Efficacy of long-term indomethacin therapy in prolonging pregnancy after fetoscopic laser surgery for twin-to-twin transfusion syndrome: a collaborative cohort study.

OBJECTIVE: To evaluate the efficacy of long-term indomethacin therapy (LIT) in prolonging pregnancy and reducing spontaneous preterm birth (PTB) in patients undergoing fetoscopic laser surgery (FLS) for the management of twin-to-twin transfusion syndrome (TTTS). DESIGN: Retrospective cohort study of prospectively collected data. SETTING: Collaborative multicentre study. POPULATION: Five hundred and fifty-seven consecutive TTTS cases that underwent FLS. METHODS: Long-term indomethacin therapy was defined as indomethacin use for at least 48 hours. Log-binomial regression was used to estimate the relative risk of PTB in the LIT group compared with a non-LIT group. Cox regression was used to evaluate the association between LIT use and FLS-to-delivery survival. MAIN OUTCOME MEASURES: Gestational age (GA) at delivery. RESULTS: Among the 411 pregnancies included, a total of 180 patients (43.8%) received LIT after FLS and 231 patients (56.2%) did not. Median GA at fetal intervention did not differ between groups (20.4 weeks). Median GA at delivery was significantly higher in the LIT group (33.6 weeks) compared with the non-LIT group (31.1 weeks; P < 0.001). FLS-to-delivery interval was significantly longer in the LIT group (P < 0.001). The risks of PTB before 34, 32, 28 and 26 weeks of gestation were all significantly lower in the LIT group compared with the non-LIT group (relative risks 0.69, 0.51, 0.37 and 0.18, respectively). The number needed to treat with LIT to prevent one PTB before 32 weeks of gestation was four, and to prevent one PTB before 34 weeks was five. CONCLUSION: Long-term indomethacin after FLS for TTTS was found to be associated with prolongation of pregnancy and reduced risk for PTB. TWEETABLE ABSTRACT: Long-term indomethacin used after fetoscopic laser surgery for twin-to-twin transfusion syndrome is effective in prolonging pregnancy and reducing the risk for preterm birth; especially extreme preterm birth.

Outcomes of laparoscopy-assisted fetoscopic laser photocoagulation for twin-twin transfusion syndrome: An established alternative for inaccessible anterior placenta.

OBJECTIVE: To evaluate laparoscopy-assisted fetoscopic laser photocoagulation (FLPC) of placental anastomoses in the treatment of twin-to-twin-transfusion syndrome (TTTS). STUDY DESIGN: We performed a retrospective cohort study analyzing pregnancies complicated by TTTS who underwent FLPC in a single university-affiliated tertiary medical-center. Outcomes were compared between patients who received laparoscopy-assisted FLPC (study group) and patients who underwent the conventional FLPC technique (control group). Baseline characteristics, sonographic findings, procedure details, and neonatal outcomes were compared between groups. RESULTS: The cohort included 278 women with 31 in the study group and 247 in the control group. Sonographic parameters, including fetal biometry and TTTS stage, were comparable between study groups. Gestational age at delivery did not differ between the groups (29.22 ± 4.55 weeks in the study group vs. 30.62 ± 4.3 weeks in the control group, p = 0.09). There were no differences in neonatal survival rates at birth and at 30 days between both groups. A subanalysis comparing the laparoscopy-assisted group to only those patients with anterior placenta in the control group, showed a lower rate of incomplete Solomonization in the laparoscopy-assisted study group (3.4% vs. 33%, p = 0.01). CONCLUSION: Laparoscopy-assisted FLPC is a reasonable and safe option that may be offered in cases of FLPC where an anterior placenta restricts adequate surgical access.

Re-evaluation of the predictive value of Quintero staging of twin-twin transfusion syndrome for fetal death after fetoscopic laser photocoagulation.

OBJECTIVE: To elucidate the significance of sonographic indices, including Doppler waveforms, that constitute the Quintero classification for predicting death of the recipient or donor after fetoscopic laser photocoagulation (FLP) for twin-twin transfusion syndrome (TTTS). METHODS: Prospectively collected data of twins who underwent FLP for TTTS were reviewed. Among the abnormal indices of ultrasound performed just before FLP, factors that were significantly associated with fetal and neonatal deaths in the log-rank test, including fetal demise of co-twins and preterm birth before 28 weeks of gestation, were introduced into the Cox proportional-hazards model to calculate risk ratio (RR). RESULTS: We included 235 cases with a prevalence of recipient and donor deaths of 7% and 14%, respectively. In the proportional-hazards model, absent or reversed umbilical artery end-diastolic velocity (UA AREDV) of recipients (n = 7) was independently associated with recipient death (RR = 6.97). In recipients without UA AREDV, reversed ductus venosus (DV) a-wave of recipients (RR = 3.55) was independently associated with recipient death. In donors, UA AREDV with a visible bladder (stage III atypical donor) was independently associated with donor death (RR = 4.24). CONCLUSION: Some individual components of the Quintero stage are associated with death of either recipient or donor twins following FLP.

Percutaneous fetoscopic spina bifida repair: effect on ambulation and need for postnatal cerebrospinal fluid diversion and bladder catheterization.

OBJECTIVE: A trial comparing prenatal with postnatal open spina bifida (OSB) repair established that prenatal surgery was associated with better postnatal outcome. However, in the trial, fetal surgery was carried out through hysterotomy. Minimally invasive approaches are being developed to mitigate the risks of open maternal-fetal surgery. The objective of this study was to investigate the impact of a novel neurosurgical technique for percutaneous fetoscopic repair of fetal OSB, the skin-over-biocellulose for antenatal fetoscopic repair (SAFER) technique, on long-term postnatal outcome. METHODS: This study examined descriptive data for all patients undergoing fetoscopic OSB repair who had available 12- and 30-month follow-up data for assessment of need for cerebrospinal fluid (CSF) diversion and need for bladder catheterization and ambulation, respectively, from eight centers that perform prenatal OSB repair via percutaneous fetoscopy using a biocellulose patch between the neural placode and skin/myofascial flap, without suture of the dura mater (SAFER technique). Univariate and multivariate logistic regression analyses were used to examine the effect of different factors on need for CSF diversion at 12 months and ambulation and need for bladder catheterization at 30 months. Potential cofactors included gestational age at fetal surgery and delivery, preoperative ultrasound findings of anatomical level of the lesion, cerebral lateral ventricular diameter, lesion type and presence of bilateral talipes, as well as postnatal findings of CSF leakage at birth, motor level, presence of bilateral talipes and reversal of hindbrain herniation. RESULTS: A total of 170 consecutive patients with fetal OSB were treated prenatally using the SAFER technique. Among these, 103 babies had follow-up at 12 months of age and 59 had follow-up at 30 months of age. At 12 months of age, 53.4% (55/103) of babies did not require ventriculoperitoneal shunt or third ventriculostomy. At 30 months of age, 54.2% (32/59) of children were ambulating independently and 61.0% (36/59) did not require chronic intermittent catheterization of the bladder. Multivariate logistic regression analysis demonstrated that significant prediction of need for CSF diversion was provided by lateral ventricular size and type of lesion (myeloschisis). Significant predictors of ambulatory status were prenatal bilateral talipes and anatomical and functional motor levels of the lesion. There were no significant predictors of need for bladder catheterization. CONCLUSION: Children who underwent prenatal OSB repair via the percutaneous fetoscopic SAFER technique achieved long-term neurological outcomes similar to those reported in the literature after hysterotomy-assisted OSB repair. © 2021 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.

Predicting fetal and neonatal demise after fetoscopy for twin-twin transfusion syndrome using recursive partitioning.

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 < 0.001], above 28.3 weeks, unlikely [p = 0.004]). Abnormal umbilical artery Doppler and weight discordance predict donor demise (p < 0.001 and p = 0.033, respectively). Cervical length under 16 mm predicts neonatal death of both twins (p < 0.001). CONCLUSIONS: Parents can gain individualized information about the survival of each fetus based on variables available from preoperative and delivery variables. Short cervix and premature delivery cause significant mortality in TTTS.

Fetoscopic myelomeningocoele closure: Is the scientific evidence enough to challenge the gold standard for prenatal surgery?

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.

A novel "video-game" simulator for training fetoscopic laser coagulation of anastomoses in twin-to-twin transfusion syndrome.

 : We have developed a high-fidelity interactive "video-game" simulator in order to teach fetoscopic laser ablation of placental anastomoses for twin-twin transfusion syndrome This simulator may be used by teachers in order to provide metrics-based simulator education to multiple trainees, in both hands-on and distanced learning settings WHAT IS ALREADY KNOWN ABOUT THIS TOPIC?: The use of simulation improves training of the fetoscopic laser techniques utilized in the treatment of twin-twin transfusion syndrome A number of mannequins have been developed to aid this education WHAT DOES THIS STUDY ADD?: Two new simulators are described for twin-twin transfusion syndrome training-silicone and digital The digital simulator is a novel digital video game virtual format This new format has enhanced interactivity and has the potential to enable distance learning.

Magnesium sulfate titration reduces maternal complications following fetoscopic closure of spina bifida.

OBJECTIVE: To evaluate if magnesium sulfate (MgSO4 ) titration following fetoscopic spina bifida closure is associated with fewer maternal complications than the Management of Myelomeningocele Study (MOMS) tocolytic regimen. METHODS: This prospective cohort study included 73 consecutive patients undergoing fetoscopic closure of spina bifida between 2015 and 2020. A policy of using the MgSO4 regimen per the MOMS trial was changed to a flexible one in which MgSO4 was titrated according to the frequency of the uterine contractions following surgery. The frequency of maternal pulmonary edema, low maternal oxygen saturation requiring oxygen supplementation, atelectasis, hypocalcemia, and preterm delivery was compared before and after the policy was changed. RESULTS: A higher proportion of women in the group that used the MOMS MgSO4 regimen had pulmonary edema compared to those in the flexible one (26.1% [6/23] vs. 6% [3/50]; p = 0.024). Multivariate analysis showed that the MOMS tocolytic regimen was independently associated with a higher risk of pulmonary edema (adjusted odds ratio: 8.57; 95% confidence interval: 1.54-47.7; p = 0.014) than a flexible one. There was no difference in the rate of preterm delivery. CONCLUSION: Following fetoscopic closure of spina bifida, the MOMS MgSO4 regimen is associated with an increased risk of pulmonary edema than a more flexible regimen.

Fetal endoscopic tracheal occlusion reverses the natural history of right-sided congenital diaphragmatic hernia: European multicenter experience.

OBJECTIVE: To evaluate the neonatal outcome of fetuses with isolated right-sided congenital diaphragmatic hernia (iRCDH) based on prenatal severity indicators and antenatal management. METHODS: This was a retrospective review of prospectively collected data on consecutive cases diagnosed with iRCDH before 30 weeks' gestation in four fetal therapy centers, between January 2008 and December 2018. Data on prenatal severity assessment, antenatal management and perinatal outcome were retrieved. Univariate and multivariate logistic regression analysis were used to identify predictors of survival at discharge and early neonatal morbidity. RESULTS: Of 265 patients assessed during the study period, we excluded 40 (15%) who underwent termination of pregnancy, two cases of unexplained fetal death, two that were lost to follow-up, one for which antenatal assessment of lung hypoplasia was not available and six cases which were found to have major associated anomalies or syndromes after birth. Of the 214 fetuses with iRCDH included in the neonatal outcome analysis, 86 were managed expectantly during pregnancy and 128 underwent fetal endoscopic tracheal occlusion (FETO) with a balloon. In the expectant-management group, lung size measured by ultrasound or by magnetic resonance imaging was the only independent predictor of survival (observed-to-expected lung-to-head ratio (o/e-LHR) odds ratio (OR), 1.06 (95% CI, 1.02-1.11); P = 0.003). Until now, stratification for severe lung hypoplasia has been based on an o/e-LHR cut-off of 45%. In cases managed expectantly, the survival rate was 15% (4/27) in those with o/e-LHR ≤ 45% and 61% (36/59) for o/e-LHR > 45% (P = 0.001). However, the best o/e-LHR cut-off for the prediction of survival at discharge was 50%, with a sensitivity of 78% and specificity of 72%. In the expectantly managed group, survivors with severe pulmonary hypoplasia stayed longer in the neonatal intensive care unit than did those with mildly hypoplastic lungs. In fetuses with an o/e-LHR ≤ 45% treated with FETO, survival rate was higher than in those with similar lung size managed expectantly (49/120 (41%) vs 4/27 (15%); P = 0.014), despite higher prematurity rates (gestational age at birth: 34.4 ± 2.7 weeks vs 36.8 ± 3.0 weeks; P < 0.0001). In fetuses treated with FETO, gestational age at birth was the only predictor of survival (OR, 1.25 (95% CI, 1.04-1.50); P = 0.02). CONCLUSIONS: Antenatal measurement of lung size can predict survival in iRCDH. In fetuses with severe lung hypoplasia, FETO was associated with a significant increase in survival without an associated increase in neonatal morbidity. © 2020 International Society of Ultrasound in Obstetrics and Gynecology.

Placental histopathological features of fetoscopic laser photocoagulation for monoaminotic diamniotic twin pregnancies.

INTRODUCTION: This study aimed to compare the histopathological placental features of monochorionic diamniotic (MCDA) twins who did and did not undergo fetoscopic laser photocoagulation (FLP). METHODS: This was a retrospective single-institution cohort study on MCDA twins who underwent FLP between October 2010 and December 2018. The control group included MCDA twins who did not undergo FLP and were delivered during the same period in the institute. The incidence of chorioamnionitis (CAM), funisitis, and other pathological findings was compared between the FLP and control groups after matching by gestational age at delivery. RESULTS: In total, 292 MCDA pregnant women who underwent FLP and 356 controls gave birth during the study period. After matching the two groups in the ratio 1:1 by gestational age at delivery, each group comprised 194 subjects. The incidence of histological CAM with Blanc association (stage I, 6.2% vs. 3.1%, crude odds ratio (cOR) = 3.1, P = 0.052; stage II, 7.2% vs. 5.7%, cOR = 1.6, P = 0.30; stage III, 2.1% vs. 2.6%, cOR = 0.66, P = 0.52) and funisitis (artery, 5.2% vs. 3.6%, cOR = 1.3, P = 0.63; vein 7.2% vs. 4.1%, cOR = 1.6, P = 0.29) was not statistically significant difference between the FLP and control groups. The FLP group demonstrated a higher incidence of partial placental infarction than the control group (10.3% vs. 3.1%, cOR = 4.3, P = 0.004, adjusted OR = 2.8, P = 0.031). DISCUSSION: FLP did not appear to increase the incidence of histological CAM or funisitis in subjects matched by gestational age at delivery. The FLP group demonstrated a higher incidence of partial placental infarction than the control group.

Neurodevelopmental impairment at 3 years of age after fetoscopic laser surgery for twin-to-twin transfusion syndrome.

BACKGROUND: Data on neurodevelopmental outcomes of children surviving after fetoscopic laser surgery (FLS) for twin-to-twin transfusion syndrome (TTTS) are scarce. METHODS: We retrospectively investigated children surviving after FLS for TTTS at 16 to 26 weeks' gestation between 2003 and 2014. Children were evaluated by standardized neurologic examinations using the Kyoto Scale 2001 at a corrected age of 3 years ± 6 months. Neurodevelopmental impairment (NDI) was defined as cerebral palsy (CP), bilateral blindness, bilateral deafness or a developmental quotient (DQ) < 70 points. Brain magnetic resonance imaging (MRI) was performed at term-equivalent age. RESULTS: A total of 188 children from 110 twin pregnancies were evaluated. NDI was detected in 16/188 (8.5%) children, including six cases of CP (3.2%). No children had bilateral blindness or deafness. An earlier gestational age at delivery was associated with a higher incidence of NDI (P < .001). Abnormal brain MRI findings were detected in 9/16 (56%) of children with NDI, including 6/6 (100%) with CP. CONCLUSION: The incidence of NDI in children following FLS at 3 years old was 8.5%. Prematurity is a strong risk factor for NDI. Brain MRI may predict the development of CP.

Intrafetal laser for midtrimester TRAP sequence-experience from a single center.

OBJECTIVE: To report our experience and evaluate outcomes in monochorionic pregnancies with Twin Reversed Arterial Perfusion sequence with intrafetal laser therapy. METHODS: Retrospective review of records of all pregnancies with TRAP sequence treated by intrafetal laser therapy between 2011 January and 2015 December that were retrieved and analysed. RESULTS: Electronic search of the scan database retrieved 57 cases of TRAP sequence during the study period, 7 triplets and 50 monochorionic twins. Intrafetal laser was done in 27 cases, 22 cases of twins and 5 cases of triplets. In the twins group, median gestational age at intervention was 22.5 weeks, the earliest done at 16.3 weeks. The median gestational age at delivery and birth weight was 37 weeks and 2.5 Kgs. The median procedure and delivery interval was 14 weeks. Live birth rate was 17/22 (77%) the pump survival rate was 16/22 (73%). Pregnancies with non-surviving pump were 5 in numbers (5/22). A repeat procedure was warranted in one case. In the triplet group, median gestational age at intervention, delivery and procedure delivery interval was 18, 35 and 17 weeks. CONCLUSION: Intrafetal laser is simple, effective and the treatment of choice to interrupt the vascular supply to acardiac twin.

Open fetal surgery for myelomeningocele - is there the learning curve at reduction mother and fetal morbidity?

OBJECTIVES: We aimed to show how increased experience of a surgery team in fMMC repair influences maternal and fetal/neonatal outcomes. MATERIAL AND METHODS: We compare perinatal results of fMMC repair in our Fetal Surgery Center (FSC) in cohort groups for the early period (2005-2011 year; previous - PFSC, n = 46) and current period (2012-2015 year; current - CFSC, n = 74) to results of the randomized Management of Myelomeningocele Study (MOMS, 78 patients). RESULTS: The maternal morbidity due to fMMC repair was low and there was no difference comparing CFSC to PFSC and MOMS. The frequency of iatrogenic preterm labor (iPTL) ≤ 30 weeks of gestation decreased from 34.1% in PFSC to 23.9% in CFSC. Iatrogenic preterm premature rupture of membranes (iPPROM) was a common complication after fMMC repair in all cohorts. The total reduction rate of hindbrain hernation (HH) was similar in CFSC - 90.3% and PFSC - 82.1%. CONCLUSIONS: The increasing experience of our surgery team in fMMC repair majorly decreased the risk of iPTL.

EXIT (ex utero intrapartum treatment) surgery for the management of fetal airway obstruction: A systematic review of the literature.

PURPOSE: To provide a comprehensive overview of the perinatal and maternal outcomes of fetuses undergoing EXIT surgery for the management of fetal airway obstruction secondary to cervical or oral tumors. METHODS: A comprehensive search from inception to September 2018 was conducted on databases including MEDLINE, EMBASE, Cochrane Library and LILACS. All studies that reported an EXIT surgery in singleton were considered eligible. A descriptive analysis was performed. RESULTS: Out of the 250 full-text study reports, 120 articles reporting 235 cases of EXIT surgery were included. EXIT surgery was performed at 35.1 weeks of gestation on average. The most frequent diagnosis was teratoma (46.4%, n = 109/235). There were 13 adverse maternal events, and the most frequent one was postpartum hemorrhage (4.7%, n = 11/235). No maternal death was reported. Fetal and neonatal death occurred in 17% (40/235) of the cases. There were 29 adverse fetal events (12.2%), and the most frequent one was the failure of intubation or tracheostomy (3.4%, n = 8/235). CONCLUSION: EXIT surgery could be considered for the management of an oral or cervical tumor that's highly suspicious of blocking the fetal airway. This systematic review reports that EXIT surgery poses substantial risks of maternal and fetal adverse events, including neonatal death. LEVEL OF EVIDENCE: IV case series with no comparison group.

Single-Center Outcome of Fetoscopic Tracheal Balloon Occlusion for Severe Congenital Diaphragmatic Hernia.

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.

Increased nuchal translucency at 11-13 weeks' gestation and outcome in twin pregnancy.

OBJECTIVE: To investigate the value of increased fetal nuchal translucency thickness (NT) at the 11-13-week scan in the prediction of adverse outcome in dichorionic (DC), monochorionic diamniotic (MCDA) and monochorionic monoamniotic (MCMA) twin pregnancies. METHODS: This was a retrospective analysis of prospectively collected data on twin pregnancies undergoing routine ultrasound examination at 11-13 weeks' gestation between 2002 and 2019. In pregnancies with no major defects or chromosomal abnormalities, we examined the value of increased NT ≥ 95th percentile in one or both fetuses in the prediction of, first, miscarriage or death of one or both fetuses at < 20 and < 24 weeks' gestation in DC, MCDA and MCMA twin pregnancies, second, death of one or both fetuses or neonates at ≥ 24 weeks in DC, MCDA and MCMA twin pregnancies, third, development of twin-twin transfusion syndrome (TTTS) or selective fetal growth restriction (sFGR) treated by endoscopic laser surgery at < 20 and ≥ 20 weeks' gestation in MCDA pregnancies, and, fourth, either fetal loss or laser surgery at < 20 weeks' gestation in MCDA pregnancies. RESULTS: The study population of 6225 twin pregnancies included 4896 (78.7%) DC, 1274 (20.5%) MCDA and 55 (0.9%) MCMA pregnancies. The incidence of NT ≥ 95th percentile in one or both fetuses in DC twin pregnancies was 8.3%; in MCDA twins the incidence was significantly higher (10.4%; P = 0.016), but in MCMA twins it was not significantly different (9.1%; P = 0.804) from that in DC twins. In DC twin pregnancies, the incidence of high NT was not significantly different between those with two survivors and those with adverse outcome. In MCMA twin pregnancies, the number of cases was too small for meaningful assessment of the relationship between high NT and adverse outcome. In MCDA twin pregnancies with at least one fetal death or need for endoscopic laser surgery at < 20 weeks' gestation, the incidence of NT ≥ 95th percentile was significantly higher than in those with two survivors (23.5% vs 9.8%; P < 0.0001). Kaplan-Meier analysis in MCDA twin pregnancies showed that, in those with NT ≥ 95th percentile, there was significantly lower survival at < 20 weeks' gestation than in those with NT < 95th percentile (P = 0.001); this was not the case for survival at ≥ 20 weeks (P = 0.960). The performance of screening by fetal NT ≥ 95th percentile for prediction of either fetal loss or need for endoscopic laser surgery at < 20 weeks' gestation was poor, with a detection rate of 23.5% at a false-positive rate of 8.9%, and the relative risk, in comparison to fetal NT < 95th percentile, was 2.640 (95% CI, 1.854-3.758; P < 0.0001). In MCDA twin pregnancies, the overall rate of fetal loss or need for laser surgery at < 20 weeks' gestation was 10.7% but, in the subgroups with NT ≥ 95th and NT ≥ 99th percentiles, which constituted 10.4% and 3.3% of the total, the rates increased to 24.1% and 40.5%, respectively. CONCLUSIONS: In MCDA twin pregnancies with no major fetal abnormalities, measurement of NT at the 11-13-week scan is a poor screening test for adverse pregnancy outcome. However, the finding in one or both fetuses of NT ≥ 95th percentile, and more so ≥ 99th percentile, is associated with a substantially increased risk of fetal loss or need for endoscopic laser surgery at < 20 weeks' gestation. The extent to which closer monitoring and earlier intervention in the high-risk group can reduce these complications remains to be determined. Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd.

The first national survey of anesthesia techniques for fetal therapies in Japan.

PURPOSE: The aim of this study was to survey the frequency of various anesthetic techniques used in the anesthetic management of both the mother and fetus during fetal therapies in Japan. METHODS: We sent a postal survey to the institutions with physicians who held membership of the Japan Society of Fetal Therapy to describe maternal and fetal anesthetic management during fetal therapies performed from January 2016 to March 2017. The therapies included were thoracoamniotic shunting (TAS), intrauterine transfusion (IUT), radiofrequency ablation (RFA), fetoscopic laser photocoagulation (FLP), fetoscopic endotracheal occlusion (FETO), and ex utero intrapartum treatment (EXIT). Survey respondents were asked to specify the standard anesthetic technique used in each of these procedures done during the study period. RESULTS: The most common anesthetic techniques used in each therapy were sedation/analgesia with local anesthesia in TAS (31%), local anesthesia alone in IUT (47%), neuraxial anesthesia in RFA (50%), FLP (66%) and FETO (100%), and general endotracheal anesthesia in EXIT. Fetal analgesia was utilized in 61% of TAS, 33% of IUT, 10% of RFA, 22% of FLP, 100% of FETO, and 50% of EXIT. In all fetal therapies, the most common route of administration for fetal anesthesia was maternal administration. CONCLUSION: In this first published description of the frequency of various anesthetic techniques used during fetal therapies in Japan, we found that anesthetic techniques varied depending on the degree of invasiveness to the mother and fetus. Fetal anesthesia was not always performed, and the most common route for fetal anesthesia was maternal administration.

TTTS-GPS: Patient-specific preoperative planning and simulation platform for twin-to-twin transfusion syndrome fetal surgery.

Twin-to-twin transfusion syndrome (TTTS) is a serious condition that may occur in pregnancies when two or more fetuses share the same placenta. It is characterized by abnormal vascular connections in the placenta that cause blood to flow unevenly between the babies. If left untreated, perinatal mortality occurs in 90% of cases, whilst neurological injuries are still present in TTTS survivors. Minimally invasive fetoscopic laser surgery is the standard and optimal treatment for this condition, but is technically challenging and can lead to complications. Acquiring and maintaining the required surgical skills need consistent practice, and a steep learning curve. An accurate preoperative planning is thus vital for complex TTTS cases. To this end, we propose the first TTTS fetal surgery planning and simulation platform. The soft tissue of the mother, the uterus, the umbilical cords, the placenta and its vascular tree are segmented and registered automatically from magnetic resonance imaging and 3D ultrasound using computer vision and deep learning techniques. The proposed state-of-the-art technology is integrated into a flexible C++ and MITK-based application to provide a full exploration of the intrauterine environment by simulating the fetoscope camera as well as the laser ablation, determining the correct entry point, training doctors' movements and trajectory ahead of operation, which allows improving upon current practice. A comprehensive usability study is reported. Experienced surgeons rated highly our TTTS planner and simulator, thus being a potential tool to be implemented in real and complex TTTS surgeries.

Outcomes of hypoplastic left heart syndrome and fetal aortic valvuloplasty in a country with suboptimal postnatal management.

BACKGROUND: Fetal aortic stenosis (AoS) may progress to hypoplastic left heart syndrome (HLHS) in utero. There are currently no data, prenatal or postnatal, describing survival of fetuses or neonates with AoS or HLHS in a country with suboptimal postnatal management. STUDY DESIGN: Prospective cohort study performed in Mexico, including cases diagnosed with AoS and HLHS within a 6-year period. AoS patients fulfilling previously published criteria for evolving HLHS (eHLHS) were offered fetal aortic valvuloplasty. Outcome variables were perinatal mortality, postnatal management, type of postnatal circulation, and overall survival. RESULTS: Fifty-four patients were included: 16 AoS and 38 HLHS. Eighteen patients had associated anomalies and/or an abnormal karyotype. Seventy-four percent of HLHS received comfort measures, with only three cases reporting an attempt at surgical palliation, and one survivor of the first stage. Fetal aortic valvuloplasty was performed successfully in nine cases of eHLHS. Overall postnatal survival was 44% in AoS with fetal aortic valvuloplasty, and one case (ongoing) in the HLHS group. CONCLUSIONS: HLHS in Mexico carries more than a 95% risk of postnatal death, with little or no experience at surgical palliation in most centers. Fetal aortic valvuloplasty in AoS may prevent progression to HLHS and in this small cohort was associated with ≈50% survival.

Primary fetal pleural effusion: Characteristics, outcomes, and the role of intervention.

BACKGROUND: We aimed to present the natural history and outcomes of fetal primary pleural effusions (PPE). METHODS: Fetuses with prenatal diagnosis of PPE delivered between January 2011 and June 2018 were included. Fetal PPE were separated into groups: resolved, stable, or progressive. Progressive PPE was diagnosed, using fetal echocardiography, by the new onset of fetal hydrops or impaired cardiac function. Data were reported as median [range] and n (%). RESULTS: Among 189 fetuses with antenatal diagnosis of pleural effusion, 30 had a PPE. A total of 26.7% (n = 8), 26.7% (n = 8), and 40.0% (n = 12) were classified as resolved, stable, and progressive, respectively; two were lost to follow-up. In 14 cases (50%), there were bilateral pleural effusions. Of the 12 cases in the progressive group, four (33.3%) had amnioreduction, six (50.0%) had thoracentesis, and eight (66.7%) had shunt placement performed. There were two fetal deaths, both in the progressive group, one of which received amnioreduction and the other underwent both thoracentesis and shunt placement prior to demise. CONCLUSION: In more than half of fetuses with prenatal PPE, the effusion remained stable or spontaneously resolved, and the perinatal outcomes were generally favorable. This information will be useful in optimizing the counseling and care of these patients.