Management Of Myelomeningocele Study Research Articles

Fetal surgery for spina bifida – An upcoming standard of care

Open spina bifida or myelomeningocele (MMC) is one of the most common serious congenital malformations that have been historically treated with a repair after birth. However, this “repair” does not avert neurological injury, reverse hindbrain herniation, or prevent hydrocephalus. With the intention to reduce numerous medical procedures, lifelong morbidities, and medical complications, fetal surgery was proposed about a quarter century ago. Fetal surgery has become the standard of care for MMC, especially after the advent of the Management of Myelomeningocele Study (MOMS) trial. Dr. Michael Harrison from the University of California was the first to birth this idea in the 1980s. While earlier research was carried out on ovine models, it was deduced that intrauterine repair reduces the secondary damage to exposed neural tissue and improves post-natal neurological outcomes. After the promising results in animal models and some clinical series, it paved the way for a prospective multicenter randomized controlled trial that compared prenatal versus postnatal MMC repair, with the aim to prove its efficacy and safety with Level-1 evidence- the MOMS. However, it was stopped early due to the improved prenatal repair outcomes, thus establishing open fetal MMC repair as the standard of care. We aim to discuss the origin story of this remarkable procedure, its rationales and advantages for the unborn child and mother and its short-term and long-term superiority and limitations. The operative procedure and the criteria with other essential aids in its diagnosis have also been discussed briefly. We also intend to eliminate the apprehension faced toward this procedure by the supporting team of obstetricians, anesthetists, neonatologists, and radiologists essential to making this procedure a success.

Fetal Spina Bifida Repair in Obese Mothers: Is Maternal and Fetal Safety Compromised?

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.

Perforation of cavum septi pellucidi in open spina bifida and need for hydrocephalus treatment by 1 year of age.

In-utero repair of an open neural tube defect (ONTD) reduces the risk of developing severe hydrocephalus postnatally. Perforation of the cavum septi pellucidi (CSP) may reflect increased intraventricular pressure in the fetal brain. We sought to evaluate the association of perforated CSP visualized on fetal imaging before and/or after in-utero ONTD repair with the eventual need for hydrocephalus treatment by 1 year of age. This was a retrospective cohort study of consecutive patients who underwent laparotomy-assisted fetoscopic ONTD repair between 2014 and 2021 at a single center. Eligibility criteria for surgery were based on those of the Management of Myelomeningocele Study (MOMS), although a maternal prepregnancy body mass index of up to 40 kg/m2 was allowed. Fetal brain imaging was performed with ultrasound and magnetic resonance imaging (MRI) at referral and 6 weeks postoperatively. Stored ultrasound and MRI scans were reviewed retrospectively to assess CSP integrity. Medical records were reviewed to determine whether hydrocephalus treatment was needed within 1 year of age. Parametric and non-parametric tests were used as appropriate to compare outcomes between cases with perforated CSP and those with intact CSP as determined on ultrasound at referral. Logistic regression analysis was performed to assess the predictive performance of various imaging markers for the need for hydrocephalus treatment. A total of 110 patients were included. Perforated CSP was identified in 20.6% and 22.6% of cases on preoperative ultrasound and MRI, respectively, and in 26.6% and 24.2% on postoperative ultrasound and MRI, respectively. Ventricular size increased between referral and after surgery (median, 11.00 (range, 5.89-21.45) mm vs 16.00 (range, 7.00-43.5) mm; P < 0.01), as did the proportion of cases with severe ventriculomegaly (ventricular width ≥ 15 mm) (12.7% vs 57.8%; P < 0.01). Complete CSP evaluation was achieved on preoperative ultrasound in 107 cases, of which 22 had a perforated CSP and 85 had an intact CSP. The perforated-CSP group presented with larger ventricles (mean, 14.32 ± 3.45 mm vs 10.37 ± 2.37 mm; P < 0.01) and a higher rate of severe ventriculomegaly (40.9% vs 5.9%; P < 0.01) compared to those with an intact CSP. The same trends were observed at 6 weeks postoperatively for mean ventricular size (median, 21.0 (range, 13.0-43.5) mm vs 14.3 (range,7.0-29.0) mm; P < 0.01) and severe ventriculomegaly (95.0% vs 46.8%; P < 0.01). Cases with a perforated CSP at referral had a lower rate of hindbrain herniation (HBH) reversal postoperatively (65.0% vs 88.6%; P = 0.01) and were more likely to require treatment for hydrocephalus (89.5% vs 22.7%; P < 0.01). The strongest predictor of the need for hydrocephalus treatment within 1 year of age was lack of HBH reversal on MRI (odds ratio (OR), 36.20 (95% CI, 5.96-219.12); P < 0.01) followed by perforated CSP on ultrasound at referral (OR, 23.40 (95% CI, 5.42-100.98); P < 0.01) and by perforated CSP at 6-week postoperative ultrasound (OR, 19.48 (95% CI, 5.68-66.68); P < 0.01). The detection of a perforated CSP in fetuses with ONTD can reliably identify those cases at highest risk for needing hydrocephalus treatment by 1 year of age. Evaluation of this brain structure can improve counseling of families considering fetal surgery for ONTD, in order to set appropriate expectations about postnatal outcome. © 2023 International Society of Ultrasound in Obstetrics and Gynecology.

Micro-Surgical Treatment of Fetal Myelomeningocele

Abstract Introduction Myelomeningocele (MMC) is the most common malformation of the central nervous system compatible with life. We will report the results obtained with the prenatal closure of MMC at the Instituto Estadual do Cérebro Paulo Niemeyer (IECPN). Objectives Clinical outcome of fetuses undergoing intrauterine MMC repair by the Peralta mini-hysterotomy. Monitor the reduction of Arnold-Chiari II secondary to MMC, reduction of hydrocephalus and also motor development in these children. Methods Descriptive study of 26 cases with intrauterine MMC repair by mini-hysterotomy, or Peralta technique, performed at the IECPN from December 2017 to February 2020. Results Between December 2017 and February 2020, 26 pregnant women with children with MMC were operated on using Peralta technique. Fetuses were born at an average gestational age of 34.2 weeks and 8% were born before 30 weeks of gestation. The average birth weight was 2096g. It was possible to observe a significant reduction in the occurrence of Arnold-Chiari II in these patients, as well as an evident improvement in motor function in the neurological examination of these babies at the end of the first month of life, where 20 of 23 babies had active movement in the lower limbs. Discussion This study demonstrates the correction of fetal MMC through a mini-hysterotomy of 2.5 to 3.5 cm, developed in order to reduce maternal and fetal mortality. This mini-hysterotomy technique is not a minimally invasive procedure, as it is based on open surgery for the treatment of fetal MMC, as recommended by the Management of Myelomeningocele Study (MOMS).

Postnatal Myelomeningocele Repair in the United States: Rates and Disparities Before and After the Management of Myelomeningocele Study Trial.

Evolving technologies have influenced the practice of myelomeningocele repair (MMCr), including mandatory folic acid fortification, advances in prenatal diagnosis, and the 2011 Management of Myelomeningocele Study (MOMS) trial demonstrating benefits of fetal over postnatal MMCr in select individuals. Postnatal MMCr continues to be performed, especially for those with limitations in prenatal diagnosis, health care access, anatomy, or personal preference. A comprehensive, updated national perspective on the trajectory of postnatal MMCr volumes and patient disparities is absent. We characterize national trends in postnatal MMCr rates before and after the MOMS trial publication (2000-2010 vs 2011-2019) and examine whether historical disparities persist. This retrospective, cross-sectional analysis queried Nationwide Inpatient Sample data for postnatal MMCr admissions. Annual and race/ethnicity-specific rates were calculated using national birth registry data. Time series analysis assessed for trends relative to the year 2011. Patient, admission, and outcome characteristics were compared between pre-MOMS and post-MOMS cohorts. Between 2000 and 2019, 12 426 postnatal MMCr operations were estimated nationwide. After 2011, there was a gradual, incremental decline in the annual rate of postnatal MMCr. Post-MOMS admissions were increasingly associated with Medicaid insurance and the lowest income quartiles, as well as increased risk indices, length of stay, and hospital charges. By 2019, race/ethnicity-adjusted rates seemed to converge. The mortality rate remained low in both eras, and there was a lower rate of same-admission shunting post-MOMS. National rates of postnatal MMCr gradually declined in the post-MOMS era. Medicaid and low-income patients comprise an increasing majority of MMCr patients post-MOMS, whereas historical race/ethnicity-specific disparities are improving. Now more than ever, we must address disparities in the care of MMC patients before and after birth.

Long-Term Imaging Follow-up from the Management of Myelomeningocele Study.

Short-term results demonstrate that prenatal repair of a myelomeningocele is associated with a reduction in hydrocephalus and an increased likelihood of the reversal of Chiari II malformations compared with postnatal repair. The purpose of this study was to identify the long-term imaging findings at school age among subjects who underwent pre- versus postnatal repair of a myelomeningocele. A subset of subjects enrolled in the Management of Myelomeningocele Study who underwent either prenatal (n = 66) or postnatal (n = 63) repair of a lumbosacral myelomeningocele and had follow-up brain MR imaging at school age were included. The prevalence of posterior fossa features of Chiari II malformation and supratentorial abnormalities and the change in these findings from fetal to school-age MR imaging were compared between the 2 groups. Prenatal repair of a myelomeningocele was associated with higher rates of normal location of fourth ventricle and lower rates of hindbrain herniation, cerebellar herniation, tectal beaking, brainstem distortion, and kinking at school age compared with postnatal repair (all P < .01). Supratentorial abnormalities, including corpus callosal abnormalities, gyral abnormalities, heterotopia, and hemorrhage, were not significantly different between the 2 groups (all P > .05). The rates of resolution of brainstem kinking, tectal beaking, cerebellar and hindbrain herniation, and normalization of fourth ventricle size from fetal to school age MR imaging were higher among the prenatal compared with postnatal surgery group (all, P < .02). Prenatal repair of a myelomeningocele is associated with persistent improvement in posterior fossa imaging findings of Chiari II malformation at school age compared with postnatal repair.

The value and limitations of using predetermined criteria in decision making for maternal-fetal interventions.

Maternal-fetal interventions &#x2013; such as prenatal fetal myelomeningocele (MMC) repair &#x2013; are at the forefront of clinical innovation within maternal-fetal medicine, pediatric surgery, and neonatology. Many centers determine eligibility for innovative procedures using inclusion and exclusion criteria based on seminal studies, such as the &#x201C;Management of Myelomeningocele Study&#x201D; for prenatal MMC repair. What if a person&#x2019;s clinical presentation does not conform to predetermined criteria for maternal-fetal intervention? Does changing criteria on a case-by-case basis (i.e., ad hoc) constitute innovation in practice and flexible personalized care or transgression of commonly held standards with potential negative consequences? We outline a principle-based, bioethically-justified method for answering these questions using fetal MMC repair as an example. We give special attention to the historical origins of inclusion and exclusion criteria; risks and benefits to the pregnant person and the fetus; and team dynamics. This article is protected by copyright. All rights reserved.

Effect of allograft patch closure on incidence of spinal inclusion cyst formation following open fetal myelomeningocele repair.

The aim of this study was to evaluate the incidence of spinal inclusion cyst (sIC) formation after open fetal myelomeningocele (fMMC) repair and the effect of dural patch closure. The authors conducted a retrospective review of patients who underwent open fMMC repair at their institution between March 2011 and June 2020. All patients met the criteria for intervention defined by the Management of Myelomeningocele Study (MOMS). The primary outcomes investigated were development of sIC and need for surgical intervention. Secondary outcomes included need for CSF diversion, extent of reversal of hindbrain herniation, and ambulatory status. Of 56 patients who underwent open fMMC repair, 52 had adequate spinal imaging for review. Twelve of these patients (23%) developed sIC (95% CI 0.11-0.35). Six patients experienced symptoms and required surgical detethering with sIC resection. Six additional patients had evidence of sIC on surveillance MRI but remained asymptomatic. The authors found a statistically significant relationship between the use of a dural allograft patch and sIC formation (p = 0.05). In terms of sIC development, there was no statistically significant difference between patients who underwent primary closure and those who received an allograft at the level of the fascia (p = 0.34) or skin (p = 0.26). The rate of hydrocephalus requiring CSF diversion was 52%. Interestingly, 98% of patients had improvement in extent of hindbrain herniation. Dural patch closure did not have any effect on the rate of progressive hydrocephalus (p = 0.33) or degree of reversal of hindbrain herniation (p > 0.99). This study suggested that children with prenatally repaired MMC are at higher risk for development of sIC and associated symptoms than those who undergo postnatal repair. The presentation of symptoms was also earlier in these patients than previously reported after postnatal repair. The use of a dural allograft patch appears to have a positive correlation with sIC formation. Future investigations evaluating the incidence of sIC after fetoscopic MMC repair, in which primary dural closure typically cannot be achieved and a dural patch is most often utilized, will be helpful in facilitating prenatal counseling for patients considering fetal intervention.

Hydrocephalus and school-age neurodevelopmental outcomes in the management of myelomeningocele prenatal surgery trial: a secondary analysis.

The purpose of this secondary analysis was to assess the role of hydrocephalus on neurodevelopmental outcomes in a cohort of school-age children enrolled in the Management of Myelomeningocele Study (MOMS) clinical trial. The sample analyzed in this report consisted of 150 of 183 children aged 5-10 years (mean ± SD 7 years 8 months ± 1.2) who were randomly assigned between 20 and 26 weeks of gestational age to undergo either prenatal or postnatal surgery and were enrolled in the school-age follow-up study of MOMS. These 150 children (76 prenatal and 74 postnatal) were placed into three groups: no hydrocephalus (n = 22), unshunted hydrocephalus (n = 31), and shunted hydrocephalus (n = 97). Comparisons were made on the basis of measures of adaptive behavior, intelligence, reading and math skills, verbal and nonverbal memory, fine motor dexterity, and sensorimotor skills. Parent ratings of executive functions, inattention, and hyperactivity-impulsivity were also compared. There were no statistically significant differences in neurodevelopmental outcomes between the groups with no hydrocephalus and unshunted hydrocephalus, or between the prenatal and postnatal groups with shunted hydrocephalus, so these groups were combined (no/unshunted vs shunted hydrocephalus). The no/unshunted group showed significantly better performance (p < 0.05) than the shunted group in terms of adaptive behavior, intelligence, verbal and nonverbal memory, reading skills (but not math), fine motor dexterity, sensorimotor skills (but not visual-motor integration), and inattention (but not hyperactivity-impulsivity or executive function ratings). An assessment of the prenatal surgery group showed that the combined no/unshunted group performed better than the shunted group in terms of adaptive behavior and verbal memory skills. Both the prenatal and postnatal surgery subgroups with unshunted hydrocephalus performed as well as the group with no hydrocephalus despite significantly enlarged ventricles. Although the primary assessment of school-age outcomes in the MOMS clinical trial did not show better adaptive behavior and cognitive skills in the prenatal group, hydrocephalus and shunting were associated with poorer neurodevelopmental outcomes (both prenatal and postnatal groups). Disease severity and dynamic changes in hydrocephalus status may be the primary factors in the need for shunting and a major determinant of adaptive behavior and cognitive outcomes after prenatal surgery.

The significance of hindbrain herniation reversal following prenatal repair of neural tube defects.

The aim of this study was to determine whether reversal of hindbrain herniation (HBH) on MRI following prenatal repair of neural tube defects (NTDs) is associated with reduced rates of ventriculoperitoneal (VP) shunt placement or endoscopic third ventriculostomy (ETV) within the 1st year of life. This is a secondary analysis of prospectively collected data from all patients who had prenatal open repair of a fetal NTD at a single tertiary care center between 2012 and 2020. Patients were offered surgery according to inclusion criteria from the Management of Myelomeningocele Study (MOMS). Patients were excluded if they were lost to follow-up, did not undergo postnatal MRI, or underwent postnatal MRI without a report assessing hindbrain status. Patients with HBH reversal were compared with those without HBH reversal. The primary outcome assessed was surgical CSF diversion (i.e., VP shunt or ETV) within the first 12 months of life. Secondary outcomes included CSF leakage, repair dehiscence, CSF diversion prior to discharge from the neonatal intensive care unit (NICU), and composite neonatal morbidity. Demographic, prenatal sonographic, and operative characteristics as well as outcomes were assessed using standard univariate statistical methods. Multivariate logistic regression models were fit to assess for independent contributions to the primary and secondary outcomes. Following exclusions, 78 patients were available for analysis. Of these patients, 38 (48.7%) had HBH reversal and 40 (51.3%) had persistent HBH on postnatal MRI. Baseline demographic and preoperative ultrasound characteristics were similar between groups. The primary outcome of CSF diversion within the 1st year of life was similar between the two groups (42.1% vs 57.5%, p = 0.17). All secondary outcomes were also similar between groups. Patients who had occurrence of the primary outcome had greater presurgical lateral ventricle width than those who did not (16.1 vs 12.1 mm, p = 0.02) when HBH was reversed, but not when HBH was persistent (12.5 vs 10.7 mm, p = 0.49). In multivariate analysis, presurgical lateral ventricle width was associated with increased rates of CSF diversion before 12 months of life (adjusted OR 1.18, 95% CI 1.03-1.35) and CSF diversion prior to NICU discharge (adjusted OR 1.18, 95% CI 1.02-1.37). HBH reversal was not associated with decreased rates of CSF diversion in this cohort. Predictive accuracy of the anticipated benefits of prenatal NTD repair may not be augmented by the observation of HBH reversal on MRI.

Primary vs patch-based skin closure for in-utero spina bifida repair.

During in-utero spina bifida (SB) repair, closure of large defects is often challenging, requiring tissue graft for watertight skin closure. No prior studies have compared primary skin closure vs patch-based repair. Our objective was to compare neonatal and 1-year outcomes associated with these two types of skin closure for in-utero SB repair. This was a prospective cohort study of 102 patients undergoing open prenatal SB repair from September 2011 to August 2021 at a single institution. All patients met the inclusion criteria of the Management of Myelomeningocele Study (MOMS), and the surgical procedure for in-utero SB repair was similar to that described in the MOMS trial. During the surgery, if primary skin approximation was not feasible due to the large size of the defect, the decision was at the discretion of the pediatric neurosurgeon to utilize a patch for closure. Neonatal outcomes at birth and 1-year outcomes were compared between the primary skin and patch-based closure groups. Of 102 patients included in the study, 70 (68.6%) underwent primary skin closure and 32 (31.4%) patch-based closure. The patch type included acellular bovine skin matrix (Durepair®; n = 31) and human acellular dermal matrix (Alloderm®; n = 1). Fetuses with myeloschisis were more likely to require patch-based repair than those with myelomeningocele. The median time of fetal repair was 4 min longer for patch-based compared with primary skin closure (37 vs 33 min; P = 0.001). Following patch-based repair, neonates had a longer length of stay in the neonatal intensive care unit (NICU) by 24 days (adjusted risk ratio, 2.40 (95% CI, 1.41-4.29)) compared to those that underwent primary skin closure. There was no difference between the two groups in the other neonatal outcomes, including the need for ventriculoperitoneal shunt placement and cerebrospinal fluid leakage. Outcome at 1 year of age was available for 90 infants. Need for wound revision within their first year after birth was more common in infants who underwent patch-based vs those with primary skin closure (19.4% vs 5.1%; P = 0.05). There was no difference between the two groups in other 1-year outcomes, including the need for ventriculoperitoneal shunt placement by 1 year of age and surgery for tethered cord. Patch-based closure during SB repair is often needed in fetuses with myeloschisis and is associated with prolonged fetal surgery time, long NICU stay and need for wound revision within the first year after birth. Further studies are required to identify optimal patches for SB repair or alternative methods to improve outcome. © 2022 International Society of Ultrasound in Obstetrics and Gynecology.

Experience of 300 Cases of Prenatal Fetoscopic Open Spina Bifida Repair: Report of the International Fetoscopic Neural Tube Defect Repair Consortium

(Am J Obstet Gynecol. 2021;225:678.e1–678.e11. [Epub June 3, 2021]) Prenatal repair of open spina bifida (OSB) showed increased chance of independent ambulation and increased outcomes of ventriculoperitoneal shunting compared with postnatal repair in a multicenter randomized controlled trial in the Management of Myelomeningocele Study (MOMS). The technique, involving a hysterotomy, has risks regarding the uterine incision used. Due to the risks, fetal surgeons worldwide have been attempting to repair OSB using minimally invasive techniques to mitigate adverse outcomes and improve neonatal neurologic benefits. One such technique is fetoscopic OSB repair.

Biomedical Engineering Advancements after Management of Myelomeningocele Study (MOMS): A Narrative Review

Spina bifida is a neural tube defect resulting from an incomplete closure of the caudal neuropore. The most debilitating form of spina bifida, myelomeningocele (MMC), can present with Chiari II malformation with concomitant hydrocephalus, bowel and bladder abnormalities, and impaired motor function of the lower limbs. The incidence rate of spina bifida is 3.4 per 10,000 live births reported within the US. On average, the US spends $1,176,000,000 annually on patient management and treatment. Advancements in the standard therapy, namely prenatal intervention pioneered by the Management of Myelomeningocele Study (MOMS), have aimed to reduce maternal and fetal complications, and yet complications were increased, calling for the need of further improvements. Beyond current standard interventions for MMC, the most promising developments have employed various biomedical methods ranging from isolated stem cell injections to biodegradable scaffold patches. These scaffolds can be biologic or synthetic and are often incorporated with bioactive proteins or stem cells. This review discusses the benefits and limitations of post-MOMS era biomedical engineering intervention articles found in 3 medical and biomedical databases consisting of systematic reviews, meta-analyses, randomized control trials, and experimental studies. After analysis of the advancements and limitations of these studies, a combination of materials could create a superior scaffold possessing watertight impermeability and cytocompatibility for successful coverage and host integration with minimal inflammation. Coupled with minimally invasive intra-amniotic injection delivery, an earlier mitigation could further prevent progression of poor neurologic outcomes, and possibly even regenerate neuronal tissue in patients with MMC.

OP054: Chromosomal microarray analysis for open neural tube defect: The prevalence of significant results and implications on in utero repair

Open neural tube defects (ONTD) are the second-most-common major congenital anomaly, and have been associated with environmental insults or genetic factors. Prenatal screening and diagnosis are widely available, and in utero repair has demonstrated superior outcomes for eligible patients. The Management of Myelomeningocele Study (MOMS) required a normal karyotype as one of the eligibility criteria for in utero repair. However, current guidelines recommend genetic testing by chromosomal microarray analysis (CMA) when a major congenital anomaly is detected prenatally.

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

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

Fetal Tracheal Occlusion for Congenital Diaphragmatic Hernia

(N Engl J Med. 2021;385:177–178) Studies on prenatal surgical interventions have historically been limited due to rarity and potential maternal complications. In 2011, the Management of Myelomeningocele Study (MOMS) found neonatal benefits associated with fetal surgery, including reduced need for cerebrospinal fluid shunting and improved infant motor outcomes at 30 months of age, but also found an increased risk of premature birth and maternal complications. This study provided an important framework for studies on fetal surgery: the inclusion of short- and long-term neonatal outcomes, attention to maternal complications, careful patient selection, and the use of multiple care centers.

Preterm birth after in-utero spina bifida repair: outcomes and risk factors

The Management of Myelomeningocele Study (MOMS) transformed practice by demonstrating that prenatal myelomeningocele (MMC) repair lowers the risk of death or shunt (ventriculoperitoneal diversion or endoscopic third ventriculostomy) procedures by 12 months of age (primary outcomes) compared to postnatal repair. Preterm birth (PTB) remains a major complication of in-utero spina bifida repair. The objective of our study was to correlate gestational age (GA) at delivery with the primary outcomes and assess the associated risk factors impact on the former findings.

Pseudomeningocele after in utero repair of myelomeningocele as an early sign of hypertensive hydrocephalus – a case report and review of the literature

Background Myelomenigocele (MMC) is the most common congenital defect of the spine. The Management of Myelomeningocele study (MOMS trial) demonstrated that the prenatal repair decreased shunt implant, reversal of hindbrain herniation and better neurologic function compared to postnatal repair. Several ultrasound findings can predict the risk of postnatal hydrocephalus after intrauterine MMC repair. This report shows a prenatal pseudomenigocele after intrauterine correction of MMC as an early sign of hydrocephalus.&#x0D; Method A 34-year-old female G2P1 with a prenatal diagnosis of MMC with anatomical level L4 and ventricular enlargement was submitted to open surgery intrauterine repair. Follow up ultrasound showed regression of the lemon sign, partial regression of hindbrain herniation and a progressive increase in the wound with local bulging characterizing a pseudomingocele. In the postnatal period, after correction of the pseudomenigocele, the neonate showed signs of hipertensive hydrocephalus. After ventriculoperitoneal shunt, the patient was discharged.&#x0D; Conclusion Presence of pseudomeningocele prenatally after in utero repair of MMC may represent an early sign of hypertensive hydrocephalus.

Postnatal Repair of Open Neural Tube Defects: A Single Center with 90-Month Interdisciplinary Follow-Up.

After publication of the Management of Myelomeningocele Study (MOMS) there is confusion regarding which treatment of open neural tube defects (NTD) is best. We report our results of postnatally repaired open NTDs born between 2007–2018 (n = 36) in critical reflection of the MOMS study. Neurosurgical, orthopedic, and urologic data were assessed. We also introduce a new entity: “status post prenatal repair”. FU ranged from 29 to 161 months (mean: 89.1 m) in 7 cases of myeloschisis and 24 myelomeningoceles in the final collective n = 31. The shunt rate was 41.9%, and the endoscopic third ventriculostomy rate was 16.1%. Hydrocephalus requiring treatment was not associated with the anatomical level, but with premature birth (p = 0.048). Myeloschisis was associated with shunt placement (p = 0.008). ROC analysis revealed birth <38.5th week predicts the necessity for hydrocephalus treatment (sensitivity: 89%; specificity: 77%; AUC= 0.71; p = 0.055). Eight (25.8%), patients are wheelchair-bound, 2 (6.5%) ambulate with a posterior walker, 10 (32.3%) with orthosis and 11 (35.5%) independently. One (3.2%) patient underwent detethering at 5.5 years. A total of three patients underwent five Chiari decompressions (9.6%). Further, nineteen orthopedic procedures were performed in nine patients (29.0%). A total of 17 (54.8%) patients self-catheterize, which was associated with an anatomical lesion at L3 or below (p = 0.032) and 23 (74.2%) take anticholinergic medication. In conclusion, shunt dependency is associated with myeloschisis, not with the anatomical defect level. Hydrocephalus treatment is associated with premature birth. In this postnatal cohort with significantly longer follow-up data than the MOMs study, the ambulation rate is better, the shunt rate lower and the secondary tethered cord rate better compared to the MOMS study.

Implementation Process and Evolution of a Laparotomy-Assisted 2-Port Fetoscopic Spina Bifida Closure Program

Introduction: Prenatal closure of open spina bifida via open fetal surgery improves neurologic outcomes for infants in selected pregnancies. Fetoscopic techniques that are minimally invasive to the uterus aim to provide equivalent fetal benefits while minimizing maternal morbidities, but the optimal technique is undetermined. We describe the development, evolution, and feasibility of the laparotomy-assisted 2-port fetoscopic technique for prenatal closure of fetal spina bifida in a newly established program. Methods: We conducted a retrospective cohort study of women consented for laparotomy-assisted fetoscopic closure of isolated fetal spina bifida. Inclusion and exclusion criteria followed the Management of Myelomeningocele Study (MOMS). Team preparation involved observation at the originating center, protocol development, ancillary staff training, and surgical rehearsal using patient-matched models through simulation prior to program implementation. The primary outcome was the ability to complete the repair fetoscopically. Secondary maternal and fetal outcomes to assess performance of the technique were collected prospectively. Results: Of 57 women screened, 19 (33%) consented for laparotomy-assisted 2-port fetoscopy between February 2017 and December 2019. Fetoscopic closure was completed in 84% (16/19) cases. Over time, the technique was modified from a single- to a multilayer closure. In utero hindbrain herniation improved in 86% (12/14) of undelivered patients at 6 weeks postoperatively. Spontaneous rupture of membranes occurred in 31% (5/16) of fetoscopic cases. For completed cases, median gestational age at birth was 37 (range 27–39.6) weeks and 50% (8/16) of women delivered at term. Vaginal birth was achieved in 56% (9/16) of patients. One newborn had a cerebrospinal fluid leak that required postnatal surgical repair. Conclusion: Implementation of a laparotomy-assisted 2-port fetoscopic spina bifida closure program through rigorous preparation and multispecialty team training may accelerate the learning curve and demonstrates favorable obstetric and perinatal outcomes.