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Introduction: The Management of Myelomeningocele Study (MOMS) eligibility criteria preclude in utero surgery for fetal spina bifida (fSB) when the maternal body mass index (BMI) is ≥35 kg/m2. Some centers still respect this criterion, while others, like ours, do not. This study aimed to assess whether maternal and fetal safety is compromised with higher maternal BMIs., Methods: Data of 192 patients with open fSB repair at our center were retrospectively analyzed. According to their BMI, patients were divided into three groups: group 1 (BMI <30 kg/m2), group 2 (BMI 30-35 kg/m2), and group 3 (BMI >35 kg/m2). Subgroup analysis was performed to assess differences in maternal and fetal outcomes. Additionally, complications were divided into grades 1 to 5 according to their severity and outcome consequences and compared among groups., Results: Out of 192 patients, 146 (76.0%) had a BMI <30 kg/m2, 28 (14.6%) had a BMI 30-35 kg/m2, and 18 (9.4%) had a BMI >35 kg/m2. Significant differences occurring more often in either group 2 or 3 compared to group 1 were maternal wound seroma (50% or 56% vs. 32%, p = 0.04), amniotic fluid leakage (14% or 6% vs. 2%, p = 0.01) as well as vaginal bleeding (11% or 35% vs. 9%, p = 0.01). On the contrary, duration of tocolysis with atosiban was shorter in patients with BMI >30 kg/m2 (4 or 5 vs. 6 days, p = 0.01). When comparing severity of maternal or fetal complications, grade 1 intervention-related complications occurred significantly more often in group 3 compared to group 1 or 2 (78% vs. 45% or 57%, p = 0.02). Gestational age at delivery was around 36 weeks in all groups without significant differences., Conclusion: This investigation did not identify clinically relevant maternal and/or fetal outcome problems related to BMIs >35 kg/m2. Additional studies are however needed to confirm our results., (© 2024 The Author(s). Published by S. Karger AG, Basel.)

Introduction: We hypothesize that after publication of the quintessence of the MOMS trial, eligibility criteria for prenatal spina bifida (SB) repair may be modified if a tenable argumentation underlies this decision., Methods: Our first 154 fetal surgery patients were analyzed with particular focus on how many, which, and why the original eligibility criteria, set forth by the MOMS Trial Protocol, were disobeyed, and what the eventually detectable, negative and positive impacts of these deviations on outcomes were., Results: A total of 152 patients (2 missing consent) were included (100%). In 69 patients (45.4%), a total of 89 eligibility criteria were disobeyed. In 54 (35.6%) cases, the following maternal criteria were concerned: gestational age at operation of >25+6 weeks in 17 (11.2%), uterine pathologies in 13 (8.6%) women, preoperative BMI ≥35 kg/m2 in 12 (7.9%), previous hysterotomy in 7 (4.6%), previous prematurity in 3 (2%), HIV/hepatitis B in 2 (1.3%), psychosocial issues in 2 (1.3%), and placenta praevia in 1 (0.7%). In 32 (21.1%) cases, fetal criteria were disobeyed 34 times: Fetal anomaly unrelated to SB in 19 (12.5%), no/minimal evidence of hindbrain herniation in 13 (8.6%), and severe kyphosis in 2 (1.3%). We could not identify cases where non-observation of criteria led to clear-cut maternal and/or fetal disadvantages., Conclusion: This study shows that MOMS trial eligibility criteria for prenatal SB repair should be modified or even abandoned with adequate medical and ethical argumentation, and with written parental informed consent after non-directive, full disclosure counseling. This clear-cut change of paradigm is a necessity as it leads toward personalized medicine, allowing more fetuses to benefit from fetal surgery than would have benefitted with the former, published, MOMS criteria in place., (© 2023 The Author(s). Published by S. Karger AG, Basel.)

Purpose:  The goal of this study was to assess the accuracy of prenatal anatomical level determination by ultrasound (US) and magnetic resonance imaging (MRI) by analyzing the congruence with the "true" anatomical level identified by postnatal MRI., Patients and Methods:  The first 60 patients undergoing fetal myelomeningocele surgery at The Zurich Center for Fetal Diangosis and Therapy were included in this study. Anatomical levels (i. e., first dysraphic vertebra) determined by prenatal US and MRI were compared to postnatal MRI. The level of agreement between the imaging modalities was evaluated with a Cohen's kappa test. Results > 0.6 were interpreted as good agreement, > 0.8 as excellent., Results:  The exact congruence between prenatal US and MRI compared to postnatal MRI was 33 % and 48 %, respectively, for an accuracy within one level difference of 80 % and 90 %, and within two levels difference of 95 % and 98 %, respectively. The level of agreement of prenatal US and MRI compared to postnatal MRI was 0.62 and 0.79, respectively. Most of the prenatally incorrectly assigned levels were assigned too high (worse) than the "true" level (US 88 % vs. MRI 65 %)., Conclusion:  Reliable exact prenatal level determination by US and MRI is not possible. However, the prenatal determination of the anatomical level of the lesion is good within one level margin of error. Prenatal US as well as MRI demonstrate a systematic error towards higher levels. The above considerations must be integrated into prenatal counselling., Competing Interests: The authors declare that they have no conflict of interest., (Thieme. All rights reserved.)

Purpose:  The aim was to describe the sonographic follow-up of hindbrain herniation (HH), the banana and lemon sign after fetal myelomeningocele (fMMC) repair, and the time of disappearance of these signs after the intervention, and to investigate any predictive value for the necessity of shunting during the infant's first year of life. Additionally, the sonographic evolution of the transcerebellar diameter (TCD) before and after fetal intervention was assessed., Patients and Methods:  The first 50 patients that underwent fMMC repair at Zurich Center for Fetal Diagnosis and Therapy (www.swissfetus.ch) were included in this study. Sonographic scans performed weekly after fMMC repair focusing on HH and banana and lemon signs were analyzed and compared between the shunted and the non-shunted group. ROC curves were generated for the time intervals of resolution of the signs in order to show their predictive accuracy for the need for shunting until 1 year of age., Results:  HH resolved in 48 fetuses (96 %) before delivery. The sonographic disappearance of HH within the first two weeks after fMMC repair was associated with a significantly lower incidence of shunt placement (OR 0.19; 95 % CI 0.4-0.9) during the first year of life (p = 0.03). All fetuses with persistent HH before delivery received a shunt. TCD growth was observed in all fetuses., Conclusion:  The reversibility of HH within two weeks after fMMC repair is associated with an 80 % lower incidence of shunt placement during the infant's first year of life. Moreover, it allows the cerebellum to grow and to normalize its configuration., Competing Interests: The authors declare that they have no conflict of interest., (Thieme. All rights reserved.)

Purpose:  The aim of this study was to describe the sonographic evolution of fetal head circumference (HC) and width of the posterior horn of the lateral ventricle (Vp) after open fetal myelomeningocele (fMMC) repair and to assess whether pre- or postoperative measurements are helpful to predict the need for shunting during the first year of life., Patients & Methods:  All 30 children older than one year by January 2017 who previously had fMMC repair at the Zurich Center for Fetal Diagnosis and Therapy were included. Sonographic evolution of fetal HC and Vp before and after fMMC repair was assessed and compared between the non-shunted (N = 16) and the shunted group (N = 14). ROC curves were generated for the fetal HC Z-score and Vp in order to show their predictive accuracy for the need for shunting until 1 year of age., Results:  HC was not an independent factor for predicting shunting. However, the need for shunting was directly dependent on the preoperative Vp as well as the Vp before delivery. A Vp > 10 mm at evaluation for fMMC repair or > 15 mm before delivery identifies 100 % of the infants needing shunt placement at a false-positive rate of 44 % and 25 %, respectively. All fetuses with a Vp > 15 mm at first evaluation received a shunt., Conclusion:  Fetuses demonstrating a Vp of > 15 mm before in utero MMC repair are extremely likely to develop hydrocephalus requiring a shunt during the first year of life. This compelling piece of evidence must be appropriately integrated into prenatal counseling., Competing Interests: The authors declare that they have no conflict of interest., (Thieme. All rights reserved.)

Introduction: The Management of Myelomeningocele Study, a.k.a. the MOMS trial, was published in 2011 in the New England Journal of Medicine. This prospective randomized controlled trial proved to be a milestone publication that provided definitive evidence that fetal surgery is a novel standard of care for select fetuses with spina bifida aperta (SB). The goal of our study is to assess whether our center can match these benchmark results., Materials and Methods: Our study was conducted according to the MOMS protocol using the same inclusion and exclusion criteria and looked at the same outcome parameters that were used in the MOMS trial. Zurich and MOMS results were compared., Results: We enrolled 20 patients between December 2010 and May 2015 all of whom underwent fetal surgery for SB. Among 51 different outcome variables, there were only 3 favorable (multiplicity-adjusted) significant differences (gestational age at birth, hindbrain herniation, and psychomotor development). There were no statistically significant differences regarding any other parameters., Conclusion: Our findings confirm that rigorous apprenticeship, training, and comprehensive prospective data collection enable centers like the Zurich Center for Fetal Diagnosis and Therapy to achieve benchmark results for open fetal surgery for myelomeningocele and myeloschisis. These results justify the existence and continuation of our program. Outcome documentation is an essential element of quality management. It is medically and ethically fundamental for fetal medicine and surgery centers offering high-end innovative medical care., (© 2019 S. Karger AG, Basel.)

Introduction: Open fetal myelomeningocele (fMMC) repair is nowadays a therapeutic option in selected cases. We aimed to evaluate changes in maternal and fetal outcome after fMMC repair during the first 8 years of experience at a tertiary referral fetal medicine center in Switzerland. -Materials and Methods: Between 2010 and 2018, fMMC repair and delivery of the neonate via planned cesarean section was performed in 67 cases. Cases were retrospectively stratified into 2 groups: a "training phase" (TP) with supervision from an external surgeon during 11 operations (2010-2014, 15 cases) followed by an "experienced phase" (EP, 2014-2018, 52 cases); each phase lasted about 4 years. Both phases were compared with regard to various maternal and fetal outcome parameters., Results: Analyses did not reveal differences between TP and EP in major outcome parameters such as gestational age at delivery, chorionic membrane separation, or the incidence of placental abruption. Although more complex surgical techniques were applied in EP (e.g., dermal closure using a rotational flap), surgery time was not different from TP. At the same time, surgical complications such as oligohydramnios (27 vs. 8%, p = 0.046) with MRI-confirmed leakage (13 vs. 4%, nonsignificant) and subchorionic hematoma (20 vs. 2%, p = 0.009) were less common in EP than TP., Conclusions: This study shows that the level of competence at our center with regard to major perinatal outcome parameters was already high in the first years of fMMC repair. However, more complex surgical techniques and significantly less minor complications were observed during the most recent years., (© 2019 S. Karger AG, Basel.)

Introduction: Neurogenic lower urinary tract dysfunction (NLUTD) represents a severe burden for patients with open spina bifida (OSB). The effect of fetal OSB repair on the urological outcome remains unclear, as controversial data exist. The aim of this study was to further increment existing outcome data and to demonstrate that our earlier published positive preliminary results are not erratic., Methods: Data from standardized urological follow-up appointments of patients with fetal OSB repair operated at our center were analyzed. Data were obtained from urodynamic studies (UDSs) and radiologic exams performed in the newborn (gestational age 37-39 weeks), at ages of 6, 12, 18, and 24 months, and then at yearly intervals., Results: Of 82 patients (mean age 2.6 years, range 6 months to 7 years), 26 (32%) had a normal bladder function as demonstrated by UDSs. Of the 56 (68%) patients with NLUTD, 29 (51%) patients showed initially a normal UDS, but developed NLUTD in the follow-up, 19 (66%) of them spontaneously and another 10 (34%) in association with growth and development, or surgery of inclusion cysts. Radiologic abnormalities (upper tract dilatation and vesico-uretero-renal reflux) were seen in 15%, mainly patients with NLUTD., Conclusion: Our results add an important set of information to the existing body of evidence. The data reconfirm our earlier published favorable preliminary results and support other studies that show a possible benefit of prenatal OSB repair on the urological outcome, but they also demonstrate that the positive effect remains limited., (© 2020 The Author(s) Published by S. Karger AG, Basel.)

Background: Fetal myelomeningocele (fMMC) repair is a therapeutic option in selected cases. This study aimed to identify risk factors for preterm birth (PTB) following open fMMC repair., Methods: Sixty-seven women underwent fMMC repair and delivered a baby between 2010 and 2018 at our center. Demographic, surgical, and pregnancy complications, including potential risk factors for PTB such as preterm premature rupture of membranes (PPROM), chorioamniotic membrane separation (CMS), and placental abruption were evaluated., Results: Maternal body mass index, maternal age, parity, previous uterine surgery, gestational age at fetal surgery, total surgery duration, surgical subcutaneous hematoma, oligohydramnios, and amniotic fluid leakage were not identified as risk factors for PTB. CMS (p = 0.028, 92 vs. 52%) and PPROM (p = 0.001, 95 vs. 52%) were highly associated with PTB. Placental abruption was found more often in women after fMMC repair than in a general obstetrical population (12 vs. 1%) and ended in premature birth in all cases (p = 0.024, 100 vs. 60%). However, the majority of women delivered at a gestational age >35 weeks., Conclusions: In our study cohort, risk factors for PTB were PPROM, CMS, and placental abruption, whereas surgery duration did not influence outcome. We conclude that the surgery technique should aim to minimize CMS and amniotic fluid leakage., (© 2019 S. Karger AG, Basel.)

Introduction: Despite undoubtable benefits of open fetal myelomeningocele (fMMC) repair, there are considerable maternal risks. The aim of this study was to evaluate and systematically categorize maternal complications after open fMMC repair., Methods: We analyzed data of 40 fMMC repairs performed at the Zurich Center for Fetal Diagnosis and Therapy. Maternal complications were classified according to a 5-level grading system based on a classification of surgical complications proposed by Clavien and Dindo., Results: We observed no grade 5 complication (death of a patient). Five (12.5%) women demonstrated severe grade 4 complications: 1 case of uterine rupture in a nullipara at 36 gestational weeks (GW), a third-degree atrioventricular block which needed short mechanical resuscitation, a bilateral lung embolism requiring intensive care unit (ICU) management due to low-output syndrome, and chorioamnionitis and urosepsis both requiring ICU management at 31 GW. Twenty-six (65%) women had minor (grade 1-3) complications., Conclusions: Only one grade 4 complication (uterine rupture, 2.5%) was a clear-cut direct consequence of fetal surgery. The other four grade 4 complications (10%) occurred in the context of, but cannot unequivocally be attributed to, fetal surgery, since they may occur also in other circumstances. The classification system used is a tenable step towards stringent documentation of maternal complications., (© 2018 S. Karger AG, Basel.)

Background: One of the intraoperative challenges of fetal spina bifida repair is skin closure when there is an extended skin defect. Thus, we examined whether distally pedicled random pattern transposition flaps (TFs) are a valid option to overcome this problem., Subjects and Methods: All patients undergoing in utero repair of spina bifida with application of a TF for back skin closure were analyzed focusing on intraoperative flap characteristics and postoperative flap performance., Results: In 30 (70%) of the 43 fetuses a primary skin closure was achieved, in 5 (12%) a skin substitute was used, and in 8 (18%) a TF was applied. Flap raising and insertion was uneventful and perfusion was sufficient in all 8 fetuses (100%). In 3 fetuses (37%) the donor sites were closed primarily, and in 5 (63%) a skin substitute was used for coverage. At birth, 7 flaps were viable and provided robust skin coverage over the center of the former lesion. Complications included a small skin defect with CSF leakage in 1 patient (13%)., Conclusion: During open fetal spina bifida repair, TFs can be safely and efficaciously used to obtain solid and durable skin coverage over lesions too large to allow conventional primary skin closure., (© 2017 S. Karger AG, Basel.)

Objectives: To describe characteristics of foetuses undergoing in utero repair of open neural tube defects (ONTD) and assess postoperative evolution of posterior fossa and brain morphology., Methods: Analysis of pre- and postoperative foetal as well as neonatal MRI of 27 foetuses who underwent in utero repair of ONTD. Type and level of ONTD, hindbrain configuration, posterior fossa and liquor space dimensions, and detection of associated findings were compared between MRI studies and to age-matched controls., Results: Level of bony spinal defect was defined with exactness of ± one vertebral body. Of surgically confirmed 18 myelomeningoceles (MMC) and 9 myeloschisis (MS), 3 MMC were misdiagnosed as MS due to non-visualisation of a flat membrane on MRI. Hindbrain herniation was more severe in MS than MMC (p < 0.001). After repair, hindbrain herniation resolved in 25/27 cases at 4 weeks and liquor spaces increased. While posterior fossa remained small (p < 0.001), its configuration normalised. Lateral ventricle diameter indexed to cerebral width decreased in 48% and increased in 12% of cases, implying a low rate of progressive obstructive hydrocephalus. Neonatally evident subependymal heterotopias were detected in 33% at preoperative and 50% at postoperative foetal MRI., Conclusion: MRI demonstrates change of Chiari malformation type II (CM-II) features., Key Points: • Hindbrain herniation is significantly more pronounced in myeloschisis than in myelomeningocele • Resolution of hindbrain herniation 4 weeks after in utero closure of ONTD • MRI is valuable for preoperative assessment and postoperative evaluation following in utero repair.

Aims: Prenatal myelomeningocele (MMC) repair has been proven to significantly reduce the need for hydrocephalus shunting and improve lower-extremity motor outcomes. The aim of this study was to evaluate the effect of prenatal MMC repair on the urological outcome., Methods: All patients who underwent fetal MMC repair at our institution are followed prospectively. Assessments include medical history, renal and bladder ultrasound, voiding cystourethrogram and urodynamic study, need for clean intermittent catheterization (CIC) and anticholinergics, and the occurrence of urinary tract infections (UTI)., Results: Of the 30 patients who underwent prenatal MMC closure from December 2010 to December 2015, eight patients with a postnatal follow-up of at least 2 years were included in this study and compared with eight patients after postnatal MMC repair. The level of the bony spinal defect was similar in both groups. Urological evaluation at 2 years revealed normal bladder function in 50% after prenatal repair. Neurogenic bladder dysfunction requiring CIC and anticholinergic therapy was seen in 50% in the prenatal and in 100% in the postnatal group. Significant bladder wall thickening was found in 37.5% and 87.5%, respectively. Febrile UTIs occurred in 37.5% in the prenatal and 62.5% in the postnatal group during the observation period., Conclusions: Our data suggest a positive effect of prenatal MMC closure on lower urinary tract function. The long-term significance of these results remains unclear. Therefore, continued close monitoring of renal and bladder function are mandatory., (© 2016 Wiley Periodicals, Inc.)

Background: There are fetuses demonstrating very large myelomeningocele lesion which can not be covered with autochothonous skin., Material and Methods: We use Integra™ artificial skin for intrauterine coverage of the back lesion. A reverse latissimus dorsi flap was used postnatally to reinforce the repair site., Conclusion: Integra™ appears to be a suitable coverage for large soft tissue defects in utero. Moreover, a postnatal reverse latissimus dorsi flap appears to markedly strengthen tissue coverage over a spinal cord rescued in utero.