Tracheomalacia and tracheomegaly in infants and children with congenital diaphragmatic hernia managed with and without fetoscopic endoluminal tracheal occlusion (FETO): a multicentre, retrospective cohort study.

BACKGROUND: Temporary fetoscopic endoluminal tracheal occlusion (FETO) promotes lung growth and increases survival in selected fetuses with congenital diaphragmatic hernia (CDH). FETO is performed percutaneously by inserting into the trachea a balloon designed for vascular occlusion. However, reports on the potential postnatal side-effects of the balloon are scarce. This study aimed to evaluate the prevalence of tracheomalacia in infants with CDH managed with and without FETO and other consequences related to the use of the balloon. METHODS: In this multicentre, retrospective cohort study, we included infants who were live born with CDH, either with FETO or without, who were managed postnatally at four centres (UZ Leuven, Leuven, Belgium; Antoine Béclère, Clamart, France; BCNatal, Barcelona, Spain; and HCor-Heart Hospital, São Paulo, Brazil) between April 5, 2002, and June 2, 2021. We primarily assessed the prevalence of all (symptomatic and asymptomatic) tracheomalacia as reported in medical records among infants with and without FETO. Secondarily we assessed the prevalence of symptomatic tracheomalacia and its resolution as reported in medical records, and compared tracheal diameters as measured on postnatal x-rays. Crude and adjusted risk ratios (aRRs) and 95% CIs were calculated via modified Poisson regression models with robust error variances for potential association between FETO and tracheomalacia. Variables included in the adjusted model were the side of the hernia, observed-to-expected lung-to-head ratio, and gestational age at birth. Crude and adjusted mean differences and 95% CIs were calculated via linear regression models to assess the presence and magnitude of association between FETO and tracheal diameters. In infants who had undergone FETO we also assessed the localisation of balloon remnants on x-rays, and the methods used for reversal of occlusion and potential complications associated with balloon remnants as documented in clinical records. Finally we investigated whether the presence of balloon remnants was influenced by the interval between balloon removal and delivery. FINDINGS: 505 neonates were included in the study, of whom 287 had undergone FETO and 218 had not. Tracheomalacia was reported in 18 (6%) infants who had undergone FETO and in three (1%) who had not (aRR 6·17 [95% CI 1·83-20·75]; p=0·0030). Tracheomalacia was first reported in the FETO group at a median of 5·0 months (IQR 0·8-13·0). Symptomatic tracheomalacia was reported in 13 (5%) infants who had undergone FETO, which resolved in ten (77%) children by 55·0 months (IQR 14·0-83·0). On average, infants who had undergone FETO had a 31·3% wider trachea (with FETO tracheal diameter 7·43 mm [SD 1·24], without FETO tracheal diameter 5·10 mm [SD 0·84]; crude mean difference 2·32 [95% CI 2·11-2·54]; p<0·0001; adjusted mean difference 2·62 [95% CI 2·35-2·89]; p<0·0001). At birth, the metallic component was visible within the body in 75 (37%) of 205 infants with available thoraco-abdominal x-rays: it was located in the gastrointestinal tract in 60 (80%) and in the lung in 15 (20%). No side-effects were reported for any of the infants during follow-up. The metallic component was more likely to be in the lung than either outside the body or the gastrointestinal tract when the interval between occlusion reversal and birth was less than 24 h. INTERPRETATION: Although FETO was associated with an increased tracheal diameter and an increased probability of tracheomalacia, symptomatic tracheomalacia typically resolved over time. There is a higher risk of retention of metallic balloon components if reversal of the occlusion occurs less than 24 h before delivery. Finally, there were no reported side-effects of the metallic component of the balloon persisting in the body during follow-up. Longer-term follow-up is needed to ensure that no tracheal problems arise later in life. FUNDING: None.

Length of hysterotomy for fetal spina bifida repair is associated with prematurity risk.

OBJECTIVE: To investigate whether prenatal repair of spina bifida aperta through mini-hysterotomy results in less prematurity, as compared to standard hysterotomy, when adjusting for known prematurity risks. METHODS: We performed a bi-centric, propensity score matched, controlled study, that is, adjusting for factors earlier reported to result in premature delivery or membrane rupture, in consecutive women having prenatal repair either through stapled hysterotomy or sutured mini-hysterotomy (≤3.5 cm). Matches were pairwise compared and cox-regression analysis was performed to define the hazard ratio of delivery <37 weeks. RESULTS: Of 346 meeting the MOMS-criteria, 78 comparable pairs were available for matched-controlled analysis. Mini-hysterotomy patients were younger and had a higher BMI. Mini-hysterotomy was associated with a 1.67-lower risk of delivery <37 weeks (hazard ratio: 0.60; 95% CI: 0.42-0.85; p = 0.004) and 1.72 for delivery <34 + 6 weeks (hazard ratio: 0.58; 95% CI: 0.34-0.97; p = 0.037). The rate of intact uterine scar at birth (mini-hysterotomy: 98.7% vs. hysterotomy: 90.4%; p = 0.070), the rate of reversal of hindbrain herniation within 1 week after surgery (88.9% vs. 97.4%; p = 0.180) and the rate of cerebrospinal fluid leakage (0% vs. 2.7%; p = 0.50) were comparable. CONCLUSION: Prenatal spina bidifa repair through mini-hysterotomy was associated with a later gestational age at delivery and a comparable intact uterus rate without apparent compromise in neuroprotection.

Predictors of fetal death, neonatal survival and neurological outcomes in severe twin-twin transfusion syndrome treated by laser ablation of placental vessels.

OBJECTIVES: To identify predictors of outcomes in severe twin oligo-polyhydramnios sequence (TOPS) with or without twin anemia-polycythemia sequence (TAPS) and/or selective fetal growth restriction (SFGR) treated by laser ablation of placental vessels (LAPV). METHODS: Analysis of cases treated from 2011 to 2022. Variables evaluated Prenatal predictors: stages of TOPS, presence of TAPS and/or SFGR; pre-LAPV fetal ultrasound parameters; peri-LAPV variables. Perinatal predictors: GA at birth; birthweight; Apgar scores; transfontanellar ultrasonography (TFUS). OUTCOME VARIABLES: fetal death, neonatal survival, infant's neurodevelopment. Binary logistic regression analyses were performed to detect predictors of outcomes. RESULTS: 265 cases were included. Predictors of post-LAPV donor fetus' death were delta EFW (p:0.045) and absent/reverse end-diastolic flow in the umbilical artery (AREDF-UA) (p < 0.001). The predictor of post-LAPV recipient fetus' death was hydrops (p:0.009). Predictors of neonatal survival were GA at birth and Apgar scores. Predictors of infant's neurodevelopment were TFUS and pre-LAPV middle cerebral artery Doppler (MCAD) for the donor twin; and pre-LAPV ductus venosus' flow and MCAD for the recipient twin. CONCLUSIONS: Prediction of fetal death, neonatal survival and infant's neurodevelopment is possible in cases of TOPS associated or not with SFGR and/or TAPS that were treated by LAPV.

Management of short cervix in twin-to-twin transfusion syndrome: a role for pessary placement following fetoscopic laser surgery?

BACKGROUND: Preterm labor and delivery is a major concern for patients with twin-to-twin transfusion syndrome undergoing fetoscopic laser surgery. A preoperative short cervix is a risk factor for preterm labor. Pessary placement is a short-acting intervention that may be useful to reduce this adverse event. OBJECTIVE: This study aimed to investigate the relationship between pessary placement and preterm delivery in monochorionic twin pregnancies with twin-to-twin transfusion syndrome and a short cervix before fetoscopic laser surgery. STUDY DESIGN: This was a retrospective study in 2 centers, including all pregnancies affected by twin-to-twin transfusion syndrome that underwent fetoscopic laser surgery with the Solomon technique between 2013 and 2022 (center A) and 2014 and 2022 (center B) with a preoperative cervical length below 25 mm. This study explored the correlation between cervical length and fetoscopic laser surgery-to-delivery interval following active or expectant management and compared perinatal outcomes between patients managed expectantly and patients managed with pessary placement, using multivariate analysis to control for potential confounders. Patients with a cervical length below 5 mm were not included in the comparative analysis. RESULTS: Of 685 patients, 134 met the inclusion criteria. Moreover, 21 patients were treated with a cervical cerclage and excluded from the analysis, leaving 113 patients for the final analysis. There was a significant negative correlation between cervical length at fetoscopic laser surgery and the risk of early delivery (adjusted odds ratio, 0.66; 95% confidence interval, 0.49-0.81; P<.001). The use of a pessary correlated with fewer patients delivering before 28 weeks of gestation (adjusted odds ratio, 0.28; 95% confidence interval, 0.09-0.75), fewer double neonatal demise (adjusted odds ratio, 0.2; 95% confidence interval, 0.05-0.75). Posthoc subgroup analysis suggested that these improvements were essentially noticeable for cervical lengths between 5 and 18 mm, where pessary placement was associated with an increased fetoscopic laser surgery-to-delivery interval (+24 days; 95% confidence interval, 0.86-42; P=.042) and later gestational age at delivery (+3.3 weeks; 95% confidence interval, 0.86-42; P=.035). CONCLUSION: Patients with a moderately shortened cervix, between 5 and 18 mm, may benefit from pessary placement after fetoscopic surgery for twin-to-twin transfusion syndrome, resulting in a reduction of adverse neonatal outcomes, double neonatal demise, and severe preterm delivery.

Percutaneous fetal endoscopic third ventriculostomy for severe isolated cerebral ventriculomegaly.

OBJECTIVE: To demonstrate the feasibility and preliminary results of percutaneous fetal endoscopic third ventriculostomy (ETV) in human fetuses (pfETV) with isolated progressive and/or severe bilateral cerebral ventriculomegaly (IPSBV). METHODS: The initial results of pfETV for IPSBV were described. Perioperative, perinatal and postnatal variables were described. The Ages and Stages Questionnaire (ASQ-3), 3rd edition (ASQ-3) was used for follow-up of all infants. RESULTS: Successful pfETV was performed in 10/11 (91%) fetuses, at a median gestational age (GA) of 28.7 weeks (25.3-30.7). There were no perioperative complications. After pfETV, 70% (7/10) of the fetuses had a decreased or stabilized lateral ventricle atria|lateral ventricle's atria. The median GA at delivery was 38.2 weeks (35.9-39.3). There were no perinatal complications. The postnatal ventriculoperitoneal shunt rate was 80% (8/10). Among neonates/infants who had prenatal stabilization or a decrease in the LVAs, 4 (4/7: 57.1%) had abnormal scores on the ASQ-3. Among neonates/infants that experienced prenatal increases in the LVAs, all of them (3/3: 100%) had abnormal scores on the ASQ-3. CONCLUSION: Percutaneous ETV is feasible in human fetuses with progressive and/or severe cerebral ventriculomegaly and seems to be a safe procedure for both the mother and the fetus.

Fetal open spinal dysraphism repair through a mini-hysterotomy: Influence of gestational age at surgery on children's ability to walk.

OBJECTIVE: To analyze the impact of gestational age (GA) at the time of fetal open spinal dysraphism (OSD) repair through a mini-hysterotomy on the ability of children to walk. METHODS: Children who underwent in utero repair of OSD and had formal neurological assessment after 2.5 years of age were compared regarding their ability to walk in relation to pre-surgical predictors. RESULTS: Sixty-nine children fulfilled the inclusion criteria. Among them, 63.7% (44/69) were able to walk with or without orthesis. Fetal OSD correction performed earlier in gestation (from 19.7 to 26.9 weeks) was associated with a higher probability of walking with or without orthesis (p = 0.033). The median GA at delivery was 35.3 weeks. Multivariate binary logistic regression showed that the upper anatomical level of the OSD ( L5) (p < 0.004; OR: 10.31 [95% CI: 2.07-51.28]) and GA at the time of fetal surgery (p = 0.026; OR = 0.68 [95% CI: 0.48-0.95]) were independent predictors of the postnatal ability to walk with or without orthesis. CONCLUSION: Fetuses with OSD who were operated on earlier in pregnancy (range: 19.7-26.9 weeks), were more likely to walk with or without orthesis.

Fetal open spinal dysraphism repair through a mini-hysterotomy: Influence of gestational age at surgery on the perinatal outcomes and postnatal shunt rates.

OBJECTIVE: To analyze the impact of gestational age (GA) at the time of fetal open spinal dysraphism (OSD) repair through a mini-hysterotomy on the perinatal outcomes and the infants' ventriculoperitoneal shunt rates. METHODS: Retrospective study of cases of fetal OSD correction performed from 2014 and 2019. RESULTS: One hundred and ninety women underwent fetal surgery for OSD through a mini-hysterotomy, and 176 (176/190:92.6%) have since delivered. Fetal OSD correction performed earlier in the gestational period, ranging from 19.7 to 26.9 weeks, was associated with lower rates of postnatal ventriculoperitoneal shunting (P: .049). Earlier fetal surgeries were associated with shorter surgical times (P: .01), smaller hysterotomy lengths (P < .001), higher frequencies of hindbrain herniation reversal (P: .003), and longer latencies from surgery to delivery (P < .001). Median GA at delivery was 35.3 weeks. Multivariate binary logistic regression showed that both fetal lateral ventricle-to-hemisphere ratio (%; P < .001; OR: 1.14 [95% CI: 1.09-1.21]) and GA at the time of fetal surgery (P: .016; OR: 1.37 [95% CI: 1.07-1.77]) were independent predictors of postnatal ventriculoperitoneal shunting. CONCLUSION: Fetuses with OSD who were operated on earlier in the gestational interval, which ranged from 19.7 to 26.9 weeks, were less prone to receiving postnatal ventriculoperitoneal shunts.

Cirurgias intrauterinas: alternativa de intervenção para as cardiopatias congênitas / Intrauterine surgeries: intervetion alternative for congenital cardiopathies

As cardiopatias congênitas são anormalidades estruturais ou funcionais do sistema cardiovascular, advindas desde o nascimento, mesmo que diagnosticadas posteriormente. Entre as malformações congênitas, as cardiopatias são as principais causas de mortalidade infantil nos Estados Unidos e em outros países desenvolvidos. Com o intuito de reduzir os efeitos progressivos dessas patologias, a intervenção intrauterina tem se destacado como opção terapêutica diante de resultados iniciais satisfatórios. O presente artigo teve por objetivo demonstrar a evolução da cirurgia intrauterina para correção cardíaca, a partir da avaliação dos benefícios ao feto e visando a riscos mínimos e aceitáveis para a mãe. Foi realizada uma pesquisa na base de dados Publisher Medline (PubMed), Scientific Electronic Library Online (SciELO) e Biblioteca Virtual da Saúde (BVS), incluindo artigos publicados entre 2008 e 2018; além da pesquisa efetuada no Manual de Medicina Fetal da SOGIMIG ­ 2018. Os critérios de elegibilidade são amplos. São necessários equipe multidisciplinar, equipamentos sofisticados e aperfeiçoamento da técnica, dessa forma dificulta-se a realização das cirurgias. Entretanto, diante dos resultados já demonstrados, a cirurgia intrauterina apresenta-se como alternativa terapêutica promissora.(AU)

The congenital cardiopathies are structural or functional abnormalities of the cardiovascular system, originated from birth, even when previously diagnosed. Among the congenic malformations, the cardiopathies are the main causes of infant mortality in the United States and in other developed countries. In order to reduce the progressive effects of these pathologies, intrauterine intervention has been highlighted as a therapeutic option in contempt of satisfactory initial results. This article has as goal to demonstrate the evolution of the intrauterine surgery for cardiac correction, based on the evaluation of the benefits to the fetus and aiming at minimum and acceptable risks to the mother. A research was made based on the Publisher Medline (PubMed) data base, Scientific Electronic Library Online (SciELO) and Biblioteca Virtual da Saúde (BVS), including published articles between 2008 and 2018. Beyond the research made on The fetal medicine manual from SOGIMIG ­ 2018. The eligibility criteria are broad. It is necessary a multidisciplinary team, sophisticated equipments and technique improvement, therefore interfering in the performance of surgeries. However, in contempt of the already demonstrated results the intrauterine surgery presents itself as a therapeutic promising alternative.(AU)

In vitro fertilization and vasa previa: A report of two cases / Fertilização in vitro e vasa previa: relato de dois casos

Abstract Vasa previa (VP) is a dangerous obstetric condition associated with perinatal mortality and morbidity. In vitro fertilization (IVF) is a risk factor for VP due to the high incidence of abnormal placentation. The diagnosis should be made prenatally, because fetal mortality can be extremely high. We report two cases to demonstrate the accuracy of transvaginal ultrasound in the prenatal diagnosis of VP. A 40-year-old primiparous Caucasian woman with IVF pregnancy was diagnosed with VP at 29 weeks of gestation and was hospitalized for observation at 31 weeks of gestation. She delivered a male newborn weighing 2,380 g, with an Apgar score of 10 at 5 minutes, by elective cesarean section at 34 weeks + 4 days of gestation, without complications. A 36-yearold primiparous Caucasian woman with IVF pregnancy was diagnosed with placenta previa, bilobed placenta increta and VP. The cord insertion was velamentous. She was hospitalized for observation at 26 weeks of gestation. She delivered a female newborn weighing 2,140 g, with an Apgar score of 9 at 5 minutes, by emergency cesarean section at 33 weeks + 4 days of gestation due to vaginal bleeding. The prenatal diagnosis of VP was associated with a favorable outcome in the two cases, supporting previous observations that IVF is a risk factor for VP and that all IVF pregnancies should be screened by transvaginal ultrasound.

Resumo Vasa previa (VP) é uma condição obstétrica perigosa associada a mortalidade e morbidade perinatais. Fertilização in vitro (FIV) é um fator de risco para VP devido à alta incidência de placentação anormal. O diagnóstico deve ser realizado no período pré-natal, pois a possibilidade de mortalidade fetal é extremamente elevada. Relatamos dois casos para demonstrar a acurácia da ultrassonografia transvaginal no diagnóstico pré-natal de VP. Mulher caucasiana, primigesta, de 40 anos, submetida a FIV, foi diagnosticada com VP na 29ª semana de gestação e hospitalizada para observação na 31ª semana de gestação. A paciente foi submetida à cesariana eletiva com 34 semanas e 4 dias, sem complicações, com recém-nascido do sexo masculino, pesando 2.380 g, e com Apgar de 10 no 5° minuto. Mulher caucasiana, primigesta, de 36 anos, subetida a FIV, foi diagnosticada com placenta prévia, placenta bilobada, acretismo placentário e VP. Cordão umbilical com inserção velamentosa. A paciente foi hospitalizada para observação na 26ª semana de gestação. Foi submetida à cesariana de emergência com33 semanas e 4 dias por sangramento vaginal. O recém nascido do sexo feminino pesou 2.140 g, com Apgar de 9 no 5°minuto. O diagnóstico de VP no período pré-natal associou-se a um desfecho favorável nos dois casos, corroborando observações anteriores de que a FIV é um fator de risco para VP e de que todas as gestações por FIV deveriam ser avaliadas por ultrassonografia transvaginal.

In Vitro Fertilization and Vasa Previa: A Report of Two Cases.

Vasa previa (VP) is a dangerous obstetric condition associated with perinatal mortality and morbidity. In vitro fertilization (IVF) is a risk factor for VP due to the high incidence of abnormal placentation. The diagnosis should be made prenatally, because fetal mortality can be extremely high. We report two cases to demonstrate the accuracy of transvaginal ultrasound in the prenatal diagnosis of VP. A 40-year-old primiparous Caucasian woman with IVF pregnancy was diagnosed with VP at 29 weeks of gestation and was hospitalized for observation at 31 weeks of gestation. She delivered a male newborn weighing 2,380 g, with an Apgar score of 10 at 5 minutes, by elective cesarean section at 34 weeks + 4 days of gestation, without complications. A 36-year-old primiparous Caucasian woman with IVF pregnancy was diagnosed with placenta previa, bilobed placenta increta and VP. The cord insertion was velamentous. She was hospitalized for observation at 26 weeks of gestation. She delivered a female newborn weighing 2,140 g, with an Apgar score of 9 at 5 minutes, by emergency cesarean section at 33 weeks + 4 days of gestation due to vaginal bleeding. The prenatal diagnosis of VP was associated with a favorable outcome in the two cases, supporting previous observations that IVF is a risk factor for VP and that all IVF pregnancies should be screened by transvaginal ultrasound.

Vasa previa (VP) é uma condição obstétrica perigosa associada a mortalidade e morbidade perinatais. Fertilização in vitro (FIV) é um fator de risco para VP devido à alta incidência de placentação anormal. O diagnóstico deve ser realizado no período pré-natal, pois a possibilidade de mortalidade fetal é extremamente elevada. Relatamos dois casos para demonstrar a acurácia da ultrassonografia transvaginal no diagnóstico pré-natal de VP. Mulher caucasiana, primigesta, de 40 anos, submetida a FIV, foi diagnosticada com VP na 29ª semana de gestação e hospitalizada para observação na 31ª semana de gestação. A paciente foi submetida à cesariana eletiva com 34 semanas e 4 dias, sem complicações, com recém-nascido do sexo masculino, pesando 2.380 g, e com Apgar de 10 no 5° minuto. Mulher caucasiana, primigesta, de 36 anos, subetida a FIV, foi diagnosticada com placenta prévia, placenta bilobada, acretismo placentário e VP. Cordão umbilical com inserção velamentosa. A paciente foi hospitalizada para observação na 26ª semana de gestação. Foi submetida à cesariana de emergência com 33 semanas e 4 dias por sangramento vaginal. O recém nascido do sexo feminino pesou 2.140 g, com Apgar de 9 no 5°minuto. O diagnóstico de VP no período pré-natal associou-se a um desfecho favorável nos dois casos, corroborando observações anteriores de que a FIV é um fator de risco para VP e de que todas as gestações por FIV deveriam ser avaliadas por ultrassonografia transvaginal.

Type II and III Selective Fetal Growth Restriction: Perinatal Outcomes of Expectant Management and Laser Ablation of Placental Vessels.

OBJECTIVES: To describe the perinatal outcomes of type II and III selective fetal growth restriction (sFGR) in monochorionic-diamniotic (MCDA) twin pregnancies treated with expectant management or laser ablation of placental vessels (LAPV). METHODS: Retrospective analysis of cases of sFGR that received expectant management (type II, n=6; type III, n=22) or LAPV (type II, n=30; type III, n=9). The main outcomes were gestational age at delivery and survival rate. RESULTS: The smaller fetus presented an absent/reversed "a" wave in the ductus venosus (arAWDV) in all LAPV cases, while none of the expectant management cases presented arAWDV. The median gestational age at delivery was within the 32nd week for expectant management (type II and III) and for type II LAPV, and the 30th week for type III LAPV. The rate of at least one twin alive at hospital discharge was 83.3% and 90.9% for expectant management type II and III, respectively, and 90% and 77.8% for LAPV type II and III, respectively. CONCLUSION: LAPV in type II and III sFGR twins with arAWDV in the smaller fetus seems to yield outcomes similar to those of less severe cases that received expectant management.

Type II and III selective fetal growth restriction: perinatal outcomes of expectant management and laser ablation of placental vessels

OBJECTIVES: To describe the perinatal outcomes of type II and III selective fetal growth restriction (sFGR) in monochorionic-diamniotic (MCDA) twin pregnancies treated with expectant management or laser ablation of placental vessels (LAPV). METHODS: Retrospective analysis of cases of sFGR that received expectant management (type II, n=6; type III, n=22) or LAPV (type II, n=30; type III, n=9). The main outcomes were gestational age at delivery and survival rate. RESULTS: The smaller fetus presented an absent/reversed "a" wave in the ductus venosus (arAWDV) in all LAPV cases, while none of the expectant management cases presented arAWDV. The median gestational age at delivery was within the 32nd week for expectant management (type II and III) and for type II LAPV, and the 30th week for type III LAPV. The rate of at least one twin alive at hospital discharge was 83.3% and 90.9% for expectant management type II and III, respectively, and 90% and 77.8% for LAPV type II and III, respectively. CONCLUSION: LAPV in type II and III sFGR twins with arAWDV in the smaller fetus seems to yield outcomes similar to those of less severe cases that received expectant management.

Balloon removal after fetoscopic endoluminal tracheal occlusion for congenital diaphragmatic hernia.

BACKGROUND: Isolated congenital diaphragmatic hernia defect allows viscera to herniate into the chest, competing for space with the developing lungs. At birth, pulmonary hypoplasia leads to respiratory insufficiency and persistent pulmonary hypertension that is lethal in up to 30% of patients. Antenatal measurement of lung size and liver herniation can predict survival after birth. Prenatal intervention aims at stimulating lung development, clinically achieved by percutaneous fetal endoscopic tracheal occlusion under local anesthesia. This in utero treatment requires a second intervention to reestablish the airway, either before birth or at delivery. OBJECTIVE: To describe our experience with in utero endotracheal balloon removal. MATERIALS AND METHODS: This is a retrospective analysis of prospectively collected data on consecutive patients with congenital diaphragmatic hernia treated in utero by fetal endoscopic tracheal occlusion from 3 centers. Maternal and pregnancy-associated variables were retrieved. Balloon removal attempts were categorized as elective or emergency and by technique (in utero: ultrasound-guided puncture; fetoscopy; ex utero: on placental circulation or postnatal tracheoscopy). RESULTS: We performed 351 balloon insertions during a 144-month period. In 9 cases removal was attempted outside fetal endoscopic tracheal occlusion centers, 3 of which were deemed impossible and led to neonatal death. We attempted 302 in-house balloon removals in 292 fetuses (217 elective [71.8%], 85 emergency [28.2%]) at 33.4 ± 0.1 weeks (range: 28.9-37.1), with a mean interval to delivery of 16.6 ± 0.8 days (0-85). Primary attempt was by fetoscopy in 196 (67.1%), by ultrasound-guided puncture in 62 (21.2%), by tracheoscopy on placental circulation in 30 (10.3%), and postnatal tracheoscopy in 4 cases (1.4%); a second attempt was required in 10 (3.4%) cases. Each center had different preferences for primary technique selection. In elective removals, we found no differences in the interval to delivery between fetoscopic and ultrasound-guided puncture removals. Difficulties during fetoscopic removal led to the development of a stylet to puncture the balloon, leading to shorter operating time and easier reestablishment of airways. CONCLUSION: In these fetal treatment centers, the balloon could always be removed successfully. In 90% this was in utero, with the use of fetoscopy preferred over ultrasound-guided puncture. Ex utero removal was a fall-back procedure. In utero removal does not seem to precipitate immediate membrane rupture, labor, or delivery, although the design of the study did not allow for a formal conclusion. For fetoscopic removals, the introduction of a stylet facilitated retrieval. Successful removal may rely on a permanently prepared team with expertise in all possible techniques.

Fetal Myelomeningocele Repair through a Mini-Hysterotomy.

OBJECTIVE: To present the feasibility of fetal myelomeningocele (MMC) repair through a mini-hysterotomy and to describe the perinatal results from our initial experience. METHODS: A descriptive study of cases of fetal MMC correction via mini-hysterotomy performed between 2014 and 2016. RESULTS: Forty-five women underwent fetal surgery and 87% (39/45) delivered. A complete multilayer correction of the MMC was possible in all cases. There were no maternal, fetal or neonatal deaths. No maternal or fetal complications occurred from fetal MMC correction until maternal hospital discharge. The average gestational age (GA) at surgery was 24.5 weeks (standard deviation, SD: 1.7; range: 20.7-26.9). The median hysterotomy length was 3.05 cm (SD: 0.39; range: 2.50-3.50). One patient (1/39; 2.6%) experienced chorioamniotic separation. Nine patients (9/39; 23.1%) had premature preterm rupture of membranes at a median GA of 34.1 weeks (range: 31.1-36.0). The average GA at delivery was 35.3 weeks (SD: 2.2; range: 27.9-39.1). Ninety-five percent (37/39) of our patients had an intact hysterotomy site at delivery. Ventriculoperitoneal shunt placement was necessary for 7.7% (3/39) of the neonates. CONCLUSION: Fetal MMC repair is feasible through a mini-hysterotomy. This approach appears to be associated with reduced risks of very preterm delivery and maternal, fetal and neonatal complications.

Twin-twin transfusion syndrome: neurodevelopment of infants treated with laser surgery.

OBJECTIVE: To assess the neurodevelopmental functions of survivors of twin-twin transfusion syndrome (TTTS) treated by fetoscopic laser coagulation (FLC), during the first year of life, comparing them to a control group; and to verify the influence of specific variables on neurodevelopment. METHOD: This was a prospective, longitudinal study. The sample comprised 33 monochorionic diamniotic twins who underwent FLC for treatment of TTTS and 22 full-term infants of single-fetus pregnancies. Bayley Scales of Infant and Toddler Development Screening Test were used for evaluation. Prenatal, perinatal and postnatal information were obtained. RESULTS: There was an increased frequency of infants in the TTTS group with inadequate performance compared to the control group. The identified variables (fetal donor, low economic income and cardiorespiratory disease) negatively impacted expressive communication and fine motor skills. CONCLUSION: Although through follow-up is recommended in all TTTS survivors, particular attention is required for the high-risk group as defined in this study.

Twin-twin transfusion syndrome: neurodevelopment of infants treated with laser surgery / Síndrome de transfusão feto-fetal: neurodesenvolvimento de lactentes tratados com cirurgia a laser

ABSTRACT Objective To assess the neurodevelopmental functions of survivors of twin-twin transfusion syndrome (TTTS) treated by fetoscopic laser coagulation (FLC), during the first year of life, comparing them to a control group; and to verify the influence of specific variables on neurodevelopment. Method This was a prospective, longitudinal study. The sample comprised 33 monochorionic diamniotic twins who underwent FLC for treatment of TTTS and 22 full-term infants of single-fetus pregnancies. Bayley Scales of Infant and Toddler Development Screening Test were used for evaluation. Prenatal, perinatal and postnatal information were obtained. Results There was an increased frequency of infants in the TTTS group with inadequate performance compared to the control group. The identified variables (fetal donor, low economic income and cardiorespiratory disease) negatively impacted expressive communication and fine motor skills. Conclusion Although through follow-up is recommended in all TTTS survivors, particular attention is required for the high-risk group as defined in this study.

RESUMO Objetivo Avaliar o desenvolvimento neurológico de sobreviventes da sindrome de transfusão feto-fetal (STFF) submetidos à coagulação a laser por fetoscopia (CLF), durante o primeiro ano de vida, comparando estes ao grupo controle; e verificar a influência de variáveis específicas no desenvolvimento. Método Tratou-se de estudo prospectivo, longitudinal. A amostra foi composta por 33 gêmeos diamnióticos monocoriônicos submetidos à CLF para tratamento da STFF e 22 lactentes a termo de gestação única. Bayley Scales of Infant and Toddler Development Screening Test foram utilizadas para avaliação. Informações pré-natal, perinatal e pós-natal foram coletadas. Resultados Houve maior número de lactentes com desempenho inadequado no grupo STFF do que no controle. As variáveis identificadas (feto doador, baixa renda econômica e doença cárdio-respiratória) influenciaram negativamente a comunicação expressiva e as habilidades motoras finas. Conclusão Embora o acompanhamento seja recomendado para todos lactentes com STFF, especial atenção deve ser dada àqueles que apresentam fatores de risco.

Twin-twin transfusion syndrome: neurodevelopmental screening test.

OBJECTIVE: To assess the neurodevelopmental functions (cognition, language and motor function) of survivors of twin-twin transfusion syndrome (TTTS). METHOD: Observational cross-sectional study of a total of 67 monochorionic diamniotic twins who underwent fetoscopic laser coagulation (FLC) for treatment of TTTS. The study was conducted at the Center for Investigation in Pediatrics (CIPED), Universidade Estadual de Campinas. Ages ranged from one month and four days to two years four months. Bayley Scales of Infant and Toddler Development Screening Test-III, were used for evaluation. RESULTS: Most children reached the competent category and were classified as having appropriate performance. The preterm children scored worse than term infants for gross motor subtest (p = 0.036). CONCLUSION: The majority of children reached the expected development according to their age. Despite the good neurodevelopment, children classified at risk should be monitored for development throughout childhood.

Twin-twin transfusion syndrome: neurodevelopmental screening test / Síndrome de transfusão feto-fetal: teste de triagem do desenvolvimento neurológico

Objective To assess the neurodevelopmental functions (cognition, language and motor function) of survivors of twin-twin transfusion syndrome (TTTS). Method Observational cross-sectional study of a total of 67 monochorionic diamniotic twins who underwent fetoscopic laser coagulation (FLC) for treatment of TTTS. The study was conducted at the Center for Investigation in Pediatrics (CIPED), Universidade Estadual de Campinas. Ages ranged from one month and four days to two years four months. Bayley Scales of Infant and Toddler Development Screening Test-III, were used for evaluation. Results Most children reached the competent category and were classified as having appropriate performance. The preterm children scored worse than term infants for gross motor subtest (p = 0.036). Conclusion The majority of children reached the expected development according to their age. Despite the good neurodevelopment, children classified at risk should be monitored for development throughout childhood. .

Objetivo Avaliar as funções do desenvolvimento neurológico (cognição, linguagem e motricidade) de sobreviventes da síndrome de transfusão feto-fetal (STFF). Método Estudo transversal observacional, total de 67 gêmeos diamnióticos monocoriônicos submetidos à coagulação a laser por fetoscopia para o tratamento da STFF. O estudo foi realizado no Centro de Investigação em Pediatria (CIPED), Universidade Estadual de Campinas. A idade variou entre um mês e quatro dias a dois anos e quatro meses. Foram utilizadas as Bayley Scales of Infant and Toddler Development Screening Test-III para avaliação. Resultados A maioria das crianças atingiu a categoria competente e foi classificada como tendo desempenho adequado. As crianças prematuras obtiveram desempenho inferior quando comparadas às nascidas a termo no subteste motor global (p = 0,036). Conclusão A maioria das crianças alcançou o desenvolvimento esperado de acordo para idade. Apesar do bom desenvolvimento neurológico, crianças classificadas na categoria de risco devem ser monitoradas para o desenvolvimento, durante a infância. .

Feasibility and reproducibility of diffusion-weighted magnetic resonance imaging of the fetal brain in twin-twin transfusion syndrome.

OBJECTIVE: The aim of this study is to test the feasibility and reproducibility of diffusion-weighted magnetic resonance imaging (DW-MRI) evaluations of the fetal brains in cases of twin-twin transfusion syndrome (TTTS). METHOD: From May 2011 to June 2012, 24 patients with severe TTTS underwent MRI scans for evaluation of the fetal brains. Datasets were analyzed offline on axial DW images and apparent diffusion coefficient (ADC) maps by two radiologists. The subjective evaluation was described as the absence or presence of water diffusion restriction. The objective evaluation was performed by the placement of 20-mm(2) circular regions of interest on the DW image and ADC maps. Subjective interobserver agreement was assessed by the kappa correlation coefficient. Objective intraobserver and interobserver agreements were assessed by proportionate Bland-Altman tests. RESULTS: Seventy-four DW-MRI scans were performed. Sixty of them (81.1%) were considered to be of good quality. Agreement between the radiologists was 100% for the absence or presence of diffusion restriction of water. For both intraobserver and interobserver agreement of ADC measurements, proportionate Bland-Altman tests showed average percentage differences of less than 1.5% and 95% CI of less than 18% for all sites evaluated. CONCLUSION: Our data demonstrate that DW-MRI evaluation of the fetal brain in TTTS is feasible and reproducible.

Fetal interventions for congenital heart disease in Brazil.

Fetal interventions have been performed for some congenital heart diseases. However, these procedures have not gained wide acceptance due to concerns about their efficacy and safety. The aim of this study was to report on a preliminary experience with fetal cardiac interventions in Brazil. Twenty-two cardiac interventions were performed in 21 fetuses. Thirteen fetuses had critical aortic stenosis (CAS), 4 had hypoplastic left heart syndrome (HLHS) and intact interatrial septum or small patent foramen ovale, 1 had pulmonary atresia with intact ventricular septum (IVS), and 3 had critical pulmonary stenosis (CPS). The main outcome variables evaluated were technical success and procedural complications as well as pregnancy and postnatal outcomes. Success was achieved in 20 of 22 procedures (91%) with 1 failed aortic and 1 failed pulmonary valvuloplasties. There was 1 fetal death. No maternal complications occurred. One patient with CAS, severe mitral regurgitation, and hydrops died postnatally within 5 months of age. All patients with HLHS and restrictive atrial septum died after interventional or surgical procedures and prolonged hospitalizations. All patients with CPS/IVS survived and achieved a biventricular (BV) circulation after neonatal valvuloplasty and ductal stenting. A BV circulation was achieved in 4 of 8 patients with CAS and evolving HLHS (one still in utero), including 2 with initial borderline left ventricles (LV) in whom surgical LV overhaul was performed at 9 months of age. In this preliminary experience, the feasibility of fetal cardiac interventions and their outcomes were similar to those previously reported.