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.

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.

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.

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.

Split cord malformation and tethered cord syndrome: case series with long-term follow-up and literature review.

PURPOSE: To date, the description of the natural course of concurrent tethered cord syndrome with a low-lying conus medullaris and split cord malformation is lacking in the literature. We report a cohort of adult and pediatric patients with concurrent malformations and long-term follow-up. METHODS: Patients with concurrent diagnoses of split cord malformation and tethered cord (radiographic evidence supporting clinical symptomatology) were identified between 2000 and 2020. Patients without sufficient documentation or at least 6-month follow-up were excluded. RESULTS: Nine patients were identified with an average of 8.9 years follow-up (range 2-31 years). The most common symptoms were radiating leg pain and lower extremity paresthesias, occurring in 44% of patients; and bladder/bowel dysfunction, worsening scoliosis, and acute motor deterioration were less common. Two patients were successfully treated conservatively for mild leg pain and paresthesias. For those who underwent surgery, all experienced symptomatic relief upon first follow-up. Two had late symptomatic recurrence; one 4 and 8 years after initial surgery; and the other, 11, 26, and 31 years after initial surgery. CONCLUSION: The rarity of concurrent split cord and tethered cord syndrome with a low-lying conus makes management difficult to formulate. This series supplements our knowledge of the long-term outcomes and lessons learned from the management of these patients. Approximately 25% of patients were managed conservatively and had symptomatic improvement. For surgically managed patients, with intractable pain or worsening neurological function, symptoms can still recur over a decade after intervention. Reoperation, however, can still be beneficial, can provide years of relief, and should be considered.

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.

Dual Myelomeningoceles in Twins: Case Report, Review, and Insights for Etiology.

INTRODUCTION: Despite folate supplementation, neural tube defects (NTDs) still occur in 0.5-1.0/1,000 pregnancies, with 30-50% not preventable with folate. Twinning has increased due to artificial fertilization and in itself predisposes to NTDs at a rate of 1.6/1,000. The contributions of genetic and environmental factors to myelomeningocele development remain poorly understood. Expression patterns of congenital pathologies in twins can sometimes provide etiological insight. Concordance of NTDs in twins is 0.03/1,000, with dual myelomeningocele reported in only 23 pairs, only one of which survived. We present the 24th pair, the 1st to maintain lower extremity motor function. We review all prior cases and discuss implications of twin concordance on the interplay of genetic and environmental influences. Case Report and Review: A new case of female monozygotic twins born to a well-nourished 24-year-old female is reported with details of perioperative care. Prenatal ultrasound showed L3-S4 and L5-S4 myelomeningoceles, Chiari II malformations, and ventriculomegaly. Copy number microarray was unrevealing. Each underwent uncomplicated repair on day of life 1, and ventriculoperitoneal shunt placement on days of life 10 and 16. Both had movement in the legs upon 6-week follow-up. All prior reported cases of concordant twin myelomeningoceles were abstracted and analyzed, revealing persistence of occurrence despite folate supplementation and a majority occurring in dizygotic pairs. The literature is also reviewed to summarize current knowledge of myelomeningocele pathophysiology as it relates to genetic and environmental influences. DISCUSSION: Meticulous surgical and perioperative care allowed for early positive outcomes in each twin. However, etiopathogenesis remains elusive. In general, only of a minority of cases have underlying genetic lesions or clear environmental triggers. Concordance in monozygotic twins argues for a strong genetic influence; yet, literature review reveals a higher rate of concordant dizygotic twins. This, along with the observation of differing resultant phenotypes in monozygotic twins as seen in this case, prompts further investigation into nonfolate environmental influences. While efforts in genetic investigation should continue, the role of teratogens and exposures should not be minimized in research efforts, public health, and family counseling. Clinical genetic testing remains of limited utility in the majority of patients until more is known.

Management of Chiari malformations: opinions from different centers-a review.

PURPOSE: Surgical decision-making in Chiari malformation type I (CM-I) patients tends to depend on the presence of neurological signs and symptoms, syringomyelia, and/or scoliosis, but significant variability exists from center to center. Here, we review the symptoms of CM-I in children and provide an overview of the differences in opinion regarding surgical indications, preferred surgical techniques, and measures of outcome. METHODS: A review of the literature was performed to identify publications relevant to the surgical management of pediatric CM-I patients. RESULTS: Most surgeons agree that asymptomatic patients without syringomyelia should not undergo prophylactic surgery, while symptoms of brainstem compression and/or lower cranial nerve dysfunction warrant surgery. Patients between these extremes, however, remain controversial, as does selection of the most appropriate surgical technique. CONCLUSIONS: The optimal surgical procedure for children with CM-I remains a point of contention, and widespread variability exists between and within centers.

Scoliosis in patients with Chiari malformation type I.

The literature about the association between Chiari malformations (CMs) and scoliosis has been growing over the last three decades; yet, no consensus on the optimal management approach in this patient population has been reached. Spinal anomalies such as isolated syrinxes, isolated CM, and CM with a syrinx are relatively common among patients with presumed idiopathic scoliosis (IS), a rule that also applies to scoliosis among CM patients as well. In CM patients, scoliosis presents with atypical features such as early onset, left apical or kyphotic curvature, and neurological deficits. While spinal X-rays are essential to confirm the diagnosis of scoliosis among CM patients, a magnetic resonance imaging (MRI) is also recommended in IS patients with atypical presentations. Hypotheses attempting to explain the occurrence of scoliosis in CM patients include cerebellar tonsillar compression of the cervicomedullary junction and uneven expansion of a syrinx in the horizontal plane of the spinal cord. Early detection of scoliosis on routine spinal examination and close follow-up on curve stability and progression are essential initial steps in the management of scoliosis, especially in patients with CM, who may require full spine MRI to screen for associated neuro-axial anomalies; bracing and spinal fusion may be subsequently pursued in high-risk patients.

Neurosurgeons' opinions on the prenatal management of myelomeningocele.

OBJECTIVE: Improvements in imaging and surgical technological innovations have led to the increasing implementation of fetal surgical techniques. Open fetal surgery has demonstrated more favorable clinical outcomes in children born with open myelomeningocele (MMC) than those following postnatal repair. However, primarily because of maternal risks but also because of fetal risks, fetal surgery for MMC remains controversial. Here, the authors evaluated the contemporary management of MMC in the hope of identifying barriers and facilitators for neurosurgeons in providing fetal surgery for MMC. METHODS: An online survey was emailed to members of the Congress of Neurological Surgeons (CNS) and the International Society for Pediatric Neurosurgery (ISPN) in March 2019. The survey focused on 1) characteristics of the respondents, 2) the practice of counseling on and managing prenatally diagnosed MMC, and 3) barriers, facilitators, and expectations of fetal surgery for MMC. Reminders were sent to improve the response rate. RESULTS: A total of 446 respondents filled out the survey, most (59.2%) of whom specialized in pediatric neurosurgery. The respondents repaired an average of 9.6 MMC defects per year, regardless of technique. Regardless of the departments in which respondents were employed, 91.0% provided postnatal repair of MMC, 13.0% open fetal repair, and 4.9% fetoscopic repair. According to the surgeons, the most important objections to performing open fetal surgery were a lack of cases available to become proficient in the technique (33.8%), the risk of maternal complications (23.6%), and concern for fetal complications (15.2%). The most important facilitators according to advocates of prenatal closure are a decreased rate of shunt dependency (37.8%), a decreased rate of hindbrain herniation (27.0%), and an improved rate of motor function (18.9%). Of the respondents, only 16.9% agreed that open fetal surgery should be the standard of care. CONCLUSIONS: The survey results showed diversity in the management of patients with MMC. In addition, significant diversity remains regarding fetal surgery for MMC closure. Despite the apparent benefits of open fetal surgery in selected pregnancies, only a minority of centers and providers offer this technique. As a more technically demanding technique that requires multidisciplinary effort with less well-established long-term outcomes, fetoscopic surgery may face similar limited implementation, although the surgery may pose fewer maternal risks than open fetal surgery. Centralization of prenatal treatment to tertiary care referral centers, as well as the use of sophisticated training models, may help to augment the most commonly cited objection to the implementation of prenatal closure, which is the overall limited caseload.

Surgical treatment of a type IV cystic sacrococcygeal teratoma with intraspinal extension utilizing a posterior-anterior-posterior approach: a case report.

Type IV sacrococcygeal teratoma with intraspinal involvement is rare and to our knowledge has not been reported previously in the literature. The authors present the case of a 2-month-old infant with a type IV sacrococcygeal teratoma diagnosed on prenatal ultrasound. Postnatal MRI revealed intraspinal extension through an enlarged sacral neuroforamina on the right side. On surgical exploration, the authors discovered a dorsal cystic tumor involving the sacral spine that extended through an enlarged S4 foramen to a large presacral component. The tumor was successfully removed to achieve a complete en bloc surgical resection. The authors review the epidemiology, pathophysiology, and treatment of sacrococcygeal teratomas with intraspinal extension.

Detection of tumor-derived DNA in cerebrospinal fluid of patients with primary tumors of the brain and spinal cord.

Cell-free DNA shed by cancer cells has been shown to be a rich source of putative tumor-specific biomarkers. Because cell-free DNA from brain and spinal cord tumors cannot usually be detected in the blood, we studied whether the cerebrospinal fluid (CSF) that bathes the CNS is enriched for tumor DNA, here termed CSF-tDNA. We analyzed 35 primary CNS malignancies and found at least one mutation in each tumor using targeted or genome-wide sequencing. Using these patient-specific mutations as biomarkers, we identified detectable levels of CSF-tDNA in 74% [95% confidence interval (95% CI) = 57-88%] of cases. All medulloblastomas, ependymomas, and high-grade gliomas that abutted a CSF space were detectable (100% of 21 cases; 95% CI = 88-100%), whereas no CSF-tDNA was detected in patients whose tumors were not directly adjacent to a CSF reservoir (P < 0.0001, Fisher's exact test). These results suggest that CSF-tDNA could be useful for the management of patients with primary tumors of the brain or spinal cord.

Cerebrofacial arteriovenous metameric syndrome (CAMS): a spectrum disorder of craniofacial vascular malformations.

Cerebrofacial arteriovenous metameric syndrome (CAMS) is a recent classification of vascular malformations that encompasses a spectrum of phenotypic expression involving arteriovenous malformations (AVMs) of the cerebral, orbital, and facial region. Recognizing the embryologic basis of CAMS is important for diagnosing other AVMs along the same metameric level. Visual loss is the most common presentation prompting ophthalmologic evaluation followed by neuroimaging. We present two pediatric patients with ipsilateral optic nerve and chiasmal AVMs without cutaneous manifestations, characteristic of CAMS 2. The diagnosis of cerebral AVMs was made by magnetic resonance imaging of the brain and confirmed with cerebral angiography. High-resolution flat-panel computed tomography was performed in one patient and was useful to demonstrate the intraneural invasion of the optic nerve by the AVM.

Instrumented fusion for spinal deformity after laminectomy or laminoplasty for resection of intramedullary spinal cord tumors in pediatric patients.

OBJECTIVE Spinal deformity has become a well-recognized complication of intramedullary spinal cord tumor (IMSCT) resection. In particular, laminectomy can result in biomechanical instability caused by loss of the posterior tension band. Therefore, laminoplasty has been proposed as an alternative to laminectomy. Here, the authors describe the largest current series of pediatric patients who have undergone laminoplasty for IMSCT resection and investigate the need for surgical fusion after both laminectomy and laminoplasty. METHODS The medical records of pediatric patients who underwent resection of an IMSCT at a single institution between November 2003 and May 2014 were reviewed retrospectively. Demographic, clinical, radiological, surgical, histopathological, and follow-up data were collected. RESULTS Sixty-six consecutive patients underwent resection of an IMSCT during the study period. Forty-three (65%) patients were male. The patients had a median age of 12.9 years (interquartile range [IQR] 7.2-16.5 years) at the time of surgery. Patients typically presented with a tumor that involved the cervical and/or thoracic spine. Nineteen (29%) patients underwent laminectomy, and 47 (71%) patients underwent laminoplasty. Patients in each cohort had a similar rate of postoperative deformity. Overall, 10 (15%) patients required instrumented spinal fusion for spinal deformity. Four patients required revision of the primary fusion. CONCLUSIONS These findings show that among pediatric patients with an IMSCT, postoperative surgical fusion rates remain high, even after laminoplasty. Known risk factors, such as the age of the patient, location of the tumor, and the number of involved levels, might play a larger role than replacement of the laminae in determining the rate of surgical fusion after IMSCT resection.

Risk factors associated with short-term complications and mortality after pediatric spinal arthrodesis.

OBJECTIVE Spinal arthrodesis is routinely performed in the pediatric population. However, there is limited information on the short-term outcomes of pediatric patients who have undergone spine fusion. Thus, the authors conducted a retrospective review of the Pediatric National Surgical Quality Improvement Program (NSQIP) database to determine the short-term mortality, complication, reoperation, and readmission rates of pediatric patients who underwent spinal arthrodesis for all indications. METHODS The Pediatric NSQIP database was queried for all patients who underwent spinal arthrodesis between 2012 and 2014. Patient demographics, comorbidities, body mass index, American Society of Anesthesiologists classification, and operative time were abstracted. Short-term mortality, reoperation, and readmission rates and complications were also noted. Univariate and multivariate analyses were performed to delineate patient risk factors that influence short-term mortality, complications, reoperation, and readmission rates. RESULTS A total of 4420 pediatric patients who underwent spinal fusion were identified. Common indications for surgical intervention included acquired/idiopathic scoliosis or kyphoscoliosis (71.2%) and genetic/syndromic scoliosis (10.7%). The mean patient age was 13.7 ± 2.9 years, and 70% of patients were female. The overall 30-day mortality was 0.14%. Multivariate analysis showed that female sex and pulmonary comorbidities significantly increased the odds of reoperation, with odds ratios of 1.43 and 1.78, respectively. CONCLUSIONS In the NSQIP database for pediatric patients undergoing spinal arthrodesis for all causes, there was a 3.6% unplanned reoperation rate, a 3.96% unplanned readmission rate, and a 9.0% complication rate. This analysis provides data for risk stratification of pediatric patients undergoing spinal arthrodesis, allowing for optimized care.

Hemorrhage Risk in Pediatric Patients with Multiple Intracranial Arteriovenous Malformations.

BACKGROUND/AIMS: This study aims to characterize the clinical features, treatment strategies, and annual hemorrhage incidence rate of pediatric patients with multiple arteriovenous malformations (MAVM). METHODS: The PubMed and EMBASE databases and the arteriovenous malformations (AVM) database at the Johns Hopkins Hospital were searched for reports of pediatric patients (under 21 years of age) with MAVM. Data related to demographics, clinical features, management, and treatment outcomes were analyzed using descriptive statistics. Twenty-four pediatric patients met the inclusion criteria. RESULTS: The annual hemorrhage incidence rate was 2.1%. The most common presenting features were neurological deficit (38%) and hemorrhage (21%). Treatment with embolization has become the most frequently used modality. In patients undergoing staged treatment of MAVM, hemorrhage of an untreated nidus (n = 1), visualization of a new nidus (n = 2), or disappearance of a draining vein (n = 1) occurred. CONCLUSION: The annual hemorrhage incidence rate for pediatric patients with MAVM approaches the upper range of previously reported hemorrhage rates for solitary AVM. A staged approach to treating MAVM requires close follow-up as changes to the remaining nidi may occur during the latency period. Limitations of this study include its small sample size and reporting bias.

Tokuhashi score is predictive of survival in a cohort of patients undergoing surgery for renal cell carcinoma spinal metastases.

PURPOSE: Renal cell carcinoma (RCC) is an aggressive disease that metastasizes to the spine often requiring surgery. However, selecting the appropriate surgical intervention can be challenging. The Tokuhashi scoring system can be used to predict survival and inform the surgical strategy. We set out to determine the Tokuhashi score for patients with RCC spine metastases and compare expected and observed survival. METHODS: Records were reviewed for all patients who underwent surgery for spinal metastases at a single institution from January 2000 to December 2011 to determine the Tokuhashi score and survival. Kaplan-Meier estimates and log-rank test for univariate analysis were performed with R version 2.15.12 (R Foundation, 2012). RESULTS: Thirty patients underwent 40 spinal operations for metastatic RCC. Median survival was 11.4 months. Preoperative Tokuhashi scores were: 12-15, 15 patients; 9-11, seven patients; 0-8, eight patients. Median survival was 32.9, 11.7, and 5.4 months, respectively. Bone (p=0.01) and visceral metastases (p=0.005), and KPS (p=0.002) significantly affected survival. Tokuhashi score predicted survival (p=0.016); survival differed between the high and low score groups (p=0.006). CONCLUSIONS: RCC is an aggressive disease with short life expectancy when metastatic to the spine. However, patients with low systemic disease burden and solitary spinal metastases can have long survival and benefit from excisional surgery. Tokuhashi score can be useful in selecting surgical intervention in patients with RCC spinal metastases, and may be more relevant than in other cancers with spinal metastases.

Alveolar soft-part sarcoma in the sacrum: a case report and review of the literature.

Alveolar soft part sarcoma (ASPS) is a rare disease of the soft tissue. Although the disease is rare, it is refractory to chemotherapy and radiation. En bloc surgical resection offers the best chance of cure. In this article we report the case of a 28-year-old woman who presented with buttock and leg pain, bowel, bladder and gait impairment and a large mass in the sacrum. Following surgical excision, the lesion was proven to be ASPS. On pathology, the mass was TFE3 (transcription factor E3) positive, indicating the presence of the ASPL-TFE3 (novel gene-transcription factor) translocation. Following surgery, the patient had improvement in her pain and ambulation; however, she refused adjuvant therapy to pursue hospice care and succumbed to her disease 2 years after surgery. On a review of the literature, it was found that ASPS of the bone constitutes a rare and formidable subset of this disease. Further, metastases related to ASPS are common in the lungs, liver, brain, and lymph nodes. The degree of dissemination is a predictor of outcome, with 5-year survival of 81-88% in patients with local disease and only 20-46% in patients with metastatic disease at the time of presentation. Brain metastases at the time of presentation portend the worst prognosis.

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.

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.