Reflections upon the intrauterine repair of myelomeningocele.

The intrauterine repair of myelomeningocele presents certain advantages and has gained widespread acceptance. It significantly reduces the incidence of Chiari-2 anomalies and hydrocephalus, and it is thought to enhance the neurologic outcome. Nevertheless, several issues remain unsettled and there are no negligible disadvantages. After working with patients with myelomeningocele for 30 years, I thought about how we currently treat them. There are ethical, organizational, neurological, obstetrical, and postnatal aspects worth discussing.

Head growth in patients with myelomeningocele treated with prenatal and postnatal surgery.

OBJECTIVE: The need for permanent CSF diversion is lower in patients who have undergone prenatal surgery for myelomeningocele (MMC) than in those who have undergone postnatal closure. Differences in brain development and head growth between treatment groups are not known, particularly for those who do not require surgical treatment for hydrocephalus. The objective of this study was to determine differences in head growth and to generate MMC-specific head circumference (HC) growth curves for patients who underwent either prenatal or postnatal surgery. METHODS: The authors retrospectively identified patients from St. Louis Children's Hospital who were treated for MMC between 2016 and 2021. HC data were obtained from birth until the most recent follow-up or hydrocephalus treatment. Nonlinear least-squares regression analysis was performed to fit the data into four models: two-term power, Gompertz, West ontogenetic, and Weibull. Subsequently, the curves were assessed for their utility in predicting hydrocephalus treatment. RESULTS: Sixty-one patients (29 females [47.5%], 25 [41%] underwent prenatal surgery, mean gestational age at birth 36.6 weeks) were included in the study. The Weibull model best fit the HC data (prenatal adjusted R2 = 0.95, postnatal adjusted R2 = 0.95), while the Gompertz model had the worst fit (prenatal adjusted R2 = 0.56, postnatal adjusted R2 = 0.39) across both cohorts. Prenatal MMC repair patients had significantly larger HC measurements than their postnatal repair counterparts. The 50th percentile of the Weibull curve was determined as a useful threshold for hydrocephalus treatment: children with HC measurements that crossed and remained above this threshold were significantly more likely to have hydrocephalus treatment regardless of time of MMC repair (prenatal relative risk [RR] 10.0 [95% CI 1.424-70.220], sensitivity 85.7% [95% CI 0.499-0.984], and specificity 82.4% [95% CI 0.600-0.948]; postnatal RR 4.750 [95% CI 1.341-16.822], sensitivity 90.5% [95% CI 0.728-0.980], and specificity 75.0% [95% CI 0.471-0.924]). The HC growth curves of the MMC patients treated prenatally were significantly larger than the WHO HC curves (p < 0.001). CONCLUSIONS: The Weibull model was identified as the HC growth curve with the best fit for MMC patients and serves as a useful predictor of hydrocephalus treatment. For MMC patients with hydrocephalus, prenatal repair patients fit the model well but postnatal repair patients did not, potentially indicating different mechanisms of hydrocephalus development. Those treated prenatally had significantly larger HC measurements compared with both the general population and those treated postnatally. Further study is needed to understand the long-term cognitive outcomes and optimal management of clinically asymptomatic patients with large HC measurements who were treated prenatally for MMC.

Predictors and timing of hydrocephalus treatment in patients undergoing prenatal versus postnatal surgery for myelomeningocele.

OBJECTIVE: Although hydrocephalus rates have decreased with intrauterine surgery for myelomeningocele (MMC), 40%-85% of children with MMC still go on to develop hydrocephalus. Prenatal ventricle size is known to be associated with later development of hydrocephalus; however, it is not known how prediction measures or timing of hydrocephalus treatment differ between pre- and postnatal surgery for MMC. The goal of this study was to determine anatomical, clinical, and radiological characteristics that are associated with the need for and timing of hydrocephalus treatment in patients with MMC. METHODS: The authors retrospectively identified patients from Barnes Jewish Hospital or St. Louis Children's Hospital between 2016 and 2021 who were diagnosed with MMC prenatally and underwent either pre- or postnatal repair. Imaging, clinical, and demographic data were examined longitudinally between treatment groups and hydrocephalus outcomes. RESULTS: Fifty-eight patients were included (27 females, 46.6%), with a mean gestational age at birth of 36.8 weeks. Twenty-three patients (39.7%) underwent prenatal surgery. For the overall cohort, the ventricle size at prenatal ultrasound (HR 1.175, 95% CI 1.071-1.290), frontal-occipital horn ratio (FOHR) at birth > 0.50 (HR 3.603, 95% CI 1.488-8.720), and mean rate of change in head circumference (HC) in the first 90 days after birth (> 0.10 cm/day: HR 12.973, 95% CI 4.262-39.486) were identified as predictors of hydrocephalus treatment. The factors associated with hydrocephalus in the prenatal cohort were FOHR at birth > 0.50 (HR 27.828, 95% CI 2.980-259.846) and the rate of change in HC (> 0.10 cm/day: HR 39.414, 95% CI 2.035-763.262). The factors associated with hydrocephalus in the postnatal cohort were prenatal ventricle size (HR 1.126, 95% CI 1.017-1.246) and the mean rate of change in HC (> 0.10 cm/day: HR 24.202, 95% CI 5.119-114.431). FOHR (r = -0.499, p = 0.008) and birth HC (-0.409, p = 0.028) were correlated with time to hydrocephalus across both cohorts. For patients who underwent treatment for hydrocephalus, those in the prenatal surgery group were significantly more likely to develop hydrocephalus after 3 months than those treated with postnatal surgery, although the overall rate of hydrocephalus was significantly higher in the postnatal surgery group (p = 0.018). CONCLUSIONS: Clinical and imaging factors associated with hydrocephalus treatment differ between those receiving pre- versus postnatal MMC repair, and while the overall rate of hydrocephalus is lower, those undergoing prenatal repair are more likely to develop hydrocephalus after 3 months of age. This has implications for clinical follow-up timing for patients treated prenatally, who may live at a distance from the treatment site.

Fetal surgery for open neural tube defect with severe ventriculomegaly.

OBJECTIVE: Prenatal open neural tube defect (ONTD) repair is performed to decrease the risk of needing treatment for hydrocephalus after birth and to preserve motor function. Some centers may not consider patients to be candidates for surgery if severe ventriculomegaly is present and there is no expected benefit in risk for hydrocephalus treatment. This study sought to compare the postnatal outcome of fetuses with ONTD and severe ventriculomegaly (ventricular width ≥ 15 mm) that underwent prenatal repair with the outcome of fetuses with severe ventriculomegaly that underwent postnatal repair and fetuses without severe ventriculomegaly (< 15 mm) that underwent prenatal repair. METHODS: This was a retrospective study of fetuses with ONTD that underwent prenatal or postnatal repair between 2012 and 2021 at a single institution. The cohort was divided into two groups based on preoperative fetal ventricular size: those with severe ventriculomegaly (ventricular width ≥ 15 mm) and those without severe ventriculomegaly (< 15 mm). Fetal ventricular size was measured by magnetic resonance imaging before surgery using the standardized approach and the mean size of the left and right ventricles was used for analysis. Motor function of the 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 the lower extremities at birth and the need for a diversion procedure for hydrocephalus treatment during the first year after birth, were collected and compared between groups. Multivariate regression analysis was used to adjust for potential confounders. RESULTS: In this study, 154 patients were included: 145 underwent fetal surgery (101 fetoscopic and 44 open hysterotomy) and nine with severe ventriculomegaly underwent postnatal repair. Among the 145 patients who underwent fetal surgery, 22 presented with severe ventriculomegaly. Fetuses with severe ventriculomegaly at referral that underwent prenatal repair were significantly more likely to need hydrocephalus treatment by 12 months after birth than those without severe ventriculomegaly (61.9% vs 28.9%, P < 0.01). However, motor function assessment at birth was similar between both prenatal repair groups (odds ratio, 0.92 (95% CI, 0.33-2.59), P = 0.88), adjusted for the anatomical level of the lesion. The prenatal repair group with severe ventriculomegaly had better preserved motor function at birth compared to the postnatal repair group with severe ventriculomegaly (median level, S1 vs L3, P < 0.01; proportion with S1 motor function, 68.2% vs 11.1%, P < 0.01). Fetuses with severe ventriculomegaly that underwent prenatal repair had an 18.9 (95% CI, 1.2-290.1)-times higher chance of having intact motor function at birth, adjusted for ethnicity, presence of club foot at referral and gestational age at delivery, compared with the postnatal repair group. There was no significant difference in the need for hydrocephalus treatment in the first year after birth between prenatal and postnatal repair groups with severe ventriculomegaly (61.9% vs 87.5%, P = 0.18). CONCLUSIONS: Although fetuses with ONTD and severe ventriculomegaly do not seem to benefit from fetal surgery in terms of postnatal hydrocephalus treatment, there is an increased chance of preserved motor function at birth. Results from this study highlight the benefit of prenatal ONTD repair for cases with severe ventriculomegaly at referral to preserve motor function. © 2024 International Society of Ultrasound in Obstetrics and Gynecology.

Fetal Surgery for Myelomeningocele: Neurosurgical Perspectives.

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.

Absence of a Hernia Sack in Patients Undergoing Prenatal Repair of Spina Bifida Increases the Risk of Developing Shunt-Dependent Hydrocephalus.

Spina bifida aperta (SBA), with (myelomeningocele) or without (myeloschisis) a hernia sack, is the most common congenital defect of the central nervous system. Prenatal surgical closure of SBA lowers the risk for developing shunt-dependent hydrocephalus, which offers a chance at improved motor, urinary, and gastrointestinal function. A total of 96 patients who had undergone open surgery prenatal repair for SBA were analyzed. The patients were divided into two groups: Group I-12 patients (12.5%)-without a hernia sack (myeloschisis) and Group II-84 patients (87.5%)-with a hernia sack (myelomeningocele). In this study, we demonstrated that prenatal SBA repair was statistically significantly less often associated with the need for ventriculoperitoneal shunting (p > 0.00001). The shunting was statistically significantly more often required in patients from Group I (p > 0.004). The absence of a hernia sack increases the risk for developing shunt-dependent hydrocephalus in patients after prenatal SBA repair. However, as prenatal SBA repair is associated with better motor, urinary, and gastrointestinal function, increased risk of developing shunt-dependent hydrocephalus in fetuses without a hernia sack should not be treated as a contraindication to prenatal intervention.

Fetoscopic Myelomeningocele Repair with Complete Release of the Tethered Spinal Cord Using a Three-Port Technique: Twelve-Month Follow-Up-A Case Report.

Open spina bifida is one of the most common congenital defects of the central nervous system. Open fetal surgery, which is one of the available therapeutic options, remains the gold standard for prenatal repairs. Fetoscopic closure may lower the number of maternal complications associated with open fetal surgery. Regardless of the approach, the outcome may be compromised by the development of tethered spinal cord (TSC) syndrome. At 24.2 weeks of gestation, a primipara was admitted due to fetal myelomeningocele and was deemed eligible for fetoscopic repair. Fetal surgery was performed at 25.0 weeks of gestation. It was the first complete untethering of the spinal cord and anatomic reconstruction (dura mater, spinal erectors, skin) achieved during a fetoscopic repair of spina bifida. Cesarean section due to placental abruption was performed at 31.1 weeks of gestation. VP shunting, with no need for revision, was performed at 5 weeks postdelivery due to progressing ventriculomegaly. No clinical or radiological signs of secondary tethering were observed. Neurological examination at 11 months postdelivery revealed cranial nerves without any signs of damage, axial hypotonia, decreased muscle tone in the lower extremities, and absent pathological reflexes. Motor development was slightly retarded. Complete untethering of the neural structures should always be performed, regardless of the surgical approach, as it is the only course of action that lowers the risk for developing secondary TSC.

Minimally Invasive Bimanual Fetal Surgery-A Review.

BACKGROUND: The aim of this review is to discuss experimental and clinical techniques and interventions of fetal surgery which have been performed minimally invasively by the means of a three-port approach for the fetoscope and instruments for the left and right hand of the surgeon (bimanual minimally invasive fetal surgery). METHODS: a print and electronic literature search was performed; the titles and abstracts were screened and included reports were reviewed in a two-step approach. First, reports other than minimally invasive fetal surgery were excluded, then a full text review and analysis of the reported data was performed. RESULTS: 17 reports were included. The heterogeneity of the included reports was high. Although reports on human fetoscopic surgical procedures can be found, most of them do not pick out bimanual fetal surgery as a central theme but rather address interventions applying a fetoscope with a working channel for a laser fiber, needle or flexible instrument. Most reports were on experimentation in animal models, the human application of minimally invasive fetoscopic bimanual surgery is rare and has at best been explored for the prenatal treatment of spina bifida. Some reported bimanual fetoscopic procedures were performed on the exteriorized uterus via a maternal laparotomy and can therefore not be classified as being truly minimally invasive. DISCUSSION: our results demonstrate that minimally invasive fetoscopic bimanual surgery is rare, even in animal models, excluding many other techniques and procedures that are loosely termed 'minimally invasive fetal surgery' which we suggest to better label as 'interventions'. Thus, more research on percutaneous minimally invasive bimanual fetoscopic surgery is warranted, with the aim to reduce the maternal, uterine and fetal trauma for correction of congenital malformations.

Fetal Surgery for Gastroschisis-A Review with Emphasis on Minimally Invasive Procedures.

(1) Background: The morbidity of gastroschisis is defined by exposure of unprotected intestines to the amniotic fluid leading to inflammatory damage and consecutive intestinal dysmotility, the viscero-abdominal disproportion which results in an abdomen too small to incorporate the herniated and often swollen intestine, and by associated pathologies, such as in complex gastroschisis. To prevent intestinal damage and to provide for growth of the abdominal cavity, fetal interventions such as amnio exchange, gastroschisis repair or covering have been evaluated in several animal models and human trials. This review aims to evaluate the reported techniques for the fetal treatment of gastroschisis by focusing on minimally invasive procedures. (2) Methods: We conducted a systematic database search, quality assessment and analyzed relevant articles which evaluate or describe surgical techniques for the prenatal surgical management of gastroschisis in animal models or human application. (3) Results: Of 96 identified reports, 42 eligible studies were included. Fetal interventions for gastroschisis in humans are only reported for EXIT procedures and amnio exchange. In animal models, particularly in the fetal sheep model, several techniques of open or minimally invasive repair of gastroschisis or covering the intestine have been described, with fetoscopic covering being the most encouraging. (4) Discussion: Although some promising minimally invasive techniques have been demonstrated in human application and animal models, most of them are still associated with relevant fetal morbidity and mortality and barely appear to be currently applicable in humans. Further research on specific procedures, instruments and materials is needed before any human application.

In utero myelomeningocele repair reduces intensification of inflammatory changes in the dura mater and the skin.

Objective: Chemical and mechanical injury in myelomeningocele (MMC) during the fetal life results in functional disorders of multiple organs. Prenatal MMC repair reduces sequelae of spinal cord injury.Design: Histopathological evaluation of dura mater and skin specimens to assess the severity of inflammatory changes.Setting: Histopathological laboratory and operated patients.Participants: 45 cases (Group I)-intrauterine surgery due to MMC and 42 cases (Group II)-postnatal surgery.Outcome measures: Specimens of the skin and of the dura mater adjacent directly to the uncovered section of the spinal cord were collected for assessment. The specimens were histopathologically evaluated to assess the severity of inflammatory changes.Results: The analysis of the severity of inflammatory changes in the skin and the dura mater showed only small lymphocytic infiltration in 5 fetuses (Group I). Medium and large infiltration in the skin and the dura mater was found in all children who underwent postnatal surgery (Group II). Lymphocytic and granulocytic infiltration in the skin and the dura mater were statistically significantly more prevalent in children who underwent postnatal surgery compared to the group of children who underwent prenatal surgery (P < 0.000003).Conclusions: By reducing the time of exposure to damaging factors, prenatal MMC repair statistically reduces the risk of inflammatory changes in the exposed spinal cord and spinal nerves. Prenatal closure of spina bifida before 24 week of gestation does not reduce the severity of inflammatory changes in the exposed spinal cord.

Pathological and sonographic review of early isolated severe lower urinary tract obstruction and implications for prenatal treatment.

OBJECTIVE: To identify favorable renal histology in fetuses with early severe lower urinary tract obstruction (LUTO) and determine the best timing and selection criteria for prenatal surgery. METHODS: This multicenter, retrospective study included male fetuses with severe LUTO which died before 24 weeks of gestation during the period January 2000 to December 2018. Age-matched controls were used as reference standard for renal histology. Prenatal ultrasound features and fetal serum and/or urine ß2microglobulin level were retrieved and kidney histology slides (hematein-eosin-safran and α-smooth-muscle-actin (αSMA) immunostaining) were prepared and reviewed. αSMA-positive staining of the blastema is due to its aberrant differentiation into myofibroblastic cells. Cases were sorted into histopathologic groups (favorable or unfavorable) according to the blastema's morphology and αSMA labeling and the data of these groups were compared. RESULTS: Included in the study were 74 fetuses with a median gestational age at outcome of 17 + 6 (range, 13 + 0 to 23 + 5) weeks. Parenchymal organization was preserved in 48% of the kidneys. A blastema was present in 90% of the kidneys, but it was morphologically normal in only 9% and αSMA-negative in only 1% of them. Most (82%) fetuses had an unfavorable prognosis, and 36% of fetuses died ≤ 18 weeks and had severe renal lesions detected on histology (early unfavorable prognosis). A favorable renal prognosis was associated with an earlier gestational age (P = 0.001). Fetuses with LUTO had a significantly lower number of mature glomeruli (P < 0.001) compared with controls. However, there was no significant difference in the number of glomeruli generations between the early-unfavorable-prognosis group (≤ 18 weeks) and the group with a favorable prognosis (P = 0.19). A comparison of prenatal ultrasound features and biochemical markers between groups could not identify any prenatal selection criteria. CONCLUSIONS: Before 18 weeks, around 30% of fetuses with severe LUTO still have potential for kidney development. Identification of these cases would enable them to be targeted for prenatal therapy. © 2021 International Society of Ultrasound in Obstetrics and Gynecology.

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

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

Impact of Brain Malformations on Neurodevelopmental Outcome in Children with a History of Prenatal Surgery for Open Spina Bifida.

INTRODUCTION: This retrospective study investigates brain malformations and their impact on neurodevelopmental outcome in children after prenatal surgery for spina bifida (SB). METHODS: Sixty-one patients were included. On neonatal MRI, SB-associated brain malformations were assessed. Ventricular size, ventriculo-peritoneal shunt (VPS), and endoscopic third ventriculostomy (ETV) were also documented. Neurodevelopment was assessed with the Bayley-III and correlated with brain malformations, ventricular size, and VPS/ETV placement. RESULTS: Chiari II malformation was detected in all patients. Corpus callosum (CC) abnormality was noted in 40%, heterotopies in 35%, and cerebellar parenchymal defects in 11%. 96% had ventriculomegaly; in 46%, VPS/ETV was performed. Cognitive and language testing yielded results in the low-average range (Bayley-III: Cognitive Composite Score 93.6, Language Composite Score 89.7), motor testing was below average (Motor Composite Score 77.4). CC abnormalities, heterotopies, and cerebellar defects were not associated with poorer Bayley-III scores, whereas patients with severe ventriculomegaly performed poorer in all subtests, significantly so for the language composite score. Patients requiring intervention for hydrocephalus had significantly lower scores in motor testing. DISCUSSION/CONCLUSION: Additional brain malformations in open SB do not seem to have an impact on cognitive function at 2 years of age. Severe ventriculomegaly is a risk factor for poorer cognitive outcome; hydrocephalus surgery adds an additional risk for delayed motor function.

Maternal Depressive Risk in Prenatal versus Postnatal Surgical Closure of Myelomeningocele: Associations with Parenting Stress and Child Outcomes.

INTRODUCTION: Depressive risk is higher for mothers of infants with chronic medical conditions. The present study examined maternal depressive risk and associations with parent and child outcomes among mothers of young children who were randomized to either prenatal or postnatal surgical closure for myelomeningocele. METHODS: Using the Management of Myelomeningocele Study database, maternal depressive risk was examined at 3 time points as follows: prior to birth, 12 months, and 30 months post birth. Separate multivariate analyses examined associations among change in depressive risk (between baseline and 30 months), parenting stress, and child outcomes at 30 months. RESULTS: Mean scores were in the minimal depressive risk range at all the time points. Post birth depressive risk did not differ by prenatal versus postnatal surgery. Mean change scores reflected a decrease in depressive risk during the first 30 months. Only 1.1-4.5% of mothers reported depressive risk in the moderate to severe range across time points. Increased depressive risk during the first 30 months was associated with increased parenting stress scores and slightly lower child cognitive scores at 30 months. CONCLUSION: Most mothers reported minimal depressive risk that decreased over time, regardless of whether their infant underwent prenatal or postnatal surgery. Only a small percentage of mothers endorsed moderate to severe depressive risk, but an increase in depressive risk over time was associated with higher parental stress and slightly lower child cognitive development.

Fetal Surgery in the Primate 4.0: A New Technique 30 Years Later.

INTRODUCTION: Open fetal surgery requires a hemostatic hysterotomy that minimizes membrane separation. For over 30 years, the standard of care for hysterotomy in the gravid uterus has been the AutoSuture Premium Poly CS*-57 stapler. OBJECTIVE: In this study, we sought to test the feasibility of hysterotomy in a rhesus monkey model with the Harmonic ACE®+7 Shears. METHODS: A gravid rhesus monkey underwent midgestation hysterotomy at approximately 90 days of gestation (2nd trimester; term = 165 ± 10 days) using the Harmonic ACE®+7 Shears. A two-layer uterine closure was completed and the dam was monitored by ultrasound intermittently throughout the pregnancy. At 58 days after hysterotomy (near term), a final surgery was performed to evaluate the uterus and hysterotomy site. RESULTS: A 3.5-cm hysterotomy was completed in 2 min 7 s. The opening was hemostatic and the membranes were sealed. Immediately after closure and throughout the pregnancy, ultrasound revealed intact membranes without separation and normal amniotic fluid levels. At term, the scar was well healed without signs of thinning or dehiscence. CONCLUSIONS: The Harmonic ACE®+7 Shears produced a hemostatic midgestation hysterotomy with membrane sealing in the rhesus monkey model. Importantly, healing was acceptable.

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.

Cost of prenatal versus postnatal myelomeningocele closure for both mother and child at 1 year of life.

OBJECTIVE: Prenatal myelomeningocele (MMC) closure has been performed in the United States for 2 decades. While prior work has focused on clinical outcomes of prenatal MMC closure, the cost of this procedure in comparison with that of postnatal MMC closure is unclear. The authors' aim was to compare the cost of prenatal versus postnatal MMC closure for both the child and mother at 1 year. METHODS: A prospective database of patients undergoing prenatal and postnatal MMC closure between 2011 and 2018 with 1-year follow-up was retrospectively reviewed. Charge data for relevant admissions were converted to a cost estimate using the authors' institution's Medicare hospital-specific cost-to-charge ratio. Children, mothers, and mother/child pairs were considered separately. The primary outcome was cost. Secondary outcomes included the need for hydrocephalus treatment, length of stay (LOS), and readmissions. Other covariates included gestational age at birth, MMC lesion level, and obstetric complications. RESULTS: The median cost of care for children in the prenatal group was greater, although not significantly so, at $58,406.71 (IQR $16,900.24-$88,951.01) compared with $49,889.95 (IQR $38,425.18-$115,163.86) for children in the postnatal group (p = 0.204). The median cost for mothers in the prenatal group was significantly greater at $24,548.29 (IQR $20,231.55-$36,862.31) compared with $5087.30 (IQR $4430.72-$5362.56) (p < 0.001). The median cost for mother/child pairs in the prenatal group was $102,377.75 (IQR $37,384.30-$118,527.74) compared with $55,667.82 (IQR $42,840.78-$120,058.06) (p = 0.45). Children in the prenatal group had a lower gestational age at birth (235.81 days vs 265.77 days, p < 0.001) and fewer readmissions (33.3% vs 72.7%, p < 0.001), and hydrocephalus treatment was less common (33.3% vs 90.9%, p < 0.001). Index LOS did not differ between children in the prenatal and postnatal groups (26.8 days vs 23.5 days, p = 0.63). Mothers in the prenatal group had longer LOS (15.92 days vs 4.68 days, p < 0.001) and more readmissions (18.5% vs 0.0%, p = 0.06). CONCLUSIONS: The median cost of prenatal versus postnatal MMC closure did not significantly differ from a hospital perspective at 1 year, although variability in cost was high for both groups. When considering the mother alone, prenatal MMC closure was costlier. Future work is needed to assess cost from a patient and societal perspective both at 1 year and beyond.

[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.

Low level myelomeningoceles: do they need prenatal surgery?

BACKGROUND: Postnatal closure of a myelomeningocele remains the standard of care in many countries. The prenatal closure has given hope for decreasing the damage to the neural placode and has challenged classic management. However, this technique presents potential sources of complications. Patients with MMC with an anatomical level of L4 and below have a better functional prognosis than higher level malformations. Are they still candidates for prenatal surgery? OBJECTIVE: To evaluate outcome of MMC with an anatomical level of L4 and below and discuss, with support of the literature, the indications to perform prenatal closure in this particular group of patients. MATERIALS AND METHODS: Twenty-nine children were included in this observational study. The level of the vertebral malformation was sacral in 12 cases (41.4%) or lumbar (level ≤ L4) in 17 cases (58.6%). All the patients was operated postnatally for closure of the MMC with microsurgical technique as soon as possible after clinical evaluation (range 0-97 days). RESULTS: Only 11 out of 29 patients (37.9%) needed of a CSF diversion. A Chiari II malformation was present before MMC closure in 17 patients (58.6%) and only in 5 (17%) after. Twenty-six patients (89.7%) were able to walk. Seven (23%) and 16 (55%) of our patients have a normal bladder and bowel control, respectively. All school-aged children attend school. CONCLUSIONS: The functional outcome for low-level MMC is good when managed with modern microneurosurgical techniques with a low risk for the patient and the mother. Therefore, we do not suggest prenatal surgery for subgroup of infant with MM.

Prenatal myelomeningocele repair improves urinary continence and reduces the risk of constipation.

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.