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Introduction: Suture tensile properties have only been tested in extrauterine environments. Amniotic fluid (AF) is a complex milieu of enzymes and inflammatory factors. This study tested the mechanical properties of sutures with a variety of inherent properties, after exposure to AF from patients with conditions prompting fetal intervention. Methods: AF was obtained from 3 patients with twin-twin transfusion syndrome (TTTS), and 3 patients with neural tube defects. Six types of 2-0 sutures were placed on 1.2 N of tension to mimic placement in vivo, and incubated in AF at 37°C (98.6°F). These included ethylene terephthalate (Ethibond), glycomer 631 (V-Loc), poliglecaprone 25 (Monocryl), poly-4-hydroxybutyrate (Monomax), polydioxanone (PDS), and polyglactin 910 (Vicryl). Failure load, stress, strain, and initial modulus were tested after 24 h of incubation and after 4 weeks, and compared with control (unincubated) sutures using t tests, Kruskal-Wallis tests, and stress-strain curves. Results: Poliglecaprone 25 and polyglactin 910 dissolve more quickly in AF compared to outside the uterus, disintegrating at 4 weeks. Ethylene terephthalate and PDS experienced little change across 4 weeks of incubation. Glycomer 631 and poly-4-hydroxybutyrate exhibited interesting behavior in AF: glycomer 631 became more deformable at 24 h but later regained toughness by 4 weeks, while poly-4-hydroxybutyrate became tougher and in some cases stronger with time in AF. As a class, braided sutures act more like rigid materials, and monofilaments act like deformable plastics. Conclusion: These findings along with other suture characteristics such as ease of handling and availability may inform fetal intervention teams as they optimize procedures in a relatively new surgical field. [ABSTRACT FROM AUTHOR]

Timely identification of fetal conditions enables comprehensive evaluation, counseling, postnatal planning, and prenatal treatments. This study assessed the existing evidence on how social determinants of health (SDOH) influence diagnosis timing of fetal conditions appropriate for care in fetal care centers (FCCs). Eligible studies were conducted in the U.S. and published in English after 1999. We employed the Healthy People 2020 SDOH framework to categorize and analyze data from 16 studies, where 86% focused solely on congenital heart disease (CHD). Studies primarily focused on individual-level SDOH, with only 36% addressing structural-level factors. A total of 31 distinct indicators of SDOH were identified, with 68% being unique to individual studies. Indicators often varied in definition and specificity. Three studies covered all five SDOH categories in the Healthy People 2020 Framework. Studies revealed varying and often conflicting associations with SDOH indicators, with race and ethnicity being the most explored (100%), followed by socioeconomic status (69%), maternal age (57%), residence (43%), and structural factors (29%). Our findings highlight the need for more comprehensive research, including conditions beyond CHD, and the establishment of consensus on indicators of SDOH. Such efforts are necessary to gain a deeper understanding of the underlying factors driving disparities in fetal diagnosis and treatment. [ABSTRACT FROM AUTHOR]

With the improvement in the detection of congenital heart disease in fetal life, fetal cardiac interventions are pushing the envelope in hopes of either altering the natural history of disease or improving survival in certain high-risk lesions. These interventions include fetal aortic valvuloplasty for evolving hypoplastic left heart syndrome, fetal atrial septoplasty with or without atrial septal stenting for hypoplastic left heart syndrome and variants with intact or severely restrictive atrial septum, and fetal pulmonary valvuloplasty for severe pulmonary stenosis or pulmonary atresia with intact ventricular septum. This review discusses their indications, technical aspects, and outcomes based on available literature. [ABSTRACT FROM AUTHOR]

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Background: This study aims to evaluate the outcomes of fetal sacrococcygeal teratoma (SCT) submitted to prenatal interventions. Methods: We performed a systematic literature review of fetal SCT patients and compared the outcomes between open fetal surgery and percutaneous intervention. In addition, we also compared the results of SCT fetuses who did not undergo any surgical intervention (NI). Results: We identified 16 cases of open fetal surgery (OS), 48 cases of percutaneous fetal intervention (PI), and 93 NI patients. The survival rate was 56.2% in OS, 45.8% in PI (p = 0.568), and 71.0% in NI patients. The gestational age at delivery was earlier in cases where there was no survival compared to cases where the fetuses did survive across all evaluated cohorts (OS: p = 0.033, PI: p < 0.001, NI: p < 0.001). The gestational weeks at delivery in OS and PI fetuses were more similar; however, OS tended to be performed later on in pregnancy, and the affected fetuses had more severe presented findings. In our evaluation, we determined that the presence of fetal hydrops and cardiac failure had no significant impact on survival in SCT cases. In NI patients, polyhydramnios was much higher in fetuses who did not survive compared to their surviving cohorts (p < 0.001). Conclusions: In conclusion, gestational age at delivery can affect the short-term prognosis of fetuses affected with sacrococcygeal teratomas. Regardless of the mode of delivery or the necessity for intervention during the fetal period, monitoring for complications, including polyhydramnios, can prevent premature delivery. [ABSTRACT FROM AUTHOR]

Congenital diaphragmatic hernia (CDH) is a complex pathology with severe pulmonary morbidity. Administration of surfactant in CDH is controversial, and the advent of fetoscopic endoluminal tracheal occlusion (FETO) has added further complexity. While FETO has been shown to improve survival outcomes, there are risks of prematurity and potential surfactant deficiency. We aim to evaluate the characteristics and outcomes of surfactant administration for CDH infants and elucidate potential benefits or risks in this unique population. A single-center retrospective cohort review of patients with unilateral CDH from September 2015 to July 2022 was performed. Demographics, prognostic perinatal imaging features, and outcomes were collected. Patients were stratified by surfactant administration and history of FETO. Data were analyzed with descriptive statistics, two-sample t-tests, chi-squared analyses, and logistic regression. Of 105 included patients, 19 (18%) underwent FETO and 25 (24%) received surfactant. Overall, surfactant recipients were born at earlier gestational ages and lower birthweights regardless of FETO history. Surfactant recipients possessed significantly worse prenatal prognostic features such as observed to expected total fetal lung volume, observed to expected lung to head ratio, and percent liver herniation. In CDH patients without FETO history, surfactant recipients demonstrated worse outcomes than nonrecipients. This association is notably absent in the FETO population, where surfactant recipients have more favorable survival and comparable outcomes. When controlling for defect severity or surfactant usage, as a proxy for respiratory status, surfactant recipients that underwent FETO trended toward improved survival and decreased ECMO use. Surfactant administration is not associated with increased morbidity and mortality and may be beneficial in CDH patients that have undergone FETO. • Surfactant administration is not associated with increased morbidity and mortality and may provide benefit to CDH patients that have undergone fetoscopic endoluminal tracheal occlusion. [ABSTRACT FROM AUTHOR]

Objectives: Non‐immune hydrops fetalis (NIHF) is the pathological accumulation of fluids in fetal compartments, without maternal isoimmunization. Fetal interventions (e.g. shunting, fetal paracentesis, fetal thoracocentesis, fetal pleurodesis) are used to alleviate fluid accumulations, but the outcome is uncertain because the underlying causes of NIHF vary. We aimed to explore the etiology and long‐term outcome of NIHF after fetal intervention. Methods: This was a retrospective review of fetuses with NIHF, defined by the presence of fetal ascites, pleural or pericardial effusion, skin edema or cystic hygroma, or a combination of these features, who underwent intervention at our institution during the period 2012–2021. Clinical surveillance, genetic analysis and viral infection screening were used to define the etiology. Chart reviews and telephone interviews were conducted to assess the long‐term outcomes. Results: In total, 55 fetuses were enrolled and 46 cases had final follow‐up data after delivery. Etiology was identified in 33 cases, including four for which the underlying causes were not identified initially using small‐gene‐panel tests but which were later diagnosed with monogenic disorders by whole‐exome sequencing (WES). Twenty‐three cases with follow‐up survived, having a follow‐up period of 2–11 years at the time of writing, of which 17 were healthy. All 11 cases initially presenting as congenital chylothorax survived with favorable outcome. Conclusions: The etiologies of NIHF are heterogeneous, and the long‐term (spanning 2–11 years) outcome of fetal intervention varies, according to the underlying etiology, with cases caused by congenital chylothorax having the best prognosis. Genome‐wide tests, such as WES, may be helpful in determining the underlying condition in cases caused by a genetic disorder, and this may affect fetal therapy approaches in the future. © 2023 International Society of Ultrasound in Obstetrics and Gynecology. Linked article: There is a comment on this article by Zhao and Li. Click here to view the Correspondence. [ABSTRACT FROM AUTHOR]

Maternal-foetal surgery is an expeditiously evolving specialty and huge progress has been made in it over the past few years. Twin reversed arterial perfusion sequence also known as “acardiac malformation” is a rare complication of twin monochorionic multiple pregnancies. One twin is acardiac and the other phenotypically normal twin drives blood through twin fetal circulations. It is called reversed perfusion because in the acardiac twin, blood flows in a reverse manner. Here, we present a case of minimally invasive fetal intervention that was successfully managed in the radiological suite itself which we believe is the first case to be reported in the literature

Objective: Twin pregnancy is associated with increased perinatal mortality and morbidity, but long‐term neurodevelopmental outcome remains underinvestigated. The primary objective of this study was to investigate the incidence of adverse neurodevelopment after 1 year of age in complicated monochorionic diamniotic (MCDA) twin pregnancies compared with uncomplicated twin pregnancies. Methods: This was a prospective cohort study conducted at St George's University Hospital NHS Foundation Trust, London, UK. Women with a twin pregnancy culminating in at least one surviving child, aged between 12 and 60 months (corrected for prematurity) at the time of assessment, were invited to complete the relevant Ages and Stages Questionnaire® version 3 (ASQ‐3) test. The two study groups were: (1) complicated MCDA twin pregnancies, including those with twin–twin transfusion syndrome, twin anemia–polycythemia sequence, selective fetal growth restriction, twin reversed arterial perfusion sequence and/or single intrauterine demise; and (2) uncomplicated MCDA and dichorionic diamniotic twin pregnancies. The primary outcome measure was an abnormal ASQ‐3 score, defined as a score of more than 2 SD below the mean in any one of the five domains. Mixed‐effects multivariable logistic regression analysis was performed to determine whether a complicated MCDA twin pregnancy was associated independently with an abnormal ASQ‐3 score. Results: The study included 174 parents who completed the questionnaire for one or both twins; therefore, 327 ASQ‐3 questionnaires were available for analysis. Of those, 117 (35.8%) were complicated MCDA twin pregnancies and 210 (64.2%) were controls. The overall rate of an abnormal ASQ‐3 score in children born of a complicated MCDA twin pregnancy was nearly double that of those from uncomplicated twin pregnancies (14.5% vs 7.6%; P = 0.056). Children born of a complicated MCDA twin pregnancy had a significantly higher rate of impairment in the gross‐motor domain compared with the control group (8.5% vs 2.9%; P = 0.031). Complicated MCDA twin pregnancies that underwent prenatal intervention had a significantly higher rate of abnormal ASQ‐3 score compared with those that did not undergo prenatal intervention (28.1% vs 1.7%; P < 0.001). On multilevel logistic regression analysis, complicated MCDA twin pregnancy was an independent predictor of abnormal ASQ‐3 score (adjusted odds ratio, 3.28 (95% CI, 3.27–3.29); P < 0.001). Conclusions: This study demonstrates that survivors of complicated MCDA twin pregnancies have a higher rate of adverse neurodevelopmental outcome, independently of prematurity. Long‐term neurodevelopmental follow‐up in these pregnancies can ensure timely and optimal management of those affected. © 2023 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology. [ABSTRACT FROM AUTHOR]

Congenital lung malformations (CLMs) are readily identified early in pregnancy with a variable natural history. Monitoring for lesion size and mediastinal shift (MS) is recommended following diagnosis. The purpose of this study is to propose a risk-stratified clinical algorithm for prenatal monitoring of CLM. After ethical approval, all fetuses with CLMs evaluated at our fetal center from January 2015 to June 2022 were retrospectively reviewed. Patient demographics, imaging characteristics, and fetal interventions were collected. Lesions were stratified by congenital lung malformation volume ratio (CVR) and the presence of MS. Descriptive statistics and receiver operating characteristic curves were employed in the analysis. We analyzed 111 patients with a mean of 23.4 wk gestational age, a median CVR of 0.5 (interquartile range, 0.3-1.2), and MS in 76 of 111(68%) patients on initial evaluation. Among low-risk patients (CVR ≤1.1), 96% remained low-risk on final evaluation. No patients transitioned from low to high risk during the growth period. Patients with CVR >1.1 often had persistent MS (P < 0.001). Hydrops (5/111, 5%) and fetal intervention (4/111, 4%) only occurred in patients with CVR >1.1 (P < 0.001, P = 0.002) and MS (P = 0.144, P = 0.214). On receiver operating characteristic curve analysis, initial CVR >1.1 had 100% sensitivity and negative predictive value for hydrops and fetal intervention. CLMs with initial CVR ≤1.1 are low risk for hydrops and fetal intervention. We propose a risk-stratified algorithm for the monitoring of CLM during the growth period based on CVR. While our experience suggests that patients with CLM and MS are at higher risk, the current subjective assessment of MS is not adequately predictive. Incorporating an MS grading system may further refine risk stratification in the management of CLM. [ABSTRACT FROM AUTHOR]

Background: Untreated critical pulmonary stenosis may develop into pulmonary atresia with intact ventricular septum, which is associated with a high risk of morbidity and mortality both in fetuses and neonates. In this meta-analysis, we sought to discover the potential of fetal pulmonary valvuloplasty that might affect patients' survival compared to other available procedures. Methods: This systematic review and meta-analysis were conducted based on the PRISMA guideline. The authors thoroughly searched the recognized and potential interventions for PA-IVS, including FPV, total ventricular repair, and palliative procedures. The primary outcome was the mortality rate. We used R software (version 4.1.3) to calculate the overall proportion using the random-effects model of proportional meta-analysis. Results: The FPV procedure was performed at a mean gestational age of 26.28 weeks (95%CI: 24.83–27.73) and was successful in 87.6 % (95 % CI: 78.3–96.3 %) of patients. A total of 52.9 % patients attained biventricular circulation postnatally (95 % CI: 31.2–74.7 %). Successful FPV was associated with a slightly higher overall mortality rate [periprocedural death 4.7 % (95%CI: 0–10.7 %) and postnatal death 8 % (95%CI: 3–13 %)] compared to the three currently available definitive therapies, namely the Fontan procedure [10 % (95%CI: 4–17 %)], 1.5V repair [11 % (95%CI: 5–17 %), and 2V repair [8 % (95%CI: 1–15 %)]. Conclusion: FPV can potentially increase the likelihood of biventricular circulation in fetuses with critical pulmonary valve stenosis.

Maternal-foetal surgery is an expeditiously evolving specialty and huge progress has been made in it over the past few years. Twin reversed arterial perfusion sequence also known as “acardiac malformation” is a rare complication of twin monochorionic multiple pregnancies. One twin is acardiac and the other phenotypically normal twin drives blood through twin fetal circulations. It is called reversed perfusion because in the acardiac twin, blood flows in a reverse manner. Here, we present a case of minimally invasive fetal intervention that was successfully managed in the radiological suite itself which we believe is the first case to be reported in the literature. [ABSTRACT FROM AUTHOR]

Timely identification of fetal conditions enables comprehensive evaluation, counseling, postnatal planning, and prenatal treatments. This study assessed the existing evidence on how social determinants of health (SDOH) influence diagnosis timing of fetal conditions appropriate for care in fetal care centers (FCCs). Eligible studies were conducted in the U.S. and published in English after 1999. We employed the Healthy People 2020 SDOH framework to categorize and analyze data from 16 studies, where 86% focused solely on congenital heart disease (CHD). Studies primarily focused on individual-level SDOH, with only 36% addressing structural-level factors. A total of 31 distinct indicators of SDOH were identified, with 68% being unique to individual studies. Indicators often varied in definition and specificity. Three studies covered all five SDOH categories in the Healthy People 2020 Framework. Studies revealed varying and often conflicting associations with SDOH indicators, with race and ethnicity being the most explored (100%), followed by socioeconomic status (69%), maternal age (57%), residence (43%), and structural factors (29%). Our findings highlight the need for more comprehensive research, including conditions beyond CHD, and the establishment of consensus on indicators of SDOH. Such efforts are necessary to gain a deeper understanding of the underlying factors driving disparities in fetal diagnosis and treatment.

Background: The purpose of this report is to describe the seminal case of a near-term human fetus with a life-threatening left diaphragmatic hernia that underwent fetoscopic tracheal occlusion (FETO) combined with fetoscopic partial removal of herniated bowel from the fetal chest by fetoscopic laparoschisis (FETO-LAP). Case summary: A life-threatening left diaphragmatic hernia (liver-up; o/e LHR of ≤25%; MRI lung volume ≤ 20%) was observed in a human fetus at 34 weeks of gestation. After counselling the mother about the high risks of postnatal demise if left untreated, the expected limitations of fetoscopic tracheal occlusion (FETO), and the previously untested option of combining FETO with fetoscopic laparoschisis, i.e., partial removal of the herniated bowel from the fetal chest (FETO-LAP), she consented to the latter novel treatment approach. FETO-LAP was performed at 36 + 5 weeks of gestation under general maternofetal anesthesia. Mother and fetus tolerated the procedure well. The neonate was delivered and the balloon removed on placental support at 37 + 2 weeks of gestation. On ECMO, a rapid increase in tidal volume was seen over the next eight days. Unfortunately, after this period, blood clots obstructed the ECMO circuit and the neonate passed away. Discussion: This seminal case shows that in a fetus with severe left diaphragmatic hernia, partial removal of the herniated organs from the fetal chest is not only possible by minimally invasive fetoscopic techniques but also well tolerated. As the effect of FETO alone is limited in saving severely affected fetuses, combining FETO with fetoscopic laparoschisis (FETO-LAP) offers a new therapeutic route with multiple, potentially life-saving implications. [ABSTRACT FROM AUTHOR]

Objectives: To evaluate the outcome of monochorionic diamniotic (MCDA) twins complicated by Type‐II selective fetal growth restriction (sFGR) who underwent fetoscopic laser photocoagulation and to validate a previously proposed subclassification system for Type‐II sFGR in a large multicenter cohort. Methods: This retrospective multicenter cohort study included all MCDA twins complicated by Type‐II sFGR who underwent laser photocoagulation of placental anastomoses at four large tertiary fetal‐care centers between 2006 and 2020. Cases were subclassified according to a recently proposed system based on Doppler evaluation of the ductus venosus (DV) and middle cerebral artery (MCA) into Type‐IIA (normal DV flow and MCA peak systolic velocity (PSV) of the growth‐restricted fetus) or Type‐IIB (absent or reversed flow in the DV during atrial contraction and/or MCA‐PSV ≥ 1.5 multiples of the median of the growth‐restricted fetus). Demographic characteristics and pregnancy outcomes were compared between the groups. Data are presented as mean ± SD or n (%) as appropriate. P‐values < 0.05 were considered statistically significant. Results: A total of 98 patients with MCDA twins met our inclusion criteria, with 56 subclassified as Type IIA and 42 as Type IIB. Demographic characteristics were similar between the groups; however, Type‐IIB cases had a significantly earlier gestational age at diagnosis and at laser surgery, as well as larger intertwin estimated fetal weight discordance, which may be a reflection of disease severity. Postnatal survival of the growth‐restricted fetus to 30 days of age was significantly lower in Type‐IIB compared to Type‐IIA cases (23.8% vs 46.4%; P = 0.034). Conclusions: In MCDA twins complicated by Type‐II sFGR and treated with laser photocoagulation of placental anastomoses, preoperative Doppler assessment of the DV and MCA helped identify a subset of patients at increased risk of demise of the growth‐restricted fetus following intervention. This study provides valuable information for guiding surgical management and patient counseling. © 2023 International Society of Ultrasound in Obstetrics and Gynecology. [ABSTRACT FROM AUTHOR]

Critical aortic stenosis that appears in mid-gestation tends to develop to growth retardation of left ventricle, known as hypoplastic left heart syndrome (HLHS). Despite better clinical management of HLHS, the morbidity and mortality rates of univentricular circulation patients remain high. In this paper, we sought to perform a systematic review and meta-analysis to know the outcomes of fetal aortic valvuloplasty in critical aortic stenosis patients. This systematic review and meta-analysis was conducted according to the Preferred Reporting Items for Systematic Review and Meta-Analyses (PRISMA) statement. A systematic search on fetal aortic valvuloplasty procedure for critical aortic stenosis was performed through PubMed, Scopus, EBSCOhost, ProQuest, and Google Scholar. The primary endpoint of each group was overall mortality. We used R software (version 4.1.3) to estimate the overall proportion of each outcome using random-effects model of proportional meta-analysis. A total of 389 fetal subjects from 10 cohort studies were included in this systematic review and meta-analysis. Fetal aortic valvuloplasty (FAV) was successfully performed in 84% of patients. It revealed a successful conversion to biventricular circulation rate of 33% with a mortality rate of 20%. Bradycardia and pleural effusion requiring treatment were two most common fetal complications, whereas maternal complication reported was only placental abruption in one patient. FAV has a high technical success rate with the ability to achieve biventricular circulation and a low rate of procedure-related mortality if carried out by experienced operators. • Patients with univentricular circulation continue to have high morbidity and mortality rates. • FAV revealed a success conversion to BV circulation rate of 33% with a mortality rate of 20%. [ABSTRACT FROM AUTHOR]

Advancements in maternal–fetal interventions have allowed for direct fetal access, shifting the focus of interventions from maternal health for fetal health to a focus on sole fetal/neonatal benefit. Given that access to the fetus can only be obtained through the mother, there are ethical considerations important to consider when counseling the maternal–fetal dyad. The goals of maternal–fetal interventions range from improved fetal/neonatal survival to decreased long-term morbidities and improved quality of life. However, interventions to improve quality of life may not always achieve their desired result. Additionally, maternal–fetal interventions have risks such as premature birth and other complications that should be heavily considered as they may offset the potential benefits of the procedure. While some families elect for a maternal–fetal intervention, doing every potential postnatal intervention may not be in alignment with their goals depending on the outcome of the intervention. Given the complex, value-laden decision-making that is crucial to counseling parents about decisions surrounding maternal–fetal interventions and subsequent neonatal care, palliative care specialists should be utilized in fetal centers. Palliative care specialists are trained to assist with complex, goal concordant decision-making and can guide families and medical teams through the decision points that arise during the treatment journey.

Introduction: In spina bifida aperta (SBA), fetal closure of the myelomeningocele (MMC) can have a neuroprotective effect and improve outcomes. In Europe, surgical MMC closure is offered by fetal-open (OSBAR), fetal-endoscopic (FSBAR), and neonatal (NSBAR) surgical techniques. Pediatric neurologists facing the challenging task of counseling the parents may therefore seek objective outcome comparisons. Until now, such data are hardly available. In SBA, we aimed to compare neurologic outcomes between OSBAR, FSBAR, and NSBAR intervention techniques. Methods: We determined intervention-related complications, neuromuscular integrity, and neurologic outcome parameters after OSBAR (n = 17) and FSBAR (n = 13) interventions by age- and lesion-matched comparisons with NSBAR-controls. Neurological outcome parameters concerned: shunt dependency, segmental alterations in muscle ultrasound density (reflecting neuromuscular integrity), segmental motor-, sensory- and reflex conditions, and the likelihood of intervention-related gain in ambulation. Results: Compared with NSBAR-controls, fetal intervention is associated with improved neuromuscular tissue integrity, segmental neurological outcomes, reduced shunt dependency, and a higher chance of acquiring ambulation in ≈20% of the operated children. Children with MMC-lesions with a cranial border at L3 revealed the most likely intervention-related motor function gain. The outcome comparison between OSBAR versus FSBAR interventions revealed no significant differences. Conclusion: In SBA, OSBAR- and FSBAR-techniques achieved similar neuroprotective results. A randomized controlled trial is helpful in revealing and compare ongoing effects by surgical learning curves. [ABSTRACT FROM AUTHOR]

Copyright of Monatsschrift Kinderheilkunde is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Fetal surgery has become a lifesaving reality for hundreds of fetuses each year. The development of a formidable spectrum of safe and effective minimally invasive techniques for fetal interventions since the early 1990s until today has led to an increasing acceptance of novel procedures by both patients and health care providers. From his vast personal experience of more than 20 years as one of the pioneers at the forefront of clinical minimally invasive fetal surgery, the author describes and comments on old and new minimally invasive approaches, highlighting their lifesaving or quality-of-life-improving potential. He provides easy-to-use practical information on how to perform partial amniotic carbon dioxide insufflation (PACI), how to assess lung function in fetuses with pulmonary hypoplasia, how to deal with giant CPAMS, how to insert shunts into fetuses with LUTO and hydrothorax when conventional devices are not available, and how to resuscitate a fetus during fetal cardiac intervention. Furthermore, the author proposes a curriculum for future fetal surgeons, solicits for the centralization of patients, for adequate maternal counseling, for adequate pain management and adequate hygienic conditions during interventions, and last but not least for starting the process of academic recognition of the matured field as an independent specialty. These steps will allow more affected expectant women and their unborn children to gain access to modern minimally invasive fetal surgery and therapy. The opportunity to treat more patients at dedicated centers will also result in more opportunities for the research of rare diseases and conditions, promising even better pre- and postnatal care in the future. [ABSTRACT FROM AUTHOR]

Background: The purpose of this report is to describe the seminal case of a near-term human fetus with a life-threatening left diaphragmatic hernia that underwent fetoscopic tracheal occlusion (FETO) combined with fetoscopic partial removal of herniated bowel from the fetal chest by fetoscopic laparoschisis (FETO-LAP). Case summary: A life-threatening left diaphragmatic hernia (liver-up; o/e LHR of ≤25%; MRI lung volume ≤ 20%) was observed in a human fetus at 34 weeks of gestation. After counselling the mother about the high risks of postnatal demise if left untreated, the expected limitations of fetoscopic tracheal occlusion (FETO), and the previously untested option of combining FETO with fetoscopic laparoschisis, i.e., partial removal of the herniated bowel from the fetal chest (FETO-LAP), she consented to the latter novel treatment approach. FETO-LAP was performed at 36 + 5 weeks of gestation under general maternofetal anesthesia. Mother and fetus tolerated the procedure well. The neonate was delivered and the balloon removed on placental support at 37 + 2 weeks of gestation. On ECMO, a rapid increase in tidal volume was seen over the next eight days. Unfortunately, after this period, blood clots obstructed the ECMO circuit and the neonate passed away. Discussion: This seminal case shows that in a fetus with severe left diaphragmatic hernia, partial removal of the herniated organs from the fetal chest is not only possible by minimally invasive fetoscopic techniques but also well tolerated. As the effect of FETO alone is limited in saving severely affected fetuses, combining FETO with fetoscopic laparoschisis (FETO-LAP) offers a new therapeutic route with multiple, potentially life-saving implications.

Congenital high airway obstruction syndrome (CHAOS) is a rare condition that can be diagnosed antenatally by ultrasound. It is usually lethal without immediate intervention at delivery. A 24-year-old woman was diagnosed with fetal CHAOS at 27 weeks’ gestation. The couple declined termination of pregnancy. A multidisciplinary team including obstetricians, geneticists, paediatric surgeons, neonatologists and anaesthetists was constituted to plan an ex utero intrapartum treatment (EXIT) procedure. After several simulations, a caesarean section was performed at 38 weeks’ gestation under deep inhalational anaesthesia. The fetus was fully delivered with placenta remaining in utero to maintain perfusion. A surgical airway was established via tracheostomy in approximately 5 minutes. The operation was then completed with no maternal complications. The child remains well at 3 years of age. To our knowledge, this is the first EXIT procedure performed for CHAOS in the public sector. This procedure can be lifesaving and is possible with proper planning.

Purpose: A growing number of fetal procedures are performed at specialized fetal care centers for congenital problems that classically would have poor outcomes despite advanced postnatal management. Consistent fetal monitoring is integral to the safety of these challenging and innovative surgeries. However, standardization of fetal monitoring during various forms of fetal surgery has yet to be established.Materials and Methods: We searched all articles on literature platforms until August 2019 using the terms "fetal surgery," "fetal monitoring," and "fetal interventions." Titles and abstracts were screened by our coauthors to determine the type of fetal monitoring used in these cases.Results: The search identified 1,625 citations, of which the 50 citations considered most pertinent were included in this review.Conclusions: Fetal monitoring during in utero fetal surgeries continues to be challenging because of limited physical fetal access and technological aspects. Innovations in fetal cardiac monitoring during fetal surgeries have the potential for continuous and high-fidelity hemodynamic and physiologic monitoring, with the goal of early detection and treatment of fetal compromise. [ABSTRACT FROM AUTHOR]

The purpose of this retrospective cohort study was to compare the outcome of human fetuses with isolated severe lower urinary tract obstructions (LUTO) that were first treated before the completion of 16 weeks of gestation to fetuses first treated later in gestation. Vesicoamniotic shunt insertion (VAS) was performed in 63 subsequent fetuses with LUTO between 12 + 5 and 30 + 3 weeks. The fetuses were analyzed in three groups: Group-I-fetuses underwent their first intervention until the completion of 16 weeks, Group–II–fetuses were first treated between 16 + 1 and 24 + 0 weeks and Group–III–fetuses beyond 24 + 1 weeks. Renal and pulmonary outcome parameters and complicating factors were assessed. – All mothers tolerated the procedures well. Overall fetal survival was 47 of 63 (75%). The mean age at delivery of survivors was 35 weeks. 68% of Group-I-fetuses, 77% of group–II–fetuses, and 100% of group–III–fetuses survived beyond postnatal hospital discharge. Amongst the survivors the chance for normal renal function was higher for group I with 79% (15/19) compared to first fetal intervention after the completion of 16 weeks with 32% (9/28, p = 0.003, OR = 7.9 [2.0, 30.8] 95% CI). Clinically relevant pulmonary hypoplasia was observed in 11% of Group-I-, 27% of Group–II–, and 20% of Group–III–fetuses. Early intervention in fetal LUTO before the completion of 16 weeks may achieve a higher rate of normal renal and pulmonary function in survivors than treatment beyond that point in time. This observation is important for the future management of this challenging patient population. [Display omitted] [ABSTRACT FROM AUTHOR]

Objectives: To investigate the survival rate of hydrops fetalis after fetal interventions and neonatal intensive care. Methods: We reviewed the medical records of patients diagnosed with hydrops fetalis from January 2009 to December 2019 at Changhua Christian Children's Hospital. All cases had abnormal fluid accumulation in at least two body compartments during pre- and postnatal examination. The primary outcome measure was the mortality rate. We also collected information regarding disease etiology, duration of hospital stay, Apgar score, gestational age at birth, initial hydrops fetalis diagnosis, fetal intervention, first albumin and pH levels, and maternal history. Results: Of the 42 cases enrolled, 30 survived and 12 died; the mortality rate was 28.6%. Furthermore, 22 cases received fetal intervention, while 20 cases did not; there was no significant difference in their survival rates (75% and 68%, respectively). Survival rate was associated with gestational age at birth, initial diagnosis time, birthweight, Apgar score, initial albumin and pH levels, and gestational hypertension. Only one case was immune-mediated. Among the nonimmune-mediated cases, the three most common etiologies were lymphatic dysplasia (12/42), idiopathic disorders (10/42), and cardiovascular disorders (5/42). Conclusions: Overall, hydrops fetalis was diagnosed early, and fetal intervention was performed in a timely manner. Preterm births were more frequent, and birthweight was lower in the cases that underwent fetal intervention than in those that did not, but there was no significant between-group difference in mortality. The initial diagnosis time, gestational age at birth, birthweight, Apgar score, and first albumin and pH levels were independently associated with mortality. [ABSTRACT FROM AUTHOR]

Background: Triploidy is commonly associated with the development of early-onset preeclampsia. While previable preeclampsia is often a contraindication to prolonging pregnancy, there may be rare circumstances in which an alternative approach may be offered. Case: A nulliparous patient with a dichorionic twin gestation, recently diagnosed triploidy in one twin, and history of chronic hypertension presented at 18 weeks of gestation with signs and symptoms suggestive of preeclampsia. After symptomatic therapy and laboratory evaluations, selective fetal termination of the affected twin was elected and performed without complications. The patient subsequently delivered a healthy newborn at 37 weeks of gestation. Conclusion: Selective fetal termination may be considered a management option for previable preeclampsia in a dichorionic gestation with triploid fetus and was associated with a favorable outcome in this case.

Introduction: In spina bifida aperta (SBA), fetal closure of the myelomeningocele (MMC) can have a neuroprotective effect and improve outcomes. In Europe, surgical MMC closure is offered by fetal-open (OSBAR), fetal-endoscopic (FSBAR), and neonatal (NSBAR) surgical techniques. Pediatric neurologists facing the challenging task of counseling the parents may therefore seek objective outcome comparisons. Until now, such data are hardly available. In SBA, we aimed to compare neurologic outcomes between OSBAR, FSBAR, and NSBAR intervention techniques. Methods: We determined intervention-related complications, neuromuscular integrity, and neurologic outcome parameters after OSBAR (n = 17) and FSBAR (n = 13) interventions by age- and lesion-matched comparisons with NSBAR-controls. Neurological outcome parameters concerned: shunt dependency, segmental alterations in muscle ultrasound density (reflecting neuromuscular integrity), segmental motor-, sensory- and reflex conditions, and the likelihood of intervention-related gain in ambulation. Results: Compared with NSBAR-controls, fetal intervention is associated with improved neuromuscular tissue integrity, segmental neurological outcomes, reduced shunt dependency, and a higher chance of acquiring ambulation in ≈20% of the operated children. Children with MMC-lesions with a cranial border at L3 revealed the most likely intervention-related motor function gain. The outcome comparison between OSBAR versus FSBAR interventions revealed no significant differences. Conclusion: In SBA, OSBAR- and FSBAR-techniques achieved similar neuroprotective results. A randomized controlled trial is helpful in revealing and compare ongoing effects by surgical learning curves.

The purpose of this review is to provide an overview of the perinatal outcomes of fetuses who underwent fetal surgery for the management of Amniotic Band Syndrome (ABS). A systematic review of studies reporting on the perinatal outcome of fetuses undergoing fetoscopic release of amniotic bands according to the (PRISMA) guidelines was performed. The MEDLINE, Embase, Scopus, and Cochrane Library databases were systematically searched. In total, 17 studies reporting 37 cases of ABS that underwent amniotic band release by fetoscopy were included. The median gestational age at which fetal surgery was performed was 22 weeks (range 18-29 weeks). PPROM occurred in 51.3%, while fetal survival reached 89.2%. The success of fetal surgery was 75.7% in preserving and maintaining the functionality of the affected limb. Fetoscopic release of amniotic bands can preserve the affected limb and its function in cases of ABS and prevent fetal death in cases of ABS involving the umbilical cord. Further studies are needed to determine the optimal criteria for selecting patients who can benefit from fetal surgery, considering that it is an intervention that is not free of perinatal complications., (© 2024 The Author(s). Prenatal Diagnosis published by John Wiley & Sons Ltd.)

Introduction: Limited data exist regarding outcomes when pre- and periviable PPROM (PPROM ≤26 weeks of gestation) occurs as a complication of fetoscopic laser surgery (FLS) for twin-twin transfusion syndrome (TTTS)., Methods: This is a retrospective cohort study of FLS cases performed at a single institution between January 2015 and May 2021. Study inclusion was limited to patients with monochorionic-diamniotic twin pregnancies complicated by TTTS who underwent FLS. Patients were grouped by pPPROM status, and further stratified to those continuing with expectant management, and outcomes were compared between groups. The primary outcome was survival to live birth of at least one twin., Results: During the study period, 171 patients underwent FLS and a total of 96 (56.1%) subjects satisfied inclusion criteria. Among included subjects, 18 (18.8%) experienced pPPROM after FLS and 78 (81.2%) did not. Baseline characteristics were similar between groups. Among patients with pPPROM, 11 (61.1%) pursued expectant management and 7 (38.9%) opted for pregnancy termination. Among expectantly managed subjects, median pPPROM-to-delivery interval was 47.0 days (6.0-66.0 IQR) with a median gestational age at delivery of 29+1 weeks (24 + 4-33 + 6 IQR). Rates of survival to live birth of at least one twin (90.9% vs. 96.2% p = 0.42) were similar between those with pPPROM undergoing expectant management and those without pPPROM. Dual survivorship (45.5% vs. 78.2%, p = 0.03), perinatal survival to live birth (68.2% vs. 87.2%, p = 0.05), and perinatal survival to newborn hospital discharge (59.1% vs. 85.9%, p = &lt;0.01) were all significantly lower among those with pPPROM. Gestational age at delivery was lower among those continuing with pregnancies complicated by pPPROM (29 + 1 vs. 32+5 weeks, p = &lt;0.01)., Conclusion: Survival of at least one twin to live birth remained high among those pursing expectant management after experiencing post-FLS pPPROM, suggesting that the outlook after this complication is not necessarily poor. However, this complication was associated with lower chances of dual survival and greater prematurity., (© 2024 S. Karger AG, Basel.)

Fetal surgery has become a lifesaving reality for hundreds of fetuses each year. The development of a formidable spectrum of safe and effective minimally invasive techniques for fetal interventions since the early 1990s until today has led to an increasing acceptance of novel procedures by both patients and health care providers. From his vast personal experience of more than 20 years as one of the pioneers at the forefront of clinical minimally invasive fetal surgery, the author describes and comments on old and new minimally invasive approaches, highlighting their lifesaving or quality-of-life-improving potential. He provides easy-to-use practical information on how to perform partial amniotic carbon dioxide insufflation (PACI), how to assess lung function in fetuses with pulmonary hypoplasia, how to deal with giant CPAMS, how to insert shunts into fetuses with LUTO and hydrothorax when conventional devices are not available, and how to resuscitate a fetus during fetal cardiac intervention. Furthermore, the author proposes a curriculum for future fetal surgeons, solicits for the centralization of patients, for adequate maternal counseling, for adequate pain management and adequate hygienic conditions during interventions, and last but not least for starting the process of academic recognition of the matured field as an independent specialty. These steps will allow more affected expectant women and their unborn children to gain access to modern minimally invasive fetal surgery and therapy. The opportunity to treat more patients at dedicated centers will also result in more opportunities for the research of rare diseases and conditions, promising even better pre- and postnatal care in the future.

Objectives: To evaluate a novel scoring system that combines several prenatal parameters for selecting ideal candidates for fetal intervention, and for predicting postnatal survival in patients with severe fetal lower urinary tract obstruction (LUTO).Methods: We retrospectively reviewed all cases of severe LUTO evaluated for fetal intervention in a single large fetal center between January 2013 and December 2017. A scoring system for determining fetal candidacy for intervention was retrospectively developed based on postnatal outcomes. The proposed scoring system included fetal urinary biochemistry, renal ultrasound parameters, initial bladder volume, and degree of bladder refill. Relevant demographic characteristics, ultrasound reports and laboratory results were reviewed. Receiver operating characteristic (ROC) curves were used to select the cut-off values for initial bladder volume and degree of bladder refill and to evaluate the performance of the scoring system in predicting postnatal death.Results: Of the 79 LUTO patients evaluated, 31 were eligible for the study. The overall 6-month postnatal survival was 64.5 % (20/31). A scoring system (0-8) was suggested with 2 points for unfavorable biochemistry, 4 points for ultrasound evidence of dysplastic kidneys, 1 point for inadequate initial bladder volume and 1 point for inadequate bladder refill. Scores>3 (N = 7) were associated with 0 % 6-month survival. The ROC curve for predicting postnatal mortality showed area under curve (AUC) of 0.82 (95 % CI 0.65-0.99). Subgroup analysis within subjects who underwent fetal intervention (N = 22) also confirmed the significance of the distribution of the scoring system between groups who survived and those who did not after adjustment for GA at delivery (p = 0.01).Conclusion: We propose a novel scoring system for antenatal evaluation of patients with severe LUTO which may be useful in selecting those candidates most appropriate for intervention and in counseling parents about predicted postnatal outcome. [ABSTRACT FROM AUTHOR]

Objectives: To investigate the survival rate of hydrops fetalis after fetal interventions and neonatal intensive care. Methods: We reviewed the medical records of patients diagnosed with hydrops fetalis from January 2009 to December 2019 at Changhua Christian Children’s Hospital. All cases had abnormal fluid accumulation in at least two body compartments during pre- and postnatal examination. The primary outcome measure was the mortality rate. We also collected information regarding disease etiology, duration of hospital stay, Apgar score, gestational age at birth, initial hydrops fetalis diagnosis, fetal intervention, first albumin and pH levels, and maternal history. Results: Of the 42 cases enrolled, 30 survived and 12 died; the mortality rate was 28.6%. Furthermore, 22 cases received fetal intervention, while 20 cases did not; there was no significant difference in their survival rates (75% and 68%, respectively). Survival rate was associated with gestational age at birth, initial diagnosis time, birthweight, Apgar score, initial albumin and pH levels, and gestational hypertension. Only one case was immune-mediated. Among the nonimmune-mediated cases, the three most common etiologies were lymphatic dysplasia (12/42), idiopathic disorders (10/42), and cardiovascular disorders (5/42). Conclusions: Overall, hydrops fetalis was diagnosed early, and fetal intervention was performed in a timely manner. Preterm births were more frequent, and birthweight was lower in the cases that underwent fetal intervention than in those that did not, but there was no significant between-group difference in mortality. The initial diagnosis time, gestational age at birth, birthweight, Apgar score, and first albumin and pH levels were independently associated with mortality.

We report a patient who presented during fetal life with severe aortic stenosis, left-ventricular dysfunction, and endocardial fibroelastosis (evolving hypoplastic left heart syndrome). Management involved in utero and postnatal balloon aortic valvuloplasty for partial relief of obstruction and early postnatal hybrid stage I palliation until recovery of left-ventricular systolic function had occurred. The infant subsequently had successful conversion to a biventricular circulation by combining resection of endocardial fibroelastosis with single-stage Ross-Konno, aortic arch reconstruction, hybrid takedown, and pulmonary artery reconstruction.

Thoracoscopic excision of a Type 0 congenital pulmonary airway malformations (CPAM) is rarely described in literature. A 1-day-old female neonate presented with prenatal diagnosis of right lung CPAM. Mother had undergone fetoscopic thoracoamniotic shunt placement which led to radiographic resolution of large CPAM cyst. Soon after delivery, the patient developed multiple premature ventricular contractions (PVCs) that were thought to be secondary to retained shunts. Thoracoscopic excision of CPAM performed.

Introduction: Supraventricular tachycardia is the most common fetal tachyarrhythmia and if persistent often associated with fetal hydrops which can cause intrauterine and neonatal death.Case Presentation: We present a case of early second trimester supraventricular tachycardia in a hydropic fetus, initially refractory to transplacental treatment.Conclusion: The supraventricular tachycardia was successfully treated when supplemented with intraperitoneal flecainide in the fetus. [ABSTRACT FROM AUTHOR]

Purpose of the Review: The authors present an overview of the mostly formative and latest literature on the current fetal management of lower urinary tract obstruction (LUTO) and postnatal renal outcome with a special focus on the bladder. Recent Findings: Since the PLUTO trial [1] in 2013, only a few retrospective studies have been published which compared fetal cystoscopy with the placement of a vesicoamniotic shunt (VAS) and showed that both interventions are superior to conservative management because they improve the 6 months survival rates in severe LUTO. In contrast, only fetal cystoscopy provides a trend of better renal outcome compared with VAS [2••, 3, 4]. The current studies focus mainly on postnatal renal function; data on bladder function are still missing. Summary: The data available today are insufficient on giving adequate answer about bladder outcome following fetal intervention and whether vesicoamniotic shunt (VAS) or fetal cystoscopy is adequate to decompress and protect the bladder. In addition to renal outcome studies, there is need for long-term bladder studies. [ABSTRACT FROM AUTHOR]