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Introduction: Spina bifida guidelines recommend neurosurgical involvement in prenatal counseling to inform decision-making between prenatal and postnatal myelomeningocele (MMC) repair. This study examines whether families with MMC presenting to one fetal center had timely neurosurgical prenatal counseling (nPNC) encounters and assesses modifiable and non-modifiable treatment-determining factors. Methods: History and timing of nPNC were quantified among infants undergoing postnatal and prenatal MMC repair, pregnant patients referred, and MMC studies in a fetal MRI database (2015–2023). Fetal repair exclusions, presentation timing, social determinants, and reported rationale for not selecting offered fetal therapy were assessed. Results: Nearly all patients (34/35; 97%) engaged in nPNC, 82% prior to 24 weeks GA. Fourteen patients were excluded from fetal repair for lack of hindbrain herniation (43%), obstetric exclusions (21%), fetal exclusions (21%), suspected closed defect (7%), and delayed presentation (7%). These patients ultimately underwent postnatal repair (71%), and pregnancy termination (14%). The 20 fetal-repair-eligible patients selected fetal repair (50%), postnatal repair (45%), and pregnancy termination (5%). Reasons for declining fetal repair included risk (55%) and cost (22%). Conclusions: Among MMC families presenting to a regional fetal therapy center, nPNC was widely extended, in a mostly timely fashion. Very few were deterred from fetal repair by potentially modifiable barriers. Plain Language Summary: Spina bifida guidelines recommend neurosurgeons help talk to families diagnosed with fetal myelomeningocele, to help them make decisions about what kind of repair treatment to select. These options include repair both after or before birth (that is, in the womb, or prenatal repair). This study examines whether families with myelomeningocele presenting to one fetal center had timely conversations with neurosurgeons, and assesses factors that helped them decide. Nearly all patients (34/35; 97%) engaged in counseling with neurosurgeons prior to birth. Most (82%) happened prior to 24 weeks gestational age. Fourteen patients were excluded from prenatal repair for lack of hindbrain herniation (43%), pregnancy-related issues (21%), fetus-related issues (21%), the wrong kind of defect (7%), and delayed timing of prenatal conversation (7%). These patients instead had after-birth repair (71%), and pregnancy termination (14%). The 20 fetal-repair-eligible patients chose fetal repair (50%), after-birth repair (45%), and pregnancy termination (5%). Reasons for not having fetal repair included patient concerns about risk (55%) and cost (22%). In summary, at one regional fetal therapy center, conversations with neurosurgeons before birth were widely extended to families with myelomeningocele, in a mostly timely fashion. Very few were deterred from fetal repair by potentially modifiable barriers. [ABSTRACT FROM AUTHOR]

Introduction: Chorionic villus sampling (CVS) remains essential for first-trimester genetic diagnosis, yet clinical volume may be insufficient to train new clinicians in the technique. Available simulation models are expensive, require animal parts or specialized resins, and cannot be stored for repeated use. Methods: We present a model for trans-abdominal CVS (TA-CVS) which is constructed from readily available materials costing less than $10 and can be refrigerated and re-used to train maternal-fetal medicine fellows in CVS. Results: All three attending physicians performing TA-CVS at our institution described the model as an accurate visual and tactile simulation, prompting its integration into our fellowship curriculum. To date, two senior fellows have achieved competency on the simulator and begun to perform clinical CVS under supervision, one of whom is an author on this paper. Both fellows and attendings indicated that the simulator provided a valuable tool for repeated practice prior to clinical CVS. Simulators are now maintained on the unit and have been re-used for 3 months and dozens of simulated procedures each without any apparent qualitative degradation in performance. Discussion/Conclusion: We describe a low-cost easily constructed, durable, high-fidelity simulator for TA-CVS. [ABSTRACT FROM AUTHOR]

Introduction: Twin-to-twin transfusion syndrome affects monochorionic twin pregnancies and can result in fetal death. Endoscopic laser treatment remains a relatively infrequent procedure for this condition. This presents difficulties for maintaining proficiency and for training new personnel.Objective: The dual mentoring program at our institution allows for continuous mentoring of new providers. We hypothesize that this approach stabilizes program proficiency despite the addition of new practitioners.Methods: Query of the fetal treatment program database returned 146 cases of laser ablation between 2000 and 2019. Patient and pregnancy characteristics as well as operative time and outcomes were recorded. The learning curve-cumulative summation method and rolling averages were used to analyze outcomes.Results: Overall survival was 69%, and survival of at least 1 twin was 89%. Mean operative time was 53.6 ± 20.9 min. Overall twin survival stabilized after the first 40 cases. Rolling averages for operative time decreased from 71 to 49 min for the most recent cases. These results were not affected by the introduction of new surgeons.Conclusions: Creative mentoring can maintain stable overall program outcomes despite changes in team composition. This training approach may be applicable to other rare procedures in fetal surgery. [ABSTRACT FROM AUTHOR]

Effective tocolysis is essential after fetal myelomeningocele repair and is associated with the development of pulmonary edema. The increased uterine activity in the immediate postoperative period is commonly treated with magnesium sulfate. However, other tocolytic agents such as nitroglycerine, nifedipine, indomethacin, terbutaline, and atosiban (outside the US) have also been used to combat uterine contractility. The ideal tocolytic regimen which balances the risks and benefits of in-utero surgery has yet to be determined. In this case report, we describe a unique case of fetal myelomeningocele repair complicated by maternal pulmonary edema and increased uterine activity resistant to magnesium sulfate therapy. [ABSTRACT FROM AUTHOR]

Introduction: To describe the incidence and risk factors for iatrogenic premature preterm rupture of membranes (iPPROM) after fetoscopic laser surgery for the twin-to-twin-transfusion syndrome. Materials and Methods: This is a retrospective review of all patients who have undergone fetoscopic laser surgery at a single fetal treatment center since 2000. We defined iPPROM as spontaneous rupture of membranes before the onset of labor prior to 34 weeks of gestation. The iPPROM cohort was compared to the cohort without iPPROM for several preoperative, operative, and delivery characteristics. Results: Ninety-two consecutive patients were reviewed. The overall rate of iPPROM was 18.5% (n = 17). The rates of iPPROM within 1 and 4 weeks were 5.4 and 10.9%, respectively. The median interval from surgery to delivery was significantly shorter in the iPPROM group (21 vs. 62 days, p = 0.01). The mean gestational age at delivery (27.0 vs. 31.1 weeks, p = 0.02) was lower in the iPPROM group. No other characteristics studied differed significantly between the groups. Discussion: The incidence of iPPROM was substantially lower than in recent multicenter reports; however, no risk factors of iPPROM could be identified. Whether this is related to variations in surgical or anesthetic management will require further investigation. © 2014 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Endoscopic fetal surgery is most commonly used for the treatment of twin-to-twin transfusion syndrome (TTTS), but the surgical techniques can be applied to other forms of fetal surgery. We present our experience with endoscopic fetal surgery over the past 10 years. From 2000 to 2010, 70 endoscopic laser ablations of placental vessels for TTTS were performed. Median number of placental vessels ablated was four. The incidence of preterm rupture of membranes (PROM) was 6%. Overall survival was 70%, with at least one twin surviving in 82%. Tocolysis was used in 73% of patients for a median of 12 hours. The combination of an open surgical approach, Seldinger technique, and uterine plugging led to outcomes similar to other reports, with a significantly lower PROM rate. Although TTTS is the most common application of endoscopic fetal surgery, this approach is applicable for other indications. Insertion and removal of tracheal occlusion balloons for severe congenital diaphragmatic hernia are currently being performed at our institution. [ABSTRACT FROM AUTHOR]

Objective Survival (≥1 twin) after laser surgery for patients with twin-to-twin transfusion syndrome (TTTS) ranges from 65 to 93%. However, most studies are noncontrolled and retrospective, and have included a limited number of patients. The aim of this study was to perform a systematic review of outcomes after laser surgery in patients with TTTS. Methods We conducted database and manual searches of reference lists and pertinent journals published between 1995 and 2009 that report outcomes of laser surgery in patients with TTTS. Two authors performed the search independently of each other. There exist only two randomized controlled trials, each with fewer than 80 patients having undergone laser surgery. Uncontrolled and retrospective series were therefore considered as well. Studies had to report sufficient information on inclusive dates, stage distribution, overall neonatal survival, and neonatal survival of at least one twin. Of the 486 studies identified, we considered 19 studies. Results For each series, 95% confidence intervals (CI) were calculated. Survival was plotted against the date of publication, number of patients/series, gestational age at delivery, and proportion of advanced cases. Univariate analysis was performed to detect significant differences. Our meta-analysis, which included 1484 patients, shows 81.2% survival of at least one twin (CI: 79.1-83.2%). The average survival of at least one twin for the entire population remained within the CI of all but one series. Neither case load, nor stage distribution, nor chronological date of the study affected the survival. Conclusion A systematic review of endoscopic laser surgery performed in patients with TTTS failed to show a significant impact of high caseloads, disease severity distribution, or improvements in technique. Copyright © 2010 John Wiley & Sons, Ltd. [ABSTRACT FROM AUTHOR]

Background: Low plasma folate concentrations in pregnancy are associated with preterm birth. Here we show an association between preconceptional folate supplementation and the risk of spontaneous preterm birth. Methods and Findings: In a cohort of 34,480 low-risk singleton pregnancies enrolled in a study of aneuploidy risk, preconceptional folate supplementation was prospectively recorded in the first trimester of pregnancy. Duration of pregnancy was estimated based on first trimester ultrasound examination. Natural length of pregnancy was defined as gestational age at delivery in pregnancies with no medical or obstetrical complications that may have constituted an indication for delivery. Spontaneous preterm birth was defined as duration of pregnancy between 20 and 37 wk without those complications. The association between preconceptional folate supplementation and the risk of spontaneous preterm birth was evaluated using survival analysis. Comparing to no supplementation, preconceptional folate supplementation for 1 y or longer was associated with a 70% decrease in the risk of spontaneous preterm delivery between 20 and 28 wk (41 [0.27%] versus 4 [0.04%] spontaneous preterm births, respectively; HR 0.22, 95% confidence interval [CI] 0.08-0.61, p = 0.004) and a 50% decrease in the risk of spontaneous preterm delivery between 28 and 32 wk (58 [0.38%] versus 12 [0.18%] preterm birth, respectively; HR 0.45, 95% CI 0.24-0.83, p = 0.010). Adjustment for maternal characteristics age, race, body mass index, education, marital status, smoking, parity, and history of prior preterm birth did not have a material effect on the association between folate supplementation for 1 y or longer and spontaneous preterm birth between 20 and 28, and 28 to 32 wk (adjusted HR 0.31, 95% CI 0.11-0.90, p = 0.031 and 0.53, 0.28-0.99, p = 0.046, respectively). Preconceptional folate supplementation was not significantly associated with the risk of spontaneous preterm birth beyond 32 wk. The association between shorter duration (,1 y) of preconceptional folate supplementation and the risk of spontaneous preterm birth was not significant after adjustment for maternal characteristics. However, the risk of spontaneous preterm birth decreased with the duration of preconceptional folate supplementation (test for trend of survivor functions, p = 0.01) and was the lowest in women who used folate supplementation for 1 y or longer. There was also no significant association with other complications of pregnancy studied after adjustment for maternal characteristics. Conclusions: Preconceptional folate supplementation is associated with a 50%-70% reduction in the incidence of early spontaneous preterm birth. The risk of early spontaneous preterm birth is inversely proportional to the duration of preconceptional folate supplementation. Preconceptional folate supplementation was specifically related to early spontaneous preterm birth and not associated with other complications of pregnancy. [ABSTRACT FROM AUTHOR]

Objective The FASTER trial compared first and second trimester screening methods for aneuploidy. We examined relationships between maternal serum markers and common congenital anomalies in the pediatric outcome data set of 36 837 subjects. Methods We used nested case-control studies, with cases defined by the most common anomalies in our follow-up database, and up to four controls matched by enrollment site, maternal age and race, enrollment gestational age, and infant gender. Serum markers were dichotomized to ≥ 2 or < 0.5 multiples of the median (MoM). Odds ratios (ORs) and 95% confidence intervals (CI) were estimated. Results Statistically significant ( p < 0.05) associations were found between inhibin A ≥ 2 MoM with fetal multicystic dysplastic kidney (MCDK) (OR = 27.5, 95% CI: 2.8-267.7) and two-vessel cord (OR = 4.22, 95% CI:1.6-10.9); hCG of ≥ 2 MoM with MCDK (OR = 19.56, 95% CI: 1.9-196.2) and hydrocele (OR = 2.48, 95% CI: 1.3-4.6); and PAPP-A ≥ 2.0 MoM with hydrocele (OR = 1.88, 95% CI:1.1-3.3). Conclusion In this large prospective study, significant associations were found between several maternal serum markers and congenital anomalies. This suggests potential additional benefits to screening programs that are primarily designed to detect aneuploidy. Copyright © 2008 John Wiley & Sons, Ltd. [ABSTRACT FROM AUTHOR]

Objective Comparison of contingent, step-wise and integrated screening policies. Methods Mid-trimester Down syndrome risks were retrospectively calculated from FaSTER trial data. For contingent screening, initial risk was calculated from ultrasound measurement of nuchal translucency (NT), maternal serum pregnancy-associated plasma protein (PAPP)-A and free β-human chorionic gonadotrophin (hCG) at 11-13 weeks, and classified positive (>1 in 30), borderline (1 in 30-1500) or negative. Borderline risks were recalculated using α-fetoprotein, hCG, unconjugated estriol (uE3) and inhibin at 15-18 weeks, and reclassified as positive (>1 in 270) or negative. For step-wise screening, initial negative risks were also recalculated. For integrated screening, a single risk was calculated from NT, PAPP-A and the second trimester markers. Results There were 86 Down syndrome and 32 269 unaffected pregancies. The detection rate for contingent screening was 91% and false-positive rate was 4.5%; initial detection rate was 60%, initial false-positive rate was 1.2% and borderline risk was 23%. Step-wise screening had 92% detection rate and 5.1% false-positive rate; integrated screening had 88% and 4.9% respectively. Conclusion As predicted by modelling, the contingent screening detection rate for a fixed false-positive rate is comparable with step-wise and integrated screening, but substantially reduces the number needing to return for second trimester testing. Copyright © 2008 John Wiley & Sons, Ltd. [ABSTRACT FROM AUTHOR]

Objectives. To evaluate the relationship between low maternal body mass index (BMI) as calculated in the first trimester and the risk of preeclampsia and gestational hypertension. Methods. Patients enrolled in the First And Second Trimester Evaluation of Risk for aneuploidy (FASTER) trial were grouped into three weight categories: low BMI (BMI <19.8 kg/m2), normal BMI (BMI 19.8 - 26 kg/m2), and overweight BMI (26.1 - 29 kg/m2). The incidences of gestational hypertension and preeclampsia were ascertained for each group. Tests for differences in crude incidence proportions were performed using Chi-square tests. Multiple logistic regression was used to adjust for maternal age, race, parity, obesity, use of assisted reproductive technology (ART), in vitro fertilization (IVF), gestational diabetes, pre-gestational diabetes, cocaine use, and smoking. Results. The proportion of patients having gestational hypertension in the low BMI group was 2.0% compared to 3.2% for normal BMI and 6.0% for overweight BMI (p < 0.0001). Women with low BMI were also less likely to develop preeclampsia, 1.1% vs. 1.9% for normal BMI and 2.8% for overweight BMI (p < 0.0001). Conclusions. We found that women with low BMI in the first trimester were significantly less likely to develop gestational hypertension or preeclampsia than women with a normal BMI. [ABSTRACT FROM AUTHOR]

Objective: Severe twin-to-twin transfusion syndrome (TTTS) is usually classified according to a staging system (I-V) based on ultrasonographic findings of polyhydramnios in the recipient, oligohydramnios in the donor, the presence or absence of the donor's bladder, Doppler waveform changes and (impending) hydrops. Stage correlates with the severity of disease, and it is assumed that, without intervention, severe TTTS will evolve in succession from stage I to stage V (fetal demise). However, this progression has not been validated in longitudinal studies. Herein, we report on the natural progression of severe TTTS in a cohort of patients from a regional Fetal Treatment Program. Methods: Eighteen patients with severe TTTS, diagnosed between 15 and 25 weeks of gestation, were managed over a 28-month period. Data were collected until delivery, endoscopic surgical intervention or dual fetal demise. Patients were evaluated at least once a week. Stage, estimated fetal weight, percent recipient/donor body weight discordance and survival were recorded. Results: The present study represents a total follow-up of 108 patient-weeks. Of 90 week-to-week evaluations, 65 showed no change in stage; 11 showed downstaging (by more than 1 stage in 3, or 27%), and 13 showed upstaging (by more than 1 stage in 8, or 62%). Nine patients (all stage II or above) underwent endoscopic laser ablation. Overall survival was 67%, and survival of at least 1 twin occurred in 78% of pregnancies. Weight discordance between the donor and recipient did not predict outcome. Conclusion: The current staging system for severe TTTS may not be helpful in predicting the direction, degree or speed of progression of the condition. Indications for intervention should remain stage-related, and not based on projected progression. [ABSTRACT FROM AUTHOR]

As laser ablation of placental vascular communications gains acceptance as treatment option for severe twin-totwin transfusion syndrome (TTTS), pathologists are increasingly confronted with the interpretation of lasertreated placentas. We present our preliminary institutional experience with the gross and microscopic analysis of these specimens. Patients underwent selective ablation for severe TTTS (Quintero stages II to V) between 16 and 25 wk gestation and the placentas were examined between < 24 h and 19 wk postoperatively. The placental vasculature was injected with gelatin-dye mixtures. The type and number of vascular anastomoses were recorded, followed by routine histopathological analysis of the placenta. Foci of laser impact were identified in all placentas examined within 1 month after laser coagulation. Located along the recipient side of the dividing membrane, the laser-treated vessels appeared hemorrhagic and showed a characteristic abrupt interruption of dye filling after vascular injection. In placentas examined more than 1 month after intervention, the most frequent gross finding was the absence or relative paucity of intertwin anastomoses, associated with subchorionic fibrin deposition. Microscopically, lasertreated vessels showed varying degrees of necrosis, associated with focal hemorrhage, avascular villi, and fibrin deposition in the underlying parenchyma. In some cases of intrauterine fetal demise or placental disruption, no definite laser scars were identified. As expected, the number of residual anastomoses (all types) was significantly smaller in laser-treated placentas than in control monochorionic placentas (2.4 ± 2.2 [n = 10] vs. 6.2 ± 3.2 [n = 70], P < 0.01). Velamentous cord insertion was noted in 50% of cases; markedly uneven placental sharing in 60%. Detailed analysis of laser-treated placentas and clinicopathological correlation may lead to a better understanding of the pathophysiology of TTTS and continued refinement of therapeutic approaches for this often lethal condition. [ABSTRACT FROM AUTHOR]

Objective: To determine the validity of existing imaging-derived formulas for predicting the fetal lung volume (FLV).Methods: In a consecutive series of postmortem lungs without pulmonary anomalies, the observed FLV (FLV(obs)) after inflation was correlated with individual fetal variables used in imaging-derived formulas. In addition, FLV(obs) was correlated with the predicted FLV calculated according to these same formulas.Results: Postmortem FLV(obs) showed a strong correlation with estimated fetal body weight, biparietal diameter, and head circumference (r > 0.9). The correlation of FLV(obs) with gestational age, femur length, and liver weight was less strong (0.8 < r < 0.9). The correlation was strongest for midgestation fetuses (between 22 and 32 weeks' gestation). The predicted FLV calculated using formulas based on fetal body weight, biparietal diameter, and head circumference showed the strongest correlation with the actual FLV(obs) (r > 0.9).Conclusions: Postmortem FLV(obs) strongly correlates with fetal body weight, biparietal diameter, and head circumference, especially in midgestation fetuses. Regression formulas based on these fetal variables provide the most accurate prediction of FLV. [ABSTRACT FROM AUTHOR]

Presents information on hypertension, diabetes and other chronic diseases in pregnant women. Hypertension risks in pregnancy; Implications of diabetes for mother and fetus; Therapeutic options for pregnant women with systemic lupus erythematosus; Effect of asthma on pregnant women.

A case of twin-to-twin transfusion syndrome (TTTS) was associated with ischemic injury of the left leg of the recipient twin. Five reduction amniocenteses were performed between 17 6/7 and 23 3/7 weeks followed by fetoscopic laser ablation of chorioangiopagus vessels at 23 6/7 weeks of gestation. Anatomic assessment of the affected limb indicated timing of the injury at 19 4/7 weeks gestational age. The published literature suggests that ischemic fetal injuries in severe TTTS are associated with blood hyperviscosity in the affected twin. [ABSTRACT FROM AUTHOR]