Fetal Surgery For Myelomeningocele Research Articles

Analysis of long-term family dynamics in mothers who have undergone fetal myelomeningocele surgery using telemedicine: a pilot study.

The aim of this study was to understand the dynamics of families with children with myelomeningocele undergoing intrauterine fetal surgery. A retrospective cohort pilot study was carried out with 11 mothers of children who had undergone intrauterine myelomeningocele repair. Participants in this study responded to an electronic questionnaire (via Google Forms), developed by the study authors, that consisted of 22 multiple-choice questions, of which 17 were closed-ended and 5 had a standardized format. The mean (± standard deviation) of the mothers' age was 37.6 (± 3.5) years. The median of gestational age at delivery and birthweight were 34.9 (range, 33 to 36.1) weeks and 2,300 (range, 1,950 to 2,763) g, respectively. The majority of mothers were white (81.8%), had university degree (81.8%), were Catholic (63.6%), and were married (100%). The majority of mothers rated their relationship with their husband, family, and friends as excellent (54.5, 72.7, and 54.5%, respectively). All 11 mothers reported that the newborn with myelomeningocele was born <37 weeks gestation and the birthweight most often<2,500 g. Approximately 64% of the mothers reported that their child required adaptations or had special needs, of which walking aids (50%) and bladder control (50%) were the most common ones. Telemedicine proved to be a useful tool in the long-term follow-up of children who underwent intrauterine surgery to correct myelomeningocele.

Fetal Surgery for Myelomeningocele: Neurosurgical Perspectives.

More than 30years 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.

Parent Decision-Making With Fetal Surgery for Myelomeningocele

IntroductionThe objectives of this study were to understand how parents made the decision to do maternal–fetal surgery for myelomeningocele and to determine the influence of the shared decision-making model on their choice.MethodThis is a qualitative study of parents in a fetal care center after fetal surgery. Interviews were recorded and transcribed. Analysis was completed using iterative code development, and thematic saturation was reached.ResultsRapid decision making, often before seeing fetal care professionals, was present, parental agency drove the decision for surgery, exposure to other parents via the Internet was key to reducing uncertainty and increasing hope for the future, positive interactions with the healthcare team played a role in parental choice for surgery, and mothers were surprised by unanticipated pain and discomfort following surgery.DiscussionTenets of shared decision making, such as patients feeling supported by their healthcare professionals, were present. Results learned from this study could be used to tailor interactions with parents seeking fetal surgery.

Ultrasound evaluation of uterine scar thickness after open fetal surgery for myelomeningocele.

This study aimed to analyse the evolution of uterine scar thickness after open fetal surgery for myelomeningocele (MMC) by ultrasonography, and to establish a cut-off point for uterine scar thickness associated with high-risk of uterine rupture. A prospective longitudinal study was conducted with 77 pregnant women who underwent open fetal surgery for MMC between 24 and 27weeks of gestation. After fetal surgery, ultrasound follow-up was performed once a week, and the scar on the uterine wall was evaluated and its thickness was measured by transabdominal ultrasound. At least five measurements of the uterine scar thickness were performed during pregnancy. A receiver operating characteristics (ROC) curve was constructed to obtain a cut-off point for the thickness of the scar capable of detecting the absence of thinning. Kaplan-Meier curves were constructed to evaluate the probability of thinning during pregnancy follow-up. The mean ± standard deviation of maternal age (years), gestational age at surgery (weeks), gestational age at delivery (weeks), and birth weight (g) were 30.6 ± 4.5, 26.1 ± 0.8, 34.3 ± 1.2 and 2287.4 ± 334.4, respectively. Thinning was observed in 23 patients (29.9%). Pregnant women with no thinning had an average of 17.1 ± 5.2min longer surgery time than pregnant women with thinning. A decrease of 1.0mm in the thickness of the uterine scar was associated with an increased likelihood of thinning by 1.81-fold (95% confidence interval [CI]: 1.32-2.47; p < 0.001). The area below the ROC curve was 0.899 (95% CI: 0.806-0.954; p < 0.001), and the cut-off point was ≤ 3.0mm, which simultaneously presented greater sensitivity and specificity. After 63days of surgery, the probability of uterine scarring was 50% (95% CI: 58-69). A cut-off point of ≤ 3.0mm in the thickness of the uterine scar after open fetal surgery for MMC may be used during ultrasonography monitoring for decision-making regarding the risk of uterine rupture and indication of caesarean section.

Sociodemographic disparities in fetal surgery for myelomeningocele: a single-center retrospective review.

Fetal surgery for myelomeningocele has become an established treatment that offers less risk of requiring a ventricular shunt and improved functional outcomes for patients. An increasing body of literature has suggested that social determinants of health have a profound influence on health outcomes. The authors sought to determine the socioeconomic and racial and ethnic backgrounds of patients who were treated with fetal surgery versus those who underwent postnatal repair. Demographic data, the method of myelomeningocele repair, insurance status, and zip code data for patients entered into the National Spina Bifida Patient Registry (NSBPR) from Children's Wisconsin were collected. The zip code was used to determine the Distressed Communities Index (DCI) score, a composite socioeconomic ranking with scores ranging from 0 (no distress) to 100 (severe distress). The zip code was also used to determine the median household income for each patient based on the US Census Bureau 2013-2017 American Community Survey 5-year estimates. A total of 205 patients were identified with zip code and insurance data. There were 23 patients in the fetal surgery group and 182 patients in the postnatal surgery group. All patients were born between 2000 and 2019. Patients in the fetal surgery group were more likely to have commercial insurance (100% vs 52.2%, p < 0.001). Fetal surgery patients were also more likely to be non-Hispanic White (95.7% vs 68.7%, p = 0.058), just missing the level of statistical significance. Patients who underwent fetal surgery tended to reside in zip codes with a higher median household income (mean $66,507 vs $59,133, p = 0.122) and less-distressed communities (mean DCI score 31.3 vs 38.5, p = 0.289); however, these differences did not reach statistical significance. Patients treated with fetal surgery were more likely to have commercial insurance and have a non-Hispanic White racial and ethnic background. The preliminary data suggest that socioeconomic and racial and ethnic disparities may exist regarding access to fetal surgery, and investigation of a larger population of spina bifida patients is warranted.

Réflexions éthiques concernant la chirurgie fœtale de réparation des myéloméningocèles

Fetal myelomeningocele surgery was introduced in France in 2014. Developments in prenatal diagnosis of neural tube defects have accompanied the development of prenatal diagnosis. This fetal surgery represents one of the three possible care paths for pregnant women faced with this prenatal diagnosis. The ethical issues of this fetal surgery are discussed and in particular regarding prenatal counselling and patient autonomy of choice.

Update on prenatal diagnosis and fetal surgery for myelomeningocele.

A seminal study titled Management of Myelomeningocele Study, from 2011, demonstrated that prenatal myelomeningocele defect repaired before 26 weeks of gestation improved neurological outcomes; based on this study, fetal surgery was introduced as a standard of care alternative. Thus, prenatal myelomeningocele diagnosis within the therapeutic window became a mandatory goal; therefore, research efforts on screening strategies were intensified, especially in the first trimester. In addition, different fetal surgery techniques were developed to improve neurological outcomes and reduce maternal risks. The objective of this review is to provide an update on the advances in prenatal screening and diagnosis during the first and second trimesters, and in open and fetoscopic fetal surgery for myelomeningocele.

Alternative Hysterotomy Technique for the Reduction of Prenatal Complications of Intrauterine Myelomeningocele Repair

Introduction: The main aim of the fetal surgery of myelomeningocele (MMC) is the improvement in prognosis for children with this defect and at the same time high safety of the procedure for mother and fetus. Objective: The aim of the study was to determine the effectiveness of alternative hysterotomy technique for fetal surgery of MMC in prevention of essential perinatal complications. Methods: Forty-nine pregnant women diagnosed with fetal MMC (fMMC) who underwent classic hysterotomy without the use of stapler formed the study cohort. Two cases of intrauterine fetal death or neonatal death in the immediate perioperative period in the study group (SG) were excluded from further analyses. In the historical control group (46 patients) from our center hysterotomy was performed according to Management of Myelomeningocele Study (MOMS) protocol. The study cohort’s outcomes were compared with the historical controls and with post-MOMS results in Children’s Hospital of Philadelphia (CHOP) and Vanderbilt University Medical Center (VUMC). Results: In the SG we did not observe delivery before 30 weeks of gestation (0/47; 0%) and the incidence of chorionamniotic membrane separation (CMS, 3/47; 6%) and preterm premature rupture of membranes (PPROM, 7/47; 15%) was lower compared to the other groups. Preterm delivery as a result of uterine contractions occurred significantly less frequently in the SG (8/47; 17%). Fetal surgery with classic hysterotomy resulted in rare use of magnesium sulfate (3/47; 6%). Conclusions: The alternative hysterotomy technique for fetal surgery of MMC results in low occurrences of CMS, PPROM, preterm delivery, and the rare use of tocolytics.

Determination of Anatomical Levels in Spina Bifida Fetuses withUltrasound and MRI.

The goal of this study was to assess the accuracy of prenatal anatomical level determination by ultrasound (US) and magnetic resonance imaging (MRI) by analyzing the congruence with the "true" anatomical level identified by postnatal MRI. The first 60 patients undergoing fetal myelomeningocele surgery at The Zurich Center for Fetal Diangosis and Therapy were included in this study. Anatomical levels (i. e., first dysraphic vertebra) determined by prenatal US and MRI were compared to postnatal MRI. The level of agreement between the imaging modalities was evaluated with a Cohen's kappa test. Results > 0.6 were interpreted as good agreement, > 0.8 as excellent. The exact congruence between prenatal US and MRI compared to postnatal MRI was 33 % and 48 %, respectively, for an accuracy within one level difference of 80 % and 90 %, and within two levels difference of 95 % and 98 %, respectively. The level of agreement of prenatal US and MRI compared to postnatal MRI was 0.62 and 0.79, respectively. Most of the prenatally incorrectly assigned levels were assigned too high (worse) than the "true" level (US 88 % vs. MRI 65 %). Reliable exact prenatal level determination by US and MRI is not possible. However, the prenatal determination of the anatomical level of the lesion is good within one level margin of error. Prenatal US as well as MRI demonstrate a systematic error towards higher levels. The above considerations must be integrated into prenatal counselling.

Fetal surgical intervention for myelomeningocele: lessons learned, outcomes, and future implications.

Fetal myelomeningocele (fMMC) closure (spina bifida aperta) has become a care option for patients that meet inclusion criteria, but it is clear that fetal intervention, while improving outcomes, is not a cure. This review will: (1) focus on the rationale for fMMC surgery based on preclinical studies and observations that laid the foundation for human pilot studies and a randomized controlled trial; (2) summarize important clinical outcomes; (3) discuss the feasibility, efficacy, and safety of recent developments in fetal surgical techniques and approaches; and (4) highlight future research directions. Given the increased risk of maternal and fetal morbidity associated with prenatal intervention, accompanied by the increasing number of centres performing interventions worldwide, teams involved in the care of these patients need to proceed with caution to maintain technical expertise, competency, and patient safety. Ongoing assessment of durability of the benefits of fMMC surgery, as well as additional refinement of patient selection criteria and counselling, is needed to further improve outcomes and reduce the risks to the mother and fetus. WHAT THIS PAPER ADDS: High-quality prospective studies are needed to broaden the indication for fetal surgery in the general myelomeningocele population. Innovative minimally invasive approaches have had promising results, yet lack comprehensive and robust experimental or clinical evaluation. Important information to help families make informed decisions regarding fetal surgery for myelomeningocele is provided.

189: Ventricle size as a predictor for ventriculoperitoneal shunt after fetal surgery for myelomeningocele

189: Ventricle size as a predictor for ventriculoperitoneal shunt after fetal surgery for myelomeningocele

Fetal myelomeningocele surgery: Only treating the tip of the iceberg.

Fetal myelomeningocele (fMMC) surgery improves infant outcomes when compared with postnatal surgery. Surgical selection criteria and the option of pregnancy termination, however, limit the number of cases that are eligible for prenatal surgery. We aimed to quantify what proportion of cases could ultimately benefit from fetal therapy. We retrospectively reviewed all cases of fMMC referred to a large tertiary care center over a 10-year period and assessed their eligibility for fetal surgery, pregnancy termination rates, and actual uptake of the surgery. Of 158 cases, 67 (42%) were ineligible for fetal surgery based on surgical exclusion criteria. Eleven fetuses (7%) had chromosomal anomalies, 10 of which (91%) had other anomalies on ultrasound. Thirty-four patients had a combination of maternal and fetal contraindications. Of the remaining 91 eligible cases (58%), 45 (49%) pregnancies were terminated, leaving only 46 (29% of initial 158 cases) as potential candidates for fetal repair. Actual uptake of fetal surgery was 15% (n=14 of 91), but this increased after a national program was started. Only a minority of fMMC cases will ultimately undergo fetal surgery. These numbers support the centralization of care in expert centers.

A study to assess global availability of fetal surgery for myelomeningocele.

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

87: Biomarkers in amniotic fluid at the time of fetal myelomeningocele surgery predict premature rupture of membranes

87: Biomarkers in amniotic fluid at the time of fetal myelomeningocele surgery predict premature rupture of membranes

Fetal Surgery for Myelomeningocele: A Systematic Review and Meta-Analysis of Outcomes in Fetoscopic versus Open Repair

Background/Objectives: The Management of Myelomeningocele (MMC) Study (MOMS) showed that prenatal repair of MMC resulted in improved neurological outcomes but was associated with high rates of obstetrical complications. This study compares outcomes of open and fetoscopic MMC repair. Data Sources: PubMed and Embase studies reporting outcomes of fetal MMC repair published since the completion of the MOMS. Results: We analyzed 11 studies and found no difference in mortality or the rate of shunt placement for hydrocephalus. Percutaneous fetoscopic repair was associated with higher rates of premature rupture of membranes (91 vs. 36%, p < 0.01) and preterm birth (96 vs. 81%, p = 0.04) compared to open repair, whereas fetoscopic repair via maternal laparotomy reduced preterm birth. The rate of dehiscence and leakage from the MMC repair site was higher after both types of fetoscopic surgery (30 vs. 7%, p < 0.01), while the rate of uterine dehiscence was higher after open repair (11 vs. 0%, p < 0.01). Discussion: Fetoscopic repair is a promising alternative to open fetal MMC repair with a lower risk of uterine dehiscence; however, fetoscopic techniques should be optimized to overcome the high rate of dehiscence and leakage at the MMC repair site. A fetoscopic approach via maternal laparotomy reduces the risk of preterm birth.

OP07.02: The relationship between the posterior fossa structures and foramen magnum using the occipitum‐dens line (ODL) in following fetal surgery for myelomeningocele

OP07.02: The relationship between the posterior fossa structures and foramen magnum using the occipitum‐dens line (ODL) in following fetal surgery for myelomeningocele

Committee Opinion No. 720: Maternal-Fetal Surgery for Myelomeningocele.

Myelomeningocele, a severe form of spina bifida, occurs in approximately 1 in 3,000 live births in the United States. The extent of disability is generally related to the level of the myelomeningocele defect, with a higher upper level of lesion generally corresponding to greater deficits. Open maternal-fetal surgery for myelomeningocele repair is a major procedure for the woman and her affected fetus. Although there is demonstrated potential for fetal and pediatric benefit, there are significant maternal implications and complications that may occur acutely, postoperatively, for the duration of the pregnancy, and in subsequent pregnancies. Women with pregnancies complicated by fetal myelomeningocele who meet established criteria for in utero repair should be counseled in a nondirective fashion regarding all management options, including the possibility of open maternal-fetal surgery. Maternal-fetal surgery for myelomeningocele repair should be offered only to carefully selected patients at facilities with an appropriate level of personnel and resources.

Committee Opinion No. 720 Summary: Maternal-Fetal Surgery for Myelomeningocele.

Myelomeningocele, a severe form of spina bifida, occurs in approximately 1 in 3,000 live births in the United States. The extent of disability is generally related to the level of the myelomeningocele defect, with a higher upper level of lesion generally corresponding to greater deficits. Open maternal-fetal surgery for myelomeningocele repair is a major procedure for the woman and her affected fetus. Although there is demonstrated potential for fetal and pediatric benefit, there are significant maternal implications and complications that may occur acutely, postoperatively, for the duration of the pregnancy, and in subsequent pregnancies. Women with pregnancies complicated by fetal myelomeningocele who meet established criteria for in utero repair should be counseled in a nondirective fashion regarding all management options, including the possibility of open maternal-fetal surgery. Maternal-fetal surgery for myelomeningocele repair should be offered only to carefully selected patients at facilities with an appropriate level of personnel and resources.

Histological evidence of reparative activity in chorioamniotic membrane following open fetal surgery for myelomeningocele.

An increased understanding of the reparative process in fetal membrane following surgical techniques may be helpful to decrease the risks to mother and fetus and avoid adverse pregnancy outcomes. The present study discusses histological evaluation of the fetal membrane following open fetal surgery. Chorioamniotic membranes (n=10) were obtained following birth from pregnancies that underwent open fetal surgery for myelomeningocele. The collagen distribution was quantified using picrosirius-polarization method comparing the suture site with non-suture site. The differences between the collagen fiber percentages at the two sites was evaluated by the paired t-test with P<0.05. The mean gestational age of fetal surgery was 26.09±0.3 and 33.81±0.82 weeks at birth. The picrosirius red sign was more intense at the suture site, primarily associated with collagen type 1. Collagen observed in the surgical area was significantly increased (13.22±2.84%) compared with the non-surgical area (6.16±1.09%; P<0.0001). It was observed that the reparative activity at the suture site of the fetal membrane was characterized by a significant increase in collagen fibers. The findings suggest nascent collagen synthesis, tissue remodeling and repair of suture site, a mechanism likely to prevent the amniotic fluid leakage and maintain pregnancy following open fetal surgery.

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.