Comparative Scoping Review of Prenatal Care Resources for Families of Children With Spinal Dysraphism and Hydrocephalus in High-Income Countries and Low- and Middle-Income Countries.

BACKGROUND AND OBJECTIVES: Low- and middle-income countries (LMICs) face higher incidences and burdens of care for neural tube defects (NTDs) and hydrocephalus compared with high-income countries (HICs), in part due to limited access to neurosurgical intervention. In this scoping review, we aim to integrate studies on prenatal care, counseling, and surgical management for families of children with spinal dysraphism and hydrocephalus in LMICs and HICs. METHODS: PubMed, Embase, Global Index Medicus, and Web of Science electronic databases were searched for English language articles pertaining to prenatal care, counseling, and surgical management for families of children with spinal dysraphism and hydrocephalus in HICs and LMICs. Identified abstracts were screened for full-text review. Studies meeting inclusion criteria were reviewed in full and analyzed. RESULTS: Seventy studies met the inclusion criteria. Twelve studies (16.9%) were conducted in HICs only, 50 studies (70.4%) were conducted in LMICs only, and 9 studies (12.7%) encompassed both. On thematic analysis, seven underlying topics were identified: epidemiology, folate deficiency and supplementation/fortification, risk factors other than folate deficiency, prenatal screening, attitudes and perceptions about NTDs and their care, surgical management, and recommendations for guideline implementation. CONCLUSION: NTDs have become a widely acknowledged public health problem in many LMICs. Prenatal counseling and care and folate fortification are critical in the prevention of spinal dysraphism. However, high-quality, standardized studies reporting their epidemiology, prevention, and management remain scarce. Compared with NTDs, research on the prevention and screening of hydrocephalus is even further limited. Future studies are necessary to quantify the burden of disease and identify strategies for improving global outcomes in treating and reducing the prevalence of NTDs and hydrocephalus. Surgical management of NTDs in LMICs is currently limited, but pediatric neurosurgeons may be uniquely equipped to address disparities in the care and counseling of families of children with spinal dysraphism and hydrocephalus.

Socioeconomic characteristics and postoperative outcomes of patients undergoing prenatal vs. postnatal repair of myelomeningoceles.

PURPOSE: To investigate differences in sociodemographic characteristics and short-term outcomes between patients undergoing prenatal versus postnatal myelomeningocele repair. METHODS: Patients who underwent myelomeningocele repair at our institution were stratified based on prenatal or postnatal timing of repair. Baseline characteristics and outcomes were compared. Multivariate analysis was performed to identify whether prenatal repair was a predictor of outcomes independent of socioeconomic measures. RESULTS: 49 patients underwent postnatal repair, and 30 underwent prenatal repair. Patients who underwent prenatal repair were more likely to have private insurance (73.3% vs. 42.9%, p = 0.03) and live farther from the hospital where they received their repair (251.5 ± 447.4 vs. 72.5 ± 205.6 miles, p = 0.02). Patients who underwent prenatal repair had shorter hospital stays (14.3 ± 22.7 days vs. 25.3 ± 20.1 days, p = 0.03), fewer complications (13.8% vs. 42.9%, p = 0.01), fewer 30-day ED visits (0.0% vs. 34.0%, p < 0.001), lower CSF diversion rates (13.8% vs. 38.8%, p = 0.02), and better functional status at 3-months (13.3% vs. 57.1% delayed, p = 0.009), 6-months (20.0% vs. 56.7% delayed, p = 0.03), and 1-year (29.4% vs. 70.6% delayed, p = 0.007). On multivariate analysis, prenatal repair was an independent predictor of inpatient complication (OR(95%CI): 0.19(0.05-0.75), p = 0.02) and 3-month (OR(95%CI): 0.14(0.03-0.80) p = 0.03), 6-month (OR(95%CI): 0.12(0.02-0.73), p = 0.02), and 1-year (OR(95%CI): 0.19(0.05-0.80), p = 0.02) functional status. CONCLUSION: Prenatal repair for myelomeningocele is associated with better outcomes and developmental functional status. However, patients receiving prenatal closure are more likely to have private health insurance and live farther from the hospital, suggesting potential barriers to care.

Potential higher risk of tethered spinal cord in children after prenatal surgery for myelomeningocele: A systematic review and meta-analysis.

INTRODUCTION: We performed a systematic review and meta-analysis on the incidence of secondary tethered spinal cord (TSC) between prenatal and postnatal closure in patients with MMC. The objectives was to understand the incidence of secondary TSC after prenatal surgery for MMC compared to postnatal surgery for MMC. MATERIAL AND METHODS: On May 4, 2023, a systematic search was conducted in Medline, Embase, and the Cochrane Library to gather relevant data. Primary studies focusing on repair type, lesion level, and TSC were included, while non-English or non-Dutch reports, case reports, conference abstracts, editorials, letters, comments, and animal studies were excluded. Two reviewers assessed the included studies for bias risk, following PRISMA guidelines. TSC frequency in MMC closure types was determined, and the relationship between TSC occurrence and closure technique was analyzed using relative risk and Fisher's exact test. Subgroup analysis revealed relative risk differences based on study designs and follow-up periods. A total of ten studies, involving 2,724 patients, were assessed. Among them, 2,293 patients underwent postnatal closure, while 431 received prenatal closure for the MMC defect. In the prenatal closure group, TSC occurred in 21.6% (n = 93), compared to 18.8% (n = 432) in the postnatal closure group. The relative risk (RR) of TSC in patients with prenatal MMC closure versus postnatal MMC closure was 1.145 (95%CI 0.939 to 1.398). Fisher's exact test indicated a statistically non-significant association (p = 0.106) between TSC and closure technique. When considering only RCT and controlled cohort studies, the overall RR for TSC was 1.308 (95%CI 1.007 to 1.698) with a non-significant association (p = .053). For studies focusing on children up until early puberty (maximum 12 years follow-up), the RR for tethering was 1.104 (95%CI 0.876 to 1.391), with a non-significant association (p = 0.409). CONCLUSION AND DISCUSSION: This review found no significant increase in relative risk of TSC between prenatal and postnatal closure in MMC patients, but a trend of increased TSC in the prenatal group. More long-term data on TSC after fetal closure is needed for better counseling and outcomes in MMC.

Consensus-Based Best Practice Guidelines for the Management of Spinal Deformity and Associated Tumors in Pediatric Neurofibromatosis Type 1: Screening and Surveillance, Surgical Intervention, and Medical Therapy.

BACKGROUND: Spinal conditions, such as scoliosis and spinal tumors, are prevalent in neurofibromatosis type 1 (NF1). Despite the recognized importance of their early detection and treatment, there remain knowledge gaps in how to approach these manifestations. The purpose of this study was to utilize the experience of a multidisciplinary committee of experts to establish consensus-based best practice guidelines (BPGs) for spinal screening and surveillance, surgical intervention, and medical therapy in pediatric patients with NF1. METHODS: Using the results of a prior systematic review, 10 key questions that required further assessment were first identified. A committee of 20 experts across medical specialties was then chosen based on their clinical experience with spinal deformity and tumors in NF1. These were 9 orthopaedic surgeons, 4 neuro-oncologists/oncologists, 3 neurosurgeons, 2 neurologists, 1 pulmonologist, and 1 clinical geneticist. An initial online survey on current practices and opinions was conducted, followed by 2 additional surveys via a formal consensus-based modified Delphi method. The final survey involved voting on agreement or disagreement with 35 recommendations. Items reaching consensus (≥70% agreement or disagreement) were included in the final BPGs. RESULTS: Consensus was reached for 30 total recommendations on the management of spinal deformity and tumors in NF1. These were 11 recommendations on screening and surveillance, 16 on surgical intervention, and 3 on medical therapy. Five recommendations did not achieve consensus and were excluded from the BPGs. CONCLUSION: We present a set of consensus-based BPGs comprised of 30 recommendations for spinal screening and surveillance, surgical intervention, and medical therapy in pediatric NF1.

Building consensus for the medical management of children with moderate and severe acute spinal cord injury: a modified Delphi study.

OBJECTIVE: The focus of this modified Delphi study was to investigate and build consensus regarding the medical management of children with moderate and severe acute spinal cord injury (SCI) during their initial inpatient hospitalization. This impetus for the study was based on the AANS/CNS guidelines for pediatric SCI published in 2013, which indicated that there was no consensus provided in the literature describing the medical management of pediatric patients with SCIs. METHODS: An international, multidisciplinary group of 19 physicians, including pediatric neurosurgeons, orthopedic surgeons, and intensivists, were asked to participate. The authors chose to include both complete and incomplete injuries with traumatic as well as iatrogenic etiologies (e.g., spinal deformity surgery, spinal traction, intradural spinal surgery, etc.) due to the overall low incidence of pediatric SCI, potentially similar pathophysiology, and scarce literature exploring whether different etiologies of SCI should be managed differently. An initial survey of current practices was administered, and based on the responses, a follow-up survey of potential consensus statements was distributed. Consensus was defined as ≥ 80% of participants reaching agreement on a 4-point Likert scale (strongly agree, agree, disagree, strongly disagree). A final meeting was held virtually to generate final consensus statements. RESULTS: Following the final Delphi round, 35 statements reached consensus after modification and consolidation of previous statements. Statements were categorized into the following eight sections: inpatient care unit, spinal immobilization, pharmacological management, cardiopulmonary management, venous thromboembolism prophylaxis, genitourinary management, gastrointestinal/nutritional management, and pressure ulcer prophylaxis. All participants stated that they would be willing or somewhat willing to change their practices based on consensus guidelines. CONCLUSIONS: General management strategies were similar for both iatrogenic (e.g., spinal deformity, traction, etc.) and traumatic SCIs. Steroids were recommended only for injury after intradural surgery, not after acute traumatic or iatrogenic extradural surgery. Consensus was reached that mean arterial pressure ranges are preferred for blood pressure targets following SCI, with goals between 80 and 90 mm Hg for children at least 6 years of age. Further multicenter study of steroid use following acute neuromonitoring changes was recommended.

Transnational Capacity Building Efforts in Global Neurosurgery: A Review and Analysis of Their Impact and Determinants of Success.

BACKGROUND: Neurosurgical capacity building efforts attempt to address the shortage of neurosurgeons and lack of neurosurgical capacity in low- and middle-income countries. This review sought to characterize neurosurgical capacity building efforts in low- and middle-income countries and the challenges they face, and identify factors that predict higher engagement, better training, and performance of high-impact activities. METHODS: A scoping review using PubMed and Embase databases was performed and relevant articles were identified. Programs were classified into 6 categories and the activities they performed were classified as having a high-, medium-, or mild impact on capacity. Programs were also classified using the Olivieri engagement and training criteria. RESULTS: Fifty-seven articles representing 42 unique efforts were included. The most important determinant of impact was a program's design and intent. Furthermore, 91% of training and twinning programs received high (class 2 or 3) engagement classifications compared to 17% of mission trips and training camps (P < 0.001); 91% of training and twinning programs received high training classifications compared to 64% of mission trips and training camps (P = 0.015); and 91% of training and twinning programs reported performing high-impact activities compared to 29% of mission trips and training camps (P < 0.001). CONCLUSIONS: Training and twinning programs are more engaged, offer better training, and are more likely to perform high-impact activities compared to mission trips and training camps, suggesting that these types of programs offer the greatest chance of producing substantial and sustainable improvements to neurosurgical capacity.

Developing consensus for the management of pediatric cervical spine disorders and stabilization: a modified Delphi study.

OBJECTIVE: Cervical spine disorders in children are relatively uncommon; therefore, paradigms for surgical and nonsurgical clinical management are not well established. The purpose of this study was to bring together an international, multidisciplinary group of pediatric cervical spine experts to build consensus via a modified Delphi approach regarding the clinical management of children with cervical spine disorders and those undergoing cervical spine stabilization surgery. METHODS: A modified Delphi method was used to identify consensus statements for the management of children with cervical spine disorders requiring stabilization. A survey of current practices, supplemented by a literature review, was electronically distributed to 17 neurosurgeons and orthopedic surgeons experienced with the clinical management of pediatric cervical spine disorders. Subsequently, 52 summary statements were formulated and distributed to the group. Statements that reached near consensus or that were of particular interest were then discussed during an in-person meeting to attain further consensus. Consensus was defined as ≥ 80% agreement on a 4-point Likert scale (strongly agree, agree, disagree, strongly disagree). RESULTS: Forty-five consensus-driven statements were identified, with all participants willing to incorporate them into their practice. For children with cervical spine disorders and/or stabilization, consensus statements were divided into the following categories: A) preoperative planning (12 statements); B) radiographic thresholds of instability (4); C) intraoperative/perioperative management (15); D) postoperative care (11); and E) nonoperative management (3). Several important statements reaching consensus included the following recommendations: 1) to obtain pre-positioning baseline signals with intraoperative neuromonitoring; 2) to use rigid instrumentation when technically feasible; 3) to provide postoperative external immobilization for 6-12 weeks with a rigid cervical collar rather than halo vest immobilization; and 4) to continue clinical postoperative follow-up at least until anatomical cervical spine maturity was reached. In addition, preoperative radiographic thresholds for instability that reached consensus included the following: 1) translational motion ≥ 5 mm at C1-2 (excluding patients with Down syndrome) or ≥ 4 mm in the subaxial spine; 2) dynamic angulation in the subaxial spine ≥ 10°; and 3) abnormal motion and T2 signal change on MRI seen at the same level. CONCLUSIONS: In this study, the authors have demonstrated that a multidisciplinary, international group of pediatric cervical spine experts was able to reach consensus on 45 statements regarding the management of pediatric cervical spine disorders and stabilization. Further study is required to determine if implementation of these practices can lead to reduced complications and improved outcomes for children.

Understanding the Ghanaian Neurosurgical Literature: A Scoping Review and Bibliometric Analysis.

BACKGROUND: Research serves to bolster clinical neurosurgery by critically assessing various disease pathologies, while identifying important challenges and opportunities. However, there is limited information on the landscape of the Ghanaian neurosurgical literature. METHODS: A scoping review and bibliometric analysis was conducted in accordance with PRISMA guidelines. PubMed, Embase, Global Index Medicus, and Web of Science electronic databases were searched from inception until December 21, 2021 for English language articles about neurosurgery in Ghana. RESULTS: 927 articles were identified and 66 were ultimately included in the analysis. A majority of them, 42.4%, were retrospective cohort studies, with 62.1% published after 2010. There were no randomized controlled or basic science studies. Most articles were published in the West African Journal of Medicine (24.2%) and non-infectious/non-traumatic spinal pathology was the most commonly discussed topic (22.7%); 66.7% of articles included only authors affiliated with Ghanaian institutions, and international collaborators frequently originated from the United States (15.9%). Only 22.7% of the manuscripts reported a funding source. Commonly reported challenges included limited sample sizes, delays in diagnosis and treatment, and lack of proper diagnostic tools and specialized care. CONCLUSIONS: This review revealed that while the Ghanaian academic neurosurgery output has been increasing over time, these have been limited to cohort studies largely assessing spine pathology. The Ghanaian neurosurgical research environment may be bolstered by an increase in research funding, the establishment of longitudinal clinical databases, training in research methodology, increased incentives for researchers, strengthening of research collaborative networks, and increased engagement of neurosurgical trainees in research.

Assessment of the Neurosurgical Capacity in Ghana: Challenges and Opportunities.

BACKGROUND: Low- and middle-income countries experience numerous challenges in the provision of neurosurgical care. However, limited information exists on the neurosurgical workforce and the constraints under which care is delivered in Ghana, West Africa. METHODS: A 19-item survey assessing neurosurgical workforce, infrastructure, and education was administered to Ghanaian consultant neurosurgeons and neurosurgeon trainees between November 8, 2021, and January 20, 2022. The data were analyzed using summary descriptions, and qualitative data were categorized into themes. RESULTS: There were 25 consultant neurosurgeons and 8 neurosurgical trainees (from 2 training centers) identified at 11 hospitals in Ghana totaling a workforce density of 1 neurosurgeon per 1,240,000. Most neurosurgical centers were located in Accra, the capital city. Almost half of the population did not have access to a hospital with a neurosurgeon in their region. Of hospitals, 82% had in-house computed tomography and/or magnetic resonance imaging scanners. In the operating room, most neurosurgeons had access to a high-speed drill (91%) but lacked microscopes and endoscopic sets (only 64% and 36% had these tools, respectively). There were no neurointensivists or neurological intensive care units in the entire country, and there was a paucity of neurovascular surgeries and functional neurosurgical procedures. CONCLUSIONS: The provision of neurosurgical care in Ghana has come a long way since the 1960s. However, the neurosurgical community continues to face significant challenges. Alleviating these barriers to care will call for systems-level changes that allow for the prioritization of neurosurgical care within the Ghanaian health care system.

Weighing the Risk Profile of Cervical Spine MRI in Evaluating Pediatric Cervical Spine Injuries.

OBJECTIVE: Screening for cervical spine injury after blunt trauma is common, but there remains varied practice patterns and clinical uncertainty regarding adequate radiographic evaluation. An oft-cited downside of MRI is the added risk compared to CT in the pediatric population; however, these specific risks have not yet been reported. This study examines the risks of cervical spine MRI in pediatric trauma patients in the context of what value MRI adds. METHODS: This was a retrospective observational study of all pediatric blunt trauma patients who were evaluated with a cervical spine MRI over a 4-year period at a level 1 pediatric trauma center. Clinical and radiographic data were abstracted, as well as anesthesia requirements and MRI-related major adverse events. CT and MRI results were compared for their ability to detect clinically unstable injuries - those requiring halo or surgery. RESULTS: There was one major adverse event related to MRI among the 269 patients who underwent cervical spine MRI - a rate of 0.37%. While 55% of children had an airway and anesthesia for MRI, only 57% of these airways were newly placed for the MRI. None of the 85 patients newly intubated for MRI developed aspiration pneumonitis or ventilator-associated pneumonia, and no patients had a significant neurologic event while at MRI. Another area of the body was imaged concurrently with the cervical spine MRI in 64% of patients and 83% of MRIs were performed within 48 h. CT and MRI were both 100% sensitive for injuries requiring halo or operative intervention. Eighty-three patients had an MRI performed after a negative CT, 11% (9/83) of these patients had a clinically stable injury detected on subsequent MRI, and none of these patients presented for delayed cervical spine complications. CONCLUSIONS: Overall, the safety profile of MRI in this setting is excellent and less than one-third of patients need new airway and anesthesia solely for MRI. In this clinical scenario, MRIs can happen relatively quickly and many patients require another body part to be imaged concurrently anyway. MRI and CT were both 100% sensitive for clinically unstable injuries. In the appropriate patients, MRI remains a safe and radiation-free alternative to CT.

Spinal screening, malignancy, medical therapy, and surgical correction of deformity in pediatric patients with neurofibromatosis type 1: a systematic review.

The objective of this systematic review was to synthesize evidence regarding spinal screening recommendations, types of spinal and thoracic neurofibromatosis type 1 (NF1) tumors, medical therapy for NF1-associated neoplasms, and treatment with magnetically controlled growing rods (MCGRs) or cervical kyphosis correction in pediatric patients with NF1. We queried PubMed, Embase, Cochrane Library, Web of Science, Scopus, Clinicaltrials.gov, and medRxiv for studies reporting spinal screening recommendations, prognosis, and medical therapy for NF1-associated spinal tumors and MCGR use or cervical kyphosis correction in pediatric NF1 patients, yielding 758 publications, 33 of which were included. There is no consensus on spinal screening interval. Computed tomography is recommended for postoperative monitoring. Patients with gangliomas and spinal neurofibromas had nearly complete symptom resolution after resection. Plexiform neurofibromas were most commonly treated with resection and laminectomy; some patients reported tumor enlargement after intervention. Malignant nerve sheath tumors have high rates of metastasis even after chemoradiation and resection. MEK-inhibitors produced limited regression in tumor size. Sirolimus and thalidomide reduced tumor size but caused more severe adverse effects than MEK-inhibitors. Improvements in major curves and T1-T12 height gain were reported after MCGR intervention. Anteroposterior arthrodesis produced the greatest correction of dystrophic cervical kyphosis. There may be value in establishing standardized spinal screening protocols for pediatric NF1 patients. Surgical correction of NF1-associated spinal deformity is effective, though current medical therapies for spinal tumors have limited success. Areas for further investigation include determining appropriate screening intervals, choice of medical therapy for spinal tumors, and long-term outcomes of MCGRs. Level of Evidence: IV.

Safety and Quality in Syndromic Neurosurgery.

The developing field of syndromic neurosurgery has significant challenges and opportunities in quality and safety. Quality care must be safe, effective, patient-centered, timely, efficient, and equitable; the Donabedian model focused on system structures, processes, and outcomes is a helpful framework to guide improvement in these areas. Ultimately, a successful syndromic neurosurgery program will bring together an interested multidisciplinary team of experts who will grow care through open communication and steady improvement efforts.

Neurosurgical Evaluation and Management of Adults with Achondroplasia.

Much of the current medical discussion for within centers for skeletal dysplasia and specifically patients with achondroplasia focuses on infancy and early childhood. Most neurosurgical concerns arise due to a defect in the endochondral ossification, resulting on early fusion of the synchondrosis. As patients age, the neurosurgical focus shifts from primarily cranial to spinal concerns. Often pediatric neurosurgeons may continue to follow their patients with skeletal dysplasia. However, general adult neurosurgeons and orthopedic surgeons may see these graduated adults in their practice. This article provides a review of the common neurosurgical concerns for patients with achondroplasia.

Neurosurgical Considerations of Neurocutaneous Syndromes.

The phakomatoses are a group of genetic and acquired disorders characterized by neurologic, cutaneous, and often ocular manifestations, thus commonly referred to as neurocutaneous syndromes. In several of these conditions the underlying genetic pathophysiology has been elucidated, which will continue to play an important role in advancing therapeutic techniques. This article focuses on several examples of such neurocutaneous syndromes, with special attention to the relevant neurosurgical considerations of these patients.

Baclofen Pump Use: Complications After Growth-friendly Instrumentation for Early-onset Scoliosis.

BACKGROUND: Patients with early-onset scoliosis (EOS) and spasticity may receive treatment with an intrathecal baclofen pump. We assessed how baclofen pumps are associated with the odds of complications and secondary interventions after growth-friendly (GF) spine surgery for EOS and analyzed infectious complications within the pump cohort. METHODS: Using a prospectively maintained, international multicenter database, we studied patients with neuromuscular EOS with baclofen pumps who underwent GF spine surgery from 2002 through 2019 (n=25). Baclofen pumps were implanted before GF instrumentation in 18 patients, during in 2 patients, and after in 5 patients. Patients with existing pumps at initial GF spine surgery were matched 1:3 with 54 patients (control group) without pumps according to treatment center, year of surgery, diagnosis, surgery type, and preoperative curve magnitude. Univariate analysis and multivariate logistic regression were performed to compare complications and secondary interventions between the 2 cohorts. RESULTS: Patients with baclofen pumps had 4.8 times the odds [95% confidence interval (CI): 1.5-16] of experiencing any complication within 1 year after initial GF spine surgery compared with controls. During mean follow-up of 6.9±4.3 years, they had 4.7 times the odds (95% CI: 1.3-16) of deep surgical site infection and 5.6 times the odds (95% CI: 1.2-26) of spinal rod removal after any complication. Differences in rates of mechanical complication, such as rod migration and breakage, were nonsignificant between the 2 groups. For the 9 patients (50%) with pumps who experienced infections, the most common microorganisms were Staphylococcus aureus (4 patients) and Pseudomonas aeruginosa (2). The pump/catheter was revised or removed, in addition to antibiotic therapy or surgical irrigation and debridement, in 2 patients. CONCLUSIONS: Among patients with neuromuscular EOS, those with baclofen pumps are much more likely to experience complications within 1 year after GF spine surgery. They are also more likely to have deep surgical site infections, with S. aureus and P. aeruginosa being the most common causative organisms, and to require spinal rod removal. LEVEL OF EVIDENCE: Level III-retrospective comparative study.

International Consensus Statement on the diagnosis, multidisciplinary management and lifelong care of individuals with achondroplasia.

Achondroplasia, the most common skeletal dysplasia, is characterized by a variety of medical, functional and psychosocial challenges across the lifespan. The condition is caused by a common, recurring, gain-of-function mutation in FGFR3, the gene that encodes fibroblast growth factor receptor 3. This mutation leads to impaired endochondral ossification of the human skeleton. The clinical and radiographic hallmarks of achondroplasia make accurate diagnosis possible in most patients. However, marked variability exists in the clinical care pathways and protocols practised by clinicians who manage children and adults with this condition. A group of 55 international experts from 16 countries and 5 continents have developed consensus statements and recommendations that aim to capture the key challenges and optimal management of achondroplasia across each major life stage and sub-specialty area, using a modified Delphi process. The primary purpose of this first International Consensus Statement is to facilitate the improvement and standardization of care for children and adults with achondroplasia worldwide in order to optimize their clinical outcomes and quality of life.

Implementation Process and Evolution of a Laparotomy-Assisted 2-Port Fetoscopic Spina Bifida Closure Program.

INTRODUCTION: Prenatal closure of open spina bifida via open fetal surgery improves neurologic outcomes for infants in selected pregnancies. Fetoscopic techniques that are minimally invasive to the uterus aim to provide equivalent fetal benefits while minimizing maternal morbidities, but the optimal technique is undetermined. We describe the development, evolution, and feasibility of the laparotomy-assisted 2-port fetoscopic technique for prenatal closure of fetal spina bifida in a newly established program. METHODS: We conducted a retrospective cohort study of women consented for laparotomy-assisted fetoscopic closure of isolated fetal spina bifida. Inclusion and exclusion criteria followed the Management of Myelomeningocele Study (MOMS). Team preparation involved observation at the originating center, protocol development, ancillary staff training, and surgical rehearsal using patient-matched models through simulation prior to program implementation. The primary outcome was the ability to complete the repair fetoscopically. Secondary maternal and fetal outcomes to assess performance of the technique were collected prospectively. RESULTS: Of 57 women screened, 19 (33%) consented for laparotomy-assisted 2-port fetoscopy between February 2017 and December 2019. Fetoscopic closure was completed in 84% (16/19) cases. Over time, the technique was modified from a single- to a multilayer closure. In utero hindbrain herniation improved in 86% (12/14) of undelivered patients at 6 weeks postoperatively. Spontaneous rupture of membranes occurred in 31% (5/16) of fetoscopic cases. For completed cases, median gestational age at birth was 37 (range 27-39.6) weeks and 50% (8/16) of women delivered at term. Vaginal birth was achieved in 56% (9/16) of patients. One newborn had a cerebrospinal fluid leak that required postnatal surgical repair. CONCLUSION: Implementation of a laparotomy-assisted 2-port fetoscopic spina bifida closure program through rigorous preparation and multispecialty team training may accelerate the learning curve and demonstrates favorable obstetric and perinatal outcomes.

Epidemiology and outcomes of pediatric intracranial aneurysms: comparison with an adult population in a 30-year, prospective database.

OBJECTIVE: Pediatric intracranial aneurysms are rare. Most large series in the last 15 years reported on an average of only 39 patients. The authors sought to report their institutional experience with pediatric intracranial aneurysms from 1991 to 2021 and to compare pediatric patient and aneurysm characteristics with those of a contemporaneous adult cohort. METHODS: Pediatric (≤ 18 years of age) and adult patients with one or more intracranial aneurysms were identified in a prospective database. Standard epidemiological features and outcomes of each pediatric patient were retrospectively recorded. These results were compared with those of adult aneurysm patients managed at a single institution over the same time period. RESULTS: From a total of 4500 patients with 5150 intracranial aneurysms admitted over 30 years, there were 47 children with 53 aneurysms and 4453 adults with 5097 aneurysms; 53.2% of children and 36.4% of adults presented with a subarachnoid hemorrhage (SAH). Pediatric aneurysms were significantly more common in males, more likely giant (≥ 25 mm), and most frequently located in the middle cerebral artery. Overall, 85.1% of the pediatric patients had a modified Rankin Scale score ≤ 2 at the last follow-up (with a mean follow-up of 65.9 months), and the pediatric mortality rate was 10.6%; all 5 patients who died had an SAH. The recurrence rate of treated aneurysms was 6.7% (1/15) in the endovascular group but 0% (0/31) in the microsurgical group. No de novo aneurysms occurred in children (mean follow-up 5.5 years). CONCLUSIONS: Pediatric intracranial aneurysms are significantly different from adult aneurysms in terms of sex, presentation, location, size, and outcomes. Future prospective studies will better characterize long-term aneurysm recurrence, rebleeds, and de novo aneurysm occurrences. The authors currently favor microsurgical over endovascular treatment for pediatric aneurysms.