Management Of Myelomeningocele Study Research Articles

Detailed Analysis of Hydrocephalus and Hindbrain Herniation After Prenatal and Postnatal Myelomeningocele Closure: Report From a Single Institution.

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

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.

Improved Postoperative Metrics with Modified Myofascial Closure in Fetal Myelomeningocele Repair.

The effect of modifications in fetal myelomeningocele (fMMC) closure techniques has not been extensively studied. To study the effect of a modified closure technique on fMMC postnatal patient outcomes: hydrocephalus, hindbrain herniation, and cyst development. We performed single-center retrospective study of a subset of post-MOMS (Management of Myelomeningocele Study) trial patients who underwent fMMC closure. After January 2015, the fetal myofascial closure technique was modified. Needlepoint monopolar cautery was used to raise dural lined myofascial flaps to create a more robust closure. Outcomes between the pre- and postmodification groups were compared with regard to hindbrain herniation, hydrocephalus, and cyst development. Families who transitioned care to local institutions were contacted via telephone for outcome information. From January 2011 to May 2016, data were reviewed from 119 fMMC closure patients. Patients without full follow-up data were excluded from the final analysis. Cerebrospinal fluid diversion was seen in 32 of 74 patients with the standard technique compared to 14 of 45 with the modified closure and was significantly decreased in postmodification when compared to that of the MOMS trial (P=.01). Hindbrain herniation resolution was significantly decreased in both the pre- and postmodification groups compared to that of the MOMS trial (P<.01). Prior to January 2015 with standard closure, 23 cysts required resection whereas no cysts required resection in the modified repair group (P<.01). Modified myofascial closure for fMMC closure is safe and feasible. The new approach reflects a decreased rate of cyst development requiring surgical resection, and a trend for improved rates of hindbrain herniation and hydrocephalus.

Pediatric neurosurgeons' views regarding prenatal surgery for myelomeningocele and the management of hydrocephalus: a national survey.

The Management of Myelomeningocele Study (MOMS) compared prenatal with postnatal surgery for myelomeningocele (MMC). The present study sought to determine how MOMS influenced the clinical recommendations of pediatric neurosurgeons, how surgeons' risk tolerance affected their views, how their views compare to those of their colleagues in other specialties, and how their management of hydrocephalus compares to the guidelines used in the MOMS trial. A cross-sectional survey was sent to all 154 pediatric neurosurgeons in the American Society of Pediatric Neurosurgeons. The effect of surgeons' risk tolerance on opinions and counseling of prenatal closure was determined by using ordered logistic regression. Compared to postnatal closure, 71% of responding pediatric neurosurgeons viewed prenatal closure as either "very favorable" or "somewhat favorable," and 51% reported being more likely to recommend prenatal surgery in light of MOMS. Compared to pediatric surgeons, neonatologists, and maternal-fetal medicine specialists, pediatric neurosurgeons viewed prenatal MMC repair less favorably (p < 0.001). Responders who believed the surgical risks were high were less likely to view prenatal surgery favorably and were also less likely to recommend prenatal surgery (p < 0.001). The management of hydrocephalus was variable, with 60% of responders using endoscopic third ventriculostomy in addition to ventriculoperitoneal shunts. The majority of pediatric neurosurgeons have a favorable view of prenatal surgery for MMC following MOMS, although less so than in other specialties. The reported acceptability of surgical risks was strongly predictive of prenatal counseling. Variation in the management of hydrocephalus may impact outcomes following prenatal closure.

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.

Fetal surgery for spina bifida.

The two-hit hypothesis of neural injury in the wake of open neural tube defects suggests an opportunity for preservation of function and potential reversibility of early morphological changes in the fetus diagnosed with myelomeningocele. The Management of Myelomeningocele Study (MOMS) demonstrated reduced need for shunting and improved neurological function in patients treated in utero relative to postnatally, thereby offering level 1 evidence supporting fetal repair. Subsequent studies have offered additional information about urological, orthopedic, radiological, and maternal factors surrounding fetal repair. The quest for robust long-term neurocognitive and motor function data is underway and poised to shape the future of fetal repair. In addition, technical innovations such as fetoscopic surgery aim to minimize maternal morbidity while conferring the beneficial effects observed with open intrauterine intervention.

Benchmarking against the MOMS Trial: Zurich Results of Open Fetal Surgery for Spina Bifida

Introduction: The Management of Myelomeningocele Study, a.k.a. the MOMS trial, was published in 2011 in the New England Journal of Medicine. This prospective randomized controlled trial proved to be a milestone publication that provided definitive evidence that fetal surgery is a novel standard of care for select fetuses with spina bifida aperta (SB). The goal of our study is to assess whether our center can match these benchmark results. Materials and Methods: Our study was conducted according to the MOMS protocol using the same inclusion and exclusion criteria and looked at the same outcome parameters that were used in the MOMS trial. Zurich and MOMS results were compared. Results: We enrolled 20 patients between December 2010 and May 2015 all of whom underwent fetal surgery for SB. Among 51 different outcome variables, there were only 3 favorable (multiplicity-adjusted) significant differences (gestational age at birth, hindbrain herniation, and psychomotor development). There were no statistically significant differences regarding any other parameters. Conclusion: Our findings confirm that rigorous apprenticeship, training, and comprehensive prospective data collection enable centers like the Zurich Center for Fetal Diagnosis and Therapy to achieve benchmark results for open fetal surgery for myelomeningocele and myeloschisis. These results justify the existence and continuation of our program. Outcome documentation is an essential element of quality management. It is medically and ethically fundamental for fetal medicine and surgery centers offering high-end innovative medical care.

Intrauterine closure of myelomeningocele is associated with superior long-term quality of life than postnatal closure: a single-center study.

The Management of Myelomeningocele Study (MOMS) established several important benefits of myelomeningocele fetal closure, including decreased need for shunting at 30 months. However, the effects of fetal closure on long-term quality of life (QOL) have not been studied. In this study, the authors aimed to analyze the differences in long-term QOL between children treated with intrauterine repair and those treated with postnatal repair. Caregivers of children treated with either intrauterine or postnatal closure at a tertiary acute care hospital between 1997 and 2003 were contacted to participate. The Pediatric Quality of Life Inventory (PedsQL 4.0) and a surgical history questionnaire were administered over the phone. Responses to the QOL survey were reverse scored and linearly transformed to a 0-100 scale, with a higher score indicating better QOL. The Mann-Whitney U-test was used to analyze differences in means. Seventy-four children had MMC repair between 1997 and 2003. Twenty-three (31%) of the patients' families responded to the PedsQL 4.0 questionnaire. Of these 23 children, 11 had intrauterine closure and 12 had postnatal closure. The intrauterine group did not differ in age (median [IQR] 17 years [14-17 years] vs 15 years [14-19 years], p = 0.926), sex (27.3% vs 41.7% male, p = 0.469), or lesion level (p = 0.199) from the postnatal group. Fewer patients in the intrauterine group underwent neurosurgical procedures than those in the postnatal group (55.6% vs 100%, p = 0.018). However, of the children receiving neurosurgical procedures, there was no difference in the number of procedures between the intrauterine and postnatal groups (median [IQR] 4 [2-10.5] vs 2.5 [1.75-6.25], p = 0.458). There was no difference in the percentage of children receiving nonneurosurgical procedures between the two groups (100% vs 100%, p > 0.99). Children who underwent intrauterine closure had significantly higher psychosocial health (median [IQR] 70.0 [56.7-83.3] vs 55.0 [42.1-60.0], p = 0.015) as well as total QOL (median [IQR] 56.5 [55.4-81.5] vs 49.5 [32.9-59.0], p = 0.019) than children with postnatal closure. Physical health was not significantly different between the two groups (median [IQR] 62.5 [37.5-78.1] vs 39.1 [18.8-59.4], p = 0.108). Relative to postnatal closure, children who underwent intrauterine closure of MMC demonstrated better long-term QOL as measured by psychosocial and overall QOL metrics. Given that not all medical and socioeconomic confounders were adjusted for due to the low sample size, validation of these results in a larger population and across multiple centers is needed.

The Use of Indomethacin with Complete Amniotic Fluid Replacement and Classic Hysterotomy for the Reduction of Perinatal Complications of Intrauterine Myelomeningocele Repair

Objective: This study presented outcomes of classical hysterotomy with modified antiprostaglandin therapy for intrauterine repair of foetal myelomeningocele (fMMC) performed in a single perinatal centre. Study Design: Forty-nine pregnant women diagnosed with fMMC underwent classic hysterotomy with anti-prostaglandin management, complete amniotic fluid replacement and high dose indomethacin application. Results: The average gestational age (GA) at delivery was 34.4 ± 3.4 weeks, with no births before 30 weeks GA. There were 2 foetal deaths. Complete reversal of hindbrain herniation (HH), assessed in magnetic resonance imaging at 30–31 weeks GA was found in 72% of foetuses (mostly with HH grade I prior to fMMC repair). Our protocol resulted in rare use of magnesium sulphate (6%), low incidence of chorioamniotic membrane separation – chorioamniotic membrane separation (6%), preterm premature rupture of membranes – preterm premature rupture of membranes (pPROM; 15%) and preterm labour – preterm labour (PTL; 17%). The postoperative wound continuity of the uterus was usually stable (in 72% of patients), with low frequency of scar thinning (23%). Conclusion: Our protocol results in rare use of tocolytics, and the low occurrences of CMS, pPROM and PTL in relation to other study cohorts: Management of Myelomeningocele Study, Children’s Hospital of Philadelphia, and Vanderbilt University Medical Centre.

MOMS Plus: Single-Institution Review of Outcomes for Extended BMI Criteria for Open Fetal Repair of Myelomeningocele

Background: In utero repair has become an accepted therapy to decrease the rate of ventriculoperitoneal shunting and improve neurologic function in select cases of myelomeningocele. The Management of Myelomeningocele Study (MOMS) trial excluded patients with a BMI >35 due to concerns for increased maternal complications and preterm delivery, limiting the population that may benefit from this intervention. Objectives: The aim of this study was to evaluate outcomes associated with extending the maternal BMI criteria to 40 in open fetal repair of myelomeningocele. Method: Retrospective review of fetal closure of myelomeningocele at a quaternary referral center between 2013 and 2016 with maternal BMI ranging from 35 to 40. Results: Eleven patients with a BMI >35 were identified. The average BMI was 37. The average maternal age at the time of evaluation was 27 years. The average gestational age at fetal surgery was 24 weeks. Gestational age at birth was an average of 32 weeks. There was one perinatal death immediately following the fetal intervention. The shunt rate at 1 year was 45% (5/11 patients). Conclusions: In this single-institution review of expanded BMI criteria for fetal repair of myelomeningocele, we did not observe any adverse maternal outcomes associated with maternal obesity; however, the gestational age at delivery was 2 weeks earlier compared to the MOMS trial.

Urinary and Fecal Continence in 5-Year-Old Patients Who Underwent in utero Myelomeningocele Repair: A Prospective Study

Introduction: After the successful results of in utero myelomeningocele (MMC) repair presented by the Management of Myelomeningocele Study, the concept of fetal surgery was introduced in our institution in 2011. Since then, we have been able to follow prospectively a group of patients with attention to urological care. In the present study, we were interested in estimating urinary and fecal continence in this new subset of patients. Material and Methods: We selected from our database patients aged 5 years or older for evaluation of urinary and fecal continence. We reviewed all charts and completed a questionnaire to study aspects of urinary and fecal continence. Results: We identified 14 patients, i.e., 4 (28.6%) males and 10 (71.4%) females. The mean age at MMC surgery was 25.6 gestational weeks. The uro­dynamic class was high-risk in 6 (42.9%), incontinent in 4 (28.6%), hypocontractile in 1 (7.1%), and normal in 3 (21.4%) patients. Three patients had undergone surgery (2 augmentations, i.e., 1 in association with a left colon ACE Macedo-Malone procedure and 1 mini-sling urethroplasty). Twelve patients underwent clean intermittent catheterization (CIC) (85.7%). Only 3 (21.4%) patients had no urinary leakage. Eleven patients (78.6%) used diapers. Eight patients (57.2%) underwent retrograde rectal irrigation and 11 (78.6%) complained of fecal loss. Eleven patients (78.6%) did not report an impact on their self-esteem. Conclusion: Despite the use of CIC in 85.7% of the cases, the continence rate in MMC patients operated on in utero was low and 78.6% of the patients used diapers. This data can be used to educate parents about future conditions of their 5-year-old children and may stimulate the debate regarding further attempts (surgical or not) to improve fecal and urinary continence.

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.

68: Prenatal open hysterotomy vs. two-port exteriorized uterus fetoscopic myelomeningocele repair. perinatal outcomes from a single center

To assess obstetrical and perinatal outcomes after fetal open or fetoscopic myelomeningocele (MMC) repair. Retrospective cohort study from 2011-2018. 66 patients diagnosed with fetal MMC who underwent a prenatal repair in our center via open-hysterotomy (N=33) or fetoscopic repair (N=33) were included. All patients fulfilled Management of Myelomeningocele Study (MOMS) inclusion and exclusion criteria. Open-hysterotomy repair was performed as reported in MOMS trial. Our standardized fetoscopic repair is a two port, exteriorized uterus, technique in CO2, using interrupted sutures for the neurosurgical repair.13 of the fetosocpic and 8 of the open repaired patients received a dural patch. Obstetrical and perinatal outcomes were compared between groups using Student's t-test for independent samples or Mann-Whitney U test if they had normal or non-normal distribution, respectively. Chi-square or Fisher's exact tests were used to compare qualitative data. Duration of surgery and spinal defect repair were lengthier in the fetoscopic group. There were no differences in intraoperative fetal bradycardia or postoperative complications. Fetoscopically repaired cases were delivered later [38.6 (29.9-40.9) weeks vs. 35.7 (27.3-37.9) weeks; p<0.01] with higher birth weight [3120(1375-4430)grams vs. 2611 (624-3623)grams; p<0.01] and a lower rate of cesarean delivery (43% vs. 100%; p<0.01). Intact surgical sites were seen in all 14 fetoscopic cases delivered by c-section and in 21 of open cases (p<0.01). Uterine adhesions were more common after open repair (36% vs. 14%; p<0.01). One neonatal death occurred after an open repair due to extreme preterm birth and sepsis. Fetoscopic repair has a lengthier surgery but similar postoperative complications to open repair, while offering significant benefits including higher gestational age at birth, vaginal delivery and a decreased risk of adhesions and scar dehiscence.

220: Prenatal open vs. two-port exteriorized uterus fetoscopic myelomeningocele repair. Neurosurgical outcomes from a single center

To assess neurosurgical and motor outcomes after an open-hysterotomy or fetoscopic fetal myelomeningocele (MMC) repair. Retrospective cohort study from 2011-2018. 66 patients diagnosed with fetal MMC who underwent a prenatal repair in our center via open-hysterotomy (N=33) or fetoscopic repair (N=33) were included. All patients fulfilled the Management of Myelomeningocele Study (MOMS) inclusion and exclusion criteria. Open-hysterotomy repair technique was performed as reported in MOMS trial. Our standardized fetoscopic repair is a two port, exteriorized uterus technique in CO2, using interrupted sutures for the neurosurgical repair. 13 and 8 of the fetoscopic and open repaired cases respectively, received a dural patch. Neurosurgical outcomes were compared between groups using Student's t-test for independent samples or Mann-Whitney U test if they had normal or non-normal distribution respectively for quantitative data. Chi-square or Fisher's exact test were used to compare qualitative data. Rate of complete hind brain herniation reversal assessed by MRI six weeks after prenatal repair, was similar between both groups [Fetoscopic: 23/32 (71.9%) vs.Open:21/33 (63.6%); p=0.47]. There were no significant differences between the groups in the rates of CSF leak, revision of repair at birth, need for ventriculo-peritoneal shunt placement or need for endoscopic third ventriculostomy (ETV) during the first year of life. Evaluation of functional and anatomical levels of the spinal defect also showed similar rates of improvement. Minimally invasive fetoscopic MMC repair shows comparable neurosurgical and motor outcomes to the open- hysterotomy approach.

Cirugía fetoscópica en mielomeningocele

El mielomeningocele se presenta en 17,8:100 000 nacidos vivos. En México se encuentra entre las primeras 10 causas de muerte en niños menores de 10 años y está asociado a alta morbilidad cognitiva, sensorial y motora. Estudios en animales y posteriormente el estudio MOMS (The Management of Myelomeningocele Study) demostró que la reparación prenatal de los defectos del tubo neural disminuye el riesgo de hidrocefalia y mejora la función motora a los 30 meses de edad. La cirugía fetal prenatal descrita en el MOMS para el mielomeningocele se realiza a través de una histerotomía, la cual se asocia con significativa morbilidad materna y fetal. Por lo tanto, se ha evolucionado a técnicas fetoscópicas con menos complicaciones maternas y fetales, obteniendo mejores resultados perinatales. En este apartado se describe las diferentes técnicas fetoscópicas, su evolución, ventajas y desventajas en cada una de ellas, así como los retos en el futuro.

Fetal surgery for myelomeningocele: Obstetric evolution and short-term perinatal outcomes of a cohort of 21 cases

ResumenObjetivo:Describir la técnica quirúrgica, evolución obstétrica y resultados perinatales de una cohorte de pacientes sometidas a cirugía intrauterina para reparación de una disrafia fetal abierta (DFA).Métodos:Análisis prospectivo de 21 casos consecutivos de DFA con diagnóstico y tratamiento quirúrgico prenatal en nuestra institución entre 2015 y 2017. La técnica de la cirugía fetal (QF) fue similar a la descripta en el estudio MOMS, excepto que se utilizó histerotomía con asa bipolar. Los cuidados postoperatorios, nacimiento y cuidados neonatales se realizaron en la misma institución. Se analizaron complicaciones perioperatorias de la QF, la evolución obstétrica y los resultados perinatales.Resultados:La QF se realizó a una edad gestacional media de 25.8 semanas (24.1-27.6). La edad gestacional media al nacer fue 34.2 semanas (29.2-37.1). El tiempo quirúrgico medio fue 138 min (101-187) con tendencia descendente y el tiempo de internación medio, 7.1 días (4-32). El 52% (11/21) de las pacientes experimentó rotura prematura de membranas (RPM). Ninguna paciente requirió transfusiones postcesárea. No hubo casos de desprendimiento placentario, rotura uterina ni muertes maternas. La cicatrización de la histerorafia fue normal en 95% de las pacientes. La sobrevida perinatal fue del 95% (20/21, una muerte intrauterina secundaria a banda amniótica). La necesidad de cierre cutáneo postnatal (CCP) fue del 5%. Ninguno de los casos (20) reparados con tejidos fetales requirió CCP. El 70% (14/20) de los pacientes no requirió ningún tratamiento para hidrocefalia. Cuatro pacientes (20%) requirieron una derivación ventriculoperitoneal (DVP) y dos adicionales, una tercer ventriculostomía endoscópica (ETV) (10%). El nivel funcional motor neonatal (NFN) fue mejor que el nivel anatómico prenatal (NAP) en 45% (9/20), igual en 50% (10/20) y peor en 5% (1/20).Conclusiones:El presente estudio confirma que la cirugía fetal de disrafias abiertas se asocia a un mayor riesgo de parto prematuro y rotura prematura de membranas, pero reduce significativamente la necesidad de tratamiento postnatal de hidrocefalia y mejora la función motora a corto plazo. Nuestros resultados son similares a los publicados en el ensayo aleatorizado MOMS.

Maternal-fetal surgery for myelomeningocele: some thoughts on ethical, legal, and psychological issues in a Western European situation.

The results of the Management of Myelomeningocele Study (MOMS) randomized controlled trial have demonstrated that maternal-fetal surgery (MFS) for myelomeningocele (MMC) compared to postnatal MMC repair has clear neurological benefits for the child at 12 and 30 months of age. Level I evidence nevertheless does not provide answers to many questions in this delicate field. Since the beginning of 2012, our fetal center has been offering MFS for spina bifida aperta (SBA) to patients from different European and non-European countries, in a societal context where termination of pregnancy is the option chosen by most patients when being informed of this diagnosis. We aim to explore in this text some of the ethical, legal, and psychological issues that we have encountered. For many of these questions, we do not have definite answers. A pregnant patient when diagnosed with a MMC fetus is a vulnerable subject. She needs to be referred to a highly specialized center with sufficient expertise in diagnosis and in all therapeutic options. Objective but compassionate counseling is of paramount importance. It is required that a multidisciplinary professional team obtains full voluntary consent from the mother after providing an appropriate information including diagnosis, short-, medium-, and long-term prognosis as well as benefits and harms of the fetal surgery. The latter should be offered with full respect for maternal choice and individual assessment and perception of potential risks taking into consideration legislation in the fetal center and the parents' country legislation.

Prenatal surgery for myelomeningocele: review of the literature and future directions

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

New directions in fetal surgery for myelomeningocele.

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

First 60 fetal in-utero myelomeningocele repairs at Saint Louis Fetal Care Institute in the post-MOMS trial era: hydrocephalus treatment outcomes (endoscopic third ventriculostomy versus ventriculo-peritoneal shunt).

The published results of the Management of Myelomeningocele Study (MOMS) trial in 2011 showed improved outcomes (reduced need for shunting, decreased incidence of Chiari II malformation, and improved scores of mental development and motor function) in the fetal prenatal repair group compared to the postnatal group. Historically, endoscopic third ventriculostomy (ETV) remains as a controversial hydrocephalus treatment option with high failure rates in pediatric patients with a history of myelomeningocele (MMC). We report hydrocephalus treatment outcomes in the fetal in-utero myelomeningocele repair patients who underwent repair at our Saint Louis Fetal Care Institute following the MOMS trial. We looked carefully at ETV outcomes in this patient population and we identified risk factors for failure. At our Saint Louis Fetal Care Institute, we followed the maternal and fetal inclusion and exclusion criteria used by the MOMS trial. The records of our first 60 fetal MMC repairs performed at our institute between 2011 and 2017 were examined. We retrospectively reviewed the charts, prenatal fetal magnetic resonance imaging (MRI) and ultrasound (US) imaging findings, postnatal brain MRI, and Bayley neurodevelopment testing results for infants and children who underwent surgical treatment of symptomatic hydrocephalus (VP shunt versus ETV). Multiple variables possibly related to ETV failure were considered for identifying risk factors for ETV failure. Between May 2011 and March 2017, 60 pregnant female patients underwent the prenatal MMC repair for their fetuses between 20 and 26weeks' gestational age (GA) utilizing the standard hysterotomy for exposure of the fetus, and microsurgical repair of the MMC defect. All MMC defects underwent successful in-utero repair, with subsequent progression of the pregnancy. At the time of this study, 58 babies have been born, 56 are alive since there were 2 mortalities in the neonatal period due to prematurity. One patient was excluded given lack of consent for research purposes. From the remaining 55 patient included in this study, a total of 30 infants and toddlers underwent treatment of hydrocephalus (ETV and VPS groups). Twenty-five patients underwent ETV (24 primary ETV and 1 after shunt failure). Nineteen patients underwent shunt placements (6 primary/13 after ETV failure). Mean GA at time of MMC repair for the ETV group was 24+6/7weeks (range 22+4/7 to 25+6/7). Mean follow up for patients who had a successful ETV was 17.25months (range 4-57months). Bayley neurodevelopmental testing results were examined pre- and post-ETV. Overall ETV success rate was 11/24 (45.8%) at the time of this study. The total number of patients who underwent shunt placement was 19/55 (34.5%), while shunting rate was 40% in the MOMS trial. Using a simple logistic regression analysis to identify predictors of ETV failure, ETV age ≤6months and gestational age ≥23weeks at repair of myelomeningocele were significant predictors for ETV failure while in-utero ventricular stability ≤4mm and in-utero ventricular size post-repair ≤15.5mm were significant predictors for ETV success. None of the listed variables independently predicted classification into ETV success versus ETV failure groups when entered into multiple logistic regression analysis. ETV, as an alternative to initial shunting, may continue to show promising results for treating fetal MMC repair patient population who present with symptomatic hydrocephalus during infancy and early childhood. Although our overall CSF diversion rate (ETV and VPS groups) in our fetal MMC group is higher than the MOMS trial, our shunting rate is lower given our higher incidence of patients with successful ETV. To our knowledge, this is the largest reported ETV series in patients who underwent fetal MMC repair. ETV deserves a closer look in the setting of improved hindbrain herniation in fetal in-utero MMC repair patients. In our series, young age (less than 6months) and late GA at time of fetal MMC repair (after 23weeks GA) were predictors for ETV failure, while in-utero stability of ventricular size (less than 4mm) and in-utero ventricular size post-repair ≤15.5mm were predictors for ETV success. Larger series and potential prospective randomized studies are required for further evaluation of risk factors for ETV failure in the fetal MMC patient population.