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Introduction: Myelomeningocele (MMC) is the most common neural tube defect, but rarely seen in premature infants. Most centers advocate for closure of MMC within 24 h of birth. However, this is not always possible in severely premature infants. Given the rarity of this patient population, we aimed to share our institutional experience and outcomes of severely premature infants with MMC., Methods: We performed a retrospective, observational review of premature infants (≤ 32 weeks gestational age) identified through our multidisciplinary spina bifida clinic (1995-2021) and surgical logs. Descriptive statistics were compiled about this sample including timing of MMC closure and incidence of adverse events such as sepsis, CSF diversion, meningitis, and death., Results: Eight patients were identified (50% male) with MMC who were born ≤ 32 weeks gestational age. Mean gestational age of the population was 27.3 weeks (SD 3.5). Median time to MMC closure was 1.5 days (IQR = 1-80.8). Five patients were taken for surgery within the recommended 48 h of birth; 2 patients underwent significantly delayed closure (107 and 139 days); and one patient's defect epithelized without surgical intervention. Six of eight patients required permanent cerebrospinal fluid (CSF) diversion (2 patients were treated with ventriculoperitoneal shunting (VPS), three were treated with endoscopic third ventriculostomy (ETV) with choroid plexus cauterization (CPC) and 1 patient treated with ETV; mean of 3 years after birth, ranging from 1 day to 16 years). Two patients required more than one permanent CSF diversion procedure. Two patients developed sepsis (defined as meeting at least 2/4 SIRS criteria). In both cases of sepsis, patients developed signs and symptoms more than 72 h after birth. Notably, both instances of sepsis occurred unrelated to operative intervention as they occurred before permanent MMC closure. Two patients had intraventricular hemorrhage (both grade III). No patients developed meningitis (defined as positive CSF cultures) prior to MMC closure. Median follow up duration was 9.7 years. During this time epoch, 3 patients died: Two before 2 years of age of causes unrelated to surgical intervention. One of the two patients with grade III IVH died within 24 h of MMC closure., Conclusions: In our institutional experience with premature infants with MMC, some patients underwent delayed MMC closure. The overall rate of meningitis, sepsis, and mortality for preterm children with MMC was similar to MMC patients born at term., (© 2024. The Author(s).)

Objective: Myelomeningocele (MMC) is a lifelong condition requiring complex multidisciplinary management. Using the National Spina Bifida Patient Registry (NSBPR), the authors tested the association between sociodemographic variables and odds of undergoing neurosurgical procedures., Methods: The authors extracted sociodemographic, clinical, and neurosurgical procedure data on participants with MMC aged ≥ 1 year who visited an NSBPR clinic between 2009 and 2020. The zip code of the participant's residence at the time of the last spina bifida clinic visit was linked to the Distressed Communities Index (DCI) tier. Multivariate models were built to identify factors associated with undergoing CSF diversion, shunt revision, tethered cord release (TCR), and Chiari decompression., Results: There were 7924 participants with a median visit age of 13 years (IQR 7-20 years); 49.1% were male, 30.2% were non-Hispanic Black or Hispanic, 54.5% had public/supplemental insurance, and 16.9% were from distressed communities. CSF diversion, shunt revision, TCR, and Chiari decompression were performed in 81.8%, 47.7%, 22.9%, and 8.7% of participants, respectively. In multivariate analyses controlling for age, sex, insurance, DCI tier, lesion level, and surgical closure timing, Hispanic individuals were less likely than their non-Hispanic White counterparts to undergo shunt revision (p = 0.013). Non-Hispanic Black and Hispanic individuals were less likely to undergo TCR (p < 0.001 each) or Chiari decompression (p < 0.001 each). Compared with privately insured individuals, publicly insured individuals were more likely to undergo CSF diversion (p = 0.031). Those in distressed communities had increased odds of undergoing CSF diversion (p = 0.004) than those in prosperous communities., Conclusions: Among individuals with MMC participating in the NSBPR, there were differences in receiving neurosurgical procedures by race/ethnicity, insurance type, and DCI tier. Additional prospective studies are necessary to elucidate the reasons for these variations and their impact on long-term outcomes for this patient population in order to created targeted interventions.

Objective: Reports on the management and survival of children with myelomeningocele defects in Bangladesh are limited. This study describes the characteristics and outcomes of these children, focusing on the timing of surgical repair and factors affecting survival., Methods: We enrolled patients with myelomeningoceles in a case-control study on arsenic exposure and spina bifida in Bangladesh. Cases were subsequently followed at regular intervals to assess survival. Demographic, clinical, and surgical characteristics were reviewed. Univariate tests identified factors affecting survival., Results: Between 2016 and 2022, we enrolled 272 patients with myelomeningocele. Postnatal surgical repair was performed in 63% of cases. However, surgery within 5 days after birth was infrequent (<10%) due to delayed presentation, and there was a high rate (29%) of preoperative deaths. Surgical repair significantly improved patient survival (P < 0.0001). Older age at time of surgery was also associated with improved survival rates, which most likely represents that those who survived to older ages prior to surgery accommodated better with their lesions. Patients who presented with ruptured lesions had lower survival rates., Conclusions: Timely neurosurgical repair of myelomeningoceles in Bangladesh is hindered by late patient presentation, resulting in a high preoperative patient death rate. Neurosurgical intervention remains a significant predictor of survival. Increased access to neurosurgical care and education of families and non-neurosurgical providers on the need for timely surgical intervention are important for improving the survival of infants with myelomeningoceles., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Open spina bifida, also known as myelomeningocele (MMC), is the most challenging and severe birth defect of the central nervous system compatible with life and it is due to a failure in the dorsal fusion of the nascent neural tube during embryonic development. MMC is often accompanied by a constellation of collateral conditions, including hydrocephalus, Arnold - Chiari II malformation, brainstem disfunction, hydrosyringomyelia, tethered cord syndrome and scoliosis. Beyond early surgical repair of the dorsal defect, MMC requires lifelong cares. Several additional surgical procedures are generally necessary to improve the long-term outcomes of patients affected by MMC and multidisciplinary evaluations are crucial for early identification and management of the various medical condition that can accompany this pathology. In this chapter, the most common pathological entities associated with MMC are illustrated, focusing on clinical manifestations, treatment strategies and follow up recommendations., (© 2024. The Author(s), under exclusive license to Springer Nature Switzerland AG.)

More than 30 years have elapsed since it was recognised that folic acid supplementation could substantially reduce the risk of open neural tube defects (ONTDs). During that time, many countries have adopted policies of food fortification with demonstrable reduction in the incidence of both cranial and spinal ONTDs. Improved prenatal detection and termination has also resulted in a reduction in the number of affected live births. Nonetheless, in the USA about 1500 children, and in the UK around 500 children are born each year with myelomeningocele (MMC) and so the management of MMC and its complications continues to constitute a significant clinical workload for many paediatric neurosurgical units around the world.Until recently, the options available following antenatal diagnosis of MMC were termination of pregnancy or postnatal repair. As a result of the MOMS trial, prenatal repair has become an additional option in selected cases (Adzick et al., N Engl J Med 364(11):993-1004, 2011). Fetal surgery for myelomeningocele is now offered in more than 30 centres worldwide. The aim of this chapter is to review the experimental basis of prenatal repair of MMC, to critically evaluate the neurosurgical implications of this intervention and to describe the technique of 'open' repair, comparing this with emerging minimally invasive alternatives., (© 2023. The Author(s), under exclusive license to Springer Nature Switzerland AG.)

Objectives: To determine factors influencing patients to choose prenatal or postnatal repair of their child's myelomeningocele (MMC) when both treatment options are offered., Methods: We distributed a retrospective survey via email and social media to parents of children with MMC who were offered both prenatal and postnatal surgery as intervention options., Results: A total of 127 surveys met all inclusion criteria. The majority of responders considered partner's opinion (85%), maternal risks of prenatal surgery (71%), and risk for preterm labor (76%) as influencers. Financially, the costs of childcare (39.4% postnatal, 13.8% prenatal, p = 0.002), relocation (57.6% postnatal, 36.2% prenatal, p = 0.019), and travel (51.5% postnatal, 33% prenatal, p = 0.033) were more influential for the postnatal group while insurance coverage (36.4% postnatal, 68.1% prenatal, p = 0.003) was more influential to the prenatal group. Of the medical factors, the consideration of maternal risk for transfusion was different between surgical groups (39.4% postnatal, 18.1% prenatal, p = 0.015). The open responses suggest that both surgical groups found the quality of life for the baby to be significant to their decision-making., Conclusions: These findings should guide providers to tailor counseling to patient's needs. Comprehensive counseling should include information on financial resources, referral to financial counselors, and psychosocial support services., (© 2021 John Wiley & Sons Ltd.)

Aim: To compare the frequencies of neurosurgical procedures to treat comorbid conditions of myelomeningocele in patients who underwent fetal surgery versus postnatal surgery for closure of the placode., Method: By utilizing the National Spina Bifida Patient Registry in a comparative effectiveness study, 298 fetal surgery patients were matched by birthdate (±3mo) and spina bifida clinic site with one to three postnatal surgery patients (n=648). Histories were obtained by record review on enrollment and yearly subsequently. Multivariable Poisson regression was used to compare frequencies of procedures between cohorts, with adjustments for sex, ethnicity, insurance status, spinal segmental level of motor function, age at last visit recorded in the Registry, and, for shunt revision in shunted patients, age at cerebrospinal fluid (CSF) diversion., Results: The median age at last visit was 4 years. In fully adjusted analyses in patients aged at least 12 months old, fetal surgery was associated with decreased frequency of CSF diversion for hydrocephalus by ventriculoperitoneal shunt insertion or endoscopic third ventriculostomy compared with postnatal surgery (46% vs 79%; incidence rate ratio=0.61; 95% confidence interval [CI] 0.53-0.71; p<0.01). Over all ages, fetal surgery was associated with decreased frequency of Chiari decompression for brainstem dysfunction (3% vs 7%; incidence rate ratio=0.41; 95% CI 0.19-0.88; p=0.02). Also over all ages, differences were not significant in frequencies of shunt revision in shunted patients (53% vs 55%; incidence rate ratio=0.87; 95% CI 0.69-1.11; p=0.27), nor tethered cord release for acquired spinal cord dysfunction (18% vs 16%; incidence rate ratio=1.11; 95% CI 0.84-1.47; p=0.46)., Interpretation: Even with the variations inherent in clinical practice, fetal surgery was associated with lower frequencies of CSF diversion and of Chiari decompression, independent of covariates. What this paper adds Fetal surgery was associated with lower frequencies of cerebrospinal fluid diversion and decompression of Chiari II malformation than postnatal surgery. Frequencies of ventriculoperitoneal shunt revision and tethered cord release were not significantly different between cohorts., (© 2021 Mac Keith Press.)

Since the completion of the Management of Myelomeningocoele Study, maternal-fetal surgery for spina bifida has become a valid option for expecting parents. More recently, multiple groups are exploring a minimally invasive approach and recent outcomes have addressed many of the initial concerns with this approach. Based on a previously published framework, we attempt to delineate the developmental stage of the surgical techniques. Furthermore, we discuss the barriers of performing randomized controlled trials comparing two surgical interventions and suggest that data collection through registries is an alternative method to gather high-grade evidence., (© 2021 The Authors. Prenatal Diagnosis published by John Wiley & Sons Ltd.)

Objectives: The objectives were to determine the prevalence of and to identify risk factors associated with constriction of the fetal ductus arteriosus (DA) following perioperative indomethacin use for fetal myelomeningocele (MMC) repair. Study design A retrospective chart review study included 100 consecutive fetuses who underwent fetal MMC repair between 2011 and 2018. All patients had fetal echocardiography (FE) on postoperative day (POD)#1 and 2 to detect constriction of the DA. All patients received indomethacin for tocolysis using a standardized protocol. Multivariate regression analysis was carried out to identify the predictors for fetal ductal constriction., Results: Eighty patients met our study eligibility criteria. Median gestational age at time of surgery was 25 (24-25) weeks. Constriction of the DA was detected in 14 fetuses (17.5%). In five fetuses, this was observed on POD# 1, in seven on POD# 2, and in two on both days. The only independent risk factor for predicting DA constriction was maternal body mass index (BMI) <25 kg/m 2 (P = .002)., Conclusion: Indomethacin therapy following fetal MMC surgery requires careful daily FE surveillance. The association of DA constriction and low BMI suggests that BMI-based dosing of indomethacin may be recommended for perioperative tocolysis in fetal MMC surgery., (© 2020 John Wiley & Sons, Ltd.)

Background: The Management of Myelomeningocele Study (MOMS) demonstrated that fetal myelomeningocele (fMMC) closure results in improved hydrocephalus and hindbrain herniation when compared to postnatal closure., Objective: To report on the outcomes of a single institution's experience in the post-MOMS era, with regard to hydrocephalus absence and hindbrain herniation resolution., Methods: A single-center retrospective study of a subset of post-MOMS patients who underwent fetal/postnatal myelomeningocele closure was performed. Primary outcomes included cerebrospinal fluid (CSF) diversion status and hindbrain herniation resolution. Families were contacted via telephone for outcome information if care was transitioned to outside institutions. Univariate/multivariable analyses were performed using several prenatal and postnatal variables., Results: From January 2011 to May 2016, data were reviewed from families of 62 postnatal and 119 fMMC closure patients. In the postnatal group, 80.6% required CSF diversion compared to 38.7% fetal cases (P < .01). Hindbrain herniation resolution occurred in 81.5% fetal repairs compared to 32.6% postnatal (P < .01). In the fetal group, fetal/premature neonatal demise occurred in 6/119 (5.0%) patients. There was a 42.0% decrease (95% CI -55.2 to -28.8) and 48.9% increase (95% CI 33.7 to 64.1) in risk difference for CSF diversion and hindbrain herniation resolution, respectively, in the fetal group. On univariate analysis for both groups, prenatal atrial diameter, frontal-occipital horn ratio, and hindbrain herniation resolution were significantly associated with the absence of clinical hydrocephalus. The treatment of hydrocephalus was significantly delayed in the fetal group compared to the postnatal group (10 mo vs 13.8 d)., Conclusion: This study demonstrates the benefits of fMMC closure with regard to CSF dynamics., (Copyright © 2019 by the Congress of Neurological Surgeons.)

Object: Spinal cord surgeries carry a high risk for significant neurological impairments. The initial techniques for spinal cord mapping emerged as an aid to identify the dorsal columns and helped select a safe myelotomy site in intramedullary tumor resection. Advancements in motor mapping of the cord have also been made recently, but exclusively with tumor surgery. We hereby present our experiences with dynamic mapping of the corticospinal tract (CST) in other types of spinal cord procedures that carry an increased risk of postoperative motor deficit, and thus could directly benefit from this technique., Case Reports: Two patients with intractable unilateral lower extremity pain due to metastatic disease of the sacrum and a thoraco-lumbar chordoma, respectively underwent thoracic cordotomy to interrupt the nociceptive pathways. A third patient with progressive leg weakness underwent cord untethering and surgical repair of a large thoracic myelomeningocele. In all three cases, multimodality intraoperative neurophysiologic testing included somatosensory and motor evoked potentials monitoring as well as dynamic mapping of the CST., Conclusion: CST mapping allowed safe advancement of the cordotomy probe and exploration of the meningocele sac with untethering of the anterior-lateral aspect of the cord respectively, resulting in postoperative preservation or improvement of motor strength from the pre-operative baseline. Stimulus thresholds varied likely with the distance between the stimulating probe and the CST as well as with the baseline motor strength in the mapped myotomes., (Copyright © 2020 Elsevier Ltd. All rights reserved.)

Objective: Lifespan outcomes of simultaneous versus sequential myelomeningocele repair and shunt placement or effects of repeated shunt revisions on specific domains of IQ or fine motor dexterity are largely unknown. The current study addressed these gaps in a large cohort of children and adults with spina bifida myelomeningocele (SBM)., Methods: Participants between 7 and 44 years of age with SBM and shunted hydrocephalus were recruited from international clinics at two time points. Each participant completed a standardized neuropsychological evaluation that included estimates of IQ and fine motor dexterity. Simultaneous versus sequential surgical repair and number of shunt revisions were examined in relation to long-term IQ and fine motor scores., Results: Simultaneous myelomeningocele repair and shunting were associated with more frequent shunt revisions, as well as to lower Full Scale and verbal IQ scores, controlling for number of shunt revisions. More shunt revisions across study time points were associated with higher nonverbal IQ (NVIQ) scores. No effects were observed on fine motor dexterity., Conclusions: Findings indicate generally greater influence of surgery type over shunt revision history on outcomes in well-managed hydrocephalus. Findings supported apparent, domain-specific benefits of sequential compared to simultaneous surgery across the lifespan in SBM. Higher NVIQ scores with greater number of additional shunt revisions across surgery type supported positive outcomes with effective surgical management for hydrocephalus.

Aim: To determine whether the timing of shunt placement affects shunt infection rate in hydrocephalus associated with myelomeningocele (MMC)., Material and Methods: The cases of 67 consecutive patients who underwent MMC repair and ventriculoperitoneal (VP) shunt placement between 2010 and 2017 were analyzed, retrospectively. Shunt infection rates were compared for three different approaches: simultaneous shunting (MMC repair and shunting in the same session; n=22), early shunting (shunting in the first week after MMC repair; n=21), and delayed shunting (shunting in the second week after MMC repair; n=24)., Results: Three patients in the simultaneous shunting group (13.6%) and two patients in the early shunting group (9.5%) developed shunt infection, whereas no such infections occurred in the delayed shunting group (p > 0.05)., Conclusion: While the shunt infection rates for the simultaneous, early and delayed shunting groups were not significant, it is of interest that no shunt infections occurred in the delayed shunting group. Investigation with a larger number of patients is warranted to assess whether delayed shunting might reduce the risk of shunt infection.

Myelomeningocele (MMC) arises from an early neural developmental anomaly and results in a variety of structural abnormalities and associated functional neurologic deficits. As such, neurologic issues are central to virtually all clinical problems. Neurosurgical intervention strives to correct or improve these defects and prevent secondary complications. These interventions include closure of the open myelomeningocele and management (across the life span) of hydrocephalus, the Chiari II malformation (C2M) and tethered spinal cord (TSC). The development of pre-natal closure techniques and reports of improved outcome with in-utero closure (IUMC) have revolutionized the neurosurgical approach to myelomeningocele. Controversies remain surrounding patient selection, maternal risks, technique of IUMC (endoscopic vs. open) and long-term outcomes. However, real gains include reduced rates of hydrocephalus, modestly improved motor capabilities and reduction in C2M morbidity. For many decades, the cornerstone of treatment of hydrocephalus for many decades has been the placement and support of ventricular shunts. Endoscopic third ventriculostomy (ETV) with or without choroid plexus coagulation (ETV/CPC) is an appealing alternate strategy that avoids the morbidity and complications associated with shunts. The exact criteria for ETV-CPC candidacy and best metrics for outcome analysis remain active areas of debate and controversy. Similarly, neurosurgical management C2M, has centered upon the indications and clinical thresholds for performing posterior fossa surgical decompression. Tethered spinal cord management incorporates the diagnosis and surgical management of adhesions formed at the initial closure site, the consequent longitudinal traction related stress on the cord and the resulting neurologic signs and symptoms.

Purpose: We investigated longer term urological outcomes in patients enrolled in the Management of Myelomeningocele Study (MOMS)., Materials and Methods: Women who participated in the original trial were asked for consent for followup for their child at age 6 years or older in a single comprehensive study visit to a MOMS center. Participating children underwent urological and radiologic procedures to provide objective evidence of current bladder functioning. Primary urological outcome was defined as any among need for clean intermittent catheterization, vesicostomy, urethral dilatation or augmentation cystoplasty., Results: A total of 156 children were evaluated, with a mean age of 7.4 years. Overall 62% vs 87% in the prenatal and postnatal surgery groups, respectively, were placed on clean intermittent catheterization (RR 0.71, 95% CI 0.58-0.86, p <0.001). Voiding status was significantly different between the groups (p <0.001) as 24% in the prenatal group vs 4% in the postnatal group (RR 5.8, 95% CI 1.8-18.7) were reported to be voiding volitionally. Augmentation cystoplasty, vesicostomy and urethral dilation did not differ between the 2 groups. Aside from a larger post-void residual urodynamic catheterization volume, there were no other statistical differences in videourodynamic data or findings on renal/bladder ultrasound., Conclusions: Prenatal closure of myelomeningocele resulted in less reported clean intermittent catheterization at school age and the mechanism for this is unclear. Although most children are in diapers or on clean intermittent catheterization, parental reports showed children who underwent prenatal closure may be more likely to void volitionally than the postnatal group. Despite these findings, urological outcomes alone should not be the sole impetus to perform in utero closure in children with spina bifida.

Objective: Many repair techniques have been proposed to treat large myelomeningocele (MMC), and although effective in many cases, some of these techniques can be complex and time consuming, with complications such as cerebrospinal fluid (CSF) leakage, flap loss, tip necrosis, and wound dehiscence. The purpose of this study was to analyze cases of large skin defects and the methods applied and to report the outcomes of the keystone design perforator island flap (KDPIF) technique for large MMC closure., Methods: The authors performed a retrospective review of all neonatal patients who had undergone KDPIF for MMC closure in the period from 2013 to 2018. All patients had a diagnosis of lumbosacral MMC based on obstetric ultrasound. The neurosurgeons and plastic surgeons had selected the cases after concluding that primary closure would be unlikely. The design of the flap is based on the randomly located vascular perforators, creating two identical opposing flaps to fashion a double keystone flap. During wound closure, V-Y advancement of each end of the double flap in the longitudinal axis creates redundancy in the central portion of the flap and reduces the horizontal tension. After discharge, both the neurosurgery and plastic surgery teams followed up all patients, tracking the results with photography., Results: No skin flap dehiscence or necrosis, infection, or CSF leakage was detected, proving the reliability of the flap. One of the patients required further surgery for the large skin defects after insufficient intrauterine closure of the MMC and successfully underwent KDPIF treatment. Another patient (14.3%) had severe neonatal sepsis, which ultimately led to death. A ventriculoperitoneal shunt was required after the skin defect repair in 5 (83.3%) of the 6 surviving patients. Exceptional aesthetic results were achieved for all patients during the follow-up., Conclusions: The KDPIF technique is based on well-known vascular perforators of the intercostal, lumbar, and gluteal regions. Wound tension is widely distributed by the flap and, as a consequence, relevant tissue bulk, reliable vascularity, and important geometrical versatility are provided. In addition, most of the muscles and fascia are preserved, which is another advantage in terms of minimizing secondary morbidity to local tissue rearrangement. The use of KDPIF closure was successfully shown to be a viable alternative for more complex MMCs that present with large skin defects.

Objective: Improvements in imaging and surgical technological innovations have led to the increasing implementation of fetal surgical techniques. Open fetal surgery has demonstrated more favorable clinical outcomes in children born with open myelomeningocele (MMC) than those following postnatal repair. However, primarily because of maternal risks but also because of fetal risks, fetal surgery for MMC remains controversial. Here, the authors evaluated the contemporary management of MMC in the hope of identifying barriers and facilitators for neurosurgeons in providing fetal surgery for MMC., Methods: An online survey was emailed to members of the Congress of Neurological Surgeons (CNS) and the International Society for Pediatric Neurosurgery (ISPN) in March 2019. The survey focused on 1) characteristics of the respondents, 2) the practice of counseling on and managing prenatally diagnosed MMC, and 3) barriers, facilitators, and expectations of fetal surgery for MMC. Reminders were sent to improve the response rate., Results: A total of 446 respondents filled out the survey, most (59.2%) of whom specialized in pediatric neurosurgery. The respondents repaired an average of 9.6 MMC defects per year, regardless of technique. Regardless of the departments in which respondents were employed, 91.0% provided postnatal repair of MMC, 13.0% open fetal repair, and 4.9% fetoscopic repair. According to the surgeons, the most important objections to performing open fetal surgery were a lack of cases available to become proficient in the technique (33.8%), the risk of maternal complications (23.6%), and concern for fetal complications (15.2%). The most important facilitators according to advocates of prenatal closure are a decreased rate of shunt dependency (37.8%), a decreased rate of hindbrain herniation (27.0%), and an improved rate of motor function (18.9%). Of the respondents, only 16.9% agreed that open fetal surgery should be the standard of care., Conclusions: The survey results showed diversity in the management of patients with MMC. In addition, significant diversity remains regarding fetal surgery for MMC closure. Despite the apparent benefits of open fetal surgery in selected pregnancies, only a minority of centers and providers offer this technique. As a more technically demanding technique that requires multidisciplinary effort with less well-established long-term outcomes, fetoscopic surgery may face similar limited implementation, although the surgery may pose fewer maternal risks than open fetal surgery. Centralization of prenatal treatment to tertiary care referral centers, as well as the use of sophisticated training models, may help to augment the most commonly cited objection to the implementation of prenatal closure, which is the overall limited caseload.

Objective: Comparing prenatal and postnatal surgical repair techniques for myelomeningocele (MMC), in utero fetal surgery has increasingly gained acceptance and is considered by many specialized centers the first choice of treatment. Despite its benefits, as demonstrated in the Management of Myelomeningocele Study (MOMS), including reduced need for CSF shunting in neonates and improved motor outcomes at 30 months, there is still an ongoing debate on fetal and maternal risks associated with the procedure. Prenatal open hysterotomy, fetoscopic MMC repair techniques, and subsequent delivery by cesarean section are associated with maternal complications. The aim of this systematic review is to assess the available literature on maternal and obstetric complication rates and perinatal maternal outcomes related to fetal MMC repair., Methods: The authors identified references for inclusion in this review by searching PubMed and MEDLINE, with restrictions to English language, case series, case reports, clinical trials, controlled clinical trials, meta-analyses, randomized controlled trials, reviews, and systematic reviews. The rate of maternal and obstetric complications was analyzed based on studies focusing on this issue and presenting clear results on the matter., Results: Of 1264 articles screened, 36 were included in this systemic review, whereof 11 were eligible for data analysis and comparison. The average overall rate of maternal and obstetric complications corresponds to 78.6%. The majority of the described events are obstetric complications, varying from chorioamniotic membrane separation in 65.6% of cases, oligohydramnios in 13.0% of cases, placental abruption in 5.0% of cases, spontaneous or preterm premature membrane rupture in 42.0% of cases, and early preterm delivery in 11.3% of cases due to uterine dehiscence, occurring in 0.9% of cases. The most common medical complications are development of pulmonary edema occurring in 2.8%, gestational diabetes in 3.7%, gestational hypertension/preeclampsia in 3.7%, and need for blood transfusions in 3.2% of cases. Limitations of the review arise from the lack of data in the current literature, with maternal and obstetric complications being underreported., Conclusions: Although the efforts of further advancement of intrauterine prenatal MMC repair aim to increase neonatal outcomes, maternal health hazard will continue to be an issue of crucial importance and further studies are required.

Background Spina bifida affects 0.5-1 in 1000 pregnancies in the United States and is often diagnosed in the mid-second trimester. The objective of the study was to directly compare ultrasounds (US) and magnetic resonance imaging (MRI) obtained in the antenatal period in the diagnosis and localization of fetal myelomeningocele (MMC) and compare these with the postnatal outcomes of these infants Methods A retrospective analysis of patients referred to the Fetal Care Center at the Cleveland Clinic from 2005 to 2017. US and MRIs were obtained from the Cleveland Clinic electronic medical record. Infants were followed-up at an interdisciplinary myelomeningocele pediatrics clinic. Results MRI and US varied in correlation with physical exam at the time of birth and surgery. While no differences were detected in demographics, pregnancy outcomes or pediatric outcomes, it was noted that the majority of patients developed neurogenic bladders irrespective of the lesion level. Conclusion MRI is not superior to US in the diagnosis of MMC. Pregnancies complicated by MMC do not vary in morbidity, and pediatric outcomes remain similar regardless of the lesion level. This data provides additional information for the counseling of patients when faced with this antenatal diagnosis.

Background: The incidence of spina bifida (SB) is higher in the developing world as compared to the United States because of folic acid deficiency during pregnancy. Advances in technology have made prenatal repair of myelomeningocele (MM) possible., Objective: The objective of this guideline was to determine if there is a difference in the rate of development of tethered cord syndrome (TCS) in infants who had prenatal closure compared to infants who had MM repair after birth., Methods: The Guidelines Task Force developed search terms and strategies to search PubMed and Embase for the relevant literature published between 1966 and September 2016. Strict inclusion/exclusion criteria were used. Full text articles were reviewed and, when appropriate, included as evidence., Results: A total of 261 abstracts were reviewed. Fifty-four full-text articles were selected for further analysis. Three studies met inclusion criteria., Conclusion: There was Class II evidence from 1 study and Class III evidence from another 2 studies demonstrating that TCS develops in infants with prenatal MM closure at an equal or higher rate than with postnatal closure. There was an increased risk of development of inclusion cysts in infants who underwent in utero closure. Continued surveillance for TCS and/or the development of inclusion cysts in children with prenatal and postnatal closure of MM is indicated (Level II). Differences between prenatal and postnatal repair with respect to the development of TCS and/or inclusion cysts should be considered alongside other relevant maternal and fetal outcomes when deciding upon a preferred method for MM closure.The full guideline can be found at https://www.cns.org/guidelines/guidelines-spina-bifida-chapter-6., (Copyright © 2019 by the Congress of Neurological Surgeons.)

Background: Myelomeningocele (MM) is an open neural tube defect treated by pediatric neurosurgeons with prenatal or postnatal closure., Objective: The objective of this systematic review was to answer the question: What is the evidence for the effectiveness of prenatal vs postnatal closure of MM regarding short and long-term ambulatory status? Treatment recommendations were provided based on the available evidence., Methods: The National Library of Medicine PubMed database and Embase were queried using MeSH headings and keywords relevant to ambulatory status after prenatal or postnatal closure of MM. Abstracts were reviewed to identify which studies met the inclusion criteria. An evidence table was assembled summarizing the studies and the quality of evidence (Classes I-III). Based on the quality of the literature, a recommendation was rendered (Level I, II, or III)., Results: One randomized controlled trial (Class II) and 3 retrospective cohort studies (Class III) were included as evidence. Initial ambulatory status depended on anatomic level of the neural tube defect. In the short term, prenatal closure may improve ambulatory status compared to postnatal closure. Spinal cord tethering or dermoid inclusion cyst has been associated with neurologic deterioration in infants closed in utero and after birth. Ambulation may cease in both groups over time. No long-term studies evaluated whether there is a difference in the ability to ambulate upon reaching adulthood., Conclusion: Prenatal closure of MM may improve ambulatory status in the short term (Level II). Spinal cord tethering in both groups caused deterioration in the ability to walk. Evaluation and treatment of spinal cord tethering may help maintain ambulatory status (Level III). No studies evaluate whether prenatal or postnatal repair provides improved ability to ambulate upon reaching adulthood.The full guideline can be found at https://www.cns.org/guidelines/guidelines-spina-bifida-chapter-3., (Copyright © 2019 by the Congress of Neurological Surgeons.)

Background: Appropriate timing for closure of myelomeningocele (MM) varies in the literature. Older studies present 48 h as the timeframe after which infection complication rates rise., Objective: The objective of this guideline is to determine if closing the MM within 48 h decreases the risk of wound infection or ventriculitis., Methods: The Guidelines Task Force developed search terms and strategies used to search PubMed and Embase for relevant literature published between 1966 and September 2016. Strict inclusion/exclusion criteria were used to screen abstracts and to develop a list of relevant articles for full-text review. Full text articles were then reviewed and when appropriate, included in the evidentiary table. The class of evidence was evaluated, discussed, and assigned to each study that met inclusion criteria., Results: A total of 148 abstracts were identified and reviewed. A total of 31 articles were selected for full text analysis. Only 4 of these studies met inclusion criteria., Conclusion: There is insufficient evidence that operating within 48 h decreases risk of wound infection or ventriculitis in 1 Class III study. There is 1 Class III study that provides evidence of global increase in postoperative infection after 48 h, but is not specific to wound infection or ventriculitis. There is 1 Class III study that provides evidence if surgery is going to be delayed greater than 48 h, antibiotics should be given.The full guideline can be found at https://www.cns.org/guidelines/guidelines-spina-bifida-chapter-4., (Copyright © 2019 by the Congress of Neurological Surgeons.)

Background: Myelomeningocele (MM) is a condition that is responsible for considerable morbidity in the pediatric population. A significant proportion of the morbidity related to MM is attributable to hydrocephalus and the surgical management thereof. Postnatal repair remains the most common form of treatment; however, increased rates of prenatal diagnosis, advances in fetal surgery, and a hypothesis that neural injury continues in utero until the MM defect is repaired have led to the development and evaluation of prenatal surgery as a means to improve outcomes in afflicted infants., Objective: The objective of this guideline is to systematically evaluate the literature to determine whether there is a difference in the proportion of patients who develop shunt-dependent hydrocephalus in infants who underwent prenatal MM repair compared to infants who had postnatal repair., Methods: The Guidelines Task Force developed search terms and strategies used to search PubMed and Embase for relevant literature published between 1966 and September 2016. Strict inclusion/exclusion criteria were used to screen abstracts and to develop a list of relevant articles for full-text review. Full-text articles were then reviewed, and when appropriate, included as evidence., Results: A total of 87 abstracts were identified and reviewed by 3 independent reviewers. Thirty-nine full-text articles were selected for analysis. Three studies met selection criteria and were included in the evidence table., Conclusion: Class I evidence from 1 study and class III evidence from 2 studies suggest that, in comparison to postnatal repair, prenatal surgery for MM reduces the risk of developing shunt-dependent hydrocephalus. Therefore, prenatal repair of MM is recommended for those fetuses who meet specific criteria for prenatal surgery to reduce the risk of developing shunt-dependent hydrocephalus (level I). Differences between prenatal and postnatal repair with respect to the requirement for permanent cerebrospinal fluid diversion should be considered alongside other relevant maternal and fetal factors when deciding upon a preferred method of MM closure. The full guideline can be found at https://www.cns.org/guidelines/guidelines-spina-bifida-chapter-2., (Copyright © 2019 by the Congress of Neurological Surgeons.)

Purpose: It is well known that the preoperative enlarged sac in occult spinal dysraphism, in which the spinal cord is attached to its dome, can be associated with neurological deficits by aggravation of spinal cord traction. We experienced a similar phenomenon during the early stage of untethering surgery for "lipomyelomeningocele in a strict sense (LMMC).", Clinical Presentation: We report a case of a 3-month-old girl with LMMC, which showed decreased motor evoked potentials (MEPs) in the lower extremities during the early stage of untethering surgery. The sac was released from the original nest by incision with separation of the overlying skin and dissection from the muscle fascia. The sac became rounder, and the spinal cord attached to the dome of the sac was further stretched. After aspiration of cerebrospinal fluid from the sac, the MEPs recovered. Postoperatively, the patient had no neurological deficits., Conclusions: Although this phenomenon rarely occurs, release of the herniated sac of LMMC from the original nest during the early stage of untethering surgery may result in neurological changes. Application of intraoperative neurophysiological monitoring helps to detect electrophysiological deterioration and can prevent neurological deficits.

Objective: Given the lack of significant responses to many queries regarding behavior, treatment options, and possible prevention of iatrogenic intraspinal inclusion tumors (IITs), we think that further clinical, radiologic, and follow-up data are mandatory to better characterize this entity. We retrospectively reviewed a series of 14 consecutive patients with IIT occurring after myelomeningocele repair. The long follow-up is a mainstay of this series and may help a better understanding of the history of this slow-growing disease. To date, this is the largest series of IITs after myelomeningocele repair reported in the literature., Methods: A retrospective chart review was performed to identify all cases of surgically repaired myelomeningocele at the Department of Neurosurgery of G. Gaslini Children's Hospital in Genoa, from January 1993 to January 2018. Among them, data regarding patients in whom an IIT developed were recovered. The medical records of these patients and their clinical history, neurologic examination, and diagnostic workup were reviewed., Results: From January 1993 to January 2018, 160 cases of myelomeningocele were surgically treated. Among them, 14 cases of IIT developed. The mean time between myelomeningocele repair and IIT diagnosis was 94.7 months. The mean follow-up time was 60.1 months., Conclusions: It is well established that IITs may be discovered in patients who have previously undergone myelomeningocele repair. In our series, with a long follow-up time, we describe the possible range of behavior of such lesions and the utility of surgical treatment of primary therapy. Our series confirms that residual IITs showed marked stability during our long follow-up., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: Neural tube defects are a large health burden for East African countries. Health strategies in the prevention of this disease include nutritional prophylaxis, prenatal diagnosis, and availability of early neonatal neurosurgery. The main objective of this study is to describe our experience in the early surgical management of neural tube defects in the Zanzibar archipelago., Methods: From December 2016 to December 2017, we prospectively collected data on all patients admitted with the diagnosis of myelomeningocele. We collected variables regarding demographics, maternal health, preoperative imaging, surgical procedures, and complications at follow-up., Results: We collected data on 19 patients. Mean age was 9.8 ± 18.7 days. Of these patients, 52.6% were male and 47.3% were female; 47.3% patients were from Unguja, 42.0% from Pemba, and 5.2% from mainland Tanzania; 68.4% of all mothers were found to have undergone prenatal ultrasonography and 89.5% of all patients received surgery. Surgical wound infection was present in 29.4% of all surgical patients and 52.9% developed secondary hydrocephalus., Conclusions: Neural tube defects are a prevailing condition in East Africa. We believe that more health initiatives should address its prevention, mainly through maternal nutrition. On the basis of our findings, we consider early neonatal neurosurgery as the most important factor in reducing immediate morbidity and mortality., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Objective: To evaluate the clinical significance of intraoperative bulbocavernosus reflex (BCR) during untethering surgery in predicting post-operative voiding function., Methods: We conducted a retrospective review of pediatric patients who underwent untethering surgery with available intraoperative baseline BCR. BCR response during surgery was classified into loss or maintenance. Post-operative voiding function was determined as worsened or maintained based on history, postvoid residual urine measurement, and urodynamic study (UDS). Data regarding demographics, diagnosis, pre-operative voiding difficulty, re-untethering, syrinx, and abnormalities in electromyography were collected for analysis., Results: We included 106 patients, with a mean age of 3.3 years, and 49 patients were male. BCR was lost in 15 patients during surgery and voiding function worsened in 14 patients after surgery. Lumbosacral lipoma was the most common diagnosis, and 16 patients were diagnosed with lipomyelomeningocele (LMMC). The sensitivity and specificity of intraoperative BCR for post-operative worsening of voiding function were 35.7%, and 88.5% at 6 months, respectively. The diagnosis of LMMC was statistically significant in a logistic regression analysis. The specificity of BCR at 6 months in patients with diagnosis other than LMMC was 93.4%, and intraoperative BCR was significant in a logistic regression analysis., Conclusions: Intraoperative BCR during untethering could predict bladder function 6 months post-operatively with high specificity (88.5%), particularly in cases other than LMMC (93.4%), indicating that voiding function deterioration will not occur if intraoperative BCR is preserved., Significance: Intraoperative BCR during untethering surgery is a useful tool to predict post-operative voiding outcome., (Copyright © 2018 International Federation of Clinical Neurophysiology. Published by Elsevier B.V. All rights reserved.)

Aim: To establish the provision of fetal surgery for myelomeningocele (MMC) worldwide., Methods: Through the International Society for Prenatal Diagnosis (ISPD) Fetal Therapy Special Interest Group and the North American Fetal Therapy Network (NAFTNet), fetal therapy centres were surveyed (September 2017-June 2018) regarding availability of fetal MMC surgical repair, patient inclusion criteria, repair techniques, number of cases, and outcome reporting. Responses were summarised on an interactive map on the ISPD website., Results: Forty-four of 59 centres responded (74.6%) of which 34 centres (77.1%) currently offered fetal surgery for MMC and seven centres (15.9%) were awaiting a first case after service set up. Patient inclusion criteria were similar and based on the Management of Myelomeningocele (MOMS) trial. Five centres (14.7%) operated beyond 26 weeks' gestational age, outside the MOMS criteria. Open fetal surgery was provided in 23 centres (67.6%), fetoscopic surgery only in five (14.7%), and six centres offered both types (17.6%). Neurosurgical closure was similar for open surgery but highly variable in fetoscopy surgery. The median number of cases per centre was 21 (range 1-253)., Conclusions: Fetal surgery for MMC is now offered globally. Two thirds of centres offer open repair via hysterotomy using criteria based on the MOMS trial., (© 2018 John Wiley & Sons, Ltd.)

Aims: The aim of the study was to evaluate the lower urinary tract function, frequency UTI, the degree of social urine continence, and the occurrence of constipation in children who underwent prenatal or postnatal surgery for MMC., Materials and Methods: Seventy-two patients with MMC were assessed and divided into the prenatal group (36 patients) and the postnatal one (36 patients). All children, regardless the time of operation, received the same treatment after birth. The urodynamic tests, USG, cystourethrography were performed in all patients along with evaluation of the UTI's, social urine continence, and presence of constipation., Results: Urodynamic and imaging studies showed no differences between the test groups. Children from the prenatally operated group showed statistically significant lower number of urinary tract infections, better urine continence, and less frequent constipation., Conclusions: Prenatal MMC repair ensures statistically significant improvement of the degree of social urinary continence, reducing the risk of urinary infections and constipation. Time of MMC repair does not statistically influence the urodynamic tests results and the urodynamic parameters are not the prognostic elements to assess the social urinary continence possibility in patients with the neurogenic bladder., (© 2018 Wiley Periodicals, Inc.)

Objective: Fetoscopy for closure of open neural tube defects (NTD) remains controversial, as the use of patches or single-layer closure is not considered to meet the standards of good neurosurgical reconstruction. In this study, we describe a fetoscopic two-layer (myofascial and skin) closure technique for the treatment of NTD in five patients and report the preliminary anatomical outcome at birth., Methods: From February to September 2017, five pregnant women with a fetus with a NTD, including three cases of myelomeningocele and two cases of myelocele, were operated on using a fetoscopic two-layer closure technique. In this technique, with the uterus exteriorized and using three 10-Fr ports, the placode is dissected from the surrounding tissue and detethered, removing the cystic tissue. The skin is undermined by blunt dissection and the defect is sutured to the midline in two layers (myofascial and skin) using a running 4/0 resorbable barbed suture., Results: Median gestational age at the procedure was 24 + 3 (range, 23 + 5 to 27 + 3) weeks. Surgery was successful in all cases, without any intraoperative complications. Median time in surgery was 180 (range, 140-180) min and median time for fetoscopy was 105 (range, 65-120) min. In terms of obstetric complications, three cases of premature rupture of membranes and one case of chorioamnionitis were recorded. Median gestational age at delivery was 34 + 1 (range, 25 + 4 to 37 + 2) weeks and two patients delivered vaginally. The closed defect was watertight with good quality tissue in all cases., Conclusion: Fetoscopic two-layer closure of NTD may improve the quality of the tissue covering the defect, diminishing the need for postnatal surgical revision, and preserving the well-documented beneficial effects of prenatal closure on the neural tissue and hindbrain herniation. However, this technique may not be appropriate for those cases with wide diastasis of the myofascial layer or with a low quantity of available tissue. Copyright © 2018 ISUOG. Published by John Wiley & Sons Ltd., (Copyright © 2018 ISUOG. Published by John Wiley & Sons Ltd.)

Objective: Surgical management of spinal dysraphism often requires the use of dural substitutes. Amniotic membrane (AM) has drawn the interest of clinicians for its valuable concentration of cytokines and factors capable of promoting wound healing, re-epithelialization, inhibiting fibrosis and regulating angiogenesis. These beneficial qualities could make AM an interesting dural substitute for spina bifida repair. In this study, we describe the use of banked homologous AM as a dural substitute for the repair of spinal dysraphism in newborns. Our purpose is to test the mechanical characteristics, as well as the safety and effectiveness of AM in preventing postoperative complications and re-tethering., Methods: The AM patch was carefully detached from the chorion of donors undergoing caesarean section, rinsed in saline solution, and cryopreserved in liquid nitrogen. Five newborns were treated using AM: three affected by open spinal dysraphism and two by spina bifida occulta. The AM patch was used as a dural substitute with two different positions and purposes: the amnion-side down covering the placode to prevent adhesions or placed extradurally facing the dura to avoid scarring and facilitating the sliding of the dural sac itself under the extradural tissue layers., Results: No adverse events occurred, and the surgical wounds healed without complications. MRI scans taken at 3 and 6 months after surgery showed a satisfying de-tethering of the spinal cord with no obvious evidence of new adherence formation., Conclusions: We present a multimodal interposition technique using AM as a reconstructive and anti-adhesive tissue for the treatment of open myelomeningocele (MMC) and lipomeningocele (LMC) treatment. In our experience, AM proved its efficacy in restoring the dural sac integrity without complications. We support the use of AM as a promising dural substitute, speculating on how the use of AM could potentially change reconstructive strategies for spinal dysraphism.

Introduction: Currarino's syndrome (CS) is an autosomal dominant disorder of embryonic development causing a rare malformating syndrome characterized by a triad of an anorectal malformations, presacral mass (most commonly an anterior sacral meningocele) and sacral bony defects. Mutations of the HLXB9 gene have been identified in most CS cases, but a precise genotype-phenotype correlation has not been described so far. Family screening is obligatory. The diagnosis is usually made during childhood and rarely in adulthood. In this context, imaging, and especially MRI plays a major role in the diagnosis of this syndrome. Surgical management is provided by pediatric surgeons or neurosurgeons., Familial Case Report: Here, we present a family case report with CS requiring different neurosurgical management. The son, a 3-year-old boy, developed a tethered spinal cord syndrome associated to a lipoma of the filum terminale, a sacro-coccygeal teratoma and an anal adhesion. A combined surgical approach permitted a good evolution on the urinary and digestive functions despite a persistent fecal incontinence. The 2-year-old daughter presented with a cyst of the thyreoglossal tract infected and fistulized to the skin. She was also followed for a very small lipoma of the filum terminale that required a neurosurgical approach. The father, 44-year-old, manifested functional digestive and urinary disorders caused by a giant anterior sacral meningocele. The ligation of the neck of the cyst and aspiration of the liquid inside in full through a posterior partial approach permit a complete collapse of the cyst with an instantly satisfactory clinical outcome., Conclusion: In these cases, cooperation between pediatric surgeons and neurosurgeons was crucial. The follow-up of these patients should be done in a spina bifida clinic. A geneticist evaluation must be offered to the patient in the case of a CS as well as a clinical evaluation of the relatives (parents, siblings)., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

Objective: To evaluate the optimal timing and type of surgical treatment of myelomeningocele (MMC)-related spinal deformities and long-term follow-up of surgical treatment., Methods: We reviewed and presented clinical pictures, treatment strategies and results of 20 patients with MMC-related spinal deformities treated at our center between 2010 and 2017., Results: The average patient age was 6.3 years. The average preoperative neurologic status according to a modified Japan Orthopedic Association (mJOA) scale was 7.3 points (Benzel's modification). Average functional status was 41 points according to a functional independent measure scale (FIM). The average angle of kyphosis was 83.7°, that of scoliosis was 36.7°, and that of lordosis was 67° (Cobb angles). The average duration of surgery was 234 minutes, and the average total blood loss was 175 mL. The average angle of kyphosis correction was 61°, that of scoliosis correction was 25°, and that of lordosis correction was 25° (Cobb angles). The average duration of hospitalization was 16.6 days, and the average follow-up was 34.5 months. The total number of complications was 13. Reoperation was required in 9 cases. Neurologic status according to the mJOA scale improved by 0.6 point on average. Functional status according to the FIM increased by 6.6 points on average., Conclusions: Early surgical correction of MMC-related spinal deformities improves body balance and quality of life. The dual growing rod technique is safe and effective in cases of moderate neuromuscular spinal deformities at an early age. Kyphectomy is a challenging procedure with high complication rates, especially skin problems, but there are no alternative procedures for cases of heavy rigid kyphosis., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: Fetoscopic meningomyelocele repair in a gas-filled uterus is a new technique performed in very few centers. There are few opportunities as well as ethical prohibitions on the initial development and subsequent refinement of innovative fetal surgery techniques in humans, and using an animal model is both very expensive and logistically difficult., Method: We developed a low-fidelity endoscopic fetal surgery simulation using a plastic doll and pieces of chicken breast to simulate a fetal meningomyelocele, and a polyurethane ball to simulate a gas-filled uterus, along with a standard endoscopy system and instruments., Experience: A unique two-port technique with significant differences from the standard laparoscopic surgery procedure was developed and refined through an iterative phase into a standardized methodology, and the simulator was then used to train three other teams to perform standardized fetoscopic meningomyelocele repair., Conclusion: A low-fidelity fetoscopic surgery simulator is a useful tool for developing new fetoscopic operations and for training multidisciplinary fetal surgery teams without the need for extensive use of an animal model. This simulator may be used to further explore the human uterus as a new surgical space for additional fetal surgeries.

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.

Background: Administering anesthesia for prenatal repair of myelomeningocele reveals several issues that are unique to this new form of treatment. This includes issues such as fetal well-being, surgical conditions and monitoring, among others. Exploring, analyzing, and understanding the different variables that are involved will help us reduce the high level of risk associated with this surgery., Objective: This review provides a systematic approach to the issues that are faced by anesthesiologists during fetal surgery.

Open spina bifida or myelomeningocele (MMC) is one of the most common serious congenital malformations. Historically, this condition has been treated with closure of the MMC defect shortly after birth. The goal of postnatal closure is to cover the exposed spinal cord and prevent infection. However, postnatal surgery does not reverse or prevent the neurologic injury seen in MMC, reverse hindbrain herniation, or prevent hydrocephalus. The neurologic defects result from primary incomplete neurulation and secondary chronic prenatal damage to the exposed neural elements through mechanical and chemical trauma. In a hope to reduce the numerous surgical procedures, medical complications, and lifelong disabilities associated with MMC, the proposal of prenatal closure was put forth more than two decades ago. After promising results in animal models and some clinical series, a randomized controlled trial, the Management of Myelomeningocele Study (MOMS), was conducted. The MOMS trial demonstrated that closure during the prenatal period could be performed relatively safely and can result in significant benefit to the child. Specifically, prenatal closure results in improved motor function, reduced hindbrain herniation, and reduced need for a cerebral spinal fluid diversion. Long-term outcomes of the patients in the MOMS trial continues in the MOMS 2 study as these children grow. Additionally, investigations are underway on modifications to the open fetal MMC closure techniques.

The treatment of children with myelomeningocele (MMC) has improved over time, from supportive management to early postnatal closure to prenatal repair of the defect. The Management of Myelomeningocele Study (MOMS) showed that prenatal repair of MMC resulted in improved neurological outcomes compared to postnatal closure. Follow-up studies showed that prenatal repair was, as with any other fetal intervention, associated with higher rates of obstetrical complications. There was no significant difference in urological outcomes. Long-term follow-up of ambulatory status, executive functioning, and urological outcomes is needed to determine the durable effects of fetal MMC repair on mobility, functional independence, and the prevalence of renal insufficiency in patients with MMC who survive to adulthood. The future of fetal MMC repair consists of developing strategies to reduce maternal morbidity and improve infant outcomes. Fetoscopic MMC repair has been suggested as an alternative to open repair that may reduce obstetrical complications and the need for cesarean delivery in subsequent pregnancies. Translational research using mesenchymal stromal cells to augment fetal repair of ovine MMC has shown improvement in motor function.

Purpose: This study aims to assess the feasibility and the effectiveness of a fetoscopic myelomeningocele (MMC) coverage using a sealed inert patch through a two-port access, in the sheep model., Methods: Forty-four fetuses underwent surgical creation of a MMC defect at day 75 and were divided into four groups according to the MMC repair technique, performed at day 90. Group 1 remained untreated. Group 2 had an open surgery using suture of the defect. Groups 3 and 4 underwent defect coverage using a Gore®-polytetrafluoroethylene patch secured with surgical adhesive (Bioglue®), with an open approach (group 3) and a fetoscopic one (group 4). Lambs were killed at term, and histological examinations were performed., Results: Fetoscopic patch coverage was achieved in all the lambs of group 4. All the fetuses of group 2 had a complete closure of the defect whereas only 38% in group 3 and 14% in group 4. Fetal loss rate seems to be lower in group 4 than in groups 2 and 3., Conclusion: Fetoscopic coverage of MMC defect can be performed using a sealed patch through a two-port access, but the patch and glue correction may not be the ideal technique to repair fetal MMC.

The current standard for fetal repair of myelomeningocele is an open exposure of the gravid uterus with control of the amniotic membranes, and then a multi-layer closure of the fetal defect. The nature of the surgical repair has evolved and requires a series of steps that take into account the delicate nature of the fetal tissues. The individual surgical steps are described including potential pitfalls.

Purpose: Although previous studies have revealed high success rates (70% to 85%) after an intradural somatic-to-autonomic nerve transfer procedure in children with spinal dysraphism, no study has had a control group or blinded observers. We report a rigorously designed study to investigate the effectiveness of the Xiao procedure., Materials and Methods: Children with neurogenic bladder dysfunction related to myelomeningocele or lipomyelomeningocele who required spinal cord detethering were randomized to 2 groups at surgery, with half undergoing only spinal cord detethering and half undergoing the Xiao procedure in addition to detethering. Double-blind evaluations were performed at regular intervals during the 3-year followup., Results: A total of 10 patients underwent spinal cord detethering only and 10 underwent detethering plus the Xiao procedure. The Xiao procedure did not result in voluntary voiding or continence in any patient, but patients undergoing spinal cord detethering plus the Xiao procedure were more likely to have greater improvements in total bladder capacity, bladder overactivity and overall quality of life than those who underwent detethering only. By the end of the study no participant or evaluator was able to accurately predict to which group the patients had been assigned., Conclusions: The results of this randomized controlled trial are in agreement with recently published similarly poor results of the Xiao procedure in patients with spinal cord injury. Improvements in bladder parameters observed in this study may be related to sacral nerve root section, a necessary portion of the Xiao procedure, instead of reinnervation. Confirmatory animal studies are recommended before further clinical trials of the Xiao procedure are performed in humans., (Copyright © 2016 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

Background and Study Aims  Although postnatal surgery can be regarded as the standard of care for open myelomeningocele (MMC), there is no uniform treatment for this condition in Germany. This study assessed the current situation regarding the management of open MMC, a first and essential step toward the development of standards of practice. Patients and Methods  In the second half of 2009, we had sent standardized questionnaires to 139 neurosurgery departments and 73 pediatric surgery departments (number of returned questionnaires: 98 and 62, respectively) to identify the principles of MMC management. Newborns with open MMC are treated in 57 of the neurosurgery departments and in 18 of the pediatric surgery departments. Results  Overall, 41 of the 57 neurosurgery departments and 16 of the 18 pediatric surgery departments providing care for newborns with MMC perform MMC closure within 24 hours of delivery. In 52 neurosurgery departments and 15 pediatric surgery departments, infants with spina bifida are delivered by planned cesarean section. The diagnostic investigations performed between the time of caesarean delivery and plastic surgical closure vary from none to magnetic resonance imaging of the spine. The criteria underlying the placement of shunts and the time to plastic surgical closure are highly variable. Conclusions  The type of treatment that infants with open MMC receive in Germany differs from institution to institution. Clear and consistent guidelines must be established using an interdisciplinary approach to improve treatment and standardize the care provided by surgical specialists for infants with MMC., (Georg Thieme Verlag KG Stuttgart · New York.)

OBJECTIVE Due to improved nutrition and early detection, myelomeningocele repair is a relatively uncommon procedure. Although previous studies have reviewed surgical trends and predictors of outcomes, they have relied largely on single-hospital experiences or on databases centered on hospital admission data. Here, the authors report 30-day outcomes of pediatric patients undergoing postnatal myelomeningocele repair from a national prospective surgical outcomes database. They sought to investigate the association between preoperative and intraoperative factors on the occurrence of 30-day complications, readmissions, and unplanned return to operating room events. METHODS The 2013 American College of Surgeons National Surgical Quality Improvement Program Pediatric database (NSQIP-P) was queried for all patients undergoing postnatal myelomeningocele repair. Patients were subdivided on the basis of the size of the repair (< 5 cm vs > 5 cm). Preoperative variables, intraoperative characteristics, and postoperative 30-day events were tabulated from prospectively collected data. Three separate outcomes for complication, unplanned readmission, and return to the operating room were analyzed using univariate and multivariate logistic regression. Rates of associated CSF diversion operations and their timing were also analyzed. RESULTS A total of 114 patients were included; 54 had myelomeningocele repair for a defect size smaller than 5 cm, and 60 had repair for a defect size larger than 5 cm. CSF shunts were placed concurrently in 8% of the cases. There were 42 NSQIP-defined complications in 31 patients (27%); these included wound complications and infections, in addition to others. Postoperative wound complications were the most common and occurred in 27 patients (24%). Forty patients (35%) had at least one subsequent surgery within 30 days. Twenty-four patients (21%) returned to the operating room for initial shunt placement. Unplanned readmission occurred in 11% of cases. Both complication and return to operating room outcomes were statistically associated with age at repair. CONCLUSIONS The NSQIP-P allows examination of 30-day perioperative outcomes from a national prospectively collected database. In this cohort, over one-quarter of patients undergoing postnatal myelomeningocele repair experienced a complication within 30 days. The complication rate was significantly higher in patients who had surgical repair within the first 24 hours of birth than in patients who had surgery after the 1st day of life. The authors also highlight limitations of investigating myelomeningocele repair using NSQIP-P and advocate the importance of disease-specific data collection.