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OBJECTIVES Many serious or life-threatening neurologic conditions are first diagnosed during the fetal period, often following a routine ultrasound or sonographic evaluation after an abnormal aneuploidy screen. Such conditions represent a worrisome or unexpected finding for expectant parents, making the perinatal period a critical time point to engage and empower families encountering complex neurologic clinical scenarios. This review covers the role of perinatal palliative care in these settings. STUDY DESIGN Topical review RESULTS:: The prenatal identification of structural abnormalities of the brain or spinal cord, radiographic signs of hemorrhage or ischemic injury, or evidence of genetic or metabolic conditions should prompt involvement of a fetal palliative care team. The inherent prognostic uncertainty is challenging for prenatally diagnosed neurologic conditions which have difficult to predict short and long-term outcomes. While many of these conditions lead to the birth of an infant with neurodevelopmental challenges, few result in in utero demise. Palliative care beginning in the perinatal period provides an additional layer of support for families navigating complex decision-making during their pregnancy and provides continuity of care into the newborn period. Palliative care principles can help guide discussions around genetic and other diagnostic testing, fetal surgery, and birth planning. A multidisciplinary team can help support families with decision-making and through bereavement care in the setting of fetal or neonatal death. CONCLUSION Early palliative care team involvement can provide a more holistic approach to counseling, facilitate planning, and ensure that a family's goals and wishes are acknowledged throughout an infant's care trajectory. KEY POINTS · Many serious or life-threatening neurologic conditions are diagnosed during the fetal period.. · Palliative care principles should be incorporated in the fetal period for affected patients.. · Palliative care clinicians can aid parents and clinicians in shared decision-making.. · Palliative care principles should be employed by all care providers in relevant cases..

Pain is a complex phenomenon that involves more than a simple physical response to external stimuli. In maternal-fetal surgical procedures, fetal analgesia is used primarily to blunt fetal autonomic responses and minimize fetal movement. The purpose of this Consult is to review the literature on what is known about the potential for fetal awareness of pain and to discuss the indications for and the risk-benefit calculus involved in the use of fetal anesthesia and analgesia. The recommendations by the Society for Maternal-Fetal Medicine are as follows: (1) we suggest that fetal paralytic agents be considered in the setting of intrauterine transfusion, if needed, for the purpose of decreasing fetal movement (GRADE 2C); (2) although the fetus is unable to experience pain at the gestational age when procedures are typically performed, we suggest that opioid analgesia should be administered to the fetus during invasive fetal surgical procedures to attenuate acute autonomic responses that may be deleterious, avoid long-term consequences of nociception and physiological stress on the fetus, and decrease fetal movement to enable the safe execution of procedures (GRADE 2C); and (3) due to maternal risk and a lack of evidence supporting benefit to the fetus, we recommend against the administration of fetal analgesia at the time of pregnancy termination (GRADE 1C).

Summary Introduction The purpose of this study was to determine the incidence of lower urinary tract surgery in patients with myelomeningocele (MMC) operated in utero as well as in the subgroup of patients with a minimum age of 5 years. Methods We use a previously published protocol to categorize bladder patterns and propose the treatment: high risk pattern-anticholinergics and clean intermittent catheterization (CIC); incontinent and normal patterns-clinical surveillance and underactivity pattern- CIC. Non-response to clinical treatment with maintenance of detrusor pressure higher than 40 cmH20 with worsening of hydronephrosis or recurrent urinary infection were imperative indications for surgery and urinary incontinence due to sphincter deficiency and leakage pressure below 40 cmH20 in patients aged 5 years and older were possible indications too. We identified the prevalence of urinary tract surgery and concomitant treatment of constipation and refractory fecal incontinence by convenience, the technique performed and surgery outcomes. Results A total of 122 patients are part of the prospective protocol. The first UE was performed in 119 patients (median age of 4 months). The categorization of the bladder pattern was high risk in 52.1%, incontinent in 25.2%, hypocontractile 4.2% and normal in 18.5%. Current follow-up was 29.9 months (1–99 months). Surgery was performed on 10 patients (8.4%): 3 vesicostomies, 2 surgeries to treat vesicoureteral reflux, 6 bladder augmentations, 3 ACE and a sling surgery. Considering only patients older than 5 years, we identified 65 patients and seven surgeries performed (10.8%). Discussion Most bladder reconstruction studies are case series of single institutions or cooperative services, most of which are retrospective. The prospective clinical interpretation and imaging and urodynamic exams over time allowed us prospectively to define the risk of surgery in patients with myelomeningocele operated in utero. It must be considered that the response to the initial urological treatment and even the failure that leads to the need for surgery have to be evaluated in a context of follow-up time. For this reason we have studied separately patients over 5 years-old and also the group considered to be at greatest risk for surgery, the high-risk group of the categorization already described. Conclusion We found an incidence of 10.8% of surgeries in patients over 5 years-old and 12.9% for the high-risk group. This information may be useful to educate parents when stating urological treatment. Download : Download high-res image (644KB) Download : Download full-size image Summary Figure . Continent catheterizable ileal reservoir (Macedo's Technique) - procedure steps.

Objective To evaluate all individual cases of dual twin demise following laser surgery for twin-twin transfusion syndrome (TTTS). Method This is an analysis of all monochorionic diamniotic twin gestations with TTTS complicated by dual demise following laser surgery from 2006 to 2019. Cases were reviewed by (1) a fetal surgeon researcher and (2) a panel of independent experienced maternal-fetal medicine specialists to code an etiology of demise for the donor and recipient, and to assess for possible preventability. Results Of 753 twins that underwent laser surgery for TTTS, 52 (6.9%) had postoperative dual demise. In this subgroup, gestational age at surgery was 19.5 (16.1-24.9) weeks, and 36 (69.2%) patients were Quintero stage III and IV. The most common etiology was the spectrum of disorders leading to preterm delivery, which included cervical insufficiency, preterm premature rupture of membranes, and preterm labor (44.2% and 48.1%, donor and recipient, respectively). Some degree of preventability was estimated for 23.1% of dual demises. Conclusions The most common cause of dual demise post laser surgery for TTTS was preterm birth, reinforcing the need for studies regarding the etiology and prevention of post-fetoscopy prematurity. Nearly one-quarter of dual demise cases were deemed potentially preventable.

As fetal gene therapies move from experimental animal models to human in utero phase I clinical trials, there is a need to consider the ethical, legal, and social implications. While fetal gene therapies are attracting more regulatory oversight than previous fetal interventions such as fetal surgery, old sociological questions should be applied to this new context. As health care pathways around fetal therapy are shaped by the ways in which a pregnant person and the fetus are constituted, and as risks and benefits are evaluated, we cannot afford to lose sight of long-term consequences, especially those pertaining to social inclusion.

Spontaneous third ventriculostomy (STV) is characterized by the spontaneous rupture of one of the ventricle walls due to increased pressure in the third ventricle caused by obstructive hydrocephalus. Clinically, STV results in resolution of signs and symptoms of intracranial hypertension and head circumference stabilization. No spontaneous STV cases in patients with myelomeningocele have been reported in the literature. The objective of this study was to report three cases of STV in patients with type 2 Chiari malformation who underwent intrauterine treatment. All patients presented clinically with increased head circumference during outpatient follow-up. Only one patient required a ventriculoperitoneal shunt implantation. The other patients did not require further intervention. STV is a rare entity that is difficult to diagnose and should always be suspected in spontaneous hydrocephalus resolution, especially in early childhood. STV is not synonymous with hydrocephalus resolution.

OBJECTIVE Fetal repair of an open neural tube defect (ONTD) by open hysterotomy has been shown to reduce the need for ventriculoperitoneal shunting and improve motor outcomes for infants, but increases the risk of Cesarean section and prematurity. Fetoscopic repair is an alternative approach that may confer similar neurological benefits but allows for vaginal delivery and reduces the incidence of hysterotomy-related complications. We sought to compare the costs of care from fetal surgery until neonatal discharge, as well as the clinical outcomes, associated with each surgical approach. METHODS This was a retrospective cohort study of patients who underwent prenatal ONTD repair, using either the open-hysterotomy or the fetoscopic approach, at a single institution between 2012 and 2018. Clinical outcomes were collected by chart review. A cost-consequence analysis was conducted from the hospital perspective, and included all inpatient and ambulatory hospital and physician costs incurred for the care of mothers and their infants, from the time of maternal admission for fetal ONTD repair up to postnatal maternal and infant discharge. Costs were estimated using cost-to-charge ratios for hospital billing and the Medicare physician fee schedule for physician billing. RESULTS Seventy-eight patients were included in the analysis, of whom 47 underwent fetoscopic repair and 31 underwent open-hysterotomy repair. In the fetoscopic-repair group, compared with the open-repair group, fewer women underwent Cesarean section (53% vs 100%; P

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

OBJECTIVE To determine the impact of the lesion type (cystic [myelomeningocele] or flat [myeloschisis]) on the fetal motor function (MF) in cases candidates for prenatal open neural tube defect (ONTD) repair. METHODS Retrospective cohort study of patients with ONTD who underwent prenatal repair at a single institution between 2011 and 2019. The lesion type and the measurements of the length and width of the lesions to calculate the surface of the ellipsoid lesion were performed using MR scans. Prenatal MF of the lower extremities was evaluated by ultrasound following a metameric distribution at the time of referral. Intact MF was defined as the observation of plantar flexion of the ankle. Logistic regression was performed to determine the predictive value of the type of lesion for having an intact MF at the time of referral. RESULTS 103 patients were included at 22.9 (19-25.4) weeks; 65% had cystic and 35% had flat lesions. At the time of referral, there was a higher proportion of cases with an intact MF in the presence of flat lesions (34/36; 94.4%) as compared to cystic lesion (48/67; 71.6%, p

Objective To describe changes in fetal Doppler parameters during a novel approach for open fetal surgery for myelomeningocele (MMC) repair. Methods Forty-four fetuses undergoing open fetal surgery for MMC repair characterized by a mini-hysterotomy (15mm), reduced fetal manipulation, and maintenance of a constant volume of amniotic fluid were evaluated. Doppler velocimetry of the umbilical artery (UA), middle cerebral artery (MCA), cerebroplacental ratio (CPR) and ductus venosus (DV) were registered at specific time points during fetal surgery and considered as abnormal with UA pulsatility index (PI) or DV-PI above the 95th centile, and MCA-PI or CPR values below the 5th centile. Results Median gestational age (GA) at procedure was 25.4 weeks (range, 22.9-27.9). Doppler recordings were successfully obtained during all time periods throughout surgery. As compared to Doppler values before surgery, there was a significant increase in the prevalence of brain vasodilation (15.9% (7/44) vs. 65.9% (29/44); p=0.001), abnormal DV-PI (0% vs. 22.7% (10/44); p=0.02), and a non-significant increase in the rate of abnormal UA-PI (0% vs. 11.4% (5/44); p=0.2). No absent or reversed end diastolic or atrial velocities in the UA or in the DV, respectively were seen in any fetus at any time. All abnormal Doppler parameters returned to normal values after surgery. Conclusions A small hysterotomy in conjunction with reduced fetal manipulation and maintenance of a normal amniotic fluid volume during open fetal surgery for MMC, seem not to significantly affect fetal Doppler parameters. The observed mild Doppler changes can be a manifestation of fetal adaptation to a stressful condition given by fetal surgery. This article is protected by copyright. All rights reserved.

Background: Fetoscopic endoluminal tracheal occlusion (FETO) improves lung maturation in severe cases of congenital diaphragmatic hernia (CDH) but it does not ameliorate lung compression by herniated abdominal organs. Surgically opening the fetal abdomen (abdominal decompression [AD]) reduces the intrathoracic pressure by diverting the abdominal organs into the amniotic cavity-a probable causal therapy for lung hypoplasia and pulmonary hypertension in CDH. Open surgical abdominal decompression has been reported: we describe a minimally invasive approach in an ovine model of CDH as a probable fetoscopic intervention. Materials and Methods: Eight fetuses were included, 2 were kept as HEALTHY controls. A CDH (left side, liver down) was created by open fetal surgery at midgestation in 6 fetuses, 2 were taken as CDH controls. Fetoscopic abdominal decompression (fAD) was performed 21 days later in 4 animals. The fetuses were retrieved at the end of gestation and evaluated by lung stereology. Results: fAD led to a near total evacuation of the thoracic cavity in 2 of the 4 animals. Fetuses with CDH had a lower total volume and fraction of alveolar air space, a lower volume fraction of the parenchyma, and an increase of the volume fraction of the alveolar septa and atelectasis, as well as an increased mean thickness of alveolar septa compared with HEALTHY fetuses. Fetuses treated with abdominal decompression showed an improvement of stereological parameters. Conclusions: In spite of relevant limitations (pilot study, small groups, spontaneous closure of the abdominal incision) we were able to demonstrate that abdominal decompression for CDH can be performed by fetoscopy. Our results support the hypothesis of causally improving lung development by abdominal decompression, thus implying increased survival in extreme cases of CDH. A refinement of the fetoscopic techniques and direct comparison to FETO appears warranted.

OBJECTIVE Prenatal closure of myelomeningocele is associated with a reduced rate of hydrocephalus treatment. This need for hydrocephalus treatment is positively correlated with fetal ventricular width. When ventricular width is 15 mm or greater, the benefits of prenatal closure, as a method to decrease hydrocephalus treatment, are reduced. Thus, fetal ventricular size is an important factor when counseling families who are considering intrauterine surgery with mitigation of hydrocephalus as the primary goal. This study sought to determine whether imaging modality (ultrasound [US] vs MRI) and interobserver variability were factors in any ventricular size disparity seen on imaging studies. METHODS The imaging studies of 15 consecutive fetuses who underwent prenatal myelomeningocele repair at Children’s Mercy Fetal Health Center, Kansas City, Missouri, were reviewed. All fetuses were imaged with US and fetal MRI; on average (range), procedures were performed 3.8 (0–20) days apart. Three comparisons were performed to analyze interobserver and intermodality variability in ventricular width measurements: 1) retrospective comparison of dictated ventricular widths measured with MRI and US by pediatric radiologists (PRs) and maternal-fetal medicine specialists (MFMs), respectively; 2) blinded measurements obtained with US by PRs versus initial US-based measurements by MFMs, and blinded measurements obtained with MRI by PRs versus initial MRI-based measurements by PRs; and 3) blinded measurements obtained with MRI by PRs versus those obtained with US. RESULTS Retrospective comparison showed that measurements with MRI by PRs were on average 2.06 mm (95% CI 1.43–2.69, p < 0.001) larger than measurements with US by MFMs. Blinded measurements with US by PRs were on average larger than dictated measurements obtained with US by MFMs, but by only 0.6 mm (95% CI 0.31–0.84, p < 0.001). When PRs measured ventricular size in a blinded fashion with both US and MRI, the mean width determined with MRI was significantly larger by 2.0 mm (95% CI 1.26–2.67, p < 0.0001). CONCLUSIONS The ventricular width of these fetuses was larger when measured with MRI than US by an amount that could impact recommendations for fetal surgery. Every center involved in counseling families about the risks and benefits of fetal intervention for spina bifida needs to be aware of these possible imaging-based disparities.

This article reviews the contemporary diagnosis and management of antenatally diagnosed congenital lung lesions. These anomalies, which include congenital pulmonary airway malformation (CPAM) (formerly congenital cystic adenomatoid malformation), bronchopulmonary sequestration (BPS), bronchogenic cyst, and congenital lobar emphysema (CLE), are relatively rare but are increasingly encountered by clinicians because of the improved resolution and enhanced sensitivity of fetal ultrasound. Serial assessment of these lesions throughout pregnancy remains the norm rather than the exception. Perinatal management strategies may differ based on initial size and growth patterns of these masses until delivery. Fetal magnetic resonance imaging and other diagnostic testing can sometimes be helpful in providing additional prognostic information. Over the last decade, maternal steroids have become standard of care in the management of larger lesions at risk for nonimmune hydrops. As a result, fetal surgical procedures, including open resection, thoracoamniotic shunting, and ex utero intrapartum treatment (EXIT), are less uncommonly performed. Decisions regarding whether delivery of these fetuses should occur in a tertiary care center with pediatric surgery coverage versus delivery at a local community hospital are now highly relevant in most prenatal counseling discussions with families. Large lung malformations may require urgent surgical removal in the early postnatal period because of respiratory distress. Other complications, such as recurrent pneumonia, pneumothorax, and cancer, are indications for postnatal lung resection on an elective basis. Many children are good candidates for minimally invasive (thoracoscopic) surgical approaches as an alternative to resection by thoracotomy. In the vast majority of cases, the overall prognosis remains excellent.

Objectives (1) To investigate the reproducibility of total fetal lung volume (TFLV) measurements using a free 3D modeling software (3DSlicer); (2) To correlate these measurements with lung-to-head ratio (LHR) or TFLV measured using PACS and; (3) To determine the role of 3DSlicer in predicting perinatal outcomes in cases with congenital diaphragmatic hernia (CDH) who had fetal tracheal occlusion (FETO). Methods Retrospective cohort study between 2012 and 2017 at Texas Children’s Hospital (2011–2017), including all patients who underwent FETO for CDH. LHR was measured by ultrasound and TFLV was measured by MRI at the time of referral and 6 weeks after FETO using 3DSlicer and PACS. We evaluated intra- and inter-rater reliability of TFLV measurement using 3DSlicer, infant survival to 1 year, need for ECMO and pulmonary hypertension. Results The intra- and inter-rater reliability of TFLV measured with 3DSlicer was excellent before and after FETO (Intra-class correlation coefficient: 0.98−0.99 and 0.94−0.99, respectively). There was a good correlation between TFLV measured with PACS and with 3DSlicer before and after FETO (r = 0.78 and r = 0.99, respectively). Similarly, there was a good correlation between TFLV measurements using PACS or 3DSlicer and LHR after FETO (r = 0.86 and r = 0.88, respectively). Infants who survived to 1 year had a significantly higher TFLV evaluated with 3DSlicer before FETO compared to non-surviving infants (OR = 1.16[1.1–1.3], p = 0.03) as well as a significantly higher TFLV evaluated by 3DSlicer after FETO (OR = 1.2[1–1.4], p = 0.04). Conclusion Lung volume measurements using free 3DSlicer in infants with severe CDH who underwent FETO are reproducible and reliable, and have comparable predictive capability for survival as those measured using conventional software.

Early and timely diagnosis of the spinal column and spinal cord pathologies, their intrauterine and postnatal correction require detailed knowledge of the anatomy and topography of the cervical spine in the fetus. The complex interactions of the cervical spine with the corresponding parts of the spinal cord and adjacent structures require detailed study during the embryonic period of ontogenesis.The aim of the study was to investigate the morphometric and topographic anatomical fetal features of the cervical spine and adjacent structures at 20–22 weeks of the intrauterine development.Material and methods. This study was performed on the sectional material from human fetuses, 20–22 weeks of development (20 human fetuses of both sexes), obtained from the fetal collection of the Department of Human Anatomy, Orenburg State Medical University. A standard set of morphological methods were applied in the study – macromicroscopic preparation, Pirogov method of cuts in three mutually perpendicular planes, production of serial histotopograms with Van Gieson staining, photography, morphometry.Results. It was found that during the studied periods of prenatal ontogenesis, the length of the cervical spine averaged 17.5±1.5 mm. The transverse size of the vertebral body in fetuses of 20–22 weeks of development throughout the cervical spine is of greater importance than the anteroposterior. The transverse and anteroposterior dimensions of the vertebral foramen gradually decrease in the craniocaudal direction. It was also demonstrated that it is necessary to remove the dura mater in order to obtain reliable dimensional characteristics of the spinal cord.Conclusion. Thus, the data obtained on the anatomy and topography of the spinal column and the spinal cord at the 20th – 22nd weeks of the human ontogenesis can serve as the morphological basis of fetal surgery.

OBJECTIVE: To determine the incidence and characterise corpus callosum (CC) abnormalities in fetuses with spina bifida aperta (SBA) between 18 and 26 weeks of gestation. METHODS: This was a retrospective study on fetuses with isolated SBA and who were assessed for fetal surgery. Digitally stored ultrasound images of the brain were reviewed for the presence/absence of the CC, and the length and diameter of its constituent parts (rostrum, genu, body and splenium). We used regression analysis to determine the relationship between CC abnormalities and gestational age, head circumference, ventricle size, lesion level and lesion type. RESULTS: Nearly three-quarters of fetuses with isolated SBA had an abnormal CC (71.7%, 76/106). Partial agenesis was most common in the splenium (18.9%, 20/ 106) and the rostrum (13.2%, 14/106). The most common abnormal pattern was of a short CC with normal diameter throughout. Of note, 20.8% (22/106) had a hypoplastic genu and 28.3% (30/106) had a thick body part. Larger lateral ventricle size was associated with partial agenesis of the CC (odds ratio [OR]: 0.14, p < 0.001) and inversely associated with a shorter CC (OR: 2.60, p < 0.01). CONCLUSION: An abnormal CC is common in fetuses with isolated SBA who are referred for fetal surgery.

Skin-over-biocellulose for Antenatal Fetoscopic Repair (SAFER) is a recently developed technique for fully percutaneous fetoscopic repair of myelomeningocele. The formation of a neo-dura mater triggered by the use of a biocellulose patch over the placode obviates the need of primary repair of fetal dura mater, and seems to further improve neurodevelopmental outcome. Insufflation of humidified carbon dioxide into the amniotic cavity requires proper training and a different equipment set from those used in the classic “under-water” fetoscopy. To shorten the learning curve of novice teams, we have developed a structured training course encompassing three critical steps: (1) visiting the proctor center, (2) selection of the first case, and (3) on-site training of the surgical team. Upon the site arrival, there will be: (1) theoretical lecture for all specialists involved in the fetal care, (2) simulation training, and (3) surgical proctoring. Proctor team can take over the surgery if the local team cannot complete. This training course has been successfully used in five different countries: Israel, Chile, Italy, USA and England and all local teams are already performing surgeries solo without any failures or maternal morbidity. Teaching new procedures in fetal medicine is challenging, because of the small number of candidate cases, and the direct relation of good outcomes and the number of cases operated. This proposed training modules may be adopted by other teams that want to embark on this type of novel minimally invasive treatment.

To estimate the significance of the association between mid-trimester maternal serum alpha-fetoprotein (MSAFP) level and fetal neuroanatomic findings in cases of open neural tube defect (ONTD).Retrospective study of patients referred for prenatal ONTD repair between 2012 and 2018. Cases were classified into three groups based on their MSAFP level: 1)High MSAFP (3.8MoM - n = 22), 2)Moderately high MSAFP (≤3.8 and ≥2.5MoM - n = 28), 3)Normal MSAFP (2.5MoM - n = 18). MRI scans at the time of referral were used to assess the relationship between MSAFP and: A)Type of ONTD; B) Ventriculomegaly; C) Size of the myeloschisis lesion; D) Volume of myelomeningocele; E) Anatomical level of the lesion (LL).Having a high MSAFP level was more likely to be associated ventriculomegaly at mid-gestation than a moderately high or normal MSAFP level (OR = 8.4;CI95[0.9-73.4];p = 0.05 and OR = 2.8;CI95[0.9-8.8];p = 0.07). There were no differences between the three groups regarding type of lesion, size of the myeloschisis lesion, anatomic LL, or volume of the myelomeningocele sac. Myeloschisis cases with normal MSAFP had a larger surface area when compared to myeloschisis cases with moderately high MSAFP (219.8[104.4-551] vs 155.4[38.5-502.4] mm², p = 0.04).A 2nd trimester MSAFP level3.8MoM in a fetus with ONTD is associated with mid-gestation ventriculomegaly.

BACKGROUND: Congenital diaphragmatic hernia (CDH) reportedly has neurologic consequences in childhood however little is known about the impact in isolated CDH. AIMS: Herein we aimed to describe the risk of neurodevelopmental complications in children born with isolated CDH. MATERIALS & METHODS: We systematically reviewed literature for reports on the neurological outcome of infants born with isolated CDH. The primary outcome was neurodevelopmental delay. Secondary outcomes included, motor skills, intelligence, vision, hearing, language and behavior abnormalities. RESULTS: Thirteen out of 87 (15%) studies reported on isolated CDH, including 2624 out of 24,146 children. Neurodevelopmental delay was investigated in four studies and found to be present in 16% (3-34%) of children. This was mainly attributed to motor problems in 13% (2-30%), whereas cognitive dysfunction only in 5% (0-20%) and hearing in 3% (1-7%). One study assessed the effect of fetal surgery. When both isolated and non-isolated children were included, these numbers were higher. DISCUSSION: This systematic review demonstrates that only a minority of studies focused on isolated CDH, with neurodevelopmental delay present in 16% of children born with CDH. CONCLUSION: To accurately counsel patients, more research should focus on isolated CDH cases and examine children that underwent fetal surgery. ispartof: PRENATAL DIAGNOSIS vol:42 issue:3 pages:318-329 ispartof: location:England status: published

Over the last three decades, advances in early diagnosis of fetal anomalies, imaging and surgical techniques have led to a huge expansion in fetal surgery. A small number of specialist centres perform fetal surgery, which involves high-risk anaesthesia for the mother and fetus. The anaesthetist plays an integral role within the large multispecialty and multidisciplinary team, involved in planning and delivering care for complex surgical procedures. This article reviews three fetal surgical procedures, congenital diaphragmatic hernia, myelomeningocele repair and ex-utero intrapartum treatment for airway obstruction. The underlying fetal pathology, surgical management, anaesthetic considerations and risks for both the mother and fetus are described for each. Fundamental to this is the understanding that clear communication and collaboration between all team members is vital to ensure successful outcomes of patients, the mother and the fetus.

Background Fetal surgery is increasingly performed to correct congenital defects. Currently, fetal brain perfusion cannot be assessed intra-operatively. The purpose of this study was to determine if contrast-enhanced ultrasound (CEUS) could be used to monitor fetal cerebral perfusion during fetal surgery and if parameters correlate with fetal hemodynamics or acid/base status. Methods Cannulated fetal sheep were insufflated with carbon dioxide gas in an extra-uterine support device and in utero to mimic fetal surgery. Fetal heart rate, mean arterial pressure, and arterial blood gases were serially measured. CEUS examinations of the brain were performed and time-dependent metrics were quantified to evaluate perfusion. The relationships between measured parameters were determined with mixed linear effects models or two-way repeated measures analysis of variance. Results 6 fetal sheep (113 ± 5 days) insufflated at multiple time-points (n = 20 experiments) in an extra-uterine support device demonstrated significant correlations between time-dependent perfusion parameters and fetal pH and carbon dioxide levels. In utero, 4 insufflated fetuses (105 ± 1 days) developed hypercarbic acidosis and had reductions in cerebral perfusion parameters compared to age-matched controls (n = 3). There was no significant relationship between cerebral perfusion parameters and fetal hemodynamics. Conclusions CEUS-derived cerebral perfusion parameters can be measured during simulated fetal surgery and strongly correlate with fetal acid/base status.

A wide range of fetal interventions are being performed worldwide to save the fetus's life, prevent permanent fetal organ damage, and allow a successful transition to extrauterine life. However, these are invasive procedures and can be associated with serious complications. This article focuses on promoting a culture of safety by highlighting five common error traps while anesthetizing patients for fetal interventions. They include failure to preserve uteroplacental perfusion and gas exchange, failure to achieve adequate uterine relaxation prior to hysterotomy, failure to monitor the fetus and prepare for fetal/neonatal resuscitation, failure to prepare for maternal hemorrhage, and failure to promptly treat uterine atony. Practical tips for avoiding these serious complications will also be discussed.

Since its conception, prenatal therapy has been successful in correction of mainly anatomical defects, although the range of application has been limited. Research into minimally invasive fetal surgery techniques and prenatal molecular diagnostics has facilitated the development of in utero stem cell transplantation (IUT)-a method of delivering healthy stem cells to the early gestation fetus with the hope of engraftment, proliferation, and migration to the appropriate hematopoietic compartment. An area of application that shows promise is the treatment of hematopoietic disorders like hemoglobinopathies. The therapeutic rationale of IUT with hematopoietic stem cells (HSCs) is based on the proposed advantages the fetal environment offers based on its unique physiology. These advantages include the immature immune system facilitating the development of donor-specific tolerance, the natural migration of endogenous hematopoietic cells providing space for homing and engraftment of donor cells, and the fetal environment providing HSCs with the same opportunity to survive and proliferate regardless of their origin (donor or host). Maternal immune tolerance to the fetus and placenta also implies that the maternal environment could be accepting of donor cells. In theory, the fetus is a perfect recipient for stem cell transplant. Clinically, however, IUT is yet to see widespread success calling into question these assumptions of fetal physiology. This review aims to discuss and evaluate research surrounding these key assumptions and the clinical success of IUT in the treatment of thalassemia.

Over the past 10 years, over 150 fetal spina bifida surgeries were performed at the Zurich Center for Fetal Diagnosis and Therapy. This study looks at surrogates for success and failure of this approach. We focused on key outcome parameters including hydrocephalus shunt rate at one year, bladder control at 4, independent ambulation at 3 years, and maternal, fetal, and neonatal complications. From the first 150 patients undergoing fetal surgery for spina bifida, 148 (98.7%) were included in the study. Maternal–fetal surgery was uneventful in 143/148 (97%) cases. Intraoperative problems included resuscitation in 4/148 fetuses (2.7%). 1/148 fetuses (0.7%) died on postoperative day 4. Maternal complications included chorioamniotic membrane separation in 22/148 (15%), lung embolism in 3/148 (2.1%), chorioamnionitis in 2/148 (1.4%), AV-block III and uterine rupture in 1/148 each (0.7%). 1/148 (0.7%) newborn death was recorded. Hindbrain herniation was identified preoperatively in 132/148 (90%) fetuses and resolved completely in 119/132 (90%). At one year, 39/106 (37%) children had required a CSF diversion. At 4 years, 4/34 patients (12%) had normal bladder control. At 3 years, 48/57 (84%) walked independently. A majority of patients benefitted from prenatal intervention, in that the shunt rate was lower and the rates of continent and walking patients were higher than reported with postnatal care.

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

Introduction: A wide range of fetal interventions are performed across fetal therapy centers (FTCs). We hypothesized that there is significant variability in anesthesia staffing and anesthetic techniques. Methods: We conducted an online survey of anesthesiology directors at every FTC within the North American Fetal Therapy Network (NAFTNet). The survey included details of fetal interventions performed in 2018, anesthesia staffing models, anesthetic techniques, fetal monitoring, and postoperative management. Results: There was a 92% response rate. Most FTCs are located within an adult hospital and employ a small team of anesthesiologists. There is heterogeneity when evaluating anesthesiology fellowship training and staffing, indicating there is a multidisciplinary specialty team-based approach even within anesthesiology. Minimally invasive fetal interventions were the most commonly performed. The majority of FTCs also performed ex utero intrapartum treatment (EXIT) and open mid-gestation procedures under general anesthesia (GA). Compared to FTCs only performing minimally invasive procedures, FTCs performing open fetal procedures were more likely to have a pediatric surgeon as director and performed more minimally invasive procedures. Conclusions: There is considerable variability in anesthesia staffing, caseload, and anesthetic techniques among FTCs in NAFTNet. Most FTCs used maternal sedation for minimally invasive procedures and GA for EXIT and open fetal surgeries.

Background: Pregnancies that are prenatally identified to have fetal anomalies are complex and require expert multidisciplinary care. As many conditions can impact the fetus prenatally and require intervention, an enhanced recovery after surgery (ERAS) for lower urinary tract obstruction (LUTO) is being evaluated to determine the level of evidenced-based data available. Problem: The percutaneous ultrasound-guided fetal surgery procedure for bladder neck obstruction is the focus for elements of preoperative counseling, intraoperative procedure/risk complications, and postoperative management. Methods: A quality improvement review Squire 2.0 (2000–2020) was undertaken for the percutaneous LUTO fetal surgery shunting (lower urinary tract obstruction), process and procedure which require 2 process pathways, one to evaluate the fetal candidate and a second to treat. This structured review is focused on identifying the process elements to allow the determination of the number of evidenced-based elements that would allow for audit and measurement of the clinical element variance for comparative feedback to the individual surgical provider or surgery center. Interventions: Titles and abstracts were screened to identify potentially relevant articles with priority given to meta-analyses, systematic reviews, randomized controlled studies, nonrandomized controlled studies, reviews, and case series. Results: A series of potential clinical elements for the diagnostic fetal evaluation and for the 3 protocol areas of surgical care for the procedures (pre-, intra-, and postoperative care) are identified using an ERAS-like process. Conclusions: The identified clinical elements have the potential for ERAS-LUTO fetal therapy guideline. Multidisciplinary collaboration (surgeon, anesthesia, nursing, imaging, and laboratory) is required for ERAS quality improvement in the pre-, intra-, and postoperative processes. Process elements in each of the operative areas can be audited, evaluated, compared, and modified/improved.

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

Background Abnormal intracranial findings are often detected at mid‐trimester ultrasound (US) in fetuses with myelomeningocele (MMC). It is unclear whether these findings constitute a spectrum of the disease or are an independent finding, which should contraindicate fetal surgery. Objective To ascertain the spectrum and frequency of US‐detected cranial findings in fetuses with MMC. Search strategy MEDLINE, Embase, Web of Science and CENTRAL were searched from January 2000 to June 2020. Selection criteria Study reporting incidence of cranial US findings in consecutive cases of second‐trimester fetuses with MMC. Data collection and analysis Publication quality was assessed by Newcastle–Ottawa Scale (NOS) and modified NOS. Meta‐analysis could not be performed as a result of high clinical diversity and study heterogeneity. Main results Fourteen cranial US findings were reported in 15 studies. Findings in classic Chiari II malformation (CIIM) spectrum included posterior fossa funnelling (96%), small transcerebellar diameter (82–96%), ‘banana’ sign (50–100%), beaked tectum (65%) and ‘lemon’ sign (53–100%). Additional cranial findings were small biparietal diameter (BPD) and head circumference (HC) (, Tweetable abstract A high rate of cranial abnormalities found on second‐trimester ultrasound in fetuses with myelomeningocele.

Background: The inherent morbidity associated with fetal ovine models of myelomeningocele (MMC) has created challenges for long-term survival of lambs. We aimed to develop a fetal ovine surgical spinal exposure model which could be used to evaluate long-term safety after direct spinal cord application of novel therapeutics for augmentation of in utero MMC repair. Methods: At gestational age (GA) 100–106, fetal lambs underwent surgical intervention. Laminectomy of L5–L6 was performed, dura was removed, and an experimental product was directly applied to the spinal cord. Paraspinal muscles and skin were closed and the fetus was returned to the uterus. Lambs were delivered via cesarean section at GA 140–142. Lambs were survived for 3 months with regular evaluation of motor function by the sheep locomotor rating scale. Spinal angulation was evaluated by magnetic resonance imaging at 2 weeks and 3 months. Results: Five fetal surgical intervention lambs and 6 control lambs who did not undergo surgical intervention were included. All lambs survived to the study endpoint of 3 months. No lambs had motor function abnormalities or increased spinal angulation. Conclusion: This model allows for long-term survival after fetal spinal cord exposure with product application directly onto the spinal cord.

Objective: Function of the lower extremities after prenatal myelomeningocele (MMC) repair is best assessed with ambulatory function at 30–36 months of age, but parents often ask about function before this milestone. Lower extremity movement can be assessed by ultrasound (US) and at the newborn exam (NE), but correlation between US, NE, and ambulation is not firmly established. Methods: This was a retrospective correlation study of fetuses that underwent open prenatal MMC repair at SSM Cardinal Glennon Fetal Care Institute, St. Louis, MO, between January 2011 and June 2017. Movement at the ankles, knees, and hips was assessed by US after open repair on postoperative days (PODs) 0–5 and at 32 weeks gestation. NE was performed by physical therapy or neurosurgery within the first month of life, and pediatric follow-up between 30 and 36 months of age was obtained to document ambulation. Results: Forty-two fetuses were included. Joint movement seen on US varied by POD: it was present on POD 1 in 7% of fetuses and 62% by POD 5. Degree of ventriculomegaly, lesion level, and lesion length did not have a significant effect on US, NE, or ambulation. Knee movement on POD 3 correlated with knee movement at NE (k = 0.58, p < 0.01), but only later knee movement correlated with ambulation (k = 0.28–0.46, p = 0.01). Hip movement at 32 weeks was the only single joint assessment that correlated with NE and ambulation (k = 0.45 and 0.46, p = 0.03 and 0.01, respectively). Conclusion: Lower extremity movement increases between POD 1 and POD 5 in fetuses after open fetal MMC repair. Knee and hip movement on US at 32 weeks correlates with ambulation at 30–36 months. These data may inform counseling, and direct therapy and spark prospective investigations.

Objective To cope with the changing health care services in the era of SARS-CoV-2 pandemic. We share the institutional framework for the management of anomalous fetuses requiring fetal intervention at Mayo Clinic, Rochester, Minnesota. To assess the success of our program during this time, we compare intraoperative outcomes of fetal interventions performed during the pandemic with the previous year. Patients We implemented our testing protocol on patients undergoing fetal intervention at our institution between March 1, and May 15, 2020, and we compared it with same period a year before. A total of 17 pregnant patients with anomalous fetuses who met criteria for fetal intervention were included: 8 from 2019 and 9 from 2020. Methods Our testing protocol was designed based on our institutional perinatal guidelines, surgical requirements from the infection prevention and control (IPAC) committee, and input from our fetal surgery team, with focus on urgency of procedure and maternal SARS-CoV-2 screening status. We compared the indications, types of procedures, maternal age, gestational age at procedure, type of anesthesia used, and duration of procedure for cases performed at our institution between March 1, 2020, and May 15, 2020, and for the same period in 2019. Results There were no statistically significant differences among the number of cases, indications, types of procedures, maternal age, gestational age, types of anesthesia, and duration of procedures (P values were all >.05) between the pre–SARS-CoV-2 pandemic in 2019 and the SARS-CoV-2 pandemic in 2020. Conclusions Adoption of new institutional protocols during SARS-CoV-2 pandemic, with appropriate screening and case selection, allows provision of necessary fetal intervention with maximal benefit to mother, fetus, and health care provider.

Two unique aspects of antenatal care occur in the setting of fetal surgery and multiple gestations. As fetal interventions increase, so do the number of cases of iatrogenic preterm prelabor rupture of membranes (PPROM). Because of the amniotic sac's inability to heal, the risk of PPROM after surgery is directly correlated with the number of interventions, the size of the defect, and the surgery performed. Higher order gestations also carry an increased risk of PPROM. This paper reviews the risks and management of PPROM in the setting of the various prenatal interventions as well as in the setting of multiple gestations.

ispartof: PRENATAL DIAGNOSIS vol:40 issue:13 pages:1627-1635 ispartof: location:England status: published

Over the last decade fetal surgery to repair open spinal dysraphisms has become an acceptable and in some cases desirable alternative to the traditional method of postnatal closure. Fetal MRI is an essential part of the workup in these patients, not only to select the appropriate candidates for fetal surgery but also to guide prenatal counseling and perinatal management. In this article we review current surgical techniques for prenatal repair, relevant imaging findings in the era of fetal surgery, and expected imaging findings of the brain and spine in the fetal and postnatal periods.

Continuous fetal hemodynamic monitoring during in-utero surgery is desirable, but it is often not feasible without intermittent interruption. We report the use of a fetal spiral electrode for continuous heart rate monitoring during fetal myelomeningocele repair. Fetal echocardiography and a fetal spiral electrode were used to monitor fetal heart rate during in-utero repair at 25 weeks' gestation. We observed good agreement between echocardiographic and spiral electrode heart rate measurements. Using the Bland-Altman approach, the mean (SD) difference between measurements was 1.8 (3.5) beats per minute with limits of agreement of -5.3 to 8.8 beats per minute. This case illuminates a potential role for a fetal spiral electrode as a real-time adjunct in fetal interventions.

Prenatal myelomeningocele (MMC) repair has been shown to provide significant benefits to the infant, decreasing the postnatal need for ventriculoperitoneal shunt and improving motor outcome. Chorioamniotic membrane separation (CAS) is a potential complication following prenatal MMC repair and may increase the risk of preterm prelabor rupture of membranes (PPROM) and preterm birth. The objectives of this study were: (1) to evaluate the incidence of CAS after prenatal MMC repair; (2) to determine risk factors associated with its occurrence; and (3) to assess its association with adverse perinatal outcomes.This was a retrospective cohort study of patients who underwent fetal MMC repair between November 2011 and December 2018. Surgery was performed using either a fetoscopic (laparotomy or exteriorized uterus) approach or an open-hysterotomy approach. Eligibility criteria were those reported in the Management of Myelomeningocele Study. If CAS was detected on ultrasound (US), its severity was graded as 'mild' if amnion detachment involved 25% of the uterine cavity, 'moderate' if it involved 25-50% and 'severe' if it involved 50%. Evolution of CAS was classified as stable, increasing or decreasing based on the difference in severity grading between the time at first diagnosis and the last US scan before delivery. Logistic regression analysis was performed to identify pre- or perisurgical factors associated with the development of CAS and to determine the risk of adverse perinatal outcome associated with CAS.In total, 91 cases were included. Fetoscopic or open-hysterotomy repair of MMC was performed in 52/91 (57.1%) and 39/91 (42.9%) cases, at a median gestational age (GA) of 25.0 weeks (range, 22.9-26.0 weeks) and 25.0 weeks (range, 21.3-25.9 weeks), respectively. CAS was diagnosed in 31/91 (34.1%) patients, at a median GA of 28.1 weeks (range, 24.4-37.6 weeks). Anterior placenta was identified as a risk factor for the postoperative development of CAS (odds ratio (OR), 3.72 (95% CI, 1.46-9.5); P 0.01). This risk was dependent on the repair technique. An anterior placenta significantly increased the risk of CAS after fetoscopic repair (OR, 3.94 (95% CI, 1.14-13.6); P = 0.03) but not after open repair (OR, 2.8 (95% CI, 0.6-12.5); P = 0.16). There was no significant difference in the rate of CAS after fetoscopic repair (21/52 (40.4%)) vs open-hysterotomy repair (10/39 (25.6%)) (P = 0.14), nor were there any differences in GA at diagnosis of CAS, interval between surgery and diagnosis, distribution of CAS severity or progression of CAS between the two groups. CAS increased the risk of PPROM (50% in those with vs 12% in those without CAS) (OR, 7.6 (95% CI, 2.5-21.9); P 0.01) and preterm delivery (70% vs 38%) (OR, 3.2 (95% CI, 1.3-8.1); P 0.01). Fetoscopically repaired cases with CAS had a higher rate of PPROM (12/20 (60.0%) vs 2/31 (6.5%); P 0.01) and preterm delivery (13/20 (65.0%) vs 5/31 (16.1%); P 0.01) than those that did not develop CAS, while the differences were not significant in cases with open-hysterotomy repair. Early detection of CAS (before 30 weeks' gestation) was a risk factor for preterm delivery (90% before 30 weeks vs 36% at or after 30 weeks) (OR, 15.7 (95% CI, 2.3-106.3); P 0.01). There was no association between PPROM or preterm delivery and the severity or progression of CAS.The presence of an anterior placenta was the only factor that increased the risk for CAS after fetoscopic MMC repair. Detection of CAS after fetoscopic MMC repair significantly increases the risk for PPROM and preterm delivery. Copyright © 2019 ISUOG. Published by John WileySons Ltd.

Twin-to-twin transfusion syndrome (TTTS) is characterized by an unbalanced blood transfer through placental abnormal vascular connections. Prenatal ultrasound (US) is the imaging technique to monitor monochorionic pregnancies and diagnose TTTS. Fetoscopic laser photocoagulation is an elective treatment to coagulate placental communications between both twins. To locate the anomalous connections ahead of surgery, preoperative planning is crucial. In this context, we propose a novel multi-task stacked generative adversarial framework to jointly learn synthetic fetal US generation, multi-class segmentation of the placenta, its inner acoustic shadows and peripheral vasculature, and placenta shadowing removal. Specifically, the designed architecture is able to learn anatomical relationships and global US image characteristics. In addition, we also extract for the first time the umbilical cord insertion on the placenta surface from 3D HD-flow US images. The database consisted of 70 US volumes including singleton, mono- and dichorionic twins at 17-37 gestational weeks. Our experiments show that 71.8% of the synthesized US slices were categorized as realistic by clinicians, and that the multi-class segmentation achieved Dice scores of 0.82 ± 0.13, 0.71 ± 0.09, and 0.72 ± 0.09, for placenta, acoustic shadows, and vasculature, respectively. Moreover, fetal surgeons classified 70.2% of our completed placenta shadows as satisfactory texture reconstructions. The umbilical cord was successfully detected on 85.45% of the volumes. The framework developed could be implemented in a TTTS fetal surgery planning software to improve the intrauterine scene understanding and facilitate the location of the optimum fetoscope entry point.

Introduction: Open fetal surgery requires a hemostatic hysterotomy that minimizes membrane separation. For over 30 years, the standard of care for hysterotomy in the gravid uterus has been the AutoSuture Premium Poly CS*-57 stapler. Objective: In this study, we sought to test the feasibility of hysterotomy in a rhesus monkey model with the Harmonic ACE®+7 Shears. Methods: A gravid rhesus monkey underwent midgestation hysterotomy at approximately 90 days of gestation (2nd trimester; term = 165 ± 10 days) using the Harmonic ACE®+7 Shears. A two-layer uterine closure was completed and the dam was monitored by ultrasound intermittently throughout the pregnancy. At 58 days after hysterotomy (near term), a final surgery was performed to evaluate the uterus and hysterotomy site. Results: A 3.5-cm hysterotomy was completed in 2 min 7 s. The opening was hemostatic and the membranes were sealed. Immediately after closure and throughout the pregnancy, ultrasound revealed intact membranes without separation and normal amniotic fluid levels. At term, the scar was well healed without signs of thinning or dehiscence. Conclusions: The Harmonic ACE®+7 Shears produced a hemostatic midgestation hysterotomy with membrane sealing in the rhesus monkey model. Importantly, healing was acceptable.

OBJECTIVES Fetal surgery for repair of open neural tube defect (ONTD) typically results in decreased need for a ventriculoperitoneal shunt (VPS). Our objectives were to determine the trend in ventricle size (VS) during pregnancy and whether VS and change in VS, as assessed by ultrasound, were predictive of the need for VPS in pregnancy with ONTD. METHODS This was a retrospective analysis of prospectively collected data of consecutive pregnancies with ONTD, evaluated in a single center from January 2012 to May 2018. Two groups were identified: the first consisted of pregnancies that underwent in-utero repair (IUR) and the second those that had postnatal repair (PNR). Penalized B splines were used to determine the trend in VS, across 2-week gestational-age (GA) epochs, between 24 and 36 weeks of gestation. VS at each GA epoch and the change in VS between each GA epoch were compared between the IUR and PNR groups. To determine whether VS at any GA was predictive of VPS, receiver-operating-characteristics (ROC) curves were used and the optimal cut-off at each GA epoch was identified. Univariate analysis and multiple logistic regression were used for further analysis. RESULTS ONTD was diagnosed in 110 fetuses, of whom 69 underwent IUR and 41 had PNR. Fetuses in the IUR group were more likely to have Chiari II malformation (100.0% vs 82.9%; P

[Background] The “Ex-Utero Intrapartum Treatment” (EXIT) procedure allows to ensure fetal airway before completion of delivery and umbilical cord clamping while keeping uteroplacental circulation. Airway obstruction in fetal oropharyngeal and cervical masses can be life-threatening at birth. In those situations, controlled access to fetal airway performed by a trained multidisciplinary team allows safe airway management, while feto-maternal circulation is preserved. We aim to review the indications and outcome of the EXIT procedure in a case series of fetal cervical and oropharyngeal masses., [Methods] We have carried out a retrospective review of all patients with fetal cervical and oropharyngeal masses who underwent an EXIT procedure between 2008 and 2019. Variables evaluated included indication for EXIT, ultrasound and MRI findings, the need of amnioreduction, gestational age at EXIT, birth weight, complications, operative time, survival rate, pathological findings, and postnatal evolution. Five patients are included in this series. One additional case has already been published., [Results] The diagnosis were cervical teratoma (n = 1), epulis (n = 1) and lymphangioma (n = 3). Polyhydramnios was present in 2 patients, requiring amnioreduction in one of them. Mean gestational age at EXIT was 36–37 weeks (range, 34–38 weeks). Median EXIT time in placental support was 9 min (range, 3–22 min). Access to airway was successfully established in EXIT in all cases. All children born by EXIT are currently healthy and without complications., [Conclusion] The localization and characteristics of the mass, its relationship to the airway, and the presence of polyhydramnios seem to be major factors determining indications for EXIT and clinical outcome.

Fetoscopic laser photocoagulation is the most effective treatment for Twin-to-Twin Transfusion Syndrome, a condition affecting twin pregnancies in which there is a deregulation of blood circulation through the placenta, that can be fatal to both babies. For the purposes of surgical planning, we design the first automatic approach to detect and segment the intrauterine cavity from axial, sagittal and coronal MRI stacks. Our methodology relies on the ability of capsule networks to successfully capture the part-whole interdependency of objects in the scene, particularly for unique class instances ( i.e., intrauterine cavity). The presented deep Q-CapsNet reinforcement learning framework is built upon a context-adaptive detection policy to generate a bounding box of the womb. A capsule architecture is subsequently designed to segment (or refine) the whole intrauterine cavity. This network is coupled with a strided nnU-Net feature extractor, which encodes discriminative feature maps to construct strong primary capsules. The method is robustly evaluated with and without the localization stage using 13 performance measures, and directly compared with 15 state-of-the-art deep neural networks trained on 71 singleton and monochorionic twin pregnancies. An average Dice score above 0.91 is achieved for all ablations, revealing the potential of our approach to be used in clinical practice.

To evaluate the impact of fetal endoscopic tracheal occlusion (FETO) on improving survival of fetuses with severe left-sided congenital diaphragmatic hernia (CDH), as compared with contemporaneous cases managed expectantly during pregnancy, in a country with suboptimal neonatal management.In this prospective cohort study, consecutive fetuses with isolated left-sided CDH, normal karyotype and severe pulmonary hypoplasia (defined as liver herniation and observed/expected lung-to-head circumference ratio below 26%) were selected for FETO at less than 32 weeks of gestation in a single tertiary referral center in Queretaro, Mexico. Postnatal outcome (survival up to 28 days after birth) was compared between fetuses treated with FETO and contemporaneous cases with similar lung size managed expectantly during pregnancy.Twenty-five fetuses with isolated severe left-sided CDH treated with FETO were matched individually with 25 cases managed expectantly during pregnancy. Endotracheal placement of the balloon was performed successfully on the first attempt in all cases. The median gestational age (GA) at balloon placement was 29.1 (range, 25.6-31.8) weeks and 34.1 (range, 30.0-36.1) weeks at balloon removal. There were no technical problems with the introduction or removal of the balloon in any cases. The median GA at delivery was significantly lower in the group treated with FETO than in those managed expectantly (35.3 vs 37.7 weeks; P = 0.04). The survival rate was significantly higher in the group treated with FETO than in those without fetal intervention (32% vs 0%; P 0.001).In settings with suboptimal neonatal management, FETO was associated with improved neonatal survival in fetuses with isolated left-sided CDH and severe pulmonary hypoplasia. Copyright © 2020 ISUOG. Published by John WileySons Ltd.Resultado de supervivencia en una hernia diafragmática congénita grave del lado izquierdo, con y sin oclusión traqueal endoscópica fetal en un país con un tratamiento neonatal subóptimo OBJETIVO: Evaluar el impacto de la oclusión traqueal endoscópica fetal (OTEF) en la mejora de la supervivencia de los fetos con hernia diafragmática congénita (HDC) grave del lado izquierdo, en comparación con los casos actuales tratados como embarazo gestante, en un país con un tratamiento neonatal subóptimo. MÉTODOS: En este estudio prospectivo de cohortes, se seleccionaron fetos consecutivos con HDC aislada del lado izquierdo, cariotipo normal e hipoplasia pulmonar grave (definida como hernia hepática y una proporción observada/esperada de la circunferencia pulmonar-cabeza inferior al 26%) para una OTEF antes de las 32 semanas de gestación, en un único centro de medicina especializada terciaria en Querétaro (México). El resultado postnatal (supervivencia hasta los 28 días después del nacimiento) se comparó entre fetos tratados con OTEF y los casos contemporáneos con tamaño pulmonar similar, tratados como embarazo gestante. RESULTADOS: Veinticinco fetos con HDC grave aislada del lado izquierdo que habían sido tratados con OTEF fueron emparejados individualmente con 25 casos tratados como embarazo gestante. La colocación endotraqueal del globo se realizó con éxito en el primer intento en todos los casos. La mediana de la edad gestacional (EG) en el momento de la colocación del globo fue de 29,1 (rango, 25,6-31,8) semanas y 34,1 (rango, 30,0-36,1) semanas cuando se retiró el globo. En ningún caso hubo problemas técnicos con la introducción o la retirada del globo. La mediana de la EG en el momento del parto fue significativamente menor en el grupo tratado con OTEF que en el grupo tratado como gestante (35,3 vs 37,7 semanas; P=0,04). La tasa de supervivencia fue significativamente más alta en el grupo tratado con OTEF que en los casos sin intervención fetal (32% vs 0%; P0,001). CONCLUSIÓN: En los entornos con un tratamiento neonatal subóptimo, la OTEF se asoció con una mejora de la supervivencia neonatal en los fetos con HDC aislada del lado izquierdo y con hipoplasia pulmonar grave. Copyright © 2020 ISUOG. Published by John WileySons Ltd.在对新生儿采取次优管理的国家里，严重先天性(左侧)膈疝在采取和未采取胎儿镜腔内气管阻塞术时的生存预后 目的: 评估胎儿镜腔内气管阻塞术(FETO)对改善严重先天性(左侧)膈疝(CDH)胎儿存活的影响，在新生儿次优管理的国家里，与在孕期采取期待治疗的同时期病例相比较。 方法: 本前瞻性群组研究中，在墨西哥克雷塔罗市的一家单个三级转诊中心内，在妊娠期不到32周的孕妇中，为FETO连续选取单纯性左侧膈疝、正常核型和严重肺发育不良(定义为肝脏疝入及观测/预期肺头比低于26%)的胎儿。就采用FETO治疗的胎儿和肺大小相似但在孕期采取期待治疗的同时期病例相比的产后预后(出生后存活至28天)。 结果: 将25个经FETO治疗的单纯性严重左侧CDH胎儿与25个在孕期采取期待治疗的病例逐个匹配。所有病例在气管内放置气球均首次尝试成功。气球放置时胎龄(GA)中位数为29.1周(范围:25.6-31.8周)，而气球移除时胎龄则为34.1周(范围:30.0-36.1周)。在任何情况下，都不存在放入或移除气球的任何技术问题。在经FETO治疗的群组中分娩时胎龄中位数远小于采取期待治疗的群组(35.3周 对 37.7周；P=0.04)。经FETO治疗的群组中胎儿的存活率远高于未对胎儿进行医疗干预的群组((32% 对 0%; P0.001)。 结论: 在对新生儿采取次优管理的情形下，FETO在治疗单纯性左侧膈疝和严重肺发育不良的胎儿时，与改善新生儿的存活情况相关。版权 © 2020 ISUOG。由威利父子公司(John WileySons Ltd)出版。.

Purpose of review Fetal intervention is a dynamic field with wide-reaching implications on neonatal ICU admissions and the care of neonates with congenital anomalies. The aim of this review is to summarize recent advancements in fetal surgery and provide a broad understanding of how these topics interrelate. Recent findings Advancements in prenatal imaging and diagnosis have dramatically expanded the indications for fetal intervention. Most recently, there has been a large focus on evaluating minimally invasive strategies of fetal intervention, notably fetoscopic surgery, and the use of stem cells for fetal treatment of myelomeningocele. With the advances in fetal intervention, neonatal care has adapted to the needs of these various patients to help improve the outcomes of this unique population. Summary Fetal intevention relies on a multidisciplinary team from prenatal imaging and maternal fetal medicine to fetal surgery and postnatal subspecialty care, particuarly neonatology. Fetal intervention uniquely involves two patients, both mother and fetus, and therefore, has unique risks and considerations, particularly in the advancement of the field. As the number of conditions suited to fetal intervention grows, awareness and advancement of the postnatal intensive care necessary for these patients are essential.