Surgery For Myelomeningocele Research Articles

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

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. 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. 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). 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.

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.

Development of a simulator for training of fetoscopic myelomeningocele surgery.

To develop a realistic simulation model for laparotomy-assisted fetoscopic spina bifida aperta (SBa) surgery, to be used for training purposes and preoperative planning. The predefined general requirement was a realistic model of an exteriorized uterus, allowing all neurosurgical steps of the intervention. The uterus was modelled using ultrasound and MRI images of a 25 weeks' gravid uterus, consisting of flexible polyurethane foam coated with pigmented silicone. The fetal model, contained an opening on the dorsal side for a customizable spinal insert with all the aspects of a SBa, including a cele, placode, and myofascial and skin layer. The model was assessed in a series of validation experiments. Production costs are low, uterus and fetus are reusable. Placental localization and the level and size of the spinal defect are adjustable, enabling case-specific adaptations. All aspects of the simulator were scored close to realistic or higher for both appearance and functional capacities. This innovative model provides an excellent training opportunity for centers that are starting a fetoscopic SBa repair program. It is the first simulation model with adjustable spinal defect and placental localisation. Further objective validation is required, but the potential for using this model in preoperative planning is promising.

Histopathological presence of dermal elements in resected margins of neural structures obtained from initial repair surgery for myelomeningocele.

Development of dermoid or epidermoid cysts in myelomeningocele (MMC) sites is generally thought to occur in a delayed fashion due to implantation of dermal elements during initial repair surgery. Another theory is that dermal and dermoid elements may already be present within dysplastic neural structures at birth. We experienced histopathological presence of dermal elements in resected tissues at initial repair surgery in four out of 18 cases with MMC who required resection of parts or margins of the neural structures to perform cord untethering. Since one of these cases has already been reported, we describe the clinicopathological findings for the remaining three cases. In Case1, cryptic dermoid elements were discovered in the terminal filum-like structure (FT-LS) caudal to the open neural placode (NP). The FT-LS had histopathological characteristics similar to the retained medullary cord. In Case 2, dermoid elements were discovered in the caudal margin of the dysplastic conus medullaris. In Case 3, a thin squamous epithelial layer overlapped the rostral margin of the NP where the NP was located near the skin. Case 1 developed an epidermoid cyst at 1 year and 2 months of age, which was totally resected. Prenatally existing cryptic dermoid elements in the caudal portion of neural structures and remnants of dermal elements overlapping the rostral margin of the NP are associated with delayed occurrence of dermoid/ epidermoid cysts. Postoperative histopathological investigation of the resected specimens is recommended. Once dermal elements are revealed, repeated imaging examination and additional surgery should be considered.

Postnatal surgery for myelomeningocele in neonates: neurodevelopmental outcomes.

The study aims to evaluate the neurodevelopmental outcomes of neonates with myelomeningocele (MMC) operated in the postnatal period. This is a prospective follow-up study in a tertiary neonatal intensive care unit. Neurodevelopmental outcomes of term neonates operated for MMC and healthy term newborns were compared with the Bayley Scales of Infant and Toddler Development -Third Edition (BSID III) at 12-18 months. A total of 57 cases were included in the study (patient group = 27; control group = 30). Demographic data between the groups were similar. Cognitive, linguistic, and motor composite scores of the patient group were lower than those of the control group (p < 0.001). In the patient group, those who underwent ventriculoperitoneal shunt had lower cognitive, language and motor scores than those without shunt (p < 0.05). The cognitive, linguistic, and motor composite scores in the patient group who underwent surgery before 72 h were better than those who underwent surgery after 72 h. In our study, it was found that the neurodevelopmental prognosis of MMC cases requiring ventriculoperitoneal shunt in the postnatal period was significantly worse than those without shunt. It is the first study in which the neurodevelopment of patients with MMC who were operated in the postnatal period was evaluated with BSID III evaluated and delays in all areas were shown in cases with MMC compared to normal cases. Better neurodevelopmental outcomes in patients operated in the first 72 h suggest that early surgery will improve neurodevelopmental outcomes in patients with MMC.

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.

Repair Surgery of Myelomeningocele

Meningomyelocele is the most common disease of the spina bifida, and repair surgery is recommended within the first 48 hours of life to prevent infection and secondary neurological damage. The basic surgical approach is a five-layer closure that closes the five layers of the spinal cord, dura matter, fascia, subcutaneous tissue, and skin. Comprehending the relationship between normal anatomy of the spinal cord and myelomeningocele is key. In this section, we discuss the basic repair surgery of meningomyelocele by describing the surgery of lumbosacral meningomyelocele.

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.

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

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

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.

Closure of large lumbosacral defect using a combined method of bilateral bipedicle flap with lateral releasing incision and Integra® dermal regeneration template

BACKGROUND: Myelomeningocele is one of the most complex congenital malformations of the central nervous system. It is one of the most common types of spina bifida which involves a failure of neural tube closure. Reconstruction surgery for myelomeningocele had always been challenging for plastic and neurosurgeons.&#x0D; CLINICAL CASE: We report a case of a new-born with lumbosacral myelomeningocele who received treatment in the Hospital Universiti Sains Malaysia. The myelomeningocele was repaired by the neurosurgery team and subsequently, the child was left with huge lumbosacral skin defect. The large defect was successfully covered by using a combined method of bilateral bipedicle flap with lateral releasing incision and remaining lumbosacral and secondary defect resurfaced using Integra dermal regeneration template (DRT). We used ACTICOAT interfaced negative pressure wound therapy (NPWT) as our main dressing in preparing the wound bed for autologous epidermal graft. The result of our closure technique provides tension free closure.&#x0D; DISCUSSION: We incorporated bilateral bipedicle fasciocutaneous flap technique together with DRT for closure of the lumbosacral defect. The bilateral bipedicle flap with lateral releasing incision served to reduce tension on the skin at bilateral lumbar region. The DRT downsized the lumbosacral defect and NPWT dressing provided an optimal sterile environment in giving time for neodermis generation. The remaining secondary defect were also resurfaced utilizing DRT and autologous skin grafting.&#x0D; CONCLUSIONS: The outcome of surgery demonstrated that the combined use of bilateral bipedicle fasciocutaneous flap with lateral releasing incision and DRT with delayed skin grafting is safe, effective and provide long term stable and supple scar for large, exposed dura defect.

Myelomeningocele Surgery over the 10 Years Following the MOMS Trial: A Systematic Review of Outcomes in Prenatal versus Postnatal Surgical Repair.

Background and Objectives: Myelomeningocele is the most severe form of spina bifida, a congenital neural tube defect arising from an incomplete neural tube closure during early development with damage worsening with advancing gestational age. The Management of Myelomeningocele Study (MOMS) Trial proved that surgery performed before 26 weeks of gestation significantly improved the prognosis, significantly changing treatment paradigms. This article aims to provide a review of the changes and updates in spina bifida repair over the 10-year period following the MOMS Trial. Material and methods: We performed a systematic review in the PubMed and Cochrane databases as well as a hand-search of high-impact journals using the reference list of all identified articles, searching for randomized controlled trials and observational studies. Results: We identified 27 articles published between 2011 and 2021 that fulfilled the inclusion criteria and review them in the present study. Conclusions: With growing experience and with the improvement of prenatal open and fetoscopic techniques, the outcome of SB-associated conditions could be improved and the risks to both the mother and the fetus reduced. A continuous follow-up of the treated infants and further randomized trials are essential to study the complications and advantages or disadvantages of any given treatment strategy.

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.

Open fetal surgery for myelomeningocele repair in France

IntroductionOpen fetal myelomeningocele (MMC) surgery is currently the standard of care option for prenatal MMC repair. We described the population referred to our center and reviewed outcome after open fetal MMC repair. Material and methodsAll patients referred to our center for MMC were reviewed from July 2014 to June 2020. For all the patients who underwent fetal MMC repair, surgical details, maternal characteristics and data from the neonatal to the three-years-old evaluations were collected. ResultsAmong the 126 patients referred to our center, 49.2% were eligible and 27.4% (n = 17) of them underwent fetal MMC repair. Average gestational age at fetal surgery was 24+6 weeks. There was no case of fetal complication and the only maternal complication was one case of transfusion. We recorded 70% of premature rupture of membranes and 47% of premature labor. Average gestational age at delivery was 34+2 weeks and no patient delivered before 30 weeks. There was no case of uterine scar dehiscence or maternal complication during cesarean section. After birth, 59% of the children had a hindbrain herniation reversal. At 1-year-old, 42% were assigned a functional level of one or more better than expected according to the prenatal anatomic level and 25% required a ventriculoperitoneal shunt. At 3-year-old, all the children attended school and 75% were able to walk with orthotics or independently. ConclusionOpen fetal surgery enables anatomical repair of the MMC lesion, a potential benefit on cerebral anomalies and motor function, with a low rate of perinatal and maternal complications.

Direct Stimulation of Neural Placode and Nerve Roots in Open Myelomeningocele: The Efficacy of Neural Tube Reconstruction

Introduction: Intraoperative electrophysiological studies are increasingly used in spinal surgery. However, its use in myelomeningocele (MMC) surgery is still not widespread. The aim of this study was to present our experience in neural placode (NP) and nerve root stimulations in newborns with open MMC. Methods: Eight newborns underwent surgical treatment for thoracolumbar and lumbosacral MMCs. Intraoperative neuromonitoring including free-running electromyography and stimulation of NP, nerve roots, and spinal cord were performed in all cases. Stimulation sites and intensities and distal response’s amplitudes and latencies were recorded. Results: Five patients had thoracolumbar and 3 patients had lumbosacral MMC. Two patients had no movements at the lower extremities while the other had some movements. No response on the lower extremities was obtained in only 1 patient. Responses from the nerve root stimulations were more robust and significant than the placode stimulations. Conclusions: It is clear that the NP and nerve roots originating from the placode are mostly functional and should be preserved during the surgery. Intraoperative neuromonitoring and direct stimulation should be performed during the MMC repair in order to obtain a better neurological outcome.

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.

Abnormal Head Size in Children and Adolescents with Congenital Nervous System Disorders or Neurological Syndromes with One or More Neurodysfunction Visible since Infancy.

The current study was designed to investigate co-occurrence of absolute/relative microcephaly, absolute/relative macrocephaly and congenital nervous system disorders or neurological syndromes with symptoms visible since infancy, based on fundamental data acquired during the admission procedure at a neurological rehabilitation ward for children and adolescents. The study applied a retrospective analysis of data collected during the hospitalization of 327 children and adolescents, aged 4–18 years, affected since infancy by congenital disorders of the nervous system and/or neurological syndromes associated with a minimum of one neurodysfunction. To identify subjects with absolute/relative microcephaly, absolute/relative macrocephaly in the group of children and adolescents, the adopted criteria took into account z-score values for head circumference (z-score hc) and head circumference index (z-score HCI). Dysmorphological (x+/−3s) and traditional (x+/−2s) criteria were adopted to diagnose developmental disorders of head size. Regardless of the adopted criteria, absolute macrocephaly often coexists with state after surgery of lumbar myelomeningocele and hydrocephalus, isolated hydrocephalus, hereditary motor and sensory polyneuropathy, and Becker’s muscular dystrophy (p < 0.001, p = 0.002). Absolute macrocephaly is often associated with neural tube defects and neuromuscular disorders (p = 0.001, p = 0.001). Relative microcephaly often occurs with non-progressive encephalopathy (p = 0.017, p = 0.029). Absolute microcephaly, diagnosed on the basis of traditional criteria, is often associated with epilepsy (p = 0.043). In children and adolescents with congenital nervous system disorders or neurological syndromes with one or more neurodysfunction visible since infancy, there is variation in abnormal head size (statistically significant relationships and clinical implications were established). The definitions used allowed for the differentiation of abnormal head size.

Open Fetal Surgical Outcomes for Myelomeningocele Closure Stratified by Maternal Body Mass Index in a Large Single-Center Cohort

Background: Open maternal-fetal surgery for in utero closure of myelomeningocele (MMC) has become an accepted treatment option for prenatally diagnosed open neural tube defects. Historically, this option has been limited to women with BMI < 35 due to concern for increasing complications in patients with obesity. Objective: The aim of this study was to evaluate maternal, obstetric, and fetal/neonatal outcomes stratified by maternal BMI classification in women who undergo open maternal-fetal surgery for fetal myelomeningocele (fMMC) closure. Methods: A single-center fMMC closure registry was queried for maternal demographics, preoperative factors, fetal surgery outcomes, delivery outcomes, and neonatal outcomes. Data were stratified based on maternal BMI: <30, 30–34.99, and ≥35–40, corresponding to normal weight/overweight, obesity class I, and obesity class II. Statistical analysis was performed using statistical software SAS v.9.4 (SAS Institute Inc., Cary, NC, USA). Results: A total of 264 patients were analyzed, including 196 (74.2%) with BMI <30, 54 (20.5%) with BMI 30–34.99, and 14 (5.3%) with BMI ≥ 35–40. Maternal demographics and preoperative characteristics were similar among the groups. Operative time increased with increasing BMI; otherwise, perioperative outcomes were similar among the groups. Obstetric and neonatal outcomes were similar among the groups. Conclusion: Increasing maternal BMI did not result in a negative impact on maternal, obstetric, and fetal/neonatal outcomes in a large cohort of patients undergoing open maternal-fetal surgery for fMMC closure. Further study is warranted to determine the generalizability of these results.