Double myelomeningocele repair by fetal surgery with a single micro-hysterotomy.

INTRODUCTION: Open spina bifida (OSB) is the most common congenital anomaly of the central nervous system. It is associated with severe neurodevelopmental delay, motor impairment, hydrocephalus, and bowel and bladder dysfunction. In selected cases, intrauterine spina bifida repair has been shown to improve neonatal outcomes. Rarely, the spine can have a double defect compromising two different segments and there is a lack of evidence on the feasibility and benefits of intrauterine repair in these cases. CASE PRESENTATION: We present a case with both cervicothoracic and lumbosacral myelomeningocele, Arnold-Chiari malformation type II, and bilateral ventriculomegaly, that was treated successfully at 25 weeks with open micro-neurosurgery. Double myelomeningocele was successfully treated through a single 2cm micro-hysterotomy, by performing external versions to sequentially expose and repair both defects. Weekly postoperative follow-up showed no progression of ventriculomegaly or complications attributable to the procedure. Preterm rupture of membranes prompted a conventional cesarean delivery at 32 weeks of gestation. Neurodevelopmental outcome at 20 months was within normal ranges, having achieved ambulation without orthopedic support, and with no need for ventriculoperitoneal shunting. CONCLUSION: This report demonstrates for the first time the feasibility of double OSB repair through a single 2cm micro-hysterotomy, suggesting that selected isolated cases of double myelomeningocele could be candidates for fetal intervention. Further prospective studies should be carried out to assess the potential benefit of double OSB intrauterine open repair.

Term planned delivery based on fetal growth assessment with or without the cerebroplacental ratio in low-risk pregnancies (RATIO37): an international, multicentre, open-label, randomised controlled trial.

BACKGROUND: The cerebroplacental ratio is associated with perinatal mortality and morbidity, but it is unknown whether routine measurement improves pregnancy outcomes. We aimed to evaluate whether the addition of cerebroplacental ratio measurement to the standard ultrasound growth assessment near term reduces perinatal mortality and severe neonatal morbidity, compared with growth assessment alone. METHODS: RATIO37 was a randomised, open-label, multicentre, pragmatic trial, conducted in low-risk pregnant women, recruited from nine hospitals over six countries. The eligibility criteria were designed to be broad; participants were required to be 18 years or older, with an ultrasound-dated confirmed singleton pregnancy in the first trimester, an alive fetus with no congenital malformations at the routine second-trimester ultrasound, an absence of adverse medical or obstetric history, and the capacity to give informed consent. Women were randomly assigned in a 1:1 ratio (block size 100) using a web-based system to either the concealed group or revealed group. In the revealed group, the cerebroplacental ratio value was known by clinicians, and if below the fifth centile, a planned delivery after 37 weeks was recommended. In the concealed group, women and clinicians were blinded to the cerebroplacental ratio value. All participants underwent ultrasound at 36 + 0 to 37 + 6 weeks of gestation with growth assessment and Doppler evaluation. In both groups, planned delivery was recommended when the estimated fetal weight was below the tenth centile. The primary outcome was perinatal mortality from 24 weeks' gestation to infant discharge. The study is registered at ClinicalTrials.gov (NCT02907242) and is now closed. FINDINGS: Between July 29, 2016, and Aug 3, 2021, we enrolled 11 214 women, of whom 9492 (84·6%) completed the trial and were eligible for analysis (4774 in the concealed group and 4718 in the revealed group). Perinatal mortality occurred in 13 (0·3%) of 4774 pregnancies in the concealed group and 13 (0·3%) of 4718 in the revealed group (OR 1·45 [95% CI 0·76-2·76]; p=0·262). Overall, severe neonatal morbidity occurred in 35 (0·73%) newborns in the concealed group and 18 (0·38%) in the revealed group (OR 0·58 [95% CI 0·40-0·83]; p=0·003). Severe neurological morbidity occurred in 13 (0·27%) newborns in the concealed group and nine (0·19%) in the revealed group (OR 0·56 [95% CI 0·25-1·24]; p=0·153). Severe non-neurological morbidity occurred in 23 (0·48%) newborns in the concealed group and nine (0·19%) in the revealed group (0·58 [95% CI 0·39-0·87]; p=0·009). Maternal adverse events were not collected. INTERPRETATION: Planned delivery at term based on ultrasound fetal growth assessment and cerebroplacental ratio at term was not followed by a reduction of perinatal mortality although significantly reduced severe neonatal morbidity compared with fetal growth assessment alone. FUNDING: La Caixa foundation, Cerebra Foundation for the Brain Injured Child, Agència per la Gestió d'Ajuts Universitaris i de Recerca, and Instituto de Salud Carlos III.

Fetal Laryngoscopy and Endoscopic Tracheal Intubation to Avoid ex utero Intrapartum Treatment in the Management of Fetuses with Suspected Airway Obstruction.

INTRODUCTION: Large congenital neck tumors can cause neonatal death due to airway obstruction. The aim of this study was to report outcomes of the first cohort of fetuses with neck masses and suspected airway obstruction managed with fetal laryngoscopy (FL) and fetal endoscopic tracheal intubation (FETI) to secure fetal airways and avoid ex utero intrapartum treatment (EXIT) procedure. METHODS: A prospective observational cohort of consecutive fetuses with neck masses that were candidates for an EXIT procedure due to suspicion of laryngeal and/or tracheal occlusion on ultrasonographic (US) or magnetic resonance imaging (MRI) examination were recruited for FL in a tertiary referral center in Queretaro, Mexico. FETI was performed if the obstruction was confirmed by FL. Maternal and perinatal outcomes were evaluated. RESULTS: Between January 2012 and March 2023, 35 cases with neck masses were evaluated. Airway obstruction was suspected in 12/35 (34.3%), either by US in 10/35 (28.6%) or by fetal MRI in 2/35 (5.7%). In all cases, FL was successfully performed at the first attempt at a median gestational age (GA) of 36+5 (range, 33+5-39+6) weeks+days, with a median surgical time of 22.5 (12-35) min. In 4 cases, airway patency was confirmed during FL and an EXIT procedure was avoided. In 8/12 cases (66.7%), airway obstruction was confirmed during fetoscopy and FETI was successfully performed at a median GA of 36+3 (33+2-38+5) weeks+days, with a median surgical time of 25.0 (range, 12-45) min. No case required an EXIT procedure. All patients underwent conventional cesarean delivery with no maternal complications and all neonates were admitted to the neonatal intensive care unit with a correctly positioned endotracheal tube (ETT) immediately after delivery. Three neonatal deaths (37.5%) were reported due to postnatal unplanned extubation, failed ETT replacement, and tumoral bleeding. CONCLUSION: In fetuses with neck masses and suspected airway obstruction, FL and FETI are feasible and could replace EXIT procedures with good maternal and perinatal outcomes.

Management of Late Twin-to-Twin Transfusion Syndrome beyond 26 Weeks: Comparison between Fetoscopic Laser Therapy versus Traditional Management.

INTRODUCTION: A proportion of monochorionic diamniotic (MCDA) twin pregnancies complicated by twin-to-twin transfusion syndrome (TTTS) can present after 26 weeks of gestation. The aim of this study was to compare perinatal outcomes of late TTTS treated by fetoscopic laser coagulation versus traditional management with amniodrainage and/or emergency preterm cesarean delivery (CD). METHODS: Retrospective cohort from January 2012 to January 2023 of consecutive MCDA twin pregnancies complicated by TTTS after 26 weeks and evaluated in our referring centers. We analyzed perinatal outcomes of cases treated with fetoscopic laser surgery at our national referral fetal surgery center in Queretaro, Mexico, and compared them with those managed with traditional management (amniodrainage and/or emergency preterm CD). The primary outcome was survival at discharge and the secondary outcome was gestational age (GA) at birth. RESULTS: Among the study population, 46 TTTS cases were treated by fetoscopy at 27+6 (26+0-31+0) weeks+days and were compared with a group of 39 cases who underwent emergency preterm CD. In comparison to the group who underwent traditional management, the group treated by laser fetoscopy showed a significantly higher GA at birth (32+3 vs. 29+1 weeks+days, p < 0.001), lower frequency of preterm delivery below 37 weeks (91.3% vs. 100%, p = 0.06), 34 weeks (63.0% vs. 100%, p < 0.001), 32 weeks (50% vs. 74.4%, p = 0.02), or 30 weeks (28.3% vs. 53.8%, p = 0.01), and significantly higher perinatal survival (89.1% vs. 71.8%, p < 0.05 of at least one twin; and 65.2% vs. 38.5%, p = 0.01 of both twins, respectively). CONCLUSION: MCDA twins complicated with TTTS can be treated with fetoscopic laser surgery between 26 and 31 weeks of gestation, which is a feasible and safe option, and such cases are associated with a higher GA at birth and better perinatal survival than those managed with amniodrainage and/or emergency preterm CD.

Flexible Video Fetoscopy: Feasibility and Outcomes of a Novel Modality for Laser Therapy in Twin-to-Twin Transfusion Syndrome Presenting with Inaccessible Anterior Placenta.

INTRODUCTION: Identification of intertwin anastomosis may be challenging during fetoscopy in cases with complete anterior placenta. The aim of this study was to describe the technique, feasibility, and outcomes of flexible video fetoscopy for laser coagulation in monochorionic (MC) twin pregnancies with twin-to-twin transfusion syndrome (TTTS) presenting with inaccessible anterior placenta. METHODS: From April 2021 to March 2022, a prospective cohort of consecutive MC twin pregnancies complicated with TTTS presenting with anterior placenta after 20 weeks was recruited. Cases with inaccessible anterior placenta during standard technique were converted into flexible video fetoscopy for completion of laser coagulation of placental anastomoses using a 270° flexible video endoscope through the same uterine port. Descriptive analysis includes feasibility, remaining anastomoses requiring laser photocoagulation, and perinatal outcomes. RESULTS: A total of 45 pregnancies with TTTS were treated with fetoscopic laser therapy during the 1-year study period. Twenty-one pregnancies presented with anterior placenta after 20 weeks, in which an inaccessible vascular equator was observed in 33.3% (7/21). Flexible video fetoscopy was successfully performed in all 7 cases at a median gestational age of 22+2 (20+0-27+1) weeks+days. Visualization of the entire placental surface, coagulation of selected vessels, and exploration of the entire vascular equator were achieved in all cases. Six cases (85.7%) required additional laser coagulation due to either vascular patency despite initial coagulation with conventional fetoscopy (1/6, 16.7%) and/or remaining noncoagulated anastomoses (5/6, 83.3%). Perinatal survival of at least one twin and both twins was achieved in 85.7% and 57.1%, respectively. DISCUSSION: Flexible video fetoscopy for completion of laser coagulation of placental anastomoses is feasible and represents a good option for TTTS cases presenting after 20 weeks with inaccessible anterior placenta.

Postnatal care setting and survival after fetoscopic tracheal occlusion for severe congenital diaphragmatic hernia: A systematic review and meta-analysis.

BACKGROUND: Fetoscopic endoluminal tracheal occlusion (FETO) was recently shown to improve postnatal survival in a multicenter, randomized controlled trial of infants with severe congenital diaphragmatic hernia (CDH). However, the external validity of this study remains unclear given a lack of standardization in postnatal management approaches. The purpose of this study was to evaluate the impact of an integrated prenatal and postnatal care setting on survival outcomes in severe CDH after FETO. STUDY DESIGN: A systematic review, meta-analysis, and individual participant analysis of FETO outcomes in severe CDH were conducted in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines. The primary outcome was survival to discharge. Subgroup analyses of patients managed in integrated versus nonintegrated settings were performed to identify predictors of outcome. RESULTS: The review generated five studies (n = 192) for the meta-analysis of FETO versus expectant prenatal management. These data revealed a significant survival benefit after FETO that was restricted to an integrated setting (OR 2.97, 95% Confidence Interval 1.69-4.26). There were nine studies (n = 150) for the individual participant analysis, which showed that FETO managed in an integrated setting had significantly increased survival rates when compared to FETO treated in a nonintegrated setting (70.7% vs. 45.7%, p = 0.003). Multi-level logistic regression identified increased availability of extracorporeal membrane oxygenation (ECMO) as the strongest determinant of postnatal survival (OR=18.8, p = 0.049). CONCLUSION: This systematic review shows that institutional integration of prenatal and postnatal care is associated with the highest overall survival in children with severe CDH. These data highlight the importance of a standardized, multidisciplinary approach, including access to ECMO, as a critical postnatal component in optimizing FETO outcomes in CDH.

Management of atypical cases of twin-to-twin transfusion syndrome.

Up to 20% of monochorionic diamniotic twin pregnancies can be complicated with twin-to-twin transfusion syndrome (TTTS). This complication is diagnosed by ultrasound demonstrating amniotic fluid discordance between both amniotic sacs, with polyhydramnios in the recipient's sac and oligohydramnios in the donor's, secondary to an imbalance in blood volume exchange between twins. Ultrasound evaluation of the amniotic fluid volume, bladder filling, and assessment of fetal Doppler parameters provide the basis for classification of TTTS, allowing severity assessment. The Quintero's staging system provides a standardized prenatal estimate on the risk of intrauterine fetal demise of one or both twins and the need for fetoscopic laser coagulation of placental vascular anastomoses or delivery depending on the gestational age. However, a proportion of TTTS cases may present without a linear progressive deterioration and no ultrasound signs of preceding staging, in rare situations, they arise even without amniotic fluid discordance. Thus, these unusual clinical presentations of TTTS have long been grouped into the category of atypical TTTS. In this review, we show the clues for diagnosis and management of different atypical cases of TTTS highlighting their underlying mechanism to improve the clinical understanding of such atypical situations, avoid misdiagnosis of TTTS, and allow a timely referral to a fetoscopic center.

Outcomes of late open fetal surgery for intrauterine spina bifida repair after 26 weeks. Should we extend the Management of Myelomeningocele Study time window?

OBJECTIVE: To evaluate maternal and perinatal outcomes of late open fetal repair for open spina bifida (OSB) between 26+0 -27+6  weeks. METHODS: A cohort of fetuses with OSB who underwent open surgery in two fetal surgery centers (Argentina and Mexico). Two groups were defined based on the gestational age (GA) at intervention: Management of Myelomeningocele Study (MOMS) time window group: GA 19+0 -25+6 , and late intervention group: GA 26+0 -27+6 . RESULTS: Intrauterine OSB repair was successfully performed in 140 cases, either before (n = 57) or after (n = 83) 26 weeks, at on average 25+0 (22+6 -25+6 ) and 26+5 (26+0 -27+6 ) weeks + days, respectively. There were no significant differences in the rate of premature rupture of membranes, chorioamnionitis, oligohydramnios, preterm delivery, perinatal death and maternal complications. The late intervention group showed a significantly lower surgical times (112.6 vs. 124.2 min, p = 0.01), lower interval between fetal surgery and delivery (7.9 vs. 9.2 weeks, p < 0.01) and similar rate of hydrocephalus requiring treatment (30.6% vs. 23.3%, p = 0.44) than the MOMS time window group. CONCLUSION: Late fetal surgery for OSB repair between 26+0 -27+6  weeks is feasible and was associated with similar outcomes than that performed before 26 weeks. These findings may allow an extension of the proposed time window for cases with late diagnosis or referral.

Twin-to-twin transfusion syndrome and coronavirus disease 2019: impact on diagnosis, referral, eligibility for fetoscopic laser therapy, and outcomes.

BACKGROUND: Because of the progressive nature of twin-to-twin transfusion syndrome, difficulties in healthcare access during the COVID-19 pandemic may lead to delayed diagnosis and referral to fetal surgery centers, which may have repercussions on outcomes. OBJECTIVE: This study aimed to assess the clinical impact of the COVID-19 pandemic on pregnancies complicated with twin-to-twin transfusion syndrome. STUDY DESIGN: A retrospective cohort study of consecutive monochorionic diamniotic twin pregnancies complicated with twin-to-twin transfusion syndrome evaluated in our national referral fetal surgery center at Queretaro, Mexico, for possible surgical fetoscopy was conducted. Maternal-fetal characteristics and perinatal outcomes of cases evaluated during the first year of the World Health Organization's COVID-19 pandemic declaration (March 11, 2020 to March 10, 2021) were retrospectively compared with outcomes of cases evaluated during the same period in the previous year (March 11, 2019 to March 10, 2020). RESULTS: Overall, 109 consecutive twin-to-twin transfusion syndrome cases were evaluated during the 2-year study period, 54 during the COVID-19 pandemic and 55 in the previous year. In the former group, a higher proportion of cases with fetal surveillance interval longer than 2 weeks (70.4% vs 47.3%; P=.01); twin-to-twin transfusion syndrome complications precluding laser therapy, such as intrauterine fetal demise, preterm rupture of membranes, or cervical dilatation with prolapsed amniotic membranes (18.5% vs 1.8%; P<.01); advanced twin-to-twin transfusion syndrome (53.7% vs 36.4%; P=.07); preoperative short cervix (25.9% vs 10.9%; P<.05); and lower overall perinatal survival (56.9% vs 80.0% [P=.01; at least 1 twin] and 39.2% vs 56.4% [P=.08; both twins], respectively) were observed. A significantly lower number of cases were selected for fetoscopic laser therapy during the pandemic (75.9% vs 92.7%; P=.01), with similar postoperative outcomes seen in both study periods. CONCLUSION: In pregnancies with twin-to-twin transfusion syndrome, the COVID-19 pandemic has shown an adverse impact involving suboptimal fetal surveillance, advanced stages at diagnosis, poorer survival rates, and higher number of complications that preclude fetoscopic laser therapy.

Prediction of neonatal survival according to lung-to-head ratio in fetuses with right congenital diaphragmatic hernia (CDH): A multicentre study from the Latin American CDH Study Group registry.

OBJECTIVE: To evaluate survival outcomes of fetuses with right sided congenital diaphragmatic hernia (CDH) treated in Latin American centres and to assess the utility of left lung area to predict neonatal survival. METHODS: A retrospective cohort including isolated right sided CDH cases managed expectantly during pregnancy in six tertiary centers from five Latin American countries. The utility of the observed/expected lung-to-head ratio (O/E-LHR) in predicting neonatal survival was assessed, and the best cut-off to predict prognosis was automatically selected by decision tree analysis. RESULTS: A total of 99 right sided CDH cases were recruited, 58 isolated fetuses were selected at a median gestational age of 26.2 weeks, showing an overall survival rate of 26.2%. A linear trend was observed between survival and the O/E-LHR, showing that at higher O/E-LHR, the greater probability of survival (r = 0.56, p < 0.001). O/E-LHR discriminates two groups with different survival outcomes: fetuses with an O/E-LHR ≥65% showed a significantly higher survival rate than those with an O/E-LHR <65% (81.8% vs. 15.6%, p < 0.01). CONCLUSIONS: Overall survival rate in right sided CDH is lower in Latin American countries. The severity category of pulmonary hypoplasia should be classified according to lung area and the survival rate in such population.

Variation in the uterine arteries Doppler parameters when obtained transvaginally or transabdominally at different sampling locations.

INTRODUCTION: To evaluate differences in Doppler velocimetry parameters when the uterine arteries (UtA) are evaluated transabdominally (TA) at different sampling locations and transvaginally (TV). MATERIALS AND METHODS: Five hundred and fifty-seven pregnant women were evaluated between 11 and 39 weeks of gestation. The mean UtA pulsatility index (PI) and prevalence of bilateral notching were obtained at four different locations: (1) TA just above the crossing with the iliac artery; (2) TA just below the crossing with the iliac artery; (3) TA well above approximately 3 cm away from the crossing with the iliac artery; and (4) TV at the point closest to the internal cervical os. Measurements obtained just above the external iliac artery were considered the standard for comparison. Differences among different locations per gestational week were calculated. RESULTS: The mean UtA-PI and prevalence of bilateral notching were similar when the uterine arteries were sampled TA just above or just below the crossing with the external iliac artery. The mean UtA-PI values and prevalence of bilateral notching were significantly higher (p < .0001) when obtained TV and significantly lower when obtained 3 cm above the crossing with the external iliac artery (p = .004), as compared to the standard plane just above the crossing. CONCLUSION: The mean UtA-PI and prevalence of bilateral notching vary significantly when the uterine arteries are sampled far above the crossing with the external iliac artery or when obtained transvaginally.Key MessageThe predictive performance of the uterine arteries during pregnancy can significantly vary in relation to the approach selected for evaluation and to the location of the Doppler sampling gate.

Impact of fetal endoscopic tracheal occlusion in fetuses with congenital diaphragmatic hernia and moderate lung hypoplasia.

OBJECTIVE: To assess the effect of Fetal Endoscopic Tracheal Occlusion (FETO) on neonatal survival in fetuses with left congenital diaphragmatic hernia (CDH) and moderate lung hypoplasia. STUDY DESIGN: CDH fetuses with moderate pulmonary hypoplasia (observed/expected lung area to head ratio between 26% and 35%, or between 36% and 45% with liver herniation) were prospectively recruited. Included patients were matched to a control group who were ineligible for FETO. Primary outcomes were survival at 28 days, at discharge, and at 6 months of age, respectively. RESULTS: 58 cases were recruited, 29 treated with FETO and 29 matched controls. Median gestational age (GA) at balloon placement and removal were 29.6 and 33.6 weeks, respectively. FETO group showed significantly lower GA at delivery (35.2 vs. 37.1 weeks, respectively, p < 0.01), higher survival at 28 days (51.7 vs. 24.1%, respectively, p = 0.03), at discharge (48.3 vs. 24.1%, respectively, p = 0.06), and at six months of age (41.4 vs. 24.1%, respectively, p = 0.16), and significantly lower length of ventilatory support (17.8 vs. 32.3 days, p = 0.01) and NICU stay (34.2 vs. 58.3 days, p = <0.01) compared to controls. CONCLUSION: FETO was associated with a non-significant increase in survival and significantly lower neonatal respiratory morbidity among CDH fetuses with moderate lung hypoplasia.

Open surgery for in utero repair of spina bifida: Microneurosurgery versus standard technique - A systematic review.

BACKGROUND/OBJECTIVES: Prenatal myelomeningocele (MMC) repair has been shown to improve neurological outcomes. It has been suggested that decreases in the hysterotomy diameter during surgery can improve perinatal outcomes without altering neurologic outcomes. The objective of this study is to describe and compare the main maternal and fetal outcomes of fetuses undergoing open surgery for MMC repair, through the different modifications (standard-classical, mini-hysterotomy, and microneurosurgery). DATA SOURCE: MEDLINE, EMBASE, Cochrane Central Register of Controlled Trials, Ovid, SciELO, LILACS, PROSPERO. RESULTS: From a total of 112 studies, seven case series were selected including 399 fetuses with open fetal surgery, five studies using the classical technique (n = 181), one with mini-hysterotomy (n = 176), and one with the microneurosurgery technique (n = 42). The mini-hysterotomy and microneurosurgery techniques presented a lower risk of preterm delivery (21.4% and 30%, respectively) compared to the classic technique (47.3%), premature rupture of membranes (78%, 62%, and 72.5 %, respectively), oligohydramnios (0% and 72.5%, respectively), dehiscence of hysterotomy, maintaining the same frequency of Chiari reversion (78%, 62%, and 72.5%, respectively), postnatal correction requirement (0%, 4.8%, and 5.8%, respectively), and lower frequency of requirement for a ventriculoperitoneal shunt placement (13.0%, 7.5%, and 29.1%, respectively). CONCLUSION: The least invasive techniques (minihysterotomy-microneurosurgery) are possible and reproduceable, as they are associated with better maternal and perinatal outcomes.

Open intrauterine repair of spina bifida aperta: Historical aspects, current availability, and clinical outcomes from the Latin American Spina Bifida Consortium.

OBJECTIVE: To determine the historical aspects, current availability, and clinical outcomes of open intrauterine repair of spina bifida aperta (IRSBA) in Spanish-speaking Latin American countries. METHODS: Cases were collected from centers with at least 2 years of experience and a minimum of 10 open IRSBA interventions by December 2020. Clinical variables were compared to the results of the Management of Myelomeningocele Study (MOMS) trial. RESULTS: Clinical experience with 314 cases from seven centers was reviewed. Most cases (n = 189, 60.2%) were performed between 24 and 25.9 weeks' gestation. Delivery at less than 30 weeks' gestation occurred in 36 cases (11.5%) and the overall perinatal mortality rate was 5.4% (17 of 314). The rate of maternal complications was low, including the need for blood transfusion (n = 3, 0.9%) and dehiscence or a thin uterine scar (n = 4, 1.3%). No cases of maternal death were recorded. Fifteen neonates required additional surgical repair of the spinal defect (4.8%) and 63 of 167 infants (37.7%) required a cerebrospinal fluid diversion procedure. Only two of the seven centers reported preliminary experience with fetoscopic IRSBA. CONCLUSIONS: Clinical experience and outcomes were within the expected results reported by the MOMS trial. There is still very limited experience with fetoscopic IRSBA in this part of the world.

Fetoscopic urethral meatotomy in fetuses with lower urinary tract obstruction by congenital megalourethra.

BACKGROUND: To describe the perinatal outcomes of fetoscopic urethral meatotomy (FUM) in fetuses with lower urinary tract obstruction (LUTO) by congenital megalourethra. STUDY DESIGN: Between 2012 and 2020, 226 cases with LUTO were referred to our fetal surgery center in Queretaro, Mexico. We report the perinatal outcome of cases with LUTO by congenital megalourethra that were selected for FUM in an attempt to release the penile urethral obstruction. RESULTS: Congenital megalourethra was diagnosed in 10 cases (4.4%) but only 3 cases (30%) with obstructive megalourethra and megacystis were selected for fetal surgery. Fetoscopic urethral metatotomy was successfully performed in all three cases at a median gestational age (GA) of 21.4 (18.0-26.7) weeks and with a median surgical time of 27 (12-43) min. A resolution of urethral dilatation and subsequent reduction of the penile length and normalization of both the bladder size and amniotic fluid were observed in all cases. The median GA at delivery was 35.2 (range: 30.6-38.0) weeks. There were no fetal deaths but one neonatal death (33%) secondary to renal failure and preterm delivery. CONCLUSION: In fetuses with LUTO by congenital obstructive megalourethra, FUM is feasible and is associated with good perinatal outcomes.

Preoperative Cervical Length Predicts the Risk of Delivery within One Week after Pleuroamniotic Shunt in Fetuses with Severe Hydrothorax.

OBJECTIVE: The objective of this study was to assess the predictive performance of preoperative cervical length (CL) for delivery within 1 week after pleuroamniotic shunting (PAS) in fetuses with severe hydrothorax. METHODS: A prospective cohort of fetuses with severe hydrothorax referred to our fetal surgery center in Querétaro, Mexico from January 2012 to July 2020. Severe fetal hydrothorax was diagnosed as an accumulation of fluid within the fetal pleural space accompanied with severe bilateral lung compression, mediastinal shift, polyhydramnios, and/or hydrops. Transvaginal CL was measured immediately before PAS, and a short cervix was defined as that <25 mm. The interval from fetal intervention to delivery, prevalence of preterm prelabor rupture of membranes (PPROMs), and associations with delivery within the first week after PAS according to a short or a normal CL, were evaluated. RESULTS: Thirty-five pregnancies with severe fetal hydrothorax treated with PAS were evaluated. Median gestational age at PAS was (weeks + days) 31+2 (range, 26+0-36+1). Two (5.7%) and 7 (20.0%) cases delivered within the first 24 h and 1 week after PAS, respectively. Ten (28.6%) women had a short cervix before PAS, while 25 (71.4%) had normal preoperative CL. Women with a short cervix showed lower mean interval between fetal intervention and delivery (2.4 vs. 5.5 weeks, p = 0.01), and higher prevalence of PPROM (50 vs. 12%, p = 0.01), as compared to women with a nonshort cervix. Preoperative short cervix was associated with significantly higher risk of delivery within the first 24 h (20.0 vs. 0%, respectively, p < 0.05) and 1 week after PAS (50.0 vs. 8.0%, respectively, p < 0.01) compared with pregnancies with normal preoperative CL. CONCLUSION: In pregnancies with severe fetal hydrothorax candidates for pleuroamniotic shunt, identification of a short cervix before fetal intervention can predict delivery within 1 week after the surgical procedure.

Single Uterine Access for Bilateral Pleuroamniotic Shunting in Fetuses with Severe Hydrothorax by an Internal Rotational Maneuver: Feasibility and Outcomes between Successful and Failed Procedures.

OBJECTIVE: The objective of this study was to describe the feasibility of single percutaneous uterine access for bilateral pleuroamniotic shunting (PAS) in fetuses with severe hydrothorax by using an internal rotational maneuver and to compare perinatal outcomes between successful and failed procedures. METHODS: A prospective cohort of 25 fetuses with isolated bilateral hydrothorax and hydrops were referred to our fetal surgery center in Queretaro, Mexico during an 8-year period. Bilateral PAS was first attempted through a percutaneous single uterine access by internal rotation of the fetus, which was achieved by using the blunt tip of the same cannula, and in case of a failed procedure, a second uterine port was used to place the second shunt. The perinatal outcomes between successful (single uterine port) and failed (2 uterine ports) fetal procedures were compared. RESULTS: Placing of bilateral shunts through a percutaneous single uterine access was feasible in 15/25 (60%) cases. Overall, median GA at delivery was 35.2 weeks with a survival rate of 64.0% (16/25). Three cases were excluded due to shunt dislodgement, leaving a final population of 22 fetuses; 13/22 (59.1%) and 9/22 (40.9%) managed using 1 and 2 uterine ports, respectively. The group with bilateral PAS placement through a successful single uterine port showed a significantly higher GA at birth (36.5 vs. 32.8 weeks, p = 0.001), lower surgical time (11.0 vs. 19.0 min, p = 0.01), longer interval between fetal intervention and delivery (5.7 vs. 2.7 weeks, p = 0.01), lower risk of preterm delivery (46.2 vs. 100%, p < 0.01), and lower rate of perinatal death (15.4 vs. 55.6%, p < 0.05) than the failed procedures requiring 2 uterine ports. CONCLUSION: In fetuses with severe bilateral hydrothorax and hydrops, bilateral pleuroamniotic shunting through a successful single percutaneous uterine access is feasible in up to 60% of cases and is associated with better perinatal outcomes.

Open Fetal Microneurosurgery for Intrauterine Spina Bifida Repair.

OBJECTIVES: The aim of the study was to describe the feasibility of open fetal microneurosurgery for intrauterine spina bifida (SB) repair and to compare perinatal outcomes with cases managed using the classic open fetal surgery technique. METHODS: In this study, we selected a cohort of consecutive fetuses with isolated open SB referred to our fetal surgery center in Queretaro, Mexico, during a 3.5-year period (2016-2020). SB repair was performed by either classic open surgery (6- to 8-cm hysterotomy with leakage of amniotic fluid, which was replaced before uterine closure) or open microneurosurgery, which is a novel technique characterized by a 15- to 20-mm hysterotomy diameter, reduced fetal manipulation by fixing the fetal back, and maintenance of normal amniotic fluid and uterine volume during the whole surgery. Perinatal outcomes of cases operated with the classic open fetal surgery technique and open microneurosurgery were compared. RESULTS: Intrauterine SB repair with a complete 3-layer correction was successfully performed in 60 cases either by classic open fetal surgery (n = 13) or open microneurosurgery (n = 47). No significant differences were observed in gestational age (GA) at fetal intervention (25.4 vs. 25.1 weeks, p = 0.38) or surgical times (107 vs. 120 min, p = 0.15) between both groups. The group with open microneurosurgery showed a significantly lower rate of oligohydramnios (0 vs. 15.4%, p = 0.01), preterm rupture of the membranes (19.0 vs. 53.8%, p = 0.01), higher GA at birth (35.1 vs. 32.7 weeks, p = 0.03), lower rate of preterm delivery <34 weeks (21.4 vs. 61.5%, p = 0.01), and lower rate of perinatal death (4.8 vs. 23.1%, p = 0.04) than the group with classic open surgery. During infant follow-up, the rate of hydrocephalus requiring ventriculoperitoneal shunting was similar between both groups (7.5 vs. 20%, p = 0.24). All patients showed an intact hysterotomy site at delivery. CONCLUSION: Intrauterine spina repair by open fetal microneurosurgery is feasible and was associated with better perinatal outcomes than classic open fetal surgery.

Prenatal diagnosis of congenital heart defects: experience of the first Fetal Cardiology Unit in Mexico.

OBJECTIVE: The purpose of this article was to describe our experience with the prenatal diagnosis of CHD in patients referred to our Fetal Cardiology Unit. METHODS: Prospective cohort study of consecutive fetuses referred for advanced fetal echocardiography to our Fetal Cardiology Unit during a 3-year period (September 2015-September 2018). RESULTS: Totally 809 fetuses were evaluated, with 1263 fetal advanced echocardiographies performed. Suspected cardiac abnormality was the most common indication for referral (62.2%). Only 7.3% of patients had known morbidities or risk factors for CHD. Mean gestational age at first examination was 25.6 ± 6.4 weeks. A total of 528 (65.3%) fetuses were found to have a cardiac defect: 40.7% had isolated CHD while 24.6% had associated anomalies. The most common defects found were ventricular septal defects (20.3%), followed by conotruncal defects (9.7%), hypoplastic left heart syndrome (9.3%), fetal arrhythmias (8.9%), and venous anomalies (8.7%). 31.6% presented abnormal genetic studies, the most frequent being Down syndrome (23/212, 10.8%), followed by DiGeorge syndrome (11/212, 5.2%). CONCLUSIONS: Prenatal screening and diagnosis of CHD in Mexico are feasible, with suspected cardiac abnormality being the main reason for referral to a specialized Fetal Cardiology Unit. Efforts must be made to make screening available to the general population in the first and second trimesters of pregnancy by fetal medicine or trained specialists, in order to identify fetal CHD and offer advanced echocardiography, genetic studies, timely fetal cardiac intervention in selected cases, and delivery in tertiary centers, to improve overall survival.