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Objective: To evaluate twin survival stratified by Quintero stage in patients with twin-to-twin transfusion syndrome (TTTS) after Solomon laser treatment., Methods: This was a single-center study at Johns Hopkins Center for Fetal Therapy, investigating a cohort of consecutive twin pregnancies treated with the Solomon laser technique for TTTS. Preoperative Quintero stage, perioperative characteristics and obstetric factors were investigated in relation to neonatal survival of the recipient and donor twins at discharge. Determinants of twin survival were evaluated using univariate logistic regression and cumulative survival probability analyses., Results: Of 402 pregnancies with TTTS that underwent Solomon laser treatment, 80 (19.9%) were diagnosed with Quintero Stage-I TTTS, 126 (31.3%) with Stage II, 169 (42.0%) with Stage III and 27 (6.7%) with Stage IV. Post-laser twin anemia polycythemia sequence or recurrent TTTS occurred in 19 (4.7%) patients and 11 (2.7%) required repeat laser surgery. Preterm prelabor rupture of membranes occurred in 150 (37.3%) patients and median gestational age at delivery was 32 + 1 weeks. In 303 (75.4%) patients, both twins were alive at discharge; 67/80 (83.8%) were Stage I, 101/126 (80.2%) were Stage II, 113/169 (66.9%) were Stage III and 22/27 (81.5%) were Stage IV (P = 0.062). Donor twin survival was lower than that of recipients in cases with Stage-III TTTS (118/169 (69.8%) vs 145/169 (85.8%) (χ 2  = 26.076, P < 0.0001)). Higher intertwin size discordance and absent or reversed umbilical artery (UA) end-diastolic velocity (EDV) were associated with donor demise (Nagelkerke R 2 , 0.38; P < 0.001). Overall, spontaneous post-laser donor demise occurred in 53 (39.6%) patients, accounting for the majority of all losses. Cumulative donor survival decreased from 92% to 65% when intertwin size discordance was >30% and to 48% when UA-EDV was absent or reversed (P < 0.001)., Conclusions: The Solomon laser technique achieves TTTS resolution and double twin survival in a high proportion of cases. Recipient and donor survival is comparable unless there is significant intertwin size discordance and placental dysfunction. This degree of unequal placental sharing, typically found in Stage-III TTTS, is the primary factor preventing double survival due to a higher rate of donor demise. © 2024 International Society of Ultrasound in Obstetrics and Gynecology., (© 2024 International Society of Ultrasound in Obstetrics and Gynecology.)

Background: We compared early neurodevelopmental morbidity in young children with severe CDH who underwent FETO to those without fetal therapy., Methods: We conducted a prospective study of severe CDH patients undergoing FETO (n = 18) at a single North American center from 2015 to 2021 (NCT02710968). Outpatient survivors (n = 12) were evaluated by a multidisciplinary team and compared to expectantly managed CDH patients. Neurodevelopmental outcomes were assessed using the Capute Scales [Clinical Linguistic and Auditory Milestone Scales (CLAMS) and Cognitive Adaptive Test (CAT)], with a developmental quotient (DQ) < 85 indicative of at-risk for delay., Results: At one year, 58% (n = 7) of FETO patients underwent evaluation, with notable concern for language delay (CLAMS median DQ, 80.1 [interquartile range, 67.6-86.7]). FETO scores improved by 24-months, whereas high severity/non-FETO scores declined [CLAMS median DQ (Difference in DQ), 92.3 (+12.2) vs. 77.1 (-13.4), respectively; p = 0.049]. On the initial CAT, FETO patients had concern for visual motor and problem-solving delays, with a median DQ of 81.3 (62.1-89.4). At 24-months, FETO patients had improving scores [Median CAT DQ, 90.8 (+9.5)], whereas high severity/non-FETO [87.5 (-3.0), p = 0.28] had declining scores., Conclusion: These initial data suggest that FETO is associated with favorable neurodevelopmental outcomes at 24-months compared to severe CDH under expectant management., Level of Evidence: III., Competing Interests: Conflicts of interest There are no competing interests to disclose., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: Although fetoscopic endoluminal tracheal occlusion (FETO) was recently shown to improve survival in a multicenter, randomized trial of severe congenital diaphragmatic hernia (CDH), morbidity outcomes remain essentially unknown. The purpose of this study was to assess long-term outcomes in children with severe CDH who underwent FETO., Methods: We conducted a prospective study of severe CDH patients undergoing FETO at an experienced North American center from 2015-2021 (NCT02710968). This group was compared to a cohort of non-FETO CDH patients with severe disease as defined by liver herniation, large defect size, and/or ECMO use. Clinical data were collected through a multidisciplinary CDH clinic. Statistics were performed with t-tests and Chi-squared analyses (p≤0.05)., Results: There were 18 FETO and 17 non-FETO patients. ECMO utilization was 56% in the FETO cohort. Despite significantly lower median observed/expected lung-to-head ratio (O/E LHR) in the FETO group, [FETO: 23% (IQR:18-25) vs. non-FETO: 36% (IQR: 28-41), p<0.001], there were comparable survival rates at discharge (FETO: 78% vs. non-FETO: 59%, p = 0.23) and at 5-years (FETO: 67% vs. non-FETO: 59%, p = 0.53) between the two cohorts. At a median follow up of 5.8 years, metrics of pulmonary hypertension, pulmonary morbidity, and gastroesophageal reflux disease improved among patients after FETO. However, most FETO patients remained on bronchodilators/inhaled corticosteroids (58%) and were feeding tube dependent (67%)., Conclusions: These North American data show that prenatal tracheal occlusion, in conjunction with a long-term multidisciplinary CDH clinic, is associated with acceptable long-term survival and morbidity in children after FETO., Level of Evidence: Level III., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Since the completion of the Management of Myelomeningocoele Study, maternal-fetal surgery for spina bifida has become a valid option for expecting parents. More recently, multiple groups are exploring a minimally invasive approach and recent outcomes have addressed many of the initial concerns with this approach. Based on a previously published framework, we attempt to delineate the developmental stage of the surgical techniques. Furthermore, we discuss the barriers of performing randomized controlled trials comparing two surgical interventions and suggest that data collection through registries is an alternative method to gather high-grade evidence., (© 2021 The Authors. Prenatal Diagnosis published by John Wiley & Sons Ltd.)

Objective: To assess feasibility and maternal and infant outcome after fetoscopic tracheal balloon occlusion in patients with severe congenital diaphragmatic hernia., Methods: We conducted a prospective cohort study of fetuses with congenital diaphragmatic hernia and observed/expected lung/head ratio less than 30%. Eligible women had planned fetoscopic tracheal balloon occlusion at 26 0/7-29 6/7 weeks of gestation and balloon removal 4-6 weeks later. Standardized prenatal and postnatal care was at a single institution. Fetoscopic tracheal balloon occlusion details, lung growth, obstetric complications, birth outcome, and infant outcome details until discharge were evaluated., Results: Of 57 women screened, 14 (25%) were enrolled between 2015 and 2019. The congenital diaphragmatic hernia was left in 12 (86%); the pre-fetoscopic tracheal balloon occlusion observed/expected lung/head ratio was 23.2% (range 15.8-29.0%). At a median gestational age of 28 5/7 weeks (range 27 3/7-29 6/7), fetoscopic tracheal balloon occlusion was successful in all cases, and balloons remained in situ. Removal was elective in 10 (71%) patients, by ultrasound-guided needle puncture in eight (57%), and occurred at a median of 33 4/7 weeks of gestation (range 32 1/7-34 4/7; median occlusion 34 days, range 17-44). The post-fetoscopic tracheal balloon occlusion observed/expected lung/head ratio increased to a median of 62.8% (44.0-108) and fell to a median of 46.6% (range 30-92) after balloon removal (all Mann Whitney U, P<.003). For prevention of preterm birth, all patients received vaginal progesterone; 11 (79%) required additional tocolytics, three (21%) had vaginal pessary placement for cervical shortening, and five (36%) had amnioreduction for polyhydramnios. Median gestational age at birth was 39 2/7 weeks (range 33 6/7-39 4/7), with term birth in eight (57%) patients. Twelve (86%) neonates required high-frequency ventilation, and seven (50%) required extracorporeal membrane oxygenation for a median of 7 days (range 3-19). All neonates needed patch repair. Neonatal survival was 93% (n=13, 95% CI 49-100%), and survival to hospital discharge was 86% (n=12, 95% CI 44-100%)., Conclusion: Fetoscopic tracheal balloon occlusion for severe congenital diaphragmatic hernia was feasible in our single-center setting, with few obstetric complications and favorable infant outcome., Clinical Trial Registration: ClinicalTrials.gov, NCT02710968.

Introduction: Spina bifida is the most common non-lethal congenital birth defect of the central nervous system that causes chronic disability due to the combined effects of local nerve damage and the sequelae of non-communicating hydrocephalus. This abnormality can be identified early in gestation and the damage can be progressive over the course of pregnancy. Advances in fetal treatment have made minimally invasive prenatal surgery a realistic consideration for spina bifida in order to improve the outcome for children affected this condition., Evidence Acquisition: Prenatal surgery for spina bifida via open fetal surgery with hysterotomy decreases the rate of ventriculoperitoneal shunt placement and improves motor function compared to standard postnatal surgery. Maternal risks of open fetal surgery are primarily related to complications of the hysterotomy including thinning or rupture that begins in the index pregnancy but persists for every future pregnancy. Minimizing maternal risks is the largest impetus to explore and optimize a minimally invasive fetoscopic alternative. Techniques vary from using a complete percutaneous approach to open fetoscopy, which requires laparotomy but is minimally invasive to the uterus. This allows vaginal delivery at term and no scar complications are reported thus far. Fetal short-term neurosurgical outcomes compare favorably with improvement in hindbrain herniation >70% and decreased need for treatment for hydrocephalus between 40-45% after prenatal surgery performed either fetoscopically or through open fetal surgery., Evidence Synthesis: Maternal obstetric outcomes are superior for fetoscopic spina bifida repair compared to open fetal surgery and avoids the ongoing risk in future pregnancy. Neonatal and infant benefits appear equivalent. The open fetoscopic approach minimizes the risk of ruptured membranes and subsequent preterm delivery as opposed to a completely percutaneous procedure. International collaboration is ongoing to share experience and assess long term treatment effects., Conclusions: Continued refinement of a minimally invasive strategy for prenatal treatment of spina bifida is necessary to maximize benefits to the child and further minimize maternal risks and preterm birth.

Introduction: The insertion site of the fetoscope for laser occlusion (FLOC) treatment of twin-twin transfusion syndrome (TTTS) determines the likelihood of treatment success. We assessed a standardized preoperative ultrasound approach for its ability to identify critical landmarks for successful FLOC., Methods: Three surgeons independently performed preoperative ultrasound and deduced the likely orientation of the intertwin membrane (ITM) and vascular equator (VE) based on the sites of the cord insertion, the lie of the donor, and the size discordance between twins. At FLOC, these landmarks were visually verified and compared to preoperative assessments., Results: Fifty consecutive FLOC surgeries had 127 preoperative assessments. Basic ITM and VE orientation were accurately predicted in 115 (90.6%), 109 (85.8%), and 105 (82.7%) assessments. Predictions were anatomically correct in 96 (75.6%), 70 (55.1%), and 58 (45.7%) assessments with no differences in accuracy between operators of different training level. The ITM/VE relationship was most poorly predicted in stage-3 TTTS (χ2, p = 0.016)., Conclusion: In TTTS, preoperative ultrasound identification of placental cord insertion sites, lie of the donor twin, and size discordance enables preoperative prediction of key landmarks for successful FLOC., (© 2018 S. Karger AG, Basel.)

Fetoscopic endotracheal occlusion (FETO) is a prenatal treatment that may increase survival in severe congenital diaphragmatic hernia (CDH). In the USA, FETO is offered for isolated severe left-sided CDH in the context of an FDA-approved feasibility study. FETO in non-isolated cases of severe CDH is only performed with a compassionate use exemption from US regulatory bodies. Anomalies frequently associated with CDH include congenital cystic lesions of the lung and cardiac defects. We describe two cases of non-isolated severe left-sided CDH that underwent prenatal FETO, survived after birth and underwent postnatal surgical repair. The potential benefit of FETO in this setting is discussed. © 2017 John Wiley & Sons, Ltd., (© 2017 John Wiley & Sons, Ltd.)

Objective: The aim of this study is to evaluate the independent and combined value of gestational age, fetal weight, fetoplacental Doppler, and myocardial performance index for the prediction of individual risk of early (≤7 days) intrauterine fetal death (IUFD) after laser therapy in twin-to-twin transfusion syndrome (TTTS)., Material and Methods: A consecutive series of 215 cases of TTTS treated with laser therapy in three centers was prospectively studied. Ultrasound evaluation within 24 h of surgery included estimated fetal weight discordance, umbilical artery, pulsatility index (PI) and diastolic flow evaluation, middle cerebral artery PI and middle cerebral artery peak systolic velocity, ductus venosus PI and atrial flow assessment, and modified myocardial performance index. Logistic regression analysis was used to explore the association of preoperative parameters with IUFD., Results: Intrauterine fetal death occurred in 17 (7.9%) of the recipients and 33 (15.3%) donors (p = 0.016). The only independent predictors of IUFD in recipients was the middle cerebral artery peak systolic velocity >1.5 MoM (OR = 22, p = 0.015), but this event was present in only 3% of recipients. In donors, reverse end diastolic flow in the umbilical artery (OR = 14.748, p = 0.033), estimated fetal weight discordance (OR = 1.054, p = 0.036), and gestational age (OR = 0.757, p = 0.046) were independent predictors., Conclusion: In TTTS, preoperative fetal assessment can identify independent risk factors for early post-operative IUFD, particularly in donors., (© 2013 John Wiley & Sons, Ltd.)

Objective: The objective of the study was to compare the outcome in women with twin-twin transfusion syndrome (TTTS) who were managed by selective laser ablation of placental anastomoses (S-LASER) vs equatorial dichorionization of the anastomoses and chorionic plate (ED-LASER)., Study Design: We conducted an analysis of 2 consecutive cohorts with TTTS treated with S-LASER and ED-LASER. Preoperative, procedural, delivery, and outcome details were compared between treatment groups., Results: A total of 147 twin pregnancies had laser (S-LASER, n = 71; ED-LASER, n = 76). Obstetric history, Quintero stage, placental location, individual arterial and venous Doppler parameters, and gestational age at laser and delivery did not differ. Use of lens fetoscope (96.1% vs 50.7%), coagulated anastomosis count (11 vs 15, P < .001), complete equatorial plate visualization (77.5% vs 92.1%, P = .019), and amniotic fluid transparency (80.3% vs 94.7%, P = .011) was greater during ED-LASER. Procedure-related complications, gestational age at delivery, and perinatal details were comparable between groups. ED-LASER was associated with significantly lower recurrence of TTTS (3.9% vs 8.5%), twin anemia polycythemia sequence (2.6% vs 4.2%), and amniotic fluid abnormalities (0% vs 5.6%, P < .05 for all comparisons). Logistic regression identified complete visualization of the equator as the primary determinant for successful TTTS treatment and coagulation of the chorionic plate as an independent cofactor. Double survival at age 6 months was significantly higher after ED-LASER (68.4% vs 50.7%, P < .05), attributable to a lower postlaser and neonatal mortality rates., Conclusion: In patients with TTTS, photocoagulation of causative anastomoses and the chorionic plate along the vascular equator decreases recurrence without increasing adverse outcome and improves survival compared with the selective technique., (Copyright © 2013 Mosby, Inc. All rights reserved.)

Objective: The purpose of this study was to evaluate the benefit of cervical cerclage for cervical length ≤ 25 mm at the time of fetoscopic laser photocoagulation (FLP) for twin-twin transfusion syndrome., Study Design: A multicenter, retrospective cohort study was conducted with 163 patients with a short cervix before FLP for twin-twin transfusion syndrome. Seventy-nine of the patients (48%) had cerclage placement at the surgeon's discretion. The outcome measures that were compared were gestational age at delivery and perinatal mortality rates for patients with cerclage and those who were treated conservatively. Outcomes were evaluated with the use of comparative statistics., Results: There were no differences in the preoperative variables, except cerclage was performed more frequently for a cervical length of ≤ 15 mm (P < .001). There were no differences in the gestational age at delivery (28.8 ± 5.4 vs 29.1 ± 5.6 weeks with and without cerclage, respectively; P = .15); perinatal mortality rates were similar between the 2 groups., Conclusion: The benefit of cerclage for patients with short cervix before FLP remains questionable., (Copyright © 2012 Mosby, Inc. All rights reserved.)

Objective: To examine effects of fetoscopic laser occlusion of placental vascular anastomoses on umbilical venous volume flow in twin-to-twin transfusion syndrome., Study Design: Absolute umbilical venous volume flow, measured preoperatively and 48 hours after fetoscopic laser occlusion was related to Doppler studies, bladder filling in donors, and anastomoses., Results: Among 45 patients, recipients had decreased ductus venosus pulsatility index (ductus venosus-pulsatility index for veins, 1.16 vs 1.01; P < .001) and unchanged umbilical venous volume flow after fetoscopic laser occlusion (74.7 vs 74.5 mL; P = .407). Donors had decreased umbilical artery pulsatility (1.34 vs 1.11; P = .008), increased ductus venous-pulsatility index for veins (0.75 vs 0.91; P < .014), and significantly increased umbilical venous volume flow per kilogram by 52.3% (136.6 vs 208.0 mL/Kg/min; P < .001). Donor bladder filling occurred at higher umbilical venous volume flow per kilogram (142.7 vs 221.4 mL/Kg/min; P < .012). Increase in umbilical venous volume flow per kilogram correlated with the net difference in arteriovenous anastomoses (Pearson r = 0.403, P = .006)., Conclusion: Fetoscopic laser occlusion in twin-to-twin transfusion syndrome corrects intertwin differences in umbilical venous volume flow by predominant effects in the donor. Reappearance of donor bladder filling correlates with correction of volume flow., (Published by Mosby, Inc.)

Fetoscopic laser ablation of placental vascular anastomoses is an intrauterine therapy targeting the source of twin-to-twin transfusion syndrome (TTTS). Our patient had successful laser treatment, with resolution of stage II TTTS, suggesting closure of all significant anastomoses. After an uneventful pregnancy course, she elected a trial of labor. Following spontaneous onset of labor, acute intrapartum twin-to-twin transfusion was suspected, suggested by sinusoidal heart rate changes, confirmed postdelivery by twins' complete blood counts. The potential risks of labor with prior fetoscopic laser ablation are reviewed in the context of current knowledge about placental architecture in TTTS.