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OBJECTIVE The aim of this study was to determine whether reversal of hindbrain herniation (HBH) on MRI following prenatal repair of neural tube defects (NTDs) is associated with reduced rates of ventriculoperitoneal (VP) shunt placement or endoscopic third ventriculostomy (ETV) within the 1st year of life. METHODS This is a secondary analysis of prospectively collected data from all patients who had prenatal open repair of a fetal NTD at a single tertiary care center between 2012 and 2020. Patients were offered surgery according to inclusion criteria from the Management of Myelomeningocele Study (MOMS). Patients were excluded if they were lost to follow-up, did not undergo postnatal MRI, or underwent postnatal MRI without a report assessing hindbrain status. Patients with HBH reversal were compared with those without HBH reversal. The primary outcome assessed was surgical CSF diversion (i.e., VP shunt or ETV) within the first 12 months of life. Secondary outcomes included CSF leakage, repair dehiscence, CSF diversion prior to discharge from the neonatal intensive care unit (NICU), and composite neonatal morbidity. Demographic, prenatal sonographic, and operative characteristics as well as outcomes were assessed using standard univariate statistical methods. Multivariate logistic regression models were fit to assess for independent contributions to the primary and secondary outcomes. RESULTS Following exclusions, 78 patients were available for analysis. Of these patients, 38 (48.7%) had HBH reversal and 40 (51.3%) had persistent HBH on postnatal MRI. Baseline demographic and preoperative ultrasound characteristics were similar between groups. The primary outcome of CSF diversion within the 1st year of life was similar between the two groups (42.1% vs 57.5%, p = 0.17). All secondary outcomes were also similar between groups. Patients who had occurrence of the primary outcome had greater presurgical lateral ventricle width than those who did not (16.1 vs 12.1 mm, p = 0.02) when HBH was reversed, but not when HBH was persistent (12.5 vs 10.7 mm, p = 0.49). In multivariate analysis, presurgical lateral ventricle width was associated with increased rates of CSF diversion before 12 months of life (adjusted OR 1.18, 95% CI 1.03–1.35) and CSF diversion prior to NICU discharge (adjusted OR 1.18, 95% CI 1.02–1.37). CONCLUSIONS HBH reversal was not associated with decreased rates of CSF diversion in this cohort. Predictive accuracy of the anticipated benefits of prenatal NTD repair may not be augmented by the observation of HBH reversal on MRI.

\ Introduction: Chorioamnion separation (CAS), preterm prelabor rupture of membranes (PPROM), and preterm delivery (PTD) remain major complications of fetoscopic laser photocoagulation (FLP) for twin-to-twin transfusion syndrome (TTTS). We sought to examine whether use of quincke-tip needles for initial entry in FLP reduces the risk of these complications. Methods: This is a secondary analysis of prospectively collected data from patients that had FLP for TTTS at a single tertiary care center (2011 – 2021). We excluded patients for whom direct trocar entry was used. Patients for whom a quincke-tip needle, versus a diamond-tip needle was used for Seldinger entry were compared. Demographics, ultrasound findings and operative characteristics were compared between groups. Post-operative outcomes and complications (including CAS, PPROM and PTD) were also compared. Multivariate logistic regression models were fit to assess independent risk factors for complications. Results: 386 patients met inclusion criteria; quincke-tip needles were used in 81 (21.0%) cases while diamond-tip needles were used in 305 (79.0%). Rates of CAS (11.1% vs 9.5%, p = 0.67) and PPROM (44.4% vs 41.0%, p = 0.57) were similar between groups. Patients in the quincke-tip group delivered 1.5 weeks earlier than those in the diamond-tip group (30.5 vs 32.0 weeks, p = 0.01). However, these patients were more likely to be delivered for maternal (35.9% vs. 19.0%) and fetal (23.1% vs. 15.3%) indications (p < 0.01). In multivariate analysis, needle type was not identified as an independent risk factor for PPROM. However, quincke-tip needle use was associated with PTD less than 32 weeks (aOR 1.83, 95% CI 1.01 – 2.95, p = 0.046). Discussion/Conclusion: Membrane complications following FLP did not depend on needle type. Earlier delivery in quincke-tip group was likely attributable to higher rates of maternal and fetal delivery indications. The needle chosen for entry is probably best determined by operator preference.

Objective Preterm delivery following fetoscopic laser surgery (FLS) of twin–twin transfusion syndrome (TTTS) is associated with severe perinatal morbidity and mortality. The role of steroid hormones in amniotic fluid (AF) after FLS remains unknown. Study Design A prospective cohort study of consecutive case series of FLS for TTTS was performed from April 2012 to February 2017. Cases were divided into early (≤27 weeks) spontaneous preterm delivery (ED) and late delivery (LD; ≥34 weeks) following FLS and compared. AF supernatants were assessed for protein, estradiol, progesterone and cortisol levels (using the ELISA kit), and normalized to total protein levels to adjust for dilution. Results A total of 294 consecutive cases of FLS for TTTS in monochorionic–diamniotic twins were performed during the study period. AF was available in 44 ED patients and 50 LD patients. On logistic regression, ED was associated with higher normalized progesterone levels (odds ratio [OR]: 1.25; 95% confidence interval [CI]: 1.12–1.41), lower normalized cortisol (OR: 0.78; 95% CI: 0.64–0.96), and higher estradiol levels (OR: 1.3; 95% CI: 1.03–1.63). Conclusion Elevated AF normalized progesterone and estradiol, and lower normalized cortisol levels were associated with ED. This novel finding requires further exploration to establish the molecular mechanism operational in pregnancies complicated by TTTS to potentially prevent early preterm birth after fetal surgery.

Microbiome exposure at birth has been associated with long-term pediatric outcomes. However, it is difficult to determine if differences in outcomes are truly due to microbiome exposure at birth or other exposures after birth and in early infancy. Using a twin cohort, we sought to determine the association between length of exposure to the maternal vaginal-fecal microbiome and long-term pediatric health outcomes by comparing outcomes between presenting and nonpresenting twins born to women who labored.We performed a mail-based survey study of women in a single maternal-fetal medicine practice who delivered twin pregnancies ≥24 weeks. The survey study was sent to women when twins were between 2 and 10 years old to assess the long-term health outcomes, including any medical diagnoses or problems with grown and development. For this study, we included all women who labored, and we compared health outcomes for the presenting versus nonpresenting twin with the primary outcome being the development of asthma/reactive airway disease and allergies. The length of exposure to the maternal vaginal-fecal microbiome was measured using the time from rupture of membranes (ROM) to delivery of each twin. Chi-square and Student'sTwo hundred fifty-seven sets of twins were eligible for analyses. The presenting twin had a longer time of ROM than the nonpresenting twin (617 ± 2408 min versus 2 ± 5 minutes,In twins born to women who labored and either delivered vaginally or via cesarean section, delivery order was not associated with any significant increase in defined adverse pediatric outcomes, including the development of asthma or allergies. Using twins as a model for microbiome exposure may help to elucidate the role of the maternal vaginal-fecal microbiome on long-term pediatric health outcomes.

OBJECTIVE: Donor demise after laser surgery for twin-twin transfusion syndrome (TTTS) is well-characterized, but recipient demise is not, nor is neonatal death. This study aims to characterize factors associated with recipient death, donor death, and dual twin death after laser, both before and after birth. METHODS: This is a prospective cohort study of monochorionic twin pairs who underwent laser ablation for TTTS. Risk factors for fetal and neonatal death of both twins were identified using univariable analysis and recursive partitioning, a novel statistical method to quantify contributions of each factor to outcomes. RESULTS: In 413 twin pairs, death of both twins occurred in 9.2% (38/413), donor death in 12.1% (50/413), and recipient death in 2.4% (10/413). Recursive partitioning showed that gestational age at delivery predicts dual twin death (below 23.7 weeks, likely [p

A consensus definition of selective fetal growth restriction in monochorionic diamniotic twins was recently proposed following a Delphi procedure involving an international panel of experts. The new definition augments the traditional definition with additional sonographic criteria.We sought to determine whether the augmentations of the "Delphi definition" identified additional morbidity and mortality compared with a traditional definition. Furthermore, we sought to determine the benefit of each definition in identifying pathologic growth restriction relative to uncomplicated monochorionic diamniotic twins.This was a retrospective analysis of unselected monochorionic diamniotic twins that underwent fortnightly ultrasound surveillance at a single center between 2011 and 2020. Patients with concomitant twin-to-twin transfusion syndrome, twin anemia polycythemia sequence, or twin reversed arterial perfusion sequence at the time of diagnosis of selective fetal growth restriction were excluded. The diagnosis of selective fetal growth restriction using the Delphi definition required either an estimated fetal weight of3rd percentile or presence of 2 of 4 observations in the smaller twin: (1) estimated fetal weight of10th percentile, (2) estimated fetal weight discordance of25% compared with the larger twin, (3) abdominal circumference of10th percentile, (4) umbilical artery pulsatility index of95th percentile. Diagnosis using the traditional definition required an estimated fetal weight of10th percentile and an estimated fetal weight discordance of25%. To determine the efficacy of the augmentations in the Delphi definition, 3 groups were compared: group I, uncomplicated monochorionic diamniotic twins; group II, twins with selective fetal growth restriction using the traditional definition (and therefore the Delphi definition); and group III, twins with selective fetal growth restriction solely using the Delphi definition. Demographic characteristics, subsequent development of twin-to-twin transfusion syndrome or twin anemia polycythemia sequence, pregnancy outcomes, and neonatal outcomes were compared.There were 325 patients with monochorionic diamniotic twins that met inclusion criteria. Of these, 213 (66%; group I) were uncomplicated, 37 (11%; group II) met the traditional definition for selective fetal growth restriction, and 112 (35%) met the Delphi definition for selective fetal growth restriction with 75 (67%) meeting solely the Delphi definition (group III). Demographic characteristics were similar between groups. Patients in group II delivered earlier than uncomplicated twins (32.1 vs 35.7 weeks of gestation; P.01) and patients in group III (32.1 vs 35.6 weeks of gestation; P.01). Furthermore, they were more likely to have critical umbilical artery Doppler abnormalities (38% vs 4%; P.01) and be delivered for deteriorating fetal status (30% vs 5%; P.01) than those in group III. Overall, survival was lower in group II than groups I and III (89% vs 96% and 100%, respectively; P=.04). Moreover, composite neonatal morbidity and mortality were greater in group II (30%) than either group I (6%; P.01) or group III (9%; P.01). The rates of composite neonatal morbidity and mortality were similar between groups I and III (P=.28).The addition of abdominal circumference and umbilical artery pulsatility index thresholds and isolated estimated fetal weight of3%, as proposed by the Delphi definition, increased the diagnosis of selective fetal growth restriction; however, there was no added benefit in the identification of growth discordant pregnancies at risk of adverse outcomes. Prospective analysis of monochorionic diamniotic twins is required to contextualize these findings.

Maternal and pediatric delivery outcomes may vary in patients who underwent open fetal myelomeningocele repair and elected to deliver at the fetal center where their fetal intervention was performed versus at the referring physician&rsquo, s hospital. A prospective cohort study of 88 patients were evaluated following in utero open fetal myelomeningocele repair at a single fetal center between the years 2011&ndash, 2019. Exclusion criteria included patients that delivered within two weeks of the procedure (n = 6), or if a patient was lost to follow-up (n = 1). Of 82 patients meeting inclusion criteria, 36 (44%) patients were delivered at the fetal center that performed fetal intervention, and 46 (56%) were delivered locally. Comparative statistics found that with the exception of parity, baseline characteristics and pre-operative variables did not differ between the groups. No differences in oligohydramnios incidence, preterm rupture of membranes, gestational age at delivery or delivery indications were found. Patients who delivered with a referring physician were more likely to be multiparous (p = 0.015). With the exception of a longer neonatal intensive care unit (NICU) stay in the fetal center group (median 30.0 vs. 11.0 days, p = 0.004), there were no differences in neonatal outcomes, including wound dehiscence, cerebrospinal fluid leakage, patch management, ventricular diversion, or prematurity complications. Therefore, we conclude that it is safe to allow patients to travel home for obstetric and neonatal management after open fetal myelomeningocele repair.

Twin anemia polycythemia sequence (TAPS) is a rare complication of monochorionic diamniotic (MCDA) twins. Middle cerebral artery peak systolic velocity (MCA-PSV) measurements are used to screen for TAPS while fetal or neonatal hemoglobin levels are required for definitive diagnosis. We sought to perform a systematic review of the efficacy of MCA-PSV in diagnosing TAPS. Search criteria were developed using relevant terms to query the Pubmed, Embase, and SCOPUS electronic databases. Publications reporting diagnostic characteristics of MCA-PSV measurements (i.e., sensitivity, specificity or receiver operator curves) were included. Each article was assessed for bias using the Quality Assessment of Diagnostic Accuracy Studies II (QUADAS II) tool. Results were assessed for uniformity to determine whether meta-analysis was feasible. Data were presented in tabular form. Among publications, five met the inclusion criteria. QUADAS II analysis revealed that four of the publications were highly likely to have bias in multiple areas. Meta-analysis was precluded by non-uniformity between definitions of TAPS by MCA-PSV and neonatal or fetal hemoglobin levels. High-quality prospective studies with consistent definitions and ultrasound surveillance protocols are still required to determine the efficacy of MCA-PSV in diagnosing TAPS. Other ultrasound findings (e.g., placenta echogenicity discordance) may augment Doppler studies.

The aim of this study was to investigate the management and outcome in the post-laser twin anemia polycythemia sequence (TAPS). Data of the international TAPS Registry, collected between 2014 and 2019, were used for this study. The primary outcomes were perinatal mortality and severe neonatal morbidity. Secondary outcomes included a risk factor analysis for perinatal mortality and severe neonatal morbidity. A total of 164 post-laser TAPS pregnancies were included, of which 92% (151/164) were diagnosed antenatally and 8% (13/164) postnatally. The median number of days between laser for TTTS and detection of TAPS was 14 (IQR: 7&ndash, 28, range: 1&ndash, 119). Antenatal management included expectant management in 43% (62/151), intrauterine transfusion with or without partial exchange transfusion in 29% (44/151), repeated laser surgery in 15% (24/151), selective feticide in 7% (11/151), delivery in 6% (9/151), and termination of pregnancy in 1% (1/151). The median gestational age (GA) at birth was 31.7 weeks (IQR: 28.6&ndash, 33.7, range: 19.0&ndash, 41.3). The perinatal mortality rate was 25% (83/327) for the total group, 37% (61/164) for donors, and 14% (22/163) for recipients (p &lt, 0.001). Severe neonatal morbidity was detected in 40% (105/263) of the cohort and was similar for donors (43%, 51/118) and recipients (37%, 54/145), p = 0.568. Independent risk factors for spontaneous perinatal mortality were antenatal TAPS Stage 4 (OR = 3.4, 95%CI 1.4-26.0, p = 0.015), TAPS donor status (OR = 4.2, 95%CI 2.1&ndash, 8.3, p &lt, 0.001), and GA at birth (OR = 0.8, 95%CI 0.7&ndash, 0.9, p = 0.001). Severe neonatal morbidity was significantly associated with GA at birth (OR = 1.5, 95%CI 1.3&ndash, 1.7, p &lt, 0.001). In conclusion, post-laser TAPS most often occurs within one month after laser for TTTS, but may develop up to 17 weeks after initial surgery. Management is mostly expectant, but varies greatly, highlighting the lack of consensus on the optimal treatment and heterogeneity of the condition. Perinatal outcome is poor, particularly due to the high rate of perinatal mortality in donor twins.

It is unknown how variations in surgical entry time in primary cesarean delivery (CD) may affect operative outcomes and maternal morbidity.Determine whether performing a primary CD in labor emergently ("stat") is associated with adverse maternal outcomes.Retrospective cohort study of patients who underwent primary CD at The Mount Sinai Hospital during the years of 2011-2016. Women with a singleton pregnancy and without a prior uterine scar attempting a trial of labor were included. An emergent CD was defined as a skin-to-uterine incision (I-U) time of ≤3 minutes. Subjects were dichotomized into those with an I-U time of ≤3 minutes or ≥5 minutes.1722 patients underwent primary CD and met eligibility criteria. 72 patients with an I-U time of 4 minutes were removed from the analysis. 196 patients (11.9%) had an I-U time ≤3 minutes and 1454 patients (88.1%) had an I-U time ≥5 minutes. There were no differences in any outcomes between groups. The likelihood of transfusion, hysterectomy, or admission to the intensive care unit (ICU) was 1.5% in the emergent group and 1.0% in the control group (p = .334). Postpartum length of stay was also similar between the groups (3.3 versus 3.2 days, p = .259). When 384 patients with I-U times10 minutes were excluded, surgical outcomes remained similar between groups. Among the subgroup of patients who reached the second stage of labor, surgical outcomes were also similar between groups.Emergent primary CD is not associated with increased maternal morbidity.

Background/Purpose: Neonates with severe cases of congenital pulmonary airway malformation (CPAM) may require early emergent/urgent surgery for cardiopulmonary compromise. The CPAM volume ratio (CVR) is often utilized for prenatal prognostication; however, the threshold that predicts early surgery is unknown. We hypothesized that a late gestation CVR threshold exists at which neonates with prenatally diagnosised CPAM would require early surgery. Methods: A single-center, retrospective cohort study was conducted of all neonates with …

Fetoscopic laser photocoagulation (FLP) of placental anastomoses is the preferred treatment for twin-to-twin transfusion syndrome (TTTS). Iatrogenic septostomy (IOS) during FLP is associated with increased risk of neonatal morbidity and mortality. We sought to identify risk factors for IOS and quantify the resultant outcomes. This is a secondary analysis of prospectively collected cases of TTTS in monochorionic diamniotic twins following FLP at a single center. Pre-operative ultrasound characteristics and operative technique (i.e., cannula size, total energy used) were compared between cases with vs. without IOS. Pregnancy and neonatal outcomes were also compared. Of 475 patients that had FLP, 33 (7%) were complicated by IOS. There was no association between operative technique and IOS. IOS was more common with later diagnosis, but less likely when selective fetal growth restriction (sFGR) was present. Survival was similar between groups (76% vs. 76% dual survivors, p = 0.95), however, IOS was associated with earlier delivery (29.7 vs. 32.0 wks, p &lt, 0.01) and greater composite neonatal morbidity (25% vs. 8% in both twins, p = 0.02). Risks of IOS at greater gestational ages without sFGR may be related to a larger collapsed intervening membrane area and the resulting increased risk of puncture on entry.

Background: Fetoscopic laser photocoagulation (FLP) is the definitive treatment for twin-twin transfusion syndrome (TTTS). Due to variability in geographic proximity to high-volume fetal centers, many patients travel great distances to receive experienced care. We sought to determine whether distance traveled (DT) is associated with gestational age (GA) at delivery and neonatal survival. Methods: A prospective cohort study of patients within the continental United States referred to our center between September 23, 2011 and July 25, 2018 undergoing planned FLP for TTTS (n = 393; GA 20.6 ± 2.5 weeks; stage I: n = 50; stage II: n = 118; stage III: n = 208; stage IV: n = 17) was performed. The great-circle distance to our center was calculated using patients’ home zip codes. DT was stratified into groups containing equal patient numbers and pregnancy outcomes assessed. Results: A total of 393 patients met the inclusion criteria. The threshold distance from our center was n = 181), 250–499 miles (n= 119), and ≥500 miles (n = 93). There was no significant difference between any of the preoperative variables among the three groups, with the exception of race and rural status. Furthermore, there was no significant association between DT and GA at delivery (p = 0.34), time interval from procedure to delivery (p = 0.37), and the number of neonatal survivors (p= 0.21). Preterm premature rupture of membranes (PPROM) at p = 0.04) in the group traveling 250–499 miles. Conclusion: To our knowledge, this is the largest study to show that in TTTS, DT is not associated with GA at delivery, time interval from procedure to delivery, or neonatal survival. Although PPROM at

Background: Sacrococcygeal teratomas (SCT) are often highly vascularized and may result in high-output cardiac failure, polyhydramnios, fetal hydrops, and demise. Delivery is guided by the SCT to fetus volume ratio (SCTratio), SCT growth rate, and cardiac output indexed for weight (CCOi). Methods: We compared measurements and outcome in 12 consecutive fetuses referred with SCT. Adverse outcomes were: fetal surgery, delivery < 32 gestational weeks or neonatal demise. Only SCTratio and CCOi were used to manage the cases. SCT vascularization index (VI%) was derived from the 3D virtual organ computer-aided analysis (VOCAL) software. The SCTModel (modified from acardiac twins) calculated a hypothetical SCT draining vein size and derived a risk line, using diameters of the superior and inferior vena cava, the azygous and umbilical veins. VI% and a model of systemic and umbilical venous volumes (SCTModel) were tested as indicators for outcome in SCT. Results: Fetuses were monitored from 20.1 to 36.4 gestational weeks and 5/12 had adverse outcomes: 1 had successful open fetal surgery at 23.8 weeks and delivered at term, 4 delivered at < 32 weeks with 3/4 having neonatal demise between 25 and 29 weeks. VI% was significantly higher in cases with adverse outcomes (mean 10.3 [8.9–11.6] vs. 4.4 [3.4–5.3], p < 0.0001). The additional fraction of the fetal cardiac output required to perfuse the SCT-draining vein (XSCO%) (p = 0.46), SCTratio (p = 0.08), and CCOi (p = 0.64) were not significant. All cases with adverse outcome had VI% > 8%. The SCTModel risk line predicted nonadverse outcomes well but lacked data in 2/5 cases with adverse outcomes. Conclusions: VI% is a significant indicator of SCT cases with adverse outcomes and combined with SCTratio may guide timing of delivery better than current measures.

The aim of the study is to determine if thrombophilic single nucleotide polymorphisms (SNPs) affect outcomes in fresh in vitro fertilization (IVF) cycles in a large general infertility population. A prospective cohort analysis was performed at a university-affiliated private IVF center of female patients undergoing fresh non-donor IVF cycles. The effect of the following thrombophilic SNPs on IVF outcomes were explored: factor V (Leiden and H1299R), prothrombin (G20210A), factor XIII (V34L), β-fibrinogen (-455G → A), plasminogen activator inhibitor-1 (4G/5G), human platelet antigen-1 (a/b9L33P), and methylenetetrahydrofolate reductase (C677T and A1298C). The main outcome measures included positive pregnancy test, clinical pregnancy, embryo implantation, live birth, and pregnancy loss. Patients (1717) were enrolled in the study, and a total of 4169 embryos were transferred. There were no statistically significant differences in positive pregnancy test, clinical pregnancy, embryo implantation, live birth, or pregnancy loss in the analysis of 1717 patients attempting their first cycle of IVF. Receiver operator characteristics and logistic regression analyses showed that outcomes cannot be predicted by the cumulative number of thrombophilic mutations present in the patient. Individual and cumulative thrombophilic SNPs do not affect IVF outcomes. Therefore, initial screening for these SNPs is not indicated.

We sought to estimate the association between cervical length (CL) and fetal fibronectin (fFN) and each pathway leading to preterm birth in twin pregnancies.Cohort study of 560 patients with twin pregnancies who underwent routine serial CL and fFN screening from 22 to 32 weeks in one maternal fetal medicine practice during 2005-2013. We calculated the association between a short CL (≤20 mm) or positive fFN with overall preterm birth32 weeks, and then subdivided the analysis into preterm birth32 weeks from preterm labor, preterm premature rupture of membranes (PPROM) and indicated causes. We excluded cases of monochorionic-monoamniotic placentation, vasa previa, twin-twin transfusion and patients with cerclage.The overall rate of preterm birth32 weeks was 6.9% (3.9% from preterm labor, 1.6% from PPROM and 1.4% indicated). A short cervix was associated with preterm birth32 weeks arising from preterm labor (12.4% versus 2.0%, p 0.001), but not PPROM (1.9% versus 1.3%, p = 0.651). Positive fFN was associated with preterm birth32 weeks both from preterm labor (17.0% versus 2.4%, p 0.001) as well as from PPROM (5.7% versus 1.0%, p = 0.034). Neither was significantly associated with preterm birth32 weeks from indicated causes.The mechanism leading toward preterm influences the accuracy of screening tests chosen to assess risk in twin pregnancies. A shortened cervical length and positive fFN is associated with spontaneous preterm labor and birth32 weeks. However, PPROM does not appear to be preceded by a short cervix, but is preceded by a positive fFN. Neither test is associated with an indicated preterm birth.