Prenatal Myelomeningocele Repair Research Articles

Orthopaedic outcomes of prenatal versus postnatal repair of myelomeningocele.

Myelomeningocele, characterized by extrusion of the spinal cord through a spinal canal defect, is the most common form of spina bifida, often resulting in lifelong disability and significant orthopaedic issues. A randomized controlled trial (RCT) has shown the efficacy of prenatal repair in decreasing the need for shunting and improving motor outcomes. However, no studies have evaluated the effects of prenatal repair on orthopaedic outcomes. The purpose of this study was to determine the rates of orthopaedic conditions in patients with prenatal and postnatal repair of myelomeningocele and compare the rates of treatment required. This study analyzes the relevant outcomes from a prospective RCT (Management of Myelomeningocele Study). Eligible women were randomized to prenatal or postnatal repair, and patients were evaluated prospectively. Outcomes of interest included rates of scoliosis, kyphosis, hip abnormality, clubfoot, tibial torsion, and leg length discrepancy (LLD) at 12 and 30 months. The need for orthopaedic intervention at the same time points was also evaluated. Statistical analyses included descriptive statistics and univariate analyses. Data for the full cohort of 183 patients were analyzed (91 prenatal, 92 postnatal). There were no differences in rates of scoliosis, kyphosis, hip abnormality, clubfoot or tibial torsion between patients treated with prenatal or postnatal repair. The rate of LLD was lower in the prenatal repair group at 12 and 30 months (7 vs. 16% at 30 months, P = 0.047). The rates of patients requiring casting or bracing were significantly lower in patients treated with prenatal repair at 12 and 30 months (78 vs. 90% at 30 months, P = 0.036). Patients treated with prenatal myelomeningocele repair may develop milder forms of orthopaedic conditions and may not require extensive orthopaedic management.

The Management of Myelomeningocele Study: Short-Term Neonatal Outcomes

Introduction: The Management of Myelomeningocele Study was a multicenter randomized trial to compare prenatal and standard postnatal repair of myelomeningocele (MMC). Neonatal outcome data for 158 of the 183 randomized women were published in The New England Journal of Medicine in 2011. Objective: Neonatal outcomes for the complete trial cohort (N = 183) are presented outlining the similarities with the original report and describing the impact of gestational age as a mediator. Methods: Gestational age, neonatal characteristics at delivery, and outcomes including common complications of prematurity were assessed. Results: Analysis of the complete cohort confirmed the initial findings that prenatal surgery was associated with an increased risk for earlier gestational age at birth. Delivery occurred before 30 weeks of gestation in 11% of neonates that had fetal MMC repair. Adverse pulmonary sequelae were rare in the prenatal surgery group despite an increased rate of oligohydramnios. There was no significant difference in other complications of prematurity including patent ductus arteriosus, sepsis, necrotizing enterocolitis, periventricular leukomalacia, and intraventricular hemorrhage. Conclusion: The benefits of prenatal surgery outweigh the complications of prematurity.

Prenatal Repair of Myelomeningocele and School-age Functional Outcomes.

The Management of Myelomeningocele Study (MOMS), a randomized trial of prenatal versus postnatal repair for myelomeningocele, found that prenatal surgery resulted in reduced hindbrain herniation and need for shunt diversion at 12 months of age and better motor function at 30 months. In this study, we compared adaptive behavior and other outcomes at school age (5.9-10.3 years) between prenatal versus postnatal surgery groups. Follow-up cohort study of 161 children enrolled in MOMS. Assessments included neuropsychological and physical evaluations. Children were evaluated at a MOMS center or at a home visit by trained blinded examiners. The Vineland composite score was not different between surgery groups (89.0 ± 9.6 in the prenatal group versus 87.5 ± 12.0 in the postnatal group; P = .35). Children in the prenatal group walked without orthotics or assistive devices more often (29% vs 11%; P = .06), had higher mean percentage scores on the Functional Rehabilitation Evaluation of Sensori-Neurologic Outcomes (92 ± 9 vs 85 ± 18; P < .001), lower rates of hindbrain herniation (60% vs 87%; P < .001), had fewer shunts placed for hydrocephalus (49% vs 85%; P < .001) and, among those with shunts, fewer shunt revisions (47% vs 70%; P = .02) than those in the postnatal group. Parents of children repaired prenatally reported higher mean quality of life z scores (0.15 ± 0.67 vs 0.11 ± 0.73; P = .008) and lower mean family impact scores (32.5 ± 7.8 vs 37.0 ± 8.9; P = .002). There was no significant difference between surgery groups in overall adaptive behavior. Long-term benefits of prenatal surgery included improved mobility and independent functioning and fewer surgeries for shunt placement and revision, with no strong evidence of improved cognitive functioning.

Progressive hydrocephalus despite early complete reversal of hindbrain herniation after prenatal open myelomeningocele repair.

Open prenatal myelomeningocele (MMC) repair is typically associated with reversal of in utero hindbrain herniation (HBH) and has been posited to be associated with a reduction in both postoperative prenatal and immediate postnatal hydrocephalus (HCP) risks. However, the long-term postnatal risk of HCP following HBH reversal in these cases has not been well defined. The authors describe the results of a long-term HCP surveillance in a cohort of patients who underwent prenatal MMC repair at their institution. A retrospective review of all prenatal MMC repair operations performed at the Mayo Clinic between 2012 and 2017 was conducted. Pertinent data regarding the clinical courses of these patients before and after MMC repair were summarized. Outcomes of interest were occurrences of HBH and HCP and the need for intervention. A total of 9 prenatal MMC repair cases were identified. There were 7 cases in which MRI clearly demonstrated prenatal HBH, and of these 86% (6/7) had evidence of HBH reversal after repair and prior to delivery. After a mean postnatal follow-up of 20 months, there were 3 cases of postnatal HCP requiring intervention. One case that failed to show complete HBH reversal after MMC repair required early ventriculoperitoneal shunting. The other 2 cases were of progressive, gradual-onset HCP despite complete prenatal HBH reversal, requiring endoscopic third ventriculostomy with choroid plexus cauterization at ages 5 and 7 months. Although prenatal MMC repair can achieve HBH reversal in a majority of well-selected cases, the prevention of postnatal HCP requiring intervention appears not to be predicated on this outcome alone. In fact, it appears that in a subset of cases in which HBH reversal is achieved, patients can experience a progressive, gradual-onset HCP within the 1st year of life. These findings support continued rigorous postnatal surveillance of all prenatal MMC repair patients, irrespective of postoperative HBH outcome.

Maternal and obstetric complications in fetal surgery for prenatal myelomeningocele repair: a systematic review.

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. 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. 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. 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.

OP17.07: Prenatal myelomeningocele repair: risk factors for developing chorioamniotic membrane separation

OP17.07: Prenatal myelomeningocele repair: risk factors for developing chorioamniotic membrane separation

EP25.06: Perinatal outcomes after detection of postoperative chorioamniotic membrane separation following prenatal myelomeningocele repair

EP25.06: Perinatal outcomes after detection of postoperative chorioamniotic membrane separation following prenatal myelomeningocele repair

EP25.07: Prenatal myelomeningocele repair complicated with chorioamniotic membrane separation: predictors of adverse perinatal outcomes

EP25.07: Prenatal myelomeningocele repair complicated with chorioamniotic membrane separation: predictors of adverse perinatal outcomes

Congress of Neurological Surgeons Systematic Review and Evidence-Based Guidelines for Pediatric Myelomeningocele: Executive Summary.

The incidence of spina bifida (SB) in the developing world is higher than in the United States because of malnutrition and folic acid deficiency during pregnancy. Advances in technology have made prenatal repair of myelomeningocele (MM) possible. The objective of the guidelines are, (1) To create clinical recommendations for best practices, based on a systematic review and analysis of available literature, (2) to obtain multi-disciplinary endorsement of these guidelines from relevant organizations, and (3) to disseminate the educational content to physicians to improve the care of infants with MM. The Guidelines Task Force developed search terms and strategies used to search PubMed and Embase for 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. Guidelines authors aimed to systematically review the literature and make evidence based recommendations about the timing of closure after birth, hydrocephalus, the impact of prenatal closure, and the effect of prenatal closure on ambulation ability and tethered spinal cord. Evidence concerning persistent ventriculomegaly and cognitive impairment was also evaluated. Hundreds of abstracts were identified and reviewed for each of the 5 topics. A total of 14 studies met stringent inclusion criteria. Based on a comprehensive systematic review, a total of 5 clinical practice recommendations were developed, with 1 Level I, 2 Level II and 2 Level III recommendations.The full guideline can be found at https://www.cns.org/guidelines/guidelines-spina-bifida-chapter-1.

Congress of Neurological Surgeons Systematic Review and Evidence-Based Guideline on the Incidence of Tethered Cord Syndrome in Infants With Myelomeningocele With Prenatal Versus Postnatal Repair.

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. 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. 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. A total of 261 abstracts were reviewed. Fifty-four full-text articles were selected for further analysis. Three studies met inclusion criteria. 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.

YouTube as a Source of Patient Information for Prenatal Repair of Myelomeningocele.

With the increasing interest in fetal repair of myelomeningoceles (MMCs) over the last decade, it is reasonable to anticipate the need for high quality and accessible educational materials for patients. Patients often look to the internet for details regarding medical topics and specifically to YouTube for informative health-related videos. This study aims to analyze the content and shortcomings of currently available videos on YouTube regarding prenatal repair of MMCs. A YouTube search was performed on December 15, 2018, using the terms "fetoscopic surgery for neural tube defect" and "fetal surgery for neural tube defect." The first 50 videos from each search were sorted by relevance and evaluated for video source (i.e., professional, personal, or other), target audience (medical professionals or general public), general descriptive statistics (i.e., video length, number of views, number of comments), and for five areas of content determined by the authors to constitute basic patient information regarding a surgical procedure: (1) procedure details, (2) eligibility criteria, (3) alternatives to surgery, (4) surgical risks, and (5) success rate. Accuracy of videos was not assessed. Of the 16 videos that met inclusion criteria, only 1 discussed fetoscopic surgery. The majority (62.5%) of videos were produced by a professional source and 81.3% were targeted toward the general public rather than medical professionals. Of the 16 videos, 10 (62.5%) included details regarding the surgery, 3 (18.8%) discussed eligibility criteria, and 8 (50.0%) mentioned alternatives to surgery. Additionally, seven videos (43.8%) discussed risks of the procedure and six (37.5%) included surgical success rate. Only 2 of the 16 videos included all five areas of content that were evaluated, and both were in regard to open fetal repair. This study not only calls attention to the initial shortcomings of YouTube videos regarding fetal surgery for neural tube defects but also demonstrates the need for further investigation and more comprehensive analysis.

Morbidity and cost burden of prenatal myelomeningocele repair

Objective This study compared the morbidities and financial burden associated with prenatal open myelomeningocele repair versus postnatal repair. Materials and Methods The retrospective study cohort included 23 mother-infant dyads undergoing prenatal repair and 30 with postnatal repair. Financial, demographic, and medical information were obtained for mother-infant dyads from each infant’s birth through the first year of life. Results Infants in the prenatal repair group were significantly affected by sequelae of prematurity, including apnea, bronchopulmonary dysplasia, and retinopathy of prematurity. Importantly, fewer of these infants required ventriculoperitoneal shunt procedures and the overall improved composite outcome of shunting or death. Infants in the postnatal repair group required more hospital readmissions in the first year of life. Financial costs for mother–infant dyads in the prenatal repair group were higher, driven by the length of stay in the neonatal intensive care unit and maternal hospital admissions. Kaplan–Meier curves analyzing the relative contributions of the length of stay and total charges in the population were constructed. The maternal contribution was markedly different between groups due to postoperative hospitalization, readmission, and close fetal surveillance required. Though these differences did not reach statistical significance, it highlights an important burden on families and the medical system. Conclusions This is the first study to report the contributions of prematurity in relation to maternal and infant morbidity and financial costs. The benefits of prenatal myelomeningocele repair include lower risk for ventriculoperitoneal shunting and fewer hospital readmissions. The risk of preterm birth and its neonatal sequelae continue to be a significant burden.

Maternal anaesthesia in open and fetoscopic surgery of foetal open spinal neural tube defects: A retrospective cohort study.

Prenatal myelomeningocele repair by open surgery can improve the neurological prognosis of children with this condition. A shift towards a fetoscopic approach seems to reduce maternal risks and improve obstetric outcomes. The aim of this study was to report on the anaesthetic management of women undergoing prenatal open or fetoscopic surgery for neural tube defects. A retrospective cohort study. Prenatal myelomeningocele repair research group, Vall d'Hebron University Hospital, Spain. Intra-uterine foetal repairs of spina bifida between 2011 and 2016 were reviewed. Anaesthetic and vasoconstrictor drugs, fluid therapy, maternal haemodynamic changes during surgery, blood gas changes during CO2 insufflation for fetoscopic surgery, and maternal and foetal complications were noted. Twenty-nine foetuses with a neural tube defect underwent surgery, seven (24.1%) with open and 22 (75.9%) with fetoscopic surgery. There were no significant differences in maternal doses of opioids or neuromuscular blocking agents. Open surgery was associated with higher dose of halogenated anaesthetic agents [maximum medium alveolar concentration (MAC) sevoflurane 1.90 vs. 1.50%, P = 0.01], higher need for intra-operative tocolytic drugs [five of seven (71.4%) and two of 22 (9.1%) required nitroglycerine, P = 0.001], higher volume of colloids (500 vs. 300 ml, P = 0.036) and more postoperative tocolytic drugs (three drugs in all seven cases (100%) of open and in one of 21 (4.76%) of fetoscopic surgery, P < 0.001). Median mean arterial pressure was lower in open than in fetoscopic surgery. SBP, DBP and mean blood pressure decreased during uterine exposure, and this descent was more acute in open surgery. Use of vasoconstrictor drugs was related to the time of uterine exposure, but not to surgical technique. Blood gas analysis was not affected by CO2 insufflation during fetoscopic surgery. Open surgery was associated with more maternal haemodynamic changes and higher doses of halogenated anaesthetic and tocolytics agents than fetoscopic surgery.

Use of plastic wound retractor at hysterotomy site in prenatal repair of myelomeningocele: a new technique

Objective: To describe the use of a self-retaining thermoplastic polyurethane wound retractor for the management of hysterotomy during prenatal repair of myelomeningocele.Methods: The preliminary experience with 16 consecutive cases of open surgery for prenatal repair of myelomeningocele using a new technique is presented. Under general anesthesia, the gravid uterus was partially exteriorized through a low transverse abdominal incision and a high 3–4-cm midline vertical mini-hysterotomy was performed. After chorioamniotic membranes were opened with scissors, the internal ring of the retractor was placed into the amniotic cavity to compress the chorioamniotic membranes against the internal uterine wall and permit full retraction of the hysterotomy. Once the neurosurgical intervention was completed, the retractor was removed manually and uterine and abdominal incisions were closed using conventional techniques. Intra- and post-operative complications, as well as short-term pregnancy outcomes, were evaluated.Results: Intrauterine surgery was performed at a mean gestational age of 25.3 weeks and all except two of the interventions were completed within 3.0 h. When compared with the technique described in the Management of Myelomeningocele study (MOMS) trial, the use of the retractor was associated with a lower, although statistically nonsignificant, rate of chorioamniotic membrane separation (20/78 (26%) versus 2/16 (13%), respectively), preterm rupture of membranes (36/78 (46%) versus 4/16 (25%), respectively), and persistent oligohydramnios (16/78 (21%) versus 1/16 (6%), respectively) as well as higher gestational age at delivery (34.1 weeks ± 3.1 versus 36.0 weeks ± 1.93, respectively) and birthweight (2383 g ± 688 versus 2790 g ± 529, respectively). There were no intra- or post-operative complications associated with the use of the device. Only one (6%) of the hysterotomy scars was noted to be thin at the time of the cesarean delivery and no cases of dehiscence occurred.Conclusions: The use of a plastic wound retractor at the hysterotomy site provides a less traumatic approach than the conventional technique for the management of the uterine incision during open intrauterine surgery. Our experience with this technique was associated with short-term pregnancy outcomes that are similar and perhaps even superior to the technique reported in the MOMS trial. Because the device is inexpensive, easy to use, and widely available, its use during open intrauterine surgery should be considered. However, further clinical experience is required to reach a definitive conclusion regarding whether this technique should be incorporated into the protocol of prenatal repair of myelomeningocele.

Doppler changes in umbilical artery and ductus venosus during fetoscopic prenatal surgical repair of myelomeningocele.

Cardiac dysfunction has been described in 60% of fetuses undergoing open myelomeningocele (MMC) repair. Routine Doppler evaluation of the umbilical artery (UA) and ductus venosus (DV) is challenging during fetal surgery. The aim of this study was to describe Doppler changes in the UA and DV in a cohort of fetuses during fetoscopic MMC repair. This was a retrospective study of fetuses that underwent fetoscopic repair of MMC at a single institution between 2014 and 2017. Ultrasound images taken intraoperatively were analyzed to describe the changes in Doppler parameters of the UA and DV during fetoscopic MMC repair. The timing of Doppler changes was matched with the phase of surgery to determine whether a pattern to their appearance could be ascertained. Of the thirty-five cases of fetoscopic MMC repair performed during the study period, Doppler data were available for 15. Of these, UA Doppler data were obtained in 12 cases, and DV Doppler data were obtained in 13 cases. Of the 12 cases for which there were UA data, nine (75%) showed transient absence of end-diastolic flow (EDF), with five (41.7%) of them progressing to reversal of EDF. Of the 13 cases for which there were DV data, nine had elevated pulsatility index; however, none of them had absent or reversed blood flow during atrial contraction. Of the nine cases with absent EDF in the UA, this abnormality first appeared during fetoscopic repair in five (55.6%), after uterus exteriorization but before CO2 insufflation in two (22.2%), after uterus replacement but before skin closure in one (11.1%) and after CO2 insufflation but before the start of the MMC repair in one (11.1%). Doppler abnormalities in four fetuses with reversed EDF and in three with absent EDF resolved during surgery. Follow-up examination 1 day after surgery demonstrated resolution of UA and DV Doppler abnormalities in all fetuses. Transient Doppler abnormalities of the UA, without significant changes in the DV, can be seen during fetoscopic MMC repair. The clinical significance of these Doppler findings remains unclear due to their transient and intermittent nature. Copyright © 2018 ISUOG. Published by John Wiley & Sons Ltd.

PRENATAL MYELOMENINGOCELE REPAIR – A CHANCE TO IMPROVE THE QUALITY OF LIFE

Spina bifida (myelomeningocele) is the most prevalent developmental defect of the nervous system. In different regions of the world it affects from 0.3 to 5 per 1000 of living neonates. It is known that the defect develops during the early fetal life however no explicit cause has been found yet. Recently it has been accepted that one of the major factors contributing to spina bifida is deficiency of folic acid in the mother's body before and during pregnancy. The first attempts to cure myelomeningocele were taken in ancient times. More extensive knowledge of aetiology and effects of spina bifida and development of new surgical techniques allowed for management not only of the defect itself but also the consequent complications. Prenatal spina bifida repair has been performed in the USA for 20 years now while European surgeons (Poland and Switzerland) have operated myelomeningocele for 12 years. Comparative analysis carried out to evaluate the patients operated for spina bifida prenatally and postnatally pointed that those after fetal repair demand the use of ventriculoperitoneal shunt for hydrocephalus twice less frequently. Thanks to shortened exposure of the spinal cord and the spinal nerves to the toxic effect of amniotic fluid and reduced exposure to mechanical injuries, prenatal myelomeningocele repair offers better opportunities to improve motor, urinary and alimentary functions, contributing then statistically to higher quality of lives of children with spina bifida.

187: Correlation between postoperative sonographic and neonatal lower extremity movement after in utero repair for meningomyelocele

Lower extremity movement (LEM) in fetuses undergoing prenatal myelomeningocele (MMC) repair is generally assessed at a neonatal evaluation (NE). Ultrasound (US) can assess LEM antenatally, but the correlation between US and NE is not firmly established, nor have differences in US findings on different postoperative days (POD) been correlated with NE. This was a retrospective cohort correlation study of fetuses that underwent open repair of myelomeningocele at SSM Cardinal Glennon Fetal Care Institute, St. Louis MO between January 2011 and December 2016. Movement of the lower extremities at the toe, ankle, knee, and hip of each leg was assessed by US at the initial visit, after open repair on POD 0-5, and at 32 weeks gestation. After delivery, NE was performed by physical therapists and a neurosurgeon, with 94% occurring in the first month of life. 41 fetuses were included. Follow-up was 100%. Movement at the ankle at the initial visit was associated with the function in the same joint on NE. US of the ankle and knee on POD 3 were strongly predictive (k=0.710, 0.420-1.000 and k= 0.577, 0.152-1.000, respectively). Ankle US on POD 4 was also strongly correlated with NE of the ankle (k=0.659, 0.308-1.000). Any LEM varied by POD: it was present on POD 1 in 45.2% of fetuses who showed neonatal movement and increased to 100% by POD 5. Postoperative US assessment of movement in the knee, ankle, and toe in fetuses undergoing open fetal MMC repair correlates with NE. POD 3 assessments at the ankles and knees and strongly associated with neonatal joint function. LEM increases between POD 1 and POD 5 in fetuses that will have neonatal function.View Large Image Figure ViewerDownload Hi-res image Download (PPT)

The Management of Myelomeningocele Study: full cohort 30-month pediatric outcomes.

Previous reports from the Management of Myelomeningocele Study demonstrated that prenatal repair of myelomeningocele reduces hindbrain herniation and the need for cerebrospinal fluid shunting, and improves motor function in children with myelomeningocele. The trial was stopped for efficacy after 183 patients were randomized, but 30-month outcomes were only available at the time of initial publication in 134 mother-child dyads. Data from the complete cohort for the 30-month outcomes are presented here. Maternal and 12-month neurodevelopmental outcomes for the full cohort were reported previously. The purpose of this study is to report the 30-month outcomes for the full cohort of patients randomized to either prenatal or postnatal repair of myelomeningocele in the original Management of Myelomeningocele Study. Eligible women were randomly assigned to undergo standard postnatal repair or prenatal repair <26 weeks gestation. We evaluated a composite of mental development and motor function outcome at 30 months for all enrolled patients as well as independent ambulation and the Bayley Scales of Infant Development, Second Edition. We assessed whether there was a differential effect of prenatal surgery in subgroups defined by: fetal leg movements, ventricle size, presence of hindbrain herniation, gender, and location of the myelomeningocele lesion. Within the prenatal surgery group only, we evaluated these and other baseline parameters as predictors of 30-month motor and cognitive outcomes. We evaluated whether presence or absence of a shunt at 1 year was associated with 30-month motor outcomes. The data for the full cohort of 183 patients corroborate the original findings of Management of Myelomeningocele Study, confirming that prenatal repair improves the primary outcome composite score of mental development and motor function (199.4 ± 80.5 vs 166.7 ± 76.7, P= .004). Prenatal surgery also resulted in improvement in the secondary outcomes of independent ambulation (44.8% vs 23.9%, P= .004), WeeFIM self-care score (20.8 vs 19.0, P= .006), functional level at least 2 better than anatomic level (26.4% vs 11.4%, P= .02), and mean Bayley Scales of Infant Development, Second Edition, psychomotor development index (17.3% vs 15.1%, P= .03), but does not affect cognitive development at 30 months. On subgroup analysis, there was a nominally significant interaction between gender and surgery, with boys demonstrating better improvement in functional level and psychomotor development index. For patients receiving prenatal surgery, the presence of in utero ankle, knee, and hip movement, absence of a sac over the lesion and a myelomeningocele lesion of ≤L3 were significantly associated with independent ambulation. Postnatal motor function showed no correlation with either prenatal ventricular size or postnatal shunt placement. The full cohort data of 30-month cognitive development and motor function outcomes validate in utero surgical repair as an effective treatment for fetuses with myelomeningocele. Current data suggest that outcomes related to the need for shunting should be counseled separately from the outcomes related to distal neurologic functioning.

Modified Hysterotomy Closure Technique for Open Fetal Surgery

Objective: We reviewed our experience with open fetal surgical myelomeningocele repair to assess the efficacy of a new modification of the hysterotomy closure technique regarding hysterotomy complication rates at the time of cesarean delivery. Methods: A modification of the standard hysterotomy closure was performed on all patients undergoing prenatal myelomeningocele repair. The closure consisted of an interrupted full-thickness #0 polydioxanone (PDS) retention suture as well as a running #0 PDS suture to re-approximate the myometrial edges, and the modification was a third imbricating layer resulting in serosal-to-serosal apposition. A standard omental patch was placed per our routine. Both operative reports and verbal descriptions of hysterotomy from delivering obstetricians were reviewed. Results: A total of 49 patients underwent prenatal repair of myelomeningocele, 43 having adequate follow-up for evaluation. Of those, 95.4% had completely intact hysterotomy closures, with only 1 partial dehiscence (2.3%) and 1 thinned scar (2.3%). There were no instances of uterine rupture. Discussion: In patients undergoing this modified hysterotomy closure technique, a much lower than expected complication rate was observed. This simple modified closure technique may improve hysterotomy healing and reduce obstetric morbidity.

Anesthesia for myelomeningocele surgery in fetus.

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. This review provides a systematic approach to the issues that are faced by anesthesiologists during fetal surgery.