c) 2005 ISUOG.)

Monoamniotic twin pregnancy discordant for anencephaly (MATDA) is a rare occurrence with only seven prior reported cases. Selective termination has been advocated in managing discordant monoamniotic twins. We report two cases managed expectantly with good outcomes and review other previously reported cases. The first case was a primigravid woman diagnosed with MATDA at 18 weeks. She was managed expectantly until 32 + 5 weeks when a Cesarean section was performed for preterm labor. The surviving female infant weighed 1610 g. The second case was a multigravid woman who was diagnosed with MATDA at 17 + 5 weeks and was managed as an outpatient. An emergency Cesarean section was performed at 31 weeks for non-reassuring monitoring and the surviving male infant weighed 1790 g. In both cases, the survivors were discharged home in good condition. A review of these two cases and those in the literature suggests that expectant management should be considered among management options for this rare condition., (Copyright 2005 ISUOG. Published by John Wiley & Sons, Ltd.)

Objective: To determine whether in screening for trisomy 21 by measurement of fetal nuchal translucency (NT) thickness in monochorionic twin pregnancies it is preferable to use the higher, smaller or average NT., Methods: We retrospectively examined 769 monochorionic twin pregnancies that had undergone NT screening. The selection criteria were that first, in each pregnancy both fetuses were alive at the 11 to 13 + 6-week scan and second, the fetal karyotype had been determined by prenatal invasive testing or the pregnancy outcome was known. In each pregnancy the risk for trisomy 21 was calculated by a combination of maternal age and fetal NT for crown-rump length (CRL). Three estimates of risk for each pregnancy were made using the higher, smaller and average NT and these were compared for detection and false positive rates., Results: The median maternal age was 33 (range, 16-45) years, the CRL was 62 (range, 45-84) mm and gestational age was 12 (range, 11 to 13 + 6) weeks. Either the fetal karyotype was normal, or phenotypically normal babies were born, in 761 cases. The karyotype was abnormal in eight cases, including six with trisomy 21. The estimated risk using the higher, smaller and average NT was 1 in 300 or more in 6 (100%), 4 (66.7%) and 6 (100%) of the trisomy 21 pregnancies and in 148 (19.4%), 57 (7.5%) and 106 (13.9%) of the normal pregnancies. For a detection rate of 100%, the false positive rates using the higher, smaller and average NT would be 5.1%, 45.9% and 4.2%, respectively., Conclusion: In monochorionic twins, effective screening for trisomy 21 is best provided by using the average NT measured in the two fetuses., (Copyright 2005 ISUOG)

The prenatal diagnosis of a complete cleft sternum was established in a fetus of a twin pregnancy at 22 weeks' gestation. We present the prenatal imaging and correlation with postnatal magnetic resonance imaging and high-resolution ultrasonography. Thinned and depressed midline anterior chest wall transmitting the cardiac pulsation was the clue to the diagnosis, and the defective sternum could be identified on close sonographic observation. Successful surgical correction was undertaken at 2 months of age. There were no major associated abnormalities such as ectopia cordis or midline abdominal wall defects.