Prenatal Myelomeningocele Repair Research Articles

Enhanced recovery after fetal spina bifida surgery: global practice.

Enhanced recovery after surgery (ERAS) protocols are multimodal evidence-based care plans that have been adopted for multiple surgical procedures to promote faster and better patient recovery and shorter hospitalization. This study aimed to explore whether worldwide fetal therapy centers offering prenatal myelomeningocele repair implement the ERAS principles and to provide recommendations for improved perioperative management of patients. In this survey study, a total of 53 fetal therapy centers offering prenatal surgery for open spina bifida were identified and invited to complete a digital questionnaire covering their pre-, intra- and postoperative management. An overall score was calculated per center based on compliance with 20 key ERAS principles, extrapolated from ERAS guidelines for Cesarean section, gynecological oncology and colorectal surgery. Each item was awarded a score of 1 or 0, depending, respectively, on whether the center did or did not comply with that principle, with a maximum score of 20. The questionnaire was completed by 46 centers in 17 countries (response rate, 87%). In total, 22 (48%) centers performed exclusively open fetal surgery (laparotomy and hysterotomy), whereas 14 (30%) offered both open and fetoscopic procedures and 10 (22%) used only fetoscopy. The perioperative management of patients undergoing fetoscopic and open surgery was very similar. The median ERAS score was 12 (range, 8-17), with a mean ± SD of 12.5 ± 2.4. Center compliance was the highest for the use of regional anesthesia (98%), avoidance of bowel preparation (96%) and thromboprophylaxis (96%), while the lowest compliance was observed for preoperative carbohydrate loading (15%), a 2-h fasting period for clear fluids (20%), postoperative nausea and vomiting prevention (33%) and early feeding (35%). ERAS scores were similar in centers with a short (2-5 days), medium (6-10 days) and long (≥ 11 days) hospital stay (mean ± SD, 12.9 ± 2.4, 12.1 ± 2.0 and 10.3 ± 3.2, respectively, P = 0.15). Furthermore, there was no significant association between ERAS score and surgical technique or case volume. The perioperative management of fetal spina bifida surgery is highly variable across fetal therapy centers worldwide. Standardized protocols integrating ERAS principles may improve patient recovery, reduce maternal morbidity and shorten the hospital stay after fetal spina bifida surgery. © 2024 The Author(s). Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.

Tethered cord prevalence among patients with prenatal or postnatal myelomeningocele repair.

Myelomeningocele (MMC) is a severe form of spina bifida characterized by spinal cord extrusion into a cerebrospinal fluid (CSF) filled sac which may lead to lifelong disability. Repair of these lesions have classically occurred shortly after birth, but more recently, prenatal myelomeningocele repair techniques have been elucidated. This study aimed to investigate the outcome of surgery, particularly with subsequent spinal cord tethering, in patients with prenatal myelomeningocele closure and those with postnatal repair surgery. In this retrospective study which assessed patients from April 2002 to April 2020, the data of 18 total patients with MMC were reviewed. Nine patients from each group were included, closed prenatally or postnatally, respectively. Demographic information including age and sex, birth week, infant comorbidities, presence of dermoid or lipoma, cutaneous stigmata, total number and timing of detethering procedures, presence of Chiari malformation, and need for VPS was obtained. Eighteen patients including nine infants closed prenatally and nine infants with myelomeningoceles closed postnatally were reviewed. The mean age was 4 ± 3years and 6.22 ± 2.4 in prenatal and postnatal retrospectively. There was a significant relationship between the number of detethering procedures (p-value = 0.03) and the need for a ventriculoperitoneal shunt (VPS) (p-value = 0.01) between the groups, with the prenatal closure group having lower rates of each. There was no significant difference between the groups in regard to the mean age at the detethering procedure (p = 0.4), sex (p = 0.09), birth week (p = 0.8), comorbidities (p = 0.8), presence of intraspinal dermoid or lipoma (p = 0.09), presence of cutaneous stigma (p = 0.08), Chiari (p = 0.6), fatty filum (p = 0.08), syrinx (p = 0.4), bone anomaly (p = 0.4), and spina bifida neurological scale (p = 0.66). There was a significant relationship between the two groups in terms of the number of detethering procedures, and the need for VPS. Our data represents a possible difference in the need for detethering surgeries and ventriculoperitoneal shunt placements between patients with prenatal and postnatal myelomeningocele closures.

MRI analysis of neurodevelopmental anatomy in myelomeningocele: prenatal vs postnatal repair.

Compared with postnatal repair, prenatal myelomeningocele (MMC) repair is associated with improved motor function and decreased need for cerebrospinal fluid (CSF) diversion. It is unknown how prenatal surgery alters neuroanatomical structures identifiable on magnetic resonance imaging (MRI). The purpose of this study was to use MRI to compare neurodevelopmental anatomy in patients undergoing fetal MMC repair compared with those undergoing postnatal repair. This was a retrospective review of neonates who underwent prenatal or postnatal MMC repair at our institution between 2016 and 2021. Imaging data, including prenatal ultrasound and pre- and postnatal MRI examinations, if available, were retrieved. We analyzed anatomical findings characteristically seen on MRI of the neuroaxis in patients with MMC and compared imaging findings between patients with prenatal vs postnatal MMC repair. The study population included 61 patients who underwent surgical repair for MMC during the 6-year study period, of whom 25 underwent prenatal repair and 36 postnatal repair. CSF diversion was required in 24% of the prenatally repaired cohort vs 67% of the postnatally repaired cohort (P = 0.001). On postnatal MRI, a syrinx was present in 13% of the prenatally repaired cohort vs 42% in the postnatally repaired cohort (P = 0.02). Postnatal corpus callosal (CC) morphology was abnormal in 54% of the prenatally repaired cohort vs 53% of the postnatally repaired cohort (P = 0.92), while falx morphology was normal in 92% of the prenatally repaired cohort vs 34% of the postnatally repaired cohort (P < 0.001). On postnatal MRI, patients in the prenatally repaired cohort had a shorter tentorium-to-foramen-magnum distance compared with those in the postnatally repaired cohort (mean, 18.43 mm vs 22.42 mm; P = 0.01), a larger foramen magnum diameter (mean, 22.87 mm vs 18.94 mm; P < 0.001) and a smaller degree of hindbrain herniation (mean, 1.53 mm vs 8.72 mm; P < 0.001). The cerebral aqueduct was patent in 79% of the prenatally repaired cohort vs 100% of the postnatally repaired cohort on postnatal MRI (P = 0.008). Between the two cohorts, at postnatal MRI there was no significant difference in the presence of gray-matter heterotopia, presence of the septum pellucidum or size of the massa intermedia. We report variations in developmental neuroanatomy in patients with MMC, including rates of CC dysgenesis, gray-matter heterotopia and additional cranial and spinal MRI findings. Compared to postnatal surgery, prenatal surgery is associated with changes to infratentorial anatomy, with minimal effect on supratentorial brain development. This information will be useful in counseling parents affected by fetal MMC and in understanding how prenatal repair of MMC affects brain development. © 2024 International Society of Ultrasound in Obstetrics and Gynecology.

Prenatal surgery in fetal myelomeningocele with severe ventriculomegaly.

Prenatal myelomeningocele (MMC) repair is performed to decrease risk for treatment of hydrocephalus after birth and to preserve motor function. Some centers may not consider patients candidates for surgery if severe ventriculomegaly is present and there is no expected benefit in decreased risk for hydrocephalus treatment. This study sought to compare postnatal outcomes of fetuses with MMC and severe ventriculomegaly (>15mm) who underwent prenatal repair, with fetuses with severe ventriculomegaly who underwent postnatal repair and fetuses with ventriculomegaly (<15mm) who underwent prenatal repair. This was a retrospective study of fetuses with MMC that underwent prenatal or postnatal repair between 2012 and 2021 at a single institution. The cohort was divided based on preoperative fetal ventricular size into two groups, those with severe ventriculomegaly (≥15 mm) and those without severe ventriculomegaly (<15 mm). Fetal ventricular size was measured by MRI prior to surgery using the standardized approach and the mean of the left and right ventricle was used for analysis. Motor function of lower extremities was assessed at the time of referral by ultrasound and if flexion-extension movements of the ankle were seen, it was considered as preserved S1 motor function. Postnatal outcomes including motor function of lower extremities assessed at birth and need for diversion procedure for hydrocephalus treatment during the first year of life were collected and compared between groups. Data was presented as median and range or number and percentages as appropriate. P value >0.05 was considered statistically significant. Multivariate regression analysis was used to adjust for potential confounders. 154 patients were included in this study: 145 patients underwent fetal surgery (101 fetoscopic and 44 open hysterotomy) and 9 patients with severe ventriculomegaly underwent postnatal repair. Among the 145 patients who underwent fetal surgery, 22 presented with severe ventriculomegaly. Prenatally repaired fetuses with severe ventriculomegaly at referral were at a significantly higher need for hydrocephalus treatment by 12 months than those without severe ventriculomegaly (62% vs. 29%, p<0.01). However, motor function assessment at birth was similar between both prenatally repaired groups (OR=0.92, 95% CI [0.33-2.59], p=0.88) adjusted for the anatomical level of the lesion. The prenatally repaired group with severe ventriculomegaly had better preserved motor function levels at birth compared to the postnatal repair group with severe ventriculomegaly (L3 with 11.1% S1 motor function; p=<0.01 and p=<0.01). Prenatally repaired patients with severe ventriculomegaly had an 18.9 times chance of having an intact motor function at birth [95% CI (1.2 - 290.1)] adjusted for ethnicity, presence of clubfeet at referral, and gestational age at delivery compared to postnatal repair. There was not a significant difference in the need for hydrocephalus treatment in the first year of life between prenatal and postnatal repair of patients with severe ventriculomegaly (61.9% vs 87.5%, p=0.18). Although fetuses with MMC and severe ventriculomegaly do not seem to benefit from fetal surgery in terms of postnatal hydrocephalus treatment, they benefit from increased chance of preserved motor function at birth. Results from this study highlight the benefits of having prenatal MMC repair for cases with severe ventriculomegaly at referral to preserve motor function. This article is protected by copyright. All rights reserved.

Maternal and perinatal outcomes after prenatal or postnatal surgical repair of myelomeningocele

Purpose: To evaluate maternal and perinatal outcomes following modified open fetal or postnatal surgery to repair myelomeningocele at a pioneering referral center for prenatal surgery in northeastern Brazil. Methods: This observational, retrospective cohort study assessed data recorded at the Instituto de Medicina Integral Prof. Fernando Figueira between December 2014 and May 2020. Pregnant women with fetuses diagnosed with myelomeningocele were enrolled. Exclusion criteria consisted of cases for which medical records were unavailable and those for which intrauterine repair was not recommended. Student’s t-test, Welch’s t-test, the chi-square test and Fisher’s exact test were used, as appropriate, in the statistical analysis. Significance level was 5%. Risk ratios (RR) and their 95% confidence intervals (95%CI) were calculated. Results: Of the 134 pregnant women identified in the database, 45 were included: 26 women underwent fetal surgery and 19 infants were operated after birth. Pregnancy complications were more common in the fetal surgery group, including premature rupture of membranes (RR: 14.0; 95%CI: 0.8-263.5; p=0.027) and other complications (RR: 5.3; 95%CI: 1.2-22.8; p=0.025). Fetal surgery was significantly longer than postnatal surgery (mean duration 226 x 71 minutes, respectively; p&lt;0.001). Prematurity was more common with fetal (79.2%) compared to postnatal surgery (13.3%) (RR: 5.9; 95%CI: 1.4-25.5; p=0.017). Although ventriculoperitoneal shunts were more common in the postnatal (18.2%) compared to the prenatal group (0%), the two groups were statistically similar (p=0.50). Conclusion: Prenatal myelomeningocele repair was associated with more cases of adverse pregnancy outcomes and prematurity. Conversely, no cases of open fetal surgery required ventriculoperitoneal shunting.

Sleep-related breathing disorders in infants with spina bifida repaired prenatally and postnatally.

Advances in prenatal repair of myelomeningocele (MMC) have improved outcomes involving different organ systems. There is limited data on respiratory outcomes following prenatal surgical repair. We hypothesize there is no difference in respiratory outcomes between spina bifida (SB) patients who have undergone prenatal versus postnatal repair. We performed a retrospective study of 46 infants <1 year with SB seen at Children's Hospital Los Angeles from 2004-2022. Demographic data, timing of closure, neonatal course, Chiari II malformation (CIIM), ventriculoperitoneal shunt (VPS), polysomnography (PSG) results, and need for supplemental oxygen were collected. Unpaired t-test and Chi-square Test were used to analyze results. 31/46 had prenatal repair of MMC; average age at repair was 27 weeks post-conception (PCA). Average age at postnatal repair was 37 PCA. There was no difference in age at PSG. There was no difference in CIIM presence (p=0.61). 60% of patients with postnatal repair and 23% in the prenatal group underwent VPS placement (p=0.01). Patients with SB who've undergone closure of neural tube defect have persistent central apneas, obstructive apneas, and significant hypoxemia. There were no differences in the frequency or severity of sleep-disordered breathing in those with prenatal repair versus postnatal repair.

Prenatal myelomeningocele repair improves social urine continence, reduces the risk of urinary tract infections and constipations

Prenatal myelomeningocele repair improves social urine continence, reduces the risk of urinary tract infections and constipations

Open fetal surgery with mini-hysterotomy for prenatal myelomeningocele repair - case report

Open fetal surgery with mini-hysterotomy for prenatal myelomeningocele repair - case report

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

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

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EP20.11: Emergency uterine repair after unintentional endoscopic lesion

This is a case report of a 33-year-old pregnant woman undergoing laparoscopic cholecystectomy at 23 weeks of gestation. A 10mm laparoscopic trocar was inserted 4 cm above the navel using an open Hasson technique. When the lens was introduced, the general surgeon observed the amniotic liquid and the placenta along with the fetus, immediately realising that the trocar was unintentionally inserted in the amniotic cavity. The trocar was immobilised and an emergency laparotomy was performed. When the fetal surgery team arrived the trocar was extracted, following a careful inspection of the 1cm uterine lesion. A typical microhysterotomy approach exactly like the one for prenatal myelomeningocele repair was used. The amnios were exteriorised and clamped to the uterine wall while the amniotic fluid was replaced with a 0.9% saline solution. A 2-layer hysterotomy using a monofilament PDS-0 suture was used for closure of the uterus and membranes. There were no complications during the surgery. At 34 weeks of gestation, the patient presented with preterm premature rupture of membranes requiring Caesarean section. A 34-week male neonate was born 11 weeks later, weighing 2620 grams, with an Apgar of 9/9, requiring admission to the neonatal intensive care unit, and discharged without complications 2 weeks later. The mother was discharged 4 days after delivery. Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.

The Construction of a Predictive Composite Index for Decision-Making of CSF Diversion Surgery in Pediatric Patients following Prenatal Myelomeningocele Repair.

There is a wide range of clinical and radiographic factors affecting individual surgeons' ultimate decision for CSF diversion for pediatric patients following prenatal myelomeningocele repair. Our aim was to construct a composite index (CSF diversion surgery index) that integrates conventional clinical measures and neuroimaging biomarkers to predict CSF diversion surgery in these pediatric patients. This was a secondary retrospective analysis of data from 33 patients with prenatal myelomeningocele repair (including 14 who ultimately required CSF diversion surgery). Potential independent variables, including the Management of Myelomeningocele Study Index (a dichotomized variable based on the shunt-placement criteria from the Management of Myelomeningocele Study), postnatal DTI measures (fractional anisotropy and mean diffusivity in the genu of the corpus callosum and the posterior limb of internal capsule), fronto-occipital horn ratio at the time of DTI, gestational ages, and sex, were evaluated using stepwise logistic regression analysis to identify the most important predictors. The CSF diversion surgery index model showed that the Management of Myelomeningocele Study Index and fractional anisotropy in the genu of the corpus callosum were significant predictors (P < .05) of CSF diversion surgery. The predictive value of the CSF diversion surgery index was also affected by fractional anisotropy in the posterior limb of the internal capsule and sex with marginal effect (.05<P < .10), but not by the fronto-occipital horn ratio (P > .10). The overall CSF diversion surgery index model fit the data well with statistical significance (eg, likelihood ratio: P < .001), with the performance (sensitivity = 78.6%; specificity = 86.5%, overall accuracy = 84.8%) superior to all individual indices in sensitivity and overall accuracy, and most of the individual indices in specificity. The CSF diversion surgery index model outperformed all single predictor models and, with additional validation, may potentially be developed and incorporated into a sensitive and robust clinical tool to assist clinicians in hydrocephalus management.

Prenatal myelomeningocele repair: report of an inaugural case and considerations about the implementation of a new fetal neurosurgery program

Myelomeningocele (MMC) or open spina bifida is a neural tube defect which consists of a cleft in the vertebral column and a correspondent defect in the skin, causing the meninges and the spinal cord to be exposed. Despite some recommendations for nutrient supplementation during pregnancy, MMC remains very prevalent, especially in poor regions. MMC causes paralysis, urinary and gastrointestinal dysfunction, motor and neurological deficits, hydrocephalus and hindbrain herniation. Until 1997, the only way to repair this defect was performing surgery right after birth. In 2011, MOMS trial revealed a positive impact of prenatal surgery compared to postnatal. This paper reports the first case of one of the first myelomeningocele prenatal surgery programs in the North and Northeast regions of Brazil and discusses some issues about the implementation of new programs. The patient was a 34 years old woman in her second pregnancy, who was diagnosed at 22 weeks. She was submitted to an open fetal surgery applying MOMS protocol at 26 weeks of pregnancy and underwent cesarean at 36 weeks and five days of pregnancy. After two-years follow up, the baby girl did not need shunt for hydrocephalus, but has motor deficit in the lower limbs. Although fetal surgery is a high-tech procedure that demands a well-trained team, many families come from low-income regions. This way, the implementation of new services has an important social impact.

Maternal decision-making about intrauterine myelomeningocele repair: a qualitative exploration

Prenatal repair of myelomeningocele may improve quality of life for the baby but can increase pregnancy complications and places significant burdens on families. We explored women’s decision-making process and understanding of the treatment options for fetal myelomeningocele.

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

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

Open surgery for in utero repair of spina bifida: Microneurosurgery versus standard technique - A systematic review.

Prenatal myelomeningocele (MMC) repair has been shown to improve neurological outcomes. It has been suggested that decreases in the hysterotomy diameter during surgery can improve perinatal outcomes without altering neurologic outcomes. The objective of this study is to describe and compare the main maternal and fetal outcomes of fetuses undergoing open surgery for MMC repair, through the different modifications (standard-classical, mini-hysterotomy, and microneurosurgery). MEDLINE, EMBASE, Cochrane Central Register of Controlled Trials, Ovid, SciELO, LILACS, PROSPERO. From a total of 112 studies, seven case series were selected including 399 fetuses with open fetal surgery, five studies using the classical technique (n=181), one with mini-hysterotomy (n=176), and one with the microneurosurgery technique (n=42). The mini-hysterotomy and microneurosurgery techniques presented a lower risk of preterm delivery (21.4% and 30%, respectively) compared to the classic technique (47.3%), premature rupture of membranes (78%, 62%, and 72.5 %, respectively), oligohydramnios (0% and 72.5%, respectively), dehiscence of hysterotomy, maintaining the same frequency of Chiari reversion (78%, 62%, and 72.5%, respectively), postnatal correction requirement (0%, 4.8%, and 5.8%, respectively), and lower frequency of requirement for a ventriculoperitoneal shunt placement (13.0%, 7.5%, and 29.1%, respectively). The least invasive techniques (minihysterotomy-microneurosurgery) are possible and reproduceable, as they are associated with better maternal and perinatal outcomes.

Ventricular size measurement methods in fetuses considered for prenatal closure of myelomeningocele.

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

Diffusion tensor imaging in children following prenatal myelomeningocele repair and its predictive value for the need and timing of subsequent CSF diversion surgery for hydrocephalus.

The aim of this study was to investigate diffusion tensor imaging (DTI), an objective and noninvasive neuroimaging technique, for its potential as an imaging biomarker to predict the need and timing of CSF diversion surgery in patients after prenatal myelomeningocele (MMC) repair. This was a retrospective analysis of data based on 35 pediatric patients after prenatal MMC repair (gestational age at birth 32.68 ± 3.42 weeks, range 24-38 weeks; 15 females and 20 males). A logistic regression analysis was used to classify patients to determine the need for CSF diversion surgery. The model performance was compared between using the frontooccipital horn ratio (FOHR) alone and using the FOHR combined with DTI values (the genu of the corpus callosum [gCC] and the posterior limb of the internal capsule [PLIC]). For patients who needed to be treated surgically, timing of the procedure was used as the clinical outcome to test the predictive value of DTI acquired prior to surgery based on a linear regression analysis. Significantly lower fractional anisotropy (FA) values in the gCC (p = 0.014) and PLIC (p = 0.037) and higher mean diffusivity (MD) values in the gCC (p = 0.013) were found in patients who required CSF diversion surgery compared with those who did not require surgery (all p values adjusted for age). Based on the logistic regression analysis, the FOHR alone showed an accuracy of performance of 0.69 and area under the receiver operating characteristic curve (AUC) of 0.60. The performance of the model was higher when DTI measures were used in the logistic regression model (accuracy = 0.77, AUC = 0.84 for using DTI values in gCC; accuracy = 0.75, AUC = 0.84 for using DTI values in PLIC). Combining the DTI values of the gCC or PLIC and FOHR did not improve the model performance when compared with using the DTI values alone. In patients who needed CSF diversion surgery, significant correlation was found between DTI values in the gCC and the time interval between imaging and surgery (FA: ρ = 0.625, p = 0.022; MD: ρ = -0.6830, p = 0.010; both adjusted for age and FOHR). The authors' data demonstrated that DTI could potentially serve as an objective biomarker differentiating patients after prenatal MMC repair regarding those who may require surgery for MMC-associated hydrocephalus. The predictive value for the need and timing of CSF diversion surgery is highly clinically relevant for improving and optimizing decision-making for the treatment of hydrocephalus in this patient population.

Functional Motor Skills in Children Who Underwent Fetal Myelomeningocele Repair: Does Anatomic Level Matter?

In this study, we evaluated children who underwent prenatal myelomeningocele (MMC) repair to investigate the influence of the anatomical level of the lesion on functional motor skills and congenital orthopedic malformations. This cross-sectional study evaluated children who underwent prenatal correction. The anatomical level of the lesion was classified by observing the magnetic resonance of the spine. The sensory/motor assessment was performed by physical examination to classify the status of ambulation, functional level, and functional performance according to chronological age using the Pediatric Evaluation of Disability Inventory (PEDI-CAT) scale. One hundred cases were evaluated; for 60%, lesions were located in the upper lumbar region and for 40%, they were located in the lower lumbar and sacral regions. The functionality, measured by the PEDI-CAT scale, showed a normal average according to age (mean 50), with 71% of children being community ambulators. Congenital orthopedic malformations were also unrelated to the injury levels, except for knee dislocation in relation to upper lumbar injury. At the functional level, we observed that for the majority, the levels of function of at least 2 vertebrae were below the anatomical level. There were no differences in functional motor skills, walking pattern, or congenital orthopedic malformation when compared with the anatomical level of injury in patients who underwent prenatal repair of MMC, except for congenital knee dislocation.

Prenatal Repair and Physical Functioning Among Children With Myelomeningocele

The Management of Myelomeningocele Study (MOMS), a randomized clinical trial of prenatal vs standard postnatal repair for myelomeningocele, found that prenatal repair reduced hydrocephalus and hindbrain herniation and improved motor function in children aged 12 to 30 months. The Management of Myelomeningocele Study Follow-up (MOMS2) was conducted in children at ages 5 to 10 years. The primary (neurocognitive) outcome has already been reported. To determine whether MOMS2 participants who had prenatal repair have better physical functioning than those with postnatal repair. Participants from MOMS were recruited for participation in the follow-up study, MOMS2, conducted from April 9, 2012, to April 15, 2017. For this secondary analysis of the randomized clinical trial, trained examiners without knowledge of the treatment group evaluated the physical characteristics, self-care skills, neurologic function, and mobility of the children. Physical functioning outcomes were compared between the prenatal and postnatal repair groups. MOMS2 was conducted at the same 3 clinical sites as MOMS. Home visits were conducted for families who were unable to travel to one of the clinical sites. Of the 161 children with myelomeningocele aged 5 to 10 years old enrolled in MOMS2, 154 had a physical examination and were included in the analyses. Prenatal repair of myelomeningocele. Prespecified secondary trial outcomes of self-care skills, functional mobility, walking skills, and motor level. This analysis included 78 children with postnatal repair (mean [SD] age, 7.4 [2.1] years; 50 girls [64.1%]; 69 White children [88.5%]) and 76 with prenatal repair (mean [SD] age, 7.5 [1.2] years; 43 boys [56.6%]; 70 White children [92.1%]). Children in the prenatal repair group were more competent with self-care skills (mean [SD] percentage of maximum FRESNO Scale score, 90.8% [9.6%] vs 85.5% [17.6%]) and were commonly community ambulators per the Modified Hoffer Classification (51.3% prenatal vs 23.1% postnatal; adjusted relative risk [aRR] for sex, 1.70; 95% CI, 1.23-2.34). Children with prenatal repair also performed the 10-m walk test 1 second faster (difference in medians, 1.0; 95% CI, 0.3-1.7), had better gait quality (adjusted mean difference for home distances of 5 m, 1.71; 95% CI, 1.14-2.54), and could perform higher-level mobility skills (adjusted mean difference for motor total, 5.70; 95% CI, 1.97-11.18). Children in the prenatal repair group were less likely to have a motor function level worse than their anatomic lesion level (aRR, 0.44; 95% CI, 0.25-0.77). This secondary analysis of a randomized clinical trial found that the physical functioning benefits of prenatal repair for myelomeningocele reported at age 30 months persisted into school age. These findings indicate the benefit of prenatal repair of myelomeningocele for school-aged children. ClinicalTrials.gov Identifier: NCT00060606.